Journal of the American Academy of Dermatology

What's new in pediatric dermatology? Part I. Diagnosis and pathogenesis

Howard B. Pride, Megha Tollefson, Robert Silverman — 2013-06-01

The field of pediatric dermatology has been rich in new developments. Part I of this continuing medical education article will focus on new diagnoses and new insights into the etiology and pathogenesis of pediatric skin disorders. Insights into the pathogenesis of atopic dermatitis, new forms of contact dermatitis, recently recognized mimickers of port wine stains, associations with infantile hemangiomas, the concept of genetic diseases being classified by common pathways (RASopathies), newly recognized genetic discoveries in mosaic disorders, the recognition of cystic fibrosis being associated with aquagenic wrinkling of the palms, new forms of epidermolysis bullosa, and genital ulcers in non–sexually active adolescent girls will be discussed.

CME examination

2013-06-01

Answers to CME examination

2013-06-01

Change of Address

2013-06-01

What's new in pediatric dermatology? Part II. Treatment

Howard B. Pride, Megha Tollefson, Robert Silverman — 2013-06-01

The field of pediatric dermatology has been rich in new developments. Part II of this continuing medical education article will focus on new therapeutic modalities for several entities encountered in pediatric dermatology. The treatment of atopic dermatitis, exciting advances in the use of propranolol and other beta-blockers for the use of infantile hemangiomas, the use of rapamycin for vascular anomalies, the use of biologics in children, the central nervous system risks of general anesthesia in young children, side effects in the use of isotretinoin, the treatment of tinea capitis, treatment of herpes simplex infections, and the use of technologies such as texting and social media in medicine will be discussed.

CME examination

2013-06-01

Important Notice Regarding JAAD Grand Rounds

2013-06-01

Answers to CME examination

2013-06-01

American Board of Dermatology Examination Dates

2013-06-01

Pediatric melanoma: Results of a large cohort study and proposal for modified ABCD detection criteria for children

2013-02-11

Background: Clinical and histopathologic features of childhood melanoma are poorly characterized. Atypical clinical presentations and ambiguous microscopic findings may contribute to diagnostic delays.Objectives: We sought to determine whether conventional ABCDE melanoma detection criteria (Asymmetry, Border irregularity, Color variegation, Diameter >6 mm, Evolution [any morphologic or symptomatic change in the lesion]) adequately detects pediatric melanoma and to evaluate clinicopathologic and outcome differences between younger and older children.Methods: This was a retrospective study of children given the diagnosis of melanoma (N = 60) or ambiguous melanocytic tumors treated as melanoma (N = 10) before age 20 years from 1984 to 2009 at the University of California, San Francisco. Seventy patients were divided into 2 age groups: 0 to 10 years (N = 19, group A) and 11 to 19 years (N = 51, group B). Clinical, histopathologic, and outcomes data were collected. Main outcome measures were time from diagnosis to death and predictors of metastasis and death.Results: In all, 60% of group A and 40% of group B children did not present with conventional ABCDE criteria. Rather, amelanosis, bleeding, “bumps,” uniform color, variable diameter, and de novo development were most common. Histopathological subtypes differed significantly between groups (P = .002). In all, 44% were histopathologically unclassifiable using current melanoma subtypes. Stage IIA disease or higher comprised 92% and 46% of groups A and B, respectively (P = .05). Ten patients died: 1 in group A and 9 in group B. Of these, 70% had amelanotic lesions, and 60% had at least 1 major risk factor. Breslow thickness predicted metastasis (adjusted odds ratio 12.8 [CI 1.4-115]).Limitations: The retrospective design resulted in incomplete data capture.Conclusion: Additional ABCD detection criteria (Amelanotic; Bleeding, Bump; Color uniformity; De novo, any Diameter) used together with conventional ABCDE criteria may facilitate earlier recognition and treatment of melanoma in children.

Pediatric dermatology inpatient consultations: A retrospective study of 427 cases

Patrick McMahon, Deborah Goddard, Ilona J. Frieden — 2013-01-28

Background: Limited reviews of inpatient pediatric dermatology (PD) exist in the current medical literature.Objective: We sought to profile the inpatient PD consultations at a large tertiary care children's hospital.Methods: We reviewed the electronic medical records of 427 consecutive PD consultations over a 52-month period from January 2006 to April 2010. The age, gender, diagnosis, requesting service, number of skin biopsies performed, and reason for admission to the hospital were recorded.Results: Patients ranged in age from newborn to 17 years old: 18% were 6 weeks to 11 months of age. General pediatrics was the service that most frequently consulted PD (44% of consultations). The most common diagnostic categories included infectious diseases, graft-versus-host disease, dermatitis, vascular anomalies, and drug eruptions. The most common diagnoses when hospitalization was primarily for skin-related disease were infections and vascular anomalies. Admission for skin disease associated with a systemic illness was most frequently for infections and drug eruptions.Limitations: The retrospective nature of the study, its reliance on electronic medical records, and occurrence at a tertiary care children's hospital are potential limitations.Conclusions: Information obtained from this study may be used to: (1) tailor teaching to relevant diagnoses for health care providers and trainees who care for children in a hospital setting, (2) inform clinicians about the array of conditions on which PD consultants provide input, and (3) understand the impact PD consultations have in a pediatric tertiary care center.

Dermatologic manifestations of ataxia-telangiectasia syndrome

2013-01-28

Background: Previous reports on the cutaneous manifestations of ataxia-telangiectasia (A-T) have relied on data from small series, in patients not genetically tested for A-T.Objective: The aim of our study was to characterize the dermatologic manifestations in patients with A-T followed up at the national A-T clinic in Israel.Methods: This retrospective cross-sectional study included 32 patients followed up at a multidisciplinary A-T clinic from 2010 to 2012. Complete skin examination was done by a single dermatologist. Information about mutations and neurologic status was extracted from the patients' charts. Relevant demographic, clinical, and laboratory characteristics of all patients were collected and summarized.Results: Of the 32 patients, 97% had ocular telangiectasia, the hallmark of the disease. Telangiectasia on other body parts was less frequent. Pigmentary anomalies included café-au-lait macules (84%), hypopigmented macules (44%), and melanocytic nevi (37%). A facial papulosquamous rash was found in 41% of cases. Other manifestations included hypertrichosis and birdlike facies. We did not observe premature hair graying or poliosis. No genotype-phenotype correlation was found in terms of skin manifestations.Limitations: There was a modest sample size, because of the rarity of the disease.Conclusion: Recognition of the ocular and dermatologic manifestations of A-T can facilitate early diagnosis in a child with neurologic deterioration.

Androgenetic alopecia and risk of prostate cancer: A systematic review and meta-analysis

Aline Amoretti, Humberto Laydner, Wilma Bergfeld — 2013-02-11

Background: Androgenetic alopecia (AGA) is a genetically determined skin condition strongly age dependent and androgens are assumed to play an important role in its development. A link between AGA and prostate cancer has been hypothesized because of their similar risk factors.Objective: We sought to systematically review the evidence available on the association between AGA and risk of prostate cancer.Methods: We searched the electronic databases MEDLINE and Cochrane for studies examining the association between AGA and risk of prostate cancer. We estimated pooled odds ratios (OR) and 95% confidence intervals. We also analyzed the OR for individual hair loss patterns, as defined by the Hamilton scale.Results: A total of 7 case-control studies including 8994 patients–4078 cases and 4916 controls–were reviewed. One cohort study was identified but did not meet our inclusion criteria. There was statistically significant association between vertex baldness and prostate cancer (OR 1.25; 95% confidence interval 1.09-1.44; Z = 3.13; P = .002). No statistically significant association between AGA (any pattern) and prostate cancer was identified (OR 1.03; 95% confidence interval 0.93-1.13; Z = 0.55; P = .58).Limitations: Only case-control studies, which may be subject to bias, met the inclusion criteria for this meta-analysis.Conclusions: Vertex pattern AGA was associated with a significant increased risk of prostate cancer. Any pattern AGA did not show a significant increase in the risk of prostate cancer.

Photoprotective habits of patients with cutaneous lupus erythematosus

Shirley Y. Yang, Ira Bernstein, Danielle Q. Lin, Benjamin F. Chong — 2013-01-28

Background: Previous studies have suggested that patients with cutaneous lupus erythematosus (CLE) are deficient in sunscreen use. Use of other photoprotective methods by patients with CLE has not been assessed to our knowledge.Objective: We sought to identify demographic and clinical characteristics of patients with CLE who have the lowest overall sun-protection habits scores, and who are least likely to practice 5 individual photoprotective methods (ie, shade, sunscreen, long sleeves, hat, and sunglasses).Methods: A total of 105 patients with CLE at the University of Texas Southwestern Medical Center in Dallas completed a survey to evaluate their photoprotective practices. Additional information including demographics and clinical indicators related to CLE was collected from the patients.Results: Patients with medium and dark skin (ie, Fitzpatrick skin types III-VI) and patients aged 31 to 50 years were the least likely CLE subgroups to practice overall photoprotection, as indicated by low sun-protection habits scores (P = .001 and P = .04, respectively). In terms of individual photoprotective methods, male patients with CLE were deficient in sunscreen use, but were more likely to wear hats than female patients with CLE. Sunscreen and sunglasses use was also significantly more infrequent in dark-skinned patients than those with Fitzpatrick skin types I to IV. Patients with CLE between the ages of 41 and 50 years were least likely to wear hats.Limitations: This study was subject to reporter bias and did not cover barriers to and knowledge of photoprotection.Conclusion: Cultural customs and misconceptions shared by those from the general population have a significant influence on the photoprotective habits of this CLE population. These need to be addressed to improve photoprotection rates in these at-risk individuals.

Sunscreen use while driving

Dennis P. Kim, Indy Chabra, Pawan Chabra, Evan C. Jones — 2013-02-05

Background: Data regarding patient perceptions and behaviors about sun-protection measures while driving are lacking.Objectives: This study evaluates patients’ awareness of the importance of sun protection while in an automobile, and assesses perceptions about and compliance with sun protection. A secondary objective was to detect any significant laterality in melanoma and nonmelanoma skin cancers.Methods: We performed a retrospective survey of patients seen at a Mohs micrographic surgery clinic.Results: Significantly fewer patients reported wearing sunscreen while in an automobile when compared with general daily sunscreen use (52% vs 27%, P < .05). Most respondents did not think they needed to use sunscreen while driving, especially if the windows were closed. Those who believed they were protected from sun damage while in a car were much less likely to use sunscreen (12% vs 46%, P < .05). There was a significant left-sided predominance of nonmelanoma skin cancers, except in patients who used automobiles with tinted windows.Limitations: This retrospective survey study design is not as ideal as a randomized controlled trial. Additional limitations of this study include small sample size, selection bias, and recall bias.Conclusion: Our results reveal poor patient awareness of and compliance with sun-protection measures while in an automobile. Skin cancer prevention efforts should be modified to specifically address automobile-related sun exposure.

Cutaneous squamous cell carcinoma: Estimated incidence of disease, nodal metastasis, and deaths from disease in the United States, 2012

Pritesh S. Karia, Jiali Han, Chrysalyne D. Schmults — 2013-02-04

Background: It is estimated that over 700,000 new cases of cutaneous squamous cell carcinoma (CSCC) are diagnosed annually in the United States. However, CSCC has been excluded from national cancer registries. Thus the precise incidence of CSCC, along with metastases and deaths resulting from it, is unknown.Objective: We sought to estimate the 2012 incidence of invasive (non-in situ) CSCC and the number of nodal metastases and deaths arising from it in the US white population.Methods: US studies reporting incidence of CSCC, or the number of nodal metastases or deaths arising from it, were reviewed. Linear regression was used to estimate current CSCC incidence based on available incidence data adjusting for higher reported incidences in southern versus northern/central United States. Reported risks of nodal metastases and death from CSCC were averaged. Averages were used to estimate current metastasis and death rates based on incidence estimates. The number of estimated CSCC deaths was compared against deaths from other cancers.Results: It is estimated that 186,157 to 419,543 whites were given a diagnosis of CSCC, 5604 to 12,572 developed nodal metastasis, and 3932 to 8791 died from CSCC in the United States in 2012.Limitations: The estimates of the 2012 incidence, nodal metastasis, and death from invasive CSCC are based on previous estimates of incidence and outcomes of CSCC.Conclusion: CSCC is an underrecognized health issue. In the central and southern United States, deaths from CSCC may be as common as deaths from renal and oropharyngeal carcinomas, and melanoma. Population-based studies reporting CSCC incidence and outcomes are required to verify these estimates.

Racial differences in mycosis fungoides: A retrospective study with a focus on eosinophilia

John G. Zampella, Ginette A. Hinds — 2013-01-31

Background: Mycosis fungoides (MF) is often associated with eosinophilia and portends a poorer prognosis. MF is more common in blacks and follows a more aggressive course compared with whites.Objective: We further elucidate racial differences between blacks and whites with MF, focusing on blood eosinophilia.Methods: The records of 345 patients with MF were reviewed for demographic, clinical, and pathologic data and evaluated by analysis of variance.Results: The average age at diagnosis for blacks was 45 years and was 55 years for white patients (P < .001). In the cohorts of patients with and without blood eosinophilia, the average maximum blood eosinophil count had a greater range in blacks. Independent of race, blood eosinophilia was predictive of more advanced disease (P < .0001), increased number of treatment types (P < .002), and less responsiveness to treatment (P < .0006).Limitations: This was a retrospective study at a single institution.Conclusions: These differences observed in eosinophil values may highlight disparities in MF diagnosis or a difference in pathophysiology between races.

Improvement in peripheral blood disease burden in patients with Sézary syndrome and leukemic mycosis fungoides after total skin electron beam therapy

2013-02-14

Background: There is a paucity of effective therapies for patients with Sézary syndrome and advanced mycosis fungoides with peripheral blood involvement. Total skin electron beam (TSEB) radiation therapy is an extremely effective skin-directed therapy for these patients, but, until recently, it was thought not to signifcantly affect the peripheral blood malignant T-cell population.Objective: We conducted this study to determine if TSEB has therapeutic effect on the peripheral blood in patients with advanced mycosis fungoides and Sézary syndrome.Methods: All patients on stable medication regimens seen in our photopheresis facility who received TSEB therapy between January 2008 and October 2011 at Temple University Hospital, Philadelphia, PA, were analyzed retrospectively for improvement in the peripheral blood, as documented by flow cytometry.Results: Six of 11 patients achieved 50% or greater decrease in their peripheral blood malignant T-cell population after TSEB therapy, for an overall response rate of 55%. Within the group of patients who had a response in the skin, 67% also had a response in the peripheral blood.Limitations: This analysis is limited in 3 ways. First, the sample described is small. Second, the results may be confounded by the fact that each patient was on other systemic therapies in addition to TSEB, albeit stable pre-existing regimens. The time interval between completion of TSEB therapy and repetition of flow cytometry was not standardized among patients, which may result in an underestimation of the overall response to TSEB therapy.Conclusion: In patients with advanced mycosis fungoides and Sézary syndrome, the peripheral blood tumor burden may improve after treatment with TSEB.

Cutaneous plasmacytosis: Report of 6 cases with or without systemic involvement

2013-01-28

Background: Cutaneous plasmacytosis and systemic plasmacytosis are rare entities arising primarily in patients of Japanese descent. The origin and exact pathogenesis are poorly understood.Objective: We sought to determine clinicopathologic features of cutaneous and systemic plasmacytosis.Methods: We describe the clinicopathologic features of 6 patients with cutaneous plasmacytosis with or without systemic involvement (male:female = 3:1; mean age: 49.7 years; median age: 50.5 years; age range: 29-64 years).Results: Patients presented clinically with multiple, red-brown infiltrated plaques and flat tumors, mainly located on the trunk. Histology revealed in all cases the presence of clusters of mature plasma cells within the dermis. Lymphoid follicles with reactive germinal centers were seen in 3 cases. Five cases showed the expected polyclonal expression of immunoglobulin light chain by the plasma cells, but monoclonality was observed in 1 case, thus showing overlapping features with cutaneous marginal zone lymphoma. One patient revealed overlapping features with multicentric Castleman disease.Limitations: This was a retrospective study on a relatively small number of patients.Conclusions: Despite typical clinical presentation, some of our patients presented with histopathologic and immunohistochemical features that deviated from the conventional appearance. Our observation confirms and expands previous observations of this elusive entity, suggesting that the spectrum of clinicopathologic presentations may be wider than previously recognized.

Oral candidiasis in patients with psoriasis: Correlation of oral examination and cytopathological evaluation with psoriasis disease severity and treatment

2013-02-05

Background: Infections are known to trigger and exacerbate psoriasis. Although oral candidiasis is often clinically diagnosed, it is not always confirmed by laboratory tests such as oral cytopathology.Objectives: The aims of this study were to determine the prevalence of oral candidiasis in patients with psoriasis through clinical and cytopathological diagnosis and to investigate the association between oral candidiasis and psoriasis with regards to the severity of the clinical presentation and the type of treatment for psoriasis.Methods: A total of 140 patients with psoriasis and 140 healthy control subjects received an oral examination. Scrapings of the tongue were also obtained for a cytopathological examination.Results: The oral examination and the results of the cytopathological smear revealed 37 (26%) cases of candidiasis in the patients with psoriasis and no cases of candidiasis in the healthy control subjects. There was no correlation between the type of psoriasis treatment and the presence of oral candidiasis (P = .616). There was a statistically significant association (P = .033) between the clinical severity of psoriasis and the presence of Candida.Limitations: This study was limited by the small number of subjects and the lack of follow-up to determine the development of psoriasis after treatment for oral candidiasis.Conclusions: The presence of oral candidiasis is higher in patients with psoriasis and it is associated with disease severity. This increased presence of oral candidiasis was apparent despite any type of treatment for the psoriasis. Cytopathology to rule out oral candidiasis should be used in the routine medical workup of patients with psoriasis.

Psoriasis is associated with an increased risk of parkinsonism: A population-based 5-year follow-up study

Jau-Jiuan Sheu, Kuo-Hsien Wang, Herng-Ching Lin, Chung-Chien Huang — 2013-01-31

Background: Few studies have examined the association between autoimmune diseases and parkinsonism.Objective: We sought to investigate the risk for parkinsonism during a 5-year follow-up period after a diagnosis of psoriasis using a population-based data set in Taiwan.Methods: We identified 4885 patients with psoriasis for the study cohort and randomly selected 24,425 patients as a control cohort. Each subject was individually followed up for a 5-year period to identify those who subsequently developed parkinsonism.Results: Stratified Cox proportional hazards regression showed that the adjusted hazard ratio for parkinsonism during the 5-year follow-up period for patients with psoriasis was 1.74 (95% confidence interval 1.35-2.20) that of control patients. Furthermore, the adjusted hazard ratios for parkinsonism within the 5-year follow-up period after the index date for subjects with psoriasis were similar between both sexes (1.78 and 1.66 for men and women, respectively).Limitation: Our data set did not provide detailed information on the severity of psoriasis, or individual factors such as cigarette smoking, alcohol consumption, body mass index, and dietary patterns.Conclusion: Patients with psoriasis were found to be at a significant risk of parkinsonism during a 5-year follow-up.

The importance of dedicated dermoscopy training during residency: A survey of US dermatology chief residents

2013-01-31

Background: Studies have shown low satisfaction levels among dermatology residents with respect to dermoscopy training. Many desire additional instruction.Objective: We surveyed graduating chief residents to assess current education practices among US dermatology training programs with respect to the role of dermoscopy as an aid in the management of pigmented lesions.Methods: An online survey was sent to 139 chief residents of US dermatology training programs.Results: A 59% response rate was achieved. Of responding chief residents, 94% use dermoscopy. Although 92% of chief residents received dermoscopy training, only 48% trained with a pigmented lesion specialist. Among those training without a specialist, less than half received classroom or bedside teaching compared with 77% of those who trained with a specialist. Of those who trained with a specialist, 77% were satisfied with their training compared with only 30% who trained without a specialist (P < .0001). Those who trained with a specialist were more likely to agree that dermoscopy can help differentiate melanoma from benign lesions (77% vs 47%; P = .0065).Limitations: Response bias and limiting the survey to chief residents potentially limits our ability to generalize these results to all US dermatology trainees.Conclusion: Although many residents use dermoscopy as a diagnostic tool, the lack of dedicated dermoscopy training remains a potential barrier to increasing residents’ diagnostic confidence in the management of pigmented lesions. Increasing the amount of dedicated instruction on this topic is one possible approach to enhance resident satisfaction, potentially increasing their competency in the management of atypical nevi.

Subcutaneous Sweet syndrome in the setting of myeloid disorders: A case series and review of the literature

May P. Chan, Lyn M. Duncan, Rosalynn M. Nazarian — 2013-02-11

Background: The association of neutrophilic dermatoses with myeloid disorders is well known, but neutrophilic panniculitis in the same setting has only been reported infrequently.Objective: We aimed to better characterize these lesions clinically and histologically, and to provide a comprehensive differential diagnosis and appropriate diagnostic approach.Methods: The pathology archives were searched for cases of neutrophilic panniculitis in patients with myeloid disorders. Clinical history and histologic features were studied. Similar cases in the English-language literature were reviewed.Results: Five biopsy specimens from 4 patients and 11 previous case reports were identified. All patients presented with erythematous tender nodules typically involving the extremities. Most were accompanied by fevers, and resolved either spontaneously or with steroids. Histologically, the subcutaneous neutrophilic infiltrates demonstrated lobular or, less frequently, septal patterns with minimal dermal involvement. Leukocytoclasis, fat necrosis, reactive stromal fibroblasts, and mild reticular dermal edema were each seen in at least 3 of our 5 biopsy specimens. No myeloid blasts, vascular changes, or non-Miescher granulomas were observed. No micro-organisms were identified in the skin nodules.Limitations: This study is limited by the small number of cases.Conclusions: Based on the fairly consistent clinical and histologic findings, neutrophilic panniculitis occurring in the setting of myeloid disorders is best classified as subcutaneous Sweet syndrome after exclusion of infectious panniculitis, id reaction, and leukemia cutis. These lesions may be associated with myeloid disorders, or induced by chemotherapy or other medications.

A syringotropic variant of cutaneous sarcoidosis: Presentation of 3 cases exhibiting defective sweating responses

Jun Hayakawa, Yoshiko Mizukawa, Maiko Kurata, Tetsuo Shiohara — 2013-01-31

Background: Although nodular collections of epithelioid histiocytes and multinuclear cells can be present at all levels of the dermis in cutaneous sarcoidosis, sarcoidal granulomas characterized by marked syringotropism of epithelioid histiocytes have not been previously reported to our knowledge.Objective: We sought to determine whether syringotropic sarcoidosis bears characteristic clinical and histologic features and exhibits defective sweating responses.Methods: We investigated the clinical, histologic, and immunohistochemical features of syringotropic sarcoidosis, and sweating responses to thermal stress in 3 patients.Results: Multiple erythematous patches/plaques predominantly affected the extensor aspect of the lower legs and thighs. Immunohistochemical analyses of the sweat glands surrounded by syringotropic granulomas exhibited profoundly decreased expression of dermcidin and aquaporin 5, both of which are constitutively expressed in sweat glands of control subjects, suggesting functional defects. Indeed, sweating responses to thermal stress were markedly decreased in the involved area, as compared with those in the uninvolved area and in healthy control subjects.Limitations: There were a limited number of cases in our study.Conclusion: A syringotropic variant of cutaneous sarcoidosis might be a distinct entity clinically and histologically or represent an abortive variant.

Contraception and the dermatologist

Kelly H. Tyler, Matthew J. Zirwas — 2013-01-21

Contraceptives are pertinent to dermatologists in 3 major instances: (1) prescribing combined oral contraceptives for the treatment of acne; (2) ensuring that women being treated with potential teratogens are on adequate contraception; and (3) counseling female patients regarding contraceptives that can worsen acne. Most modern combined oral contraceptives will benefit acne; however, there are some agents that may be more effective than others, primarily because of the progestin used in the agent. Long-acting reversible contraceptives should be first line for women on teratogenic medications, but some of these agents can worsen acne because they release progestins.

E-professionalism at the dermatology office: New challenges to confidentiality in the era of social networking

Lauren A.V. Orenstein, Jeffrey Benabio, Benjamin K. Stoff — 2013-06-01

Dr Wyatt, a dermatologist in a small group practice, receives a telephone call from an irate mother about her adult daughter, Sarah. The mother noted that Sarah had “Liked” the practice's Facebook page and written a comment about a coupon on the site that provided a discount for tattoo removal. Sarah's mother fears that now “everyone will know” about Sarah's tattoo. She is appalled that the practice allows patients to post confidential information on the Internet and demands that the practice inactivate the Facebook page immediately.

Announcing New Features in JAAD Online

2013-06-01

Diabetes insipidus, bone lesions, and new-onset red-brown papules in a 42-year-old man

2013-03-04

A 42-year old Caucasian man was referred to the National Institutes of Health (NIH) for evaluation. His symptoms began approximately 10 years before referral with pituitary dysfunction that manifested as diabetes insipidus, secondary hypogonadism, and hyperprolactinemia, treated with desmopressin and testosterone gel. Eight years later, he began to experience vertigo, dizziness, diplopia, and difficulty focusing and tracking. This was followed by progressive left-sided weakness, poor coordination, and balance difficulties. Magnetic resonance imaging without contrast revealed multiple nonspecific white-matter lesions. Subsequent evaluation failed to identify an underlying infection or inflammatory origin. Systemic corticosteroid therapy was initiated with minimal improvement. Corticosteroids were tapered off 2 months later and the patient noted new asymptomatic skin lesions on his bilateral upper and lower extremities, leading to reinitiation of systemic steroids and introduction of interferon alfa. He denied fevers, chills, weight loss, or difficulty breathing.

Internet dermatology resources

Robert P. Dellavalle — 2013-06-01

As part of the celebration of the Academy's 75th anniversary, this year each monthly issue of JAAD features an expert commentary on a previously published article of particular significance to practicing dermatologists.

Physician Global Assessment (PGA) and Psoriasis Area and Severity Index (PASI): Why do both? A systematic analysis of randomized controlled trials of biologic agents for moderate to severe plaque psoriasis

Alexander Nast, Jochen Schmitt — 2013-06-01

To the Editor: With great interest we read the publication “Physician Global Assessment (PGA) and Psoriasis Area and Severity Index (PASI): Why do both? A systematic analysis of randomized controlled trials of biologic agents for moderate to severe plaque psoriasis” by Robinson, Kardos, and Kimball. Systematic research on outcomes methodology in psoriasis—such as the study by Robinson et al—is very valuable and may help to standardize the reporting of efficacy in future clinical trials. Standardization of outcome measures is a prerequisite for evidence-based medicine and enables indirect comparisons of different interventions by means of meta-analyses. However, it has not been systematically investigated whether clinical disease severity measured by means of the PASI is the most important outcome from the patient’s perspective. Although many psoriasis patients report symptoms such as pain and itching, symptoms are not being regularly assessed in psoriasis trials. Ideally, a formal consensus process involving key stakeholders such as patients, clinicians, methodologists, and regulators, should be undertaken to define core outcome domains for psoriasis trials. Based on a systematic review, the international multidisciplinary harmonizing outcome measures for eczema (HOME) initiative recently defined symptoms, clinical severity, health-related quality of life, and long-term control of flares as the core outcome domains for atopic eczema trials. A similar research project for psoriasis should be initiated.

Novel systemic drugs for psoriasis: Mechanism of action for apremilast, a specific inhibitor of PDE4

Peter H. Schafer, Robert M. Day — 2013-06-01

To the Editor: The article, “Novel systemic drugs under investigation for the treatment of psoriasis,” provides a valuable overview of investigational treatments for patients with psoriasis. However, the description of apremilast’s mechanism of action is too narrow. Apremilast should not be considered only a T-cell inhibitor, as is indicated in Table I and Fig 2 of the original article. While it is accurate that inhibition of phosphodiesterase 4 (PDE4) by apremilast affects T-cell production of inflammatory cytokines, recent data on apremilast’s mechanism of action illustrate additional effects in psoriasis.

Add Pittsburgh teledermatology “with a twist” to the map!

Joseph C. English, Robin Gehris, William Leyva — 2013-06-01

To the Editor: We applaud the effort by Armstrong et al for providing a systematic survey of the teledermatology programs in the United States. Unfortunately, the teledermatology efforts in Western Pennsylvania were not queried in this survey and were left off the US Map of Teledermatology sites. Just writing to state we have a program would not be worthy of publication. However, our programs offer a unique twist to the standard outpatient teledermatology. We believe our experience could help other institutions advance their teledermatology programs and put us on the map.

Reply

April W. Armstrong, Julie Wu — 2013-06-01

To the Editor: We are delighted to have Dr English introduce the UPMC telemedicine program and VAHPTH, a teledermatology program “with a twist.” For our study, we identified active teledermatology programs by using a 2003 ATA survey and surveying members of the American Telemedicine Association (ATA), Teledermatology SIG, and the American Academy of Dermatology’s Telemedicine Task Force. Regrettably, at the time of the online search, the initial results for the Pennsylvania programs failed to reveal this innovative program.

Methotrexate for the treatment of cutaneous dermatomyositis

James W. Click, Abrar A. Qureshi, Ruth Ann Vleugels — 2013-06-01

To the Editor: Cutaneous dermatomyositis is challenging to treat, often not responding to initial corticosteroid and antimalarial therapy. Numerous steroid-sparing agents have been used with varied success. Two retrospective chart reviews and one case series have previously reported the efficacy of methotrexate (MTX) for the treatment of cutaneous dermatomyositis. These reports prompted an investigation of our institution’s experience in treating cutaneous dermatomyositis with MTX.

Cantharidin therapy: Practice patterns and attitudes of health care providers

2013-06-01

To the Editor: Cantharidin is difficult for clinicians in the United States to obtain and utilize in practice. Cantharidin met the safety requirements of the original federal Food, Drug, and Cosmetic Act of 1938, but failure of manufacturers to provide drug efficacy data required by a 1961 amendment to the Act led to its removal from the market. Despite having no Federal Food and Drug Administration (FDA)–approved indication, cantharidin is regularly used off-label in the United States for the treatment of molluscum contagiosum (MC) and verruca vulgaris (VV). Practitioners who use cantharidin acquire the compound from Canadian distributors or compounding pharmacies. Since our institution currently prohibits the use of cantharidin, we sought to investigate prescribing patterns among other practitioners. We surveyed the attitudes and behaviors of dermatologists toward this therapeutic agent at the 2012 American Academy of Dermatology annual meeting.

Investigation of molluscum contagiosum virus, orf and other parapoxviruses in lymphomatoid papulosis

2013-06-01

To the Editor: Lymphomatoid papulosis (LyP) is a primary cutaneous CD30+ lymphoproliferative disorder with large, atypical CD-30+ cells, which, despite their ominous appearance, also occur in reactive processes, such as CD30+ cutaneous lymphoid hyperplasia. As most causes of CD30+ cutaneous lymphoid hyperplasia are viral, we explored the possibility that poxvirus or parapoxvirus could be detected in lesions of LyP.

Successful treatment of alopecia totalis with hydroxychloroquine: Report of 2 cases

Farid Stephan, Maya Habre, Roland Tomb — 2013-06-01

To the Editor: Alopecia areata (AA) is a nonscarring hair loss of unknown origin. AA is chronic and unpredictably relapsing, thus treatment of severe cases, especially alopecia totalis (AT), remains challenging.

Dermatology Calendar

2013-06-01

Cover Sheet for Index

2013-06-01

Author index

2013-06-01

Abd-El-Raheem T (see El-Domyati et al). 2013;68:103-12 (Derm. surgery) Abdel-Wahab H (see El-Domyati et al). 2013;68:103-12 (Derm. surgery)

Subject index

2013-06-01

AAD 75th Anniversary Articles. 2013;68:177-8, 338-9, 507-8, 675-6, 858-9, 1039

A young boy with a perioral eruption

Laura Sandoval, Steven Feldman — 2013-06-01

An 11-year-old Asian American boy presented to our department after a several-day history of an itchy perioral eruption. The patient had 1 previous similar incident that resolved without treatment. He denied any changes in medications or personal hygiene products. A review of his diet revealed a recent increased consumption of fruits, specifically watermelon and mango. His history was negative for eczema or acne. The patient did have a previous episode of poison ivy dermatitis. The physical examination revealed a sharply demarcated, erythematous, eczematous eruption in a perioral distribution. The lips were involved, with mild erythema and edema ( and ). His neck and upper extremities were not involved.

Asymptomatic crusted lesions

Emiliano Grillo, Sergio Vano-Galván, Angela Miguel-Morrondo, Pedro Jaén — 2013-06-01

A 92-year-old man was admitted to our hospital because of a 4-month history of an asymptomatic rash with widespread crusting. Five months earlier, the patient had been diagnosed with chronic lymphocytic leukemia and began anticoagulant therapy for chronic atrial fibrillation. The physical examination revealed large, malodorous, crusted plaques located on the chest, limbs, and stomach and sparing the face and back (). The patient denied pruritus, although he often manipulated and scratched the crusted lesions. The patient had no previous or family history of psoriasis or other skin diseases. He had lived alone for many years and had not traveled abroad. The patient denied any other drug intake.

Patients' educational needs about topical treatments for psoriasis

Sabrina L. Martin, Suzanne T. McGoey, Bruce F. Bebo, Steven R. Feldman — 2012-06-11

Background: Topical medications are a mainstay of psoriasis treatment. Many patients lack education about topicals. This may contribute to low adherence with long-term disease management.Objective: We sought to describe educational needs concerning topical treatment for patients with psoriasis.Methods: Patients' questions regarding topical therapy were collected from a National Psoriasis Foundation webcast on topical medications. The prebroadcast question responses and the postwebcast survey responses were categorized into common themes and ranked by frequency.Results: Thirty percent asked about side effects, with a major emphasis on topical steroids; 16% asked about proper use; and 11% asked about efficacy. Popular new and useful education concerned specific medication facts and information on medication (especially steroid) safety, the need for treatment adherence, and the variety of options available in topical form.Limitations: The study population consisted of online users expressing interest in the National Psoriasis Foundation educational material, not the general population of patients with psoriasis.Conclusions: Patient needs can be better met by providing information regarding side effects, proper use, and efficacy of topical medications. Communication regarding treatment changes and adherence to the treatment regimen should also occur.

Melanoma death prevention: Moving away from the sun

Jeanette Kamell Mitchell, Kieron S. Leslie — 2013-04-01

This evidence-backed editorial addresses the limitations of solely primary prevention campaigns and outlines the proven efficacy of early detection/secondary prevention strategies with respect to melanoma. It synthesizes experience from several outreach efforts that have resulted in sustained improvements in knowledge and self-skin examination behaviors. Data demonstrate that educational campaigns emphasizing increased knowledge about melanoma and self-screening practices correlate with thinner tumors. The editorial also confronts the lack of data around skin cancer screening per the US Preventative Services Task Force. It explains how we might address the issue to obtain solid evidence to back a recommendation for screening of high-risk populations in the future. Cost-efficacy of skin cancer screening is also addressed. Lastly, lessons learned from other cancers, particularly breast cancer, with respect to successful educational campaign creation and development of an effective cause marketing campaign for advocacy are discussed. Hypothetical ideas for a screening algorithm and for educational/media campaigns are presented with the hope of triggering thoughtful discussion and forward momentum.

A de novo SOX10 mutation in a patient with Waardenburg syndrome type IV

Ho Joo Jung, Sun A Jin, Soo Jin Na Choi, Seung-Chul Lee, Sook Jung Yun — 2013-06-01

To the Editor: Waardenburg syndrome type IV (WS4), a rare congenital disorder caused by defective functioning of the embryonic neural crest, is characterized by a combination of pigmentary abnormalities of the skin, eyes, and hair; sensorineural deafness; and Hirschsprung disease (HD). SOX10 is a key transcription factor in the development of 2 neural crest–derived lineages, melanocytes and neurons of the myenteric ganglia. To date, only 3 nontruncating missense mutations of SOX10 are known in WS4: p. Ala157Val, p. Arg106Trp, and p. Leu145Pro. The clinical spectrum as a result of SOX10 mutation has different syndromes manifesting variable combination of phenotypes, including depigmentation, hearing loss, peripheral and central neuropathy, and HD. However, the genotype-phenotype correlations according to specific mutation location and type are not clear.

Disseminated keratotic spicules: A rare manifestation in multiple myeloma and successful response to lenalidomide

2013-06-01

To the Editor: One of the characteristic but rare paraneoplastic signs of multiple myeloma (MM) are hyperkeratotic spicules defined as circumscribed hyperkeratosis containing a column of keratotic material. At least 18 cases have been reported to date, most of them presenting with limited distribution in head and neck. We describe one of the very few cases presenting with a generalized distribution and report the successful treatment with lenalidomide.

Pityriasis rosea–like eruption associated with lamotrigine

2013-06-01

To the Editor: Pityriasis rosea (PR)–a common, acute, self-limited eruption–is characterized by an initial herald patch followed by a diffuse papulosquamous rash without systemic manifestations. Occasionally a lesional skin biopsy is important to rule out other papulosquamous and erythematous disorders. PR-like drug eruptions from angiotensin-converting enzyme inhibitors, nonsteroidal anti-inflammatory drugs, barbiturates, and beta-blockers, but not from lamotrigine have been reported.

Effectiveness of topical maxacalcitol for acquired perforating disorder

Hiroyuki Yoshiya, Ryuhei Okuyama, Hisashi Uhara — 2013-06-01

To the Editor: We report the case of a 69-year-old Japanese woman with numerous pruritic eruptions on her trunk, arms, and legs that had existed for 5 months. She had had diabetes mellitus for 20 years and had received hemodialysis for renal failure 2 months prior to her presentation. On examination, multiple erythematous and umbilicated papules with a central keratotic plug, 3 to 5 mm in diameter, were scattered on the trunk and extremities (, A). Histopathologic examination revealed crusts containing keratin, neutrophil debris, and degenerated basophilic collagen fibers in the center of the acanthotic epidermis, an appearance consistent with acquired perforating disorder. Because the eruptions showed no response to topical corticosteroids and antihistamines, 0.0025% maxacalcitol ointment was applied twice a day. The patient’s pruritus was immediately alleviated after the treatment, and the keratotic papules disappeared within 2 months (, B). The total amount of the maxacalcitol ointment used during this period was 60 g.

Tumor necrosis factor inhibition and glucose homeostasis

Ammon J. Larsen, Jennie T. Clarke — 2013-06-01

To the Editor: Tumor necrosis factor (TNF) inhibitors are commonly used in the treatment of psoriasis. A large cross-sectional study demonstrated a strong correlation between psoriasis and the metabolic syndrome. Recently a retrospective cohort study suggested a reduced risk of diabetes mellitus in patients with rheumatoid arthritis or psoriasis receiving treatment with TNF inhibitors compared with those receiving other disease-modifying anti-rheumatic drugs. The authors suggest that TNF inhibitors may aid in the prevention of diabetes in patients with psoriasis. We report the development of hyperglycemia related to adalimumab therapy in a patient with psoriasis.

Pasteurella multocida cellulitis in a 15-year-old male with chronic lymphedema

Rachel C. Blasiak, Dean S. Morrell, Craig N. Burkhart — 2013-06-01

To the Editor: Pasteurella species are known to colonize the oropharynx of many animals, with Pasteurella canis and multocida being the most commonly isolated organisms from dog and cat bites, respectively. P multocida is a gram-negative coccobacillus that can cause local wound infections, bacteremia, pneumonia, sepsis, and meningitis associated with a history of animal bite or exposure. We report an unusual case of successfully treated P multocida cellulitis.

Acquired perforating dermatosis associated with natalizumab

Enric Piqué-Duran, Pablo Eguía, Odalys García-Vázquez — 2013-06-01

To the Editor: Natalizumab is a biologic agent used to treat relapsing-remitting multiple sclerosis (RRMS). It is a monoclonal anti-α-4 integrin antibody that interferes with the migration of immune cells into the central nervous system by binding to the α4 subunit integrin subunit of the α4-β1-integrin expressed by lymphocytes. Thus, the binding between this integrin and the endothelial receptor VCAM-1 is blocked, and the lymphocytes are retained inside the blood vessels, preventing the inflammation in the central nervous system. Cutaneous side effects associated with natalizumab include melanoma, urticaria, and hypersensitivity reactions.

Acute treatment of generalized pustular psoriasis of von Zumbusch with single-dose infliximab

2013-06-01

To the Editor: Generalized pustular psoriasis of von Zumbusch (GPP-VZ) is an acute form of psoriasis with erythematous, painful skin and widespread sterile pustules. In severe cases, pustules coalesce and form lakes of pus that rupture, resulting in desquamation. Disruption of the skin barrier predisposes to life-threatening complications, including infection, hemodynamic instability, and electrolyte imbalances, notably hypocalcemia. Systemic abnormalities include fever, malaise, and leukocytosis.

Localized myxedema of thyroid disease

Nooshin K. Brinster, Algin B. Garrett, Tassia C. Kostopoulos — 2013-06-01

To the Editor: A 57-year-old woman presented with a several-month history of a painful growth on her right great toe at the site of bunion removal surgery 4 years earlier. Her medical history was significant for Graves disease diagnosed 20 years ago and treated with radioactive iodine. Levothyroxine postradioablation was started.

Dermoscopy of a Sister Mary Joseph nodule

Je-Ho Mun, Jeong-Min Kim, Hyun-Chang Ko, Byung-Soo Kim, Moon-Bum Kim — 2013-06-01

To the Editor: Dermoscopy is a useful tool for the diagnosis of cutaneous benign lesions and skin malignancy because it facilitates rapid and magnified in vivo observation of the lesion. However, few studies have been published regarding the use of dermoscopy for detecting cutaneous metastasis from internal malignancies.

Bullous pemphigoid associated with chronic renal allograft rejection

Juliano Peruzzo, Lia Dias Pinheiro Dantas, Marcia Zampese — 2013-06-01

To the Editor: We report a case of bullous pemphigoid (BP) in a young woman who presented with chronic renal allograft rejection, an unusual association. A 15-year-old girl developed nephrotic syndrome as a result of mesangial glomerulonephritis. She developed loss of renal function, requiring dialysis, and underwent kidney transplantation 3 years later. Ten years after the transplantation, she again presented with renal function impairment. A kidney biopsy specimen demonstrated chronic rejection, and she was again submitted to dialysis.

Red fingers syndrome in a patient with pseudolymphoma

Barry Ladizinski, Elise A. Olsen — 2013-06-01

To the Editor: A 31-year-old woman presented with a 10-year history of erythematous plaques on the face consistent with cutaneous pseudolymphoma. She incidentally was noted to also have dusky discoloration of her distal fingers and hypothenar eminences bilaterally (), but not toes, and without associated pain, numbness, or accentuation in the cold. Onycholysis was noted on several fingernails, but clubbing or periungual telangiectasias were not present. The patient related that the redness was not present at birth and appeared during her adult years before her pseudolymphoma lesions. She also noted that the redness appeared on the fingers and palms simultaneously and did not wax and wane. She was not using any prescribed or over-the-counter medications at the time of onset or at presentation. She further denied use of alcohol or illicit drugs and was otherwise without medical problems. Screening serologies for HIV, hepatitis B virus (HBV), hepatitis C virus (HCV), Lyme disease and mononucleosis produced unremarkable findings. A biopsy was offered, but the patient declined as the redness was asymptomatic. She had complete clearance of pseudolymphoma lesions with interferon alfa-2b therapy, but no change in the distal finger discoloration was observed.

Carcinomatous lymphangitis in lymphoepithelial carcinoma of the parotid

2013-06-01

To the Editor: A 73-year-old Caucasian woman presented with a 9-month history of an erythematous patch on the right side of her neck. Her medical history was unremarkable, except for a lymphoepithelial carcinoma of the parotid treated with radiotherapy and chemotherapy in 2010 that had been asymptomatic in all the follow-up visits with only mild radiodermatitis and lymphedema.

Bullae and psoriasiform hyperkeratosis after treatment with golimumab

Lauren M. Madigan, Benjamin H. Kaffenberger, Henry K. Wong — 2013-06-01

To the Editor: Golimumab is a human monoclonal antibody that binds with high specificity and affinity to tumor necrosis factor (TNF)-alfa and is Food and Drug Administration approved for the treatment of moderate to severe rheumatoid arthritis, active psoriatic arthritis, and ankylosing spondylitis. This agent has also shown promise in the treatment of severe psoriasis. In the GO-REVEAL study, greater than 60% of patients with significant skin disease experienced a 75% reduction in the Psoriasis Area and Severity Index score when treated for 52 weeks. The safety of golimumab has been evaluated in at least 13 clinical trials, and although these studies did not demonstrate a large number of dermatologic complications, reports of such adverse effects have been observed. One striking outcome is the finding that golimumab, and other TNF-alfa blockers, are paradoxically associated with the onset/worsening of psoriatic skin lesions. A recent literature review discovered 104 cases of new onset or worsening cutaneous psoriasis in patients treated with anti–TNF agents for various conditions.

Iotaderma #233

Robert I. Rudolph — 2013-06-01

May iotaderma (#232)

Robert I. Rudolph — 2013-06-01

Editorial Board

2013-06-01

Information for Readers

2013-06-01

Journal of the American Academy of Dermatology

What's new in pediatric dermatology? Part I. Diagnosis and pathogenesis

CME examination

Answers to CME examination

Change of Address

What's new in pediatric dermatology? Part II. Treatment

CME examination

Important Notice Regarding JAAD Grand Rounds

Answers to CME examination

American Board of Dermatology Examination Dates

Pediatric melanoma: Results of a large cohort study and proposal for modified ABCD detection criteria for children

Pediatric dermatology inpatient consultations: A retrospective study of 427 cases

Dermatologic manifestations of ataxia-telangiectasia syndrome

Androgenetic alopecia and risk of prostate cancer: A systematic review and meta-analysis

Photoprotective habits of patients with cutaneous lupus erythematosus

Sunscreen use while driving

Cutaneous squamous cell carcinoma: Estimated incidence of disease, nodal metastasis, and deaths from disease in the United States, 2012

Racial differences in mycosis fungoides: A retrospective study with a focus on eosinophilia

Improvement in peripheral blood disease burden in patients with Sézary syndrome and leukemic mycosis fungoides after total skin electron beam therapy

Cutaneous plasmacytosis: Report of 6 cases with or without systemic involvement

Oral candidiasis in patients with psoriasis: Correlation of oral examination and cytopathological evaluation with psoriasis disease severity and treatment

Psoriasis is associated with an increased risk of parkinsonism: A population-based 5-year follow-up study

The importance of dedicated dermoscopy training during residency: A survey of US dermatology chief residents

Subcutaneous Sweet syndrome in the setting of myeloid disorders: A case series and review of the literature

A syringotropic variant of cutaneous sarcoidosis: Presentation of 3 cases exhibiting defective sweating responses

Contraception and the dermatologist

E-professionalism at the dermatology office: New challenges to confidentiality in the era of social networking

Announcing New Features in JAAD Online

Diabetes insipidus, bone lesions, and new-onset red-brown papules in a 42-year-old man

Internet dermatology resources

Physician Global Assessment (PGA) and Psoriasis Area and Severity Index (PASI): Why do both? A systematic analysis of randomized controlled trials of biologic agents for moderate to severe plaque psoriasis

Novel systemic drugs for psoriasis: Mechanism of action for apremilast, a specific inhibitor of PDE4

Add Pittsburgh teledermatology “with a twist” to the map!

Reply

Methotrexate for the treatment of cutaneous dermatomyositis

Cantharidin therapy: Practice patterns and attitudes of health care providers

Investigation of molluscum contagiosum virus, orf and other parapoxviruses in lymphomatoid papulosis

Successful treatment of alopecia totalis with hydroxychloroquine: Report of 2 cases

Dermatology Calendar

Cover Sheet for Index

Author index

Subject index

A young boy with a perioral eruption

Asymptomatic crusted lesions

Patients' educational needs about topical treatments for psoriasis

Melanoma death prevention: Moving away from the sun

A de novo SOX10 mutation in a patient with Waardenburg syndrome type IV

Disseminated keratotic spicules: A rare manifestation in multiple myeloma and successful response to lenalidomide

Pityriasis rosea–like eruption associated with lamotrigine

Effectiveness of topical maxacalcitol for acquired perforating disorder

Tumor necrosis factor inhibition and glucose homeostasis

Pasteurella multocida cellulitis in a 15-year-old male with chronic lymphedema

Acquired perforating dermatosis associated with natalizumab

Acute treatment of generalized pustular psoriasis of von Zumbusch with single-dose infliximab

Localized myxedema of thyroid disease

Dermoscopy of a Sister Mary Joseph nodule

Bullous pemphigoid associated with chronic renal allograft rejection

Red fingers syndrome in a patient with pseudolymphoma

Carcinomatous lymphangitis in lymphoepithelial carcinoma of the parotid

Bullae and psoriasiform hyperkeratosis after treatment with golimumab

Iotaderma #233

May iotaderma (#232)

Editorial Board

Table of Contents

Information for Readers