Journal of the American Academy of Dermatology

Alopecia areata update: Part I. Clinical picture, histopathology, and pathogenesis

Abdullah Alkhalifah, Adel Alsantali, Eddy Wang, Kevin J. McElwee, Jerry Shapiro — 2010-02-01

Alopecia areata (AA) is an autoimmune disease that presents as nonscarring hair loss, although the exact pathogenesis of the disease remains to be clarified. Disease prevalence rates from 0.1% to 0.2% have been estimated for the United States. AA can affect any hair-bearing area. It often presents as well demarcated patches of nonscarring alopecia on skin of overtly normal appearance. Recently, newer clinical variants have been described. The presence of AA is associated with a higher frequency of other autoimmune diseases. Controversially, there may also be increased psychiatric morbidity in patients with AA. Although some AA features are known poor prognostic signs, the course of the disease is unpredictable and the response to treatment can be variable. Part one of this two-part series on AA describes the clinical presentation and the associated histopathologic picture. It also proposes a hypothesis for AA development based on the most recent knowledge of disease pathogenesis.Learning objectives: After completing this learning activity, participants should be familiar with the most recent advances in AA pathogenesis, recognize the rare and recently described variants of AA, and be able to distinguish between different histopathologic stages of AA.

CME examination

2010-02-01

Answers to CME examination

2010-02-01

Announcing a New Online E-Blue Feature

2010-02-01

Alopecia areata update: Part II. Treatment

Abdullah Alkhalifah, Adel Alsantali, Eddy Wang, Kevin J. McElwee, Jerry Shapiro — 2010-02-01

Various therapeutic agents have been described for the treatment of alopecia areata (AA), but none are curative or preventive. The aim of AA treatment is to suppress the activity of the disease. The high rate of spontaneous remission and the paucity of randomized, double-blind, placebo-controlled studies make the evidence-based assessment of these therapies difficult. The second part of this two-part series on AA discusses treatment options in detail and suggests treatment plans according to specific disease presentation. It also reviews recently reported experimental treatment options and potential directions for future disease management.Learning objectives: After completing this learning activity, participants should be able to compare the efficacy and safety of various treatment options, formulate a treatment plan tailored to individual patients, and recognize recently described treatments and potential therapeutic approaches.

CME examination

2010-02-01

Answers to CME examination

2010-02-01

American Board of Dermatology Examination Dates

2010-02-01

Infliximab therapy for patients with moderate to severe hidradenitis suppurativa: A randomized, double-blind, placebo-controlled crossover trial

2010-02-01

Background: Biologic therapies with anti–tumor necrosis factor agents are promising treatments for hidradenitis suppurativa (HS).Objective: We assessed the efficacy and safety of infliximab (IFX) for the treatment of moderate to severe HS.Methods: A prospective double-blind treatment phase of 8 weeks where patients received IFX or placebo was followed by an open-label phase where patients taking placebo were given the opportunity to cross over to IFX, and an observational phase. Primary treatment efficacy was based on HS Severity Index. Secondary end points included Dermatology Life Quality Index, visual analog scale, and Physician Global Assessment scores. Inflammatory markers erythrocyte sedimentation rate and C-reactive protein were also assessed.Results: More patients in the IFX than in the placebo group showed a 50% or greater decrease from baseline HS Severity Index score. In addition, statistically and clinically significant improvement from baseline was observed at week 8 in Dermatology Life Quality Index score, visual analog scale score, erythrocyte sedimentation rate, and C-reactive protein compared with placebo. Patients in the placebo group treated with IFX after week 8 (crossover) responded similarly to the original IFX group. Many patients withdrew during the observational phase to continue anti–tumor necrosis factor-alfa therapy. No unexpected serious adverse events were observed.Limitations: Results are representative of a single center, patients were treated by a single physician, some patients did not return after their last infusion, and the HS Severity Index requires validation.Conclusions: This clinical study represents the first formal assessment of IFX for treatment of moderate to severe HS. IFX was well tolerated, no unexpected safety issues were identified, and improvements in pain intensity, disease severity, and quality of life were demonstrated with concomitant reduction in clinical markers of inflammation.

The relation between the amount of sunscreen applied and the sun protection factor in Asian skin

Sang Min Kim, Byung Ho Oh, Yang Won Lee, Yong Beom Choe, Kyu Joong Ahn — 2009-12-07

Background: The measurement of the sun protection factor (SPF) is the usual method in the examination of the effectiveness of sunscreen. The declared SPF is based on the use of a sunscreen layer of 2 mg/cm2. However, only around a quarter (0.5 mg/cm2) of this amount is generally used in real life. Theoretical calculations have suggested that the effectiveness of SPF is related to sunscreen quantity in an exponential way but this was not confirmed in Asian skin.Objectives: This study was performed to investigate the change in SPF values when less than the recommended amount of sunscreen was applied.Methods: A board divided into 10 areas measuring 7 × 4 cm was placed on the back of 15 healthy volunteers. Low- and high-SPF standard reference sunscreens, and two types of sunscreen (SPF 30 and 35) were each applied on 4 areas, 0.5, 1.0, 1.5, and 2.0 mg/cm2, respectively, and were left to dry for 20 minutes. The irradiation was conducted at a distance of 50 cm using a template (1 × 1 cm) placed directly on the skin with 10 windows allowing ultraviolet (UV) radiation to pass through with a dose increment of 20%. Erythema was evaluated 20 to 24 hours after exposure to UV radiation.Results: Sunscreen showed its expected SPF value when 2.0 mg/cm2 was applied. The SPF values of the different amounts were significantly different from each other and decreased when less was applied (P < .05). The relation between the amount of sunscreen applied and the SPF provided was most likely to follow exponential growth.Limitations: Spectral differences between our solar simulator and the UV sources of commercial laboratories are likely to be important. In addition, differences in sunscreen application techniques may have influenced the ultimate SPF values.Conclusions: This study concludes that to get the expected SPF value, it is important to apply the UV protective sunscreen precisely in the amount of 2.0 mg/cm2 on Asian skin as recommended by the Food and Drug Administration. In addition, it was difficult to predict the SPF values when the usual amount of 0.5 mg/cm2 was applied

Standards for genital protection in phototherapy units

Farah R. Abdulla, Christopher Breneman, Brian Adams, Debra Breneman — 2009-12-11

Background: Phototherapy is a useful therapy for many dermatologic disorders and is known for its low side-effect profile. However, one potential notable side effect is genital skin cancer. Unfortunately, no standards for genital protection currently exist for this preventable complication. Patients treated with phototherapy may already have a decreased quality of life because of their primary dermatologic disorder. Development of squamous cell carcinoma of the genitalia may certainly further affect the quality of life.Objective: The objective was to determine which readily available materials afford the best photoprotection of the male genitalia.Methods: Seven common materials used in phototherapy units for genital protection were placed over ultraviolet (UV) B and UVA monitors and placed in broadband UVB, narrowband UVB, and UVA full-body units. The percentage of light blocked was then calculated.Results: Blue and white cotton underwear, blue surgical towels, an athletic supporter with or without a cup, and the psoralen plus UVA pouch provided acceptable means of genital protection; however, surgical masks did not.Limitations: Only the most commonly used materials were tested in the phototherapy units. The materials were not of a single material type or similar masses. In addition, only one of each type of full-body phototherapy unit was used to obtain the data.Conclusion: Although a polyester composition provides better UV protection, factors such as low porosity and higher mass are intrinsic to decreasing the amount of UV penetration of any fabric. Of the commonly used objects, surgical masks do not provide sufficient protection to the genital area.

A population-based study of cutaneous melanoma in Alberta, Canada (1993-2002)

2009-12-17

Background: There is evidence to suggest that melanoma incidence rates continue to rise in Canada and the United States.Objective: Our objective was to determine cutaneous melanoma trends from 1993 to 2002 in the province of Alberta and to compare the results to previously published provincial analyses for the decade of 1967-1976.Methods: A retrospective study of 3479 patients with cutaneous melanoma diagnosed in Alberta between 1993 and 2002 was conducted. Estimates of relative survival compared the survival of melanoma patients with the Alberta population to derive the likelihood of surviving melanoma in the absence of other causes of death. Further comparison to published Canadian data was also conducted.Results: For the period 1993-2002, the annual melanoma age-standardized incidence rates per 100,000 person-years ranged between 11.1 and 15.9 and between 9.8 and 14.1 among men and women, respectively. These rates are considerably higher than the previously reported (1976) highest Alberta incidence rates of 4.1 and 4.8 in men and women, respectively. The rates increased slightly for the period 1993-1999 with an average annual percentage change of +3.5%, but appeared to decrease for the interval 1999-2002 with an average annual percentage change of −6.4%. The majority of the tumors were less than 1.0 mm in thickness for both genders. On univariate analysis the following parameters were associated with decreasing patient survival: male gender, increasing age, head and neck tumors, Clark level of invasion, and Breslow tumor thickness. Multivariate analysis demonstrated that the strongest determinant of survival was Breslow tumor thickness.Limitations: Melanomas in-situ were not included in this study.Conclusions: Although melanoma incidence rates in Alberta are higher than previously reported, the incidence rates over the study period of 1993 to 2002 appear to have leveled and may in fact be declining over the past several years.

Cheilitis glandularis: A clinicopathological study in 22 patients

Marcello Menta Simonsen Nico, Juliana Nakano de Melo, Silvia Vanessa Lourenço — 2009-12-11

Background: Cheilitis glandularis (CG) is a condition in which thick saliva is secreted by minor labial salivary glands and adheres to a swollen lip causing discomfort to the patient. Most publications refer to single case reports or small case series.Objective: We sought to report and to analyze clinical, pathological, and therapeutic data on 22 patients with CG seen at the department of dermatology at our university.Method: Retrospective data about 22 patients with CG are reviewed and presented.Results: Seventeen patients were male and 5 were female. All were fair skinned, including 6 albino individuals. Several of them presented significant signs of photodamage on the lips. Surgical treatment was performed in 10 severely affected patients and consisted of a vermilionectomy followed by minor salivary gland removal. Histopathological study revealed various degrees of chronic sialadenitis and vermilion epithelial changes. Superficially invasive and in situ squamous cell carcinoma of the vermilion was detected in 3 cases.Limitations: Biopsy and surgery were not performed in all patients.Conclusions: CG is strongly related to sun sensitivity and may be more severe in albino patients. The swollen, sun-exposed lip may become more susceptible to the occurrence of squamous cell carcinoma.

Efficacy of imipenem therapy for Nocardia actinomycetomas refractory to sulfonamides

2009-12-11

Background: Actinomycetomas are chronic, granulomatous, subcutaneous infections caused by actinomycetes bacteria. Despite prolonged high-dose and combination antibiotic therapies, some cases remain resistant with risks of bone and visceral involvement.Objectives: We sought to evaluate the efficacy and safety of imipenem monotherapy, and in combination with amikacin for the treatment of severe and refractory disease, and to identify the disease characteristics that might predict therapy failure with first-line sulfonamides.Methods: A retrospective study was performed of all microbiologically confirmed cases of actinomycetomas treated since 1995 at a tertiary center for mycology. Eleven patients (Nocardia, n = 10) were treated with sulfonamide combinations (trimethoprim/sulfamethoxazole and dapsone). Eight patients (Nocardia, n = 7) refractory to previous therapies including sulfonamides received a 3-week course of either parenteral imipenem monotherapy (1.5 g daily, n = 3) or combination therapy with amikacin (1 g daily, n = 5), which was repeated at 6-month intervals.Results: Eleven patients with limited disease and mean disease duration of 1.7 years responded successfully to sulfonamides after a mean treatment period of 15 months (range 6-48 months). Patients receiving imipenem had mean disease duration of 10 years, with visceral and bone involvement in 4 patients. Imipenem treatment was well tolerated, and 4 patients achieved clinical and microbiological cure after one to two courses of treatment, the others demonstrating greater than 75% clinical improvement and negative culture results.Limitations: Patient cohorts in this study were small because strict criteria for inclusion included species identification and adequate follow-up periods. The efficacy data for imipenem ± amikacin therapy cannot be extrapolated to all Nocardia mycetomas, as the cohort treated in this study had particularly refractory infection.Conclusions: Sulfonamides are effective for limited disease of relatively short duration. Imipenem monotherapy or in combination with amikacin is well tolerated and demonstrates efficacy in severe disease refractory to sulfonamides.

Clinical usefulness of variable-frequency ultrasound in localized lesions of the skin

Ximena Wortsman, Jacobo Wortsman — 2009-12-04

Background: High variable-frequency ultrasound is a recently available technique capable of clearly defining skin layers and deeper structures that also provides local perfusion patterns obtained in real time.Objectives: The aim of the study was to assess the performance of variable-frequency ultrasound in the evaluation of skin lesions.Methods: We performed a retrospective study of 4338 skin ultrasound examinations in predominantly localized skin lesions, and in a group of 130 healthy controls. We determined ultrasound sensitivity, specificity, and statistical level of certainty, and compared ultrasound diagnoses with clinical diagnoses.Results: Referring diagnosis was correct in 73% of the lesions, and addition of ultrasound increased correctness to 97% (P < .001 for the difference). Ultrasound overall sensitivity was 99%, specificity was 100%, and statistical diagnostic certainty was 99%Limitations: Ultrasound in its current version cannot detect lesions that are epidermal only or that measure less than 0.1 mm in depth.Conclusions: Ultrasound is a reliable adjuvant for the accurate and precise diagnosis of skin lesions.

How informative are dermatopathology requisition forms completed by dermatologists? A review of the clinical information provided for 100 consecutive melanocytic lesions

Jeanette M. Waller, Daniel C. Zedek — 2009-12-07

Background: Accurate clinicopathologic correlation can be crucial to arriving at the correct microscopic diagnosis.Objective: We reviewed the clinical information provided on the dermatopathology requisition forms for melanocytic lesions submitted by community dermatologists.Methods: The clinical information provided and the microscopic diagnoses rendered were recorded in a retrospective, unblinded fashion for 100 consecutive melanocytic lesions submitted as wet tissue to our dermatopathology department.Results: Biopsy specimens were received from 60 community dermatologists and 5 nurse practitioners/physician assistants. Clinical morphology (ie, papule) was provided in 33% of cases. With respect to the ABCDE criteria, 55% of cases had none, 12% had one criterion, 21% had two criteria, 10% had 3 criteria, 2% had 4 criteria, and none had all 5 criteria. No forms stated whether the biopsy specimen was a partial or complete sampling of the lesion. Asymmetry was provided 4% of the time, border irregularity 8%, color 39%, diameter 22%, and evolution 10%. A family or personal history of melanoma was provided in 8% of cases. No requisition forms mentioned the “ugly duckling” sign. Dermatoscopy information and a clinical photograph were provided once each. In 19 cases, the only information on the requisition form was one of the phrases: “r/o atypia,” “r/o atypical nevus,” “r/o Clark's,” or “r/o dysplastic nevus.” In 10 cases, the only information was “r/o nevus.”Limitations: Only 100 consecutive melanocytic lesions were studied in a retrospective, unblinded fashion.Conclusion: Important clinical information regarding pigmented lesions is often not provided on the requisition form. Potential reasons for this deficit and suggestions for improvement are discussed.

Ultrasound tightening of facial and neck skin: A rater-blinded prospective cohort study

2010-02-01

Background: Nonablative skin tightening technologies offer the prospect of reduction of wrinkles and skin sagging with minimal downtime, discomfort, and risk of adverse events. The excellent safety profile is mitigated by the limited efficacy of such procedures.Objective: We sought to assess the efficacy of ultrasound skin tightening for brow-lift in the context of a procedure treating the full face and neck.Methods: This was a rater-blinded, prospective cohort study at a dermatology clinic in an urban academic medical center. Subjects were medicated with topical anesthetic and then treated with an investigational focused intense ultrasound tightening device to the forehead, temples, cheeks, submental region, and side of neck using the following probes: 4 MHz, 4.5-mm focal depth; 7 MHz, 4.5-mm focal depth; and 7 MHz, 3.0-mm focal depth. Standardized photographs of front and side views were obtained at 2, 7, 28, 60, and 90 days; rating scales of pain, adverse events, physical findings, and patient satisfaction were also completed. Primary outcome measure was detection of improvement in paired comparison of pretreatment and posttreatment (day 90) photographs by 3 masked expert physician assessors, cosmetic and laser dermatologists, and plastic surgeons who were not authors. Second primary outcome measure was objective brow elevation as quantitated by a standard procedure using fixed landmarks. Secondary outcomes measure was patient satisfaction as measured by a questionnaire.Results: A total of 36 subjects (34 female) were enrolled, one subject dropped out, and 35 subjects were evaluated. Median age was 44 years (range 32-62). On the first primary outcome measure, 30 of 35 subjects (86%) were judged by the 3 masked experienced clinician raters to show clinically significant brow-lift 90 days after treatment (P = .00001). On the second primary outcome measure, mean value of average change in eyebrow height as assessed by measurement of photographs at 90 days was 1.7 mm.Limitations: Limitations of this study include the inability to quantitatively measure lower face tightening because of the lack of fixed anatomic landmarks in this area.Conclusion: Ultrasound appears to be a safe and effective modality for facial skin tightening. A single ultrasound treatment of the forehead produced on average brow height elevation of slightly less than 2 mm. Most treated individuals responded, commonly with accompanying transitory mild erythema and edema.

Perineural invasion progressing to leptomeningeal carcinomatosis: Is the absence of peripheral nerves an important sign?

Martin Dunn, Michael B. Morgan — 2010-02-01

Background: Leptomeningeal carcinomatosis (LMC) is a rare, frequently fatal complication of cranial nerve invasion by a primary cutaneous carcinoma.Objective: Complete absence of nerve tissue was observed in retrospect in a Mohs surgery case that had subsequently proceeded to LMC. We theorized that the cancer invaded the perineural space and spread contiguously until meeting a point of resistance, in this case the mental foramen. Pressure on the nerve resulted in Wallerian degeneration of the peripheral nerve, causing progressive anesthesia and obscuring the presence of perineural invasion (PNI). Our objective was to find out if this was an isolated phenomenon or an important sign of PNI progressing to LMC.Methods: We report and describe a case and review similar case reports in the literature.Results: Twenty-two case reports fit our criteria. In only two cases was carcinomatous PNI reported in initial surgical specimens.Limitations: There are relatively few similar case reports in the literature. In many reports, histopathologic findings are not detailed enough to be helpful. Absence of peripheral nerves, although inferred, is not specifically stated in any other case report.Conclusion: The absence of nerve tissue in a Mohs surgery specimen, coupled with signs of cranial nerve involvement, could be important early indicators of PNI progressing to LMC.

Trichoadenoma of Nikolowski is a distinct neoplasm within the spectrum of follicular tumors

Iakov Shimanovich, Dieter Krahl, Christian Rose — 2010-02-01

Background: Trichoadenoma is a rare benign follicular tumor first described by Nikolowski 50 years ago. Both trichoadenoma and desmoplastic trichoepithelioma are composed of cords of epithelial cells and cornifying cysts embedded in sclerotic stroma. In trichoadenoma the cystic component predominates, while desmoplastic trichoepithelioma is a mostly solid neoplasm. Therefore trichoadenoma was suggested to represent a cystic variant of desmoplastic trichoepithelioma.Objective: The aim of this study was to investigate whether the morphologic overlap between trichoadenoma and desmoplastic trichoepithelioma translates into a similar immunohistochemical profile.Methods: We studied 19 trichoadenomas and 21 desmoplastic trichoepitheliomas for cytokeratin 20, Ber-EP4, and androgen receptor expression.Results: Eighteen of 19 trichoadenomas and all desmoplastic trichoepitheliomas demonstrated the presence of Merkel cells as detected by a monoclonal antibody against cytokeratin 20. In contrast, while all desmoplastic trichepitheliomas were positive for Ber-EP4, only 4 of 19 trichoadenomas showed any kind of reactivity for this marker. None of the trichoadenomas or desmoplastic trichoepitheliomas expressed androgen receptor.Limitations: This study is limited by the moderate number of these rare tumors available for immunohistochemical analysis.Conclusion: Our data demonstrate that trichoadenoma typically retains cytokeratin 20–positive Merkel cells but lacks Ber-EP4 and androgen receptor expression. Trichoadenoma is a distinct follicular tumor related but not identical to desmoplastic trichoepithelioma.

Analysis of alterations adjacent to invasive squamous cell carcinoma of the penis and their relationship with associated carcinoma

2010-02-01

Background: In contrast to vulvar squamous cell carcinoma (SCC), the etiologic factors and precancerous lesions associated with penile carcinoma remain uncertain.Objectives: To describe the morphologic features of lesions adjacent to invasive penile SCC and their relationship with the associated carcinoma and to compare these associations with vulvar carcinoma.Methods: This was a retrospective histologic analysis of 68 cases of penile SCC. Adjacent lesions were considered to be premalignant lesions. They were classified as penile intraepithelial neoplasia (PIN), squamous hyperplasia (SH), and lichen sclerosus (LS). PIN cases were divided into two subtypes depending on the extension of atypia throughout the epithelium and, by analogy, with the classification of the vulvar intraepithelial neoplasia (VIN). Thus they were designated as undifferentiated (or bowenoid) PIN, defined by full-thickness atypia throughout the epithelium, and differentiated PIN, characterized by atypia confined to the lower third of the epithelium. SCC subtypes were classified as usual, verrucous, warty (condylomatous), basaloid, and mixed.Results: Undifferentiated PIN was observed in 22 cases; LS was observed in 26 cases. Differentiated PIN and SH (except for two cases) were associated with underlying LS. Undifferentiated PIN was always associated with warty (condylomatous) (4 cases), basaloid (16 cases) or mixed SCC (2 cases), and LS with usual (19 cases) or verrucous SCC (7 cases).Limitations: This was a retrospective analysisConclusion: This study suggests that, similarly to vulvar carcinoma, penile SCC occurs in association with two types of penile lesions: undifferentiated (or bowenoid) PIN and LS-linked differentiated PIN and/or SH. It appears that the subtype of these carcinomas is related to these adjacent lesions.

Psoriasis in patients with HIV infection: From the Medical Board of the National Psoriasis Foundation

2009-08-03

Background: Patients with psoriasis and HIV infection often present with more severe and treatment-refractory cutaneous disease. In addition, many of these patients have significant psoriatic arthritis. Many effective drugs for psoriasis and psoriatic arthritis are immunosuppressive. Therefore, therapy for the HIV-infected patient is more challenging, requiring both careful consideration of the potential risks and benefits of treatment and more fastidious monitoring for potential adverse events.Objective: A task force of the National Psoriasis Foundation Medical Board was convened to evaluate treatment options. Our aim was to arrive at a consensus on therapy for psoriasis in patients with HIV.Methods: A MEDLINE search of the terms “psoriasis,” “psoriatic arthritis,” “human immunodeficiency virus (HIV),” and “HIV skin diseases” was performed and literature relevant to HIV-associated psoriasis and the treatment of HIV-associated psoriasis were reviewed.Results: Based on a review of the literature, 29 reports were included as evidence in this review. Topical therapy is the first-line recommended treatment for mild to moderate disease. For moderate to severe disease, phototherapy and antiretrovirals are the recommended first-line therapeutic agents. Oral retinoids may be used as second-line treatment. For more refractory, severe disease, cautious use of cyclosporine, methotrexate, hydroxyurea, and tumor necrosis factor-α inhibitors may also be considered.Limitations: There are no randomized, placebo-controlled trials evaluating the therapeutic efficacy or safety of treatments for patients with HIV-associated psoriasis; consequently, the evidence supporting this review consists mainly of case reports or case series.Conclusions: HIV-associated psoriasis is often refractory to traditional treatments. Treatment is challenging and requires careful consideration and should be tailored to patients based on disease severity and the input from an infectious disease specialist. Close monitoring for potential adverse events is necessary.

The National Institute of Allergy and Infectious Diseases to Seek Public Comment on Food Allergy Practice Guidelines

2010-02-01

Primary cutaneous aggressive epidermotropic CD8+ T-cell lymphoma

2009-11-30

Cutaneous T-cell lymphomas most commonly have a CD4+ memory T-cell phenotype with relatively indolent course, but may in rare cases present with a CD8+ cytotoxic phenotype exhibiting strikingly more aggressive clinical behavior. We present two cases of the clinically aggressive subtype of primary cutaneous epidermotropic CD8+ cutaneous T-cell lymphoma and review the current literature, clinical behavior, and recommendations for treatment distinct from that of more common CD4+ variants of cutaneous T-cell lymphoma.

Juvenile temporal arteritis

Olga K. Kolman, Henry M. Spinelli, Cynthia M. Magro — 2009-09-14

Juvenile temporal arteritis (JTA) is a nongranulomatous inflammation of the temporal artery with fragmentation of the internal elastic lamina and no concurrent systemic manifestations. It is a rare clinicopathologic entity with fewer than 20 reported cases, most of which represent localized disease with no recurrence or systemic symptoms at follow-up of up to 2 years. Histopathologic features can include lymphoeosinophilic infiltrate and endothelial proliferation. As the histology may resemble angiolymphoid hyperplasia with eosinophilia or Kimura disease, whether JTA is a discrete localized disease or a manifestation of these systemic conditions has been debated. We present a case of a 36-year-old Jamaican woman with a painful forehead nodule that showed histologic features of JTA, including intimal hyperplasia, lymphoeosinophilic inflammation of the vessel wall, and disruption of the internal elastic lamina; distinctive signet ringlike cytomorphologic alterations of the endothelial cells were noted as well. The lesion also showed extensive subcutaneous lymphoeosinophilic infiltrates and neovascularization with extension into the underlying muscle consistent with angiolymphoid hyperplasia with eosinophilia or Kimura disease. As the connection between JTA and angiolymphoid hyperplasia with eosinophilia and Kimura disease is currently debated and most reported cases of JTA have had only brief follow-up, the long-term sequelae of JTA are not known and careful patient monitoring may be necessary.

Minocycline-induced drug reaction with eosinophilia and systemic symptoms (DRESS) syndrome with persistent myocarditis

2009-08-10

Drug reaction with eosinophilia and systemic symptoms (DRESS) syndrome is a rare serious adverse effect associated with a variety of medications. We present a case of minocycline-induced DRESS syndrome, which resulted in acute renal failure, transient thyroiditis, and transaminitis, and a persistent lymphocytic myocarditis resulting in congestive heart failure. To our knowledge, this is the third reported case of minocycline-induced myocarditis. Additionally, we report successful plasmapheresis and rituximab treatment for minocycline-induced myocarditis associated with the DRESS syndrome.

Erratum

2010-02-01

Warshaw EM, Lederle FA, Grill JP, Gravely AA, Bangerter AK, Fortier LA, et al. Accuracy of teledermatology for pigmented neoplasms. J Am Acad Dermatol 2009;61:753-65. In the article above, the final row of Table V printed incorrectly. The corrected table appears below.

Change of Address

2010-02-01

Cutaneous B-cell lymphoma with histologic features of mycosis fungoides

Sarah Chiang, David J. DiCaudo, Riccardo Valdez, David L. Swanson — 2009-10-08

Histologic examination of skin biopsy specimens from a 57-year-old man with a pruritic rash on his chest, abdomen, and thighs revealed a dense atypical dermal lymphoid infiltrate bordering the dermoepidermal junction and scattered intraepithelial lymphocytes. Histopathologic and clinical features were suggestive of mycosis fungoides. Immunophenotyping studies, however, identified CD20+ B lymphocytes with aberrant expression of CD43. Clonal immunoglobulin gene rearrangement was demonstrated, and no clonal T-cell gene rearrangement was identified. Morphologic and immunophenotypic features were most consistent with extranodal marginal zone B-cell lymphoma. Systemic evaluation identified involvement of the bone marrow and possibly the peripheral blood, spleen, and splenic lymph nodes. Cutaneous manifestations were treated with narrowband ultraviolet B phototherapy for 3 months, resulting in complete resolution of the pruritus and rash. Primary and secondary cutaneous B-cell lymphomas seldom mimic mycosis fungoides histologically. Immunophenotyping studies were critical in correctly classifying this rare example of an epidermotropic B-cell lymphoma.

Congenital pigment synthesizing melanoma of the scalp

2010-02-01

The pigment synthesizing melanoma, so-called animal type melanoma, is a rare variant of melanoma that is characterized by prominent melanin production and an unpredictable prognosis. Congenital onset of this melanoma is exceedingly rare. A 2-month-old Korean girl had a black nodule and a satellite black macule on the scalp which were noticed at birth. She received a surgical resection 3 months later because of rapidly growing lesions and the histopathologic features of a pigment synthesizing melanoma. Two months later, she returned with cervical area swelling, and the excised multiple lymph nodes showed metastatic malignant melanoma. The exact origin and pathogenesis of congenital pigment synthesizing melanoma is different from the more common forms of melanoma and remains poorly understood.

A child with coexistent juvenile xanthogranuloma and Langerhans cell histiocytosis

Hong Yu, Jincheng Kong, Yan Gu, Bo Ling, Zhengjun Xi, Zhirong Yao — 2009-12-07

A 15-month-old boy was seen because of two distinct types of lesions, namely, yellowish papules on the scalp and face, and hemorrhagic macules and papules on the trunk. A biopsy specimen from one of the yellowish papules showed histopathologic and immunohistochemical changes of both juvenile xanthogranuloma and Langerhans cell histiocytosis. The section from the center of the biopsy specimen showed a proliferation of foamy histiocytes, among them Touton giant cells, which were positive for CD68, but negative for S-100 and CD1a. At the edges of the specimen was a predominantly histiocytic infiltration in the papillary dermis that was positive for S-100 and CD1a, but negative for CD68. The patient died 12 days after hospital admission consequent to disseminated intravascular coagulopathy. We did not biopsy the hemorrhagic lesions; however, this combination of findings suggests a possible relationship between juvenile xanthogranuloma and Langerhans cell histiocytosis, as previously reported.

Synovitis-acne-pustulosis-hyperostosis-osteitis (SAPHO) syndrome complicated by seven pulmonary emboli in a 15-year old patient

Jacquelyn Coloe, Stephanie Diamantis, Frederick Henderson, Dean S. Morrell — 2010-02-01

SAPHO (synovitis, acne, pustulosis, hyperostosis, and osteitis) syndrome represents a spectrum of various dermatologic and musculoskeletal conditions. Thromboses have infrequently been reported in SAPHO syndrome, most often in the subclavian vein. There have been no reported cases of pulmonary emboli associated with SAPHO. We report a case of a young patient with SAPHO syndrome who later presented with extensive iliofemoral deep vein thromboses and seven pulmonary emboli.

Tissue gadolinium deposition and fibrosis mimicking nephrogenic systemic fibrosis (NSF)–subclinical nephrogenic systemic fibrosis?

Alan S. Boyd, Soma Sanyal, Jerrold L. Abraham — 2009-11-25

Nephrogenic systemic fibrosis is a condition with significant and often debilitating cutaneous manifestations. Recent research on this disease has delineated an association between nephrogenic systemic fibrosis and exposure to magnetic resonance imaging studies using gadolinium-based contrast agents in patients with ongoing renal failure. This metal has been detected in cutaneous biopsy specimens taken from lesional skin suggesting that gadolinium provides an impetus for the deposition of circulating fibrocytes in the skin and subsequent fibrosis. We describe a hemodialysis-dependent liver transplant recipient who received a gadolinium-based contrast agent and demonstrated insoluble gadolinium deposition in a fibrotic dermis and subcutaneous septum using scanning electron microscopy/energy dispersive x-ray spectroscopy. He has yet to manifest symptoms and signs of nephrogenic systemic fibrosis 3 years after his magnetic resonance imaging study.

Rapid improvement of nephrogenic systemic fibrosis with rapamycin therapy: Possible role of phospho-70-ribosomal-S6 kinase

2010-02-01

Nephrogenic systemic fibrosis (NSF) is a fibrosing disorder that occurs in some patients with renal insufficiency. Exposure to gadolinium-based contrast agents (GdCA) has been associated with the development of NSF. No uniformly effective treatment options exist. We present immunohistochemical evidence to show that the proliferating fibrocytes of NSF express phospho-70-s6 kinase (PI-3-K), a protein downstream of PI-3-K, and the target of the drug rapamycin. In our patient, use of rapamycin resulted in rapid clinical improvement marked by reduced edema, reduced skin induration, and decreased pain. This suggests a possible role for PI-3-K and rapamycin (mTOR) pathways in the pathogenesis of NSF. Drugs that inhibit these pathways may be a target for future therapy. While our patient did attribute disease onset to GdCA exposure, used on a single occasion for abdominal imaging, he was also exposed to iron, calcium, and darbepoetin alpha at the time of imaging.

Botox and Dysport: Is there a dose conversion ratio in dermatology and aesthetic medicine?

Syrus Karsai, Christian Raulin — 2010-02-01

To the Editor: The use of botulinum neurotoxin A is the most frequent intervention in aesthetic medicine. Sales of the two main preparations—Botox (Allergan Inc, Irvine, CA) and Dysport (Ipsen Ltd, Slough, Berkshire, UK)—amounted to USD $1 billion in 2007, and the trend is still growing.

Conflict of interest?

Eric W. Hossler — 2010-02-01

To the Editor: I read with great interest the article comparing various treatment modalities for nonmelanoma skin cancer in the July 2009 issue of the Journal. As a critical reader, I typically pay attention to the “financial disclosure” section listed for each article. In this article, no financial conflicts were declared.

A comparison of sunburn protection of high–sun protection factor (SPF) sunscreens: SPF 85 sunscreen is significantly more protective than SPF 50

Julie E. Russak, Theresa Chen, Yohini Appa, Darrell S. Rigel — 2010-02-01

To the Editor: On August 27, 2007, the US Food and Drug Administration (FDA) released a proposed amendment to the 1999 sunscreen monograph. An important aspect of this amendment is that the FDA has proposed that manufacturers label their products with a specific sun protection factor (SPF) value up to 50; sunscreens with SPFs >50 would be labeled as 50+. We conducted a double-blind, randomized, split-face clinical study to evaluate whether a statistically significant difference exists between the sunburn protective effects of a SPF 50 sunscreen versus a SPF 85 formulation.

Thyroid autoimmunity and pemphigus vulgaris: Is there a significant association?

2010-02-01

To the Editor: The association of pemphigus with autoimmune thyroid diseases, namely Hashimoto thyroiditis and Graves disease, is well known. Nevertheless, controlled studies evaluating thyroidal autoimmunity in pemphigus patients are limited in the English-language literature.

The set-back buried dermal suture: An alternative to the buried vertical mattress for layered wound closure

Jonathan Kantor — 2010-02-01

To the Editor: Key surgical principles include minimizing dead space, encouraging wound eversion, minimizing tension across the epidermal component of the wound, restricting absorbable suture material to the reticular dermis, and maintaining adequate vascular supply to the healing wound. Twenty years ago, Zitelli and Moy described the buried vertical mattress suture. Several other modifications of absorbable dermal suturing techniques have been proposed, including the modified buried vertical mattress technique of Sadick et al, the percutaneous buried vertical mattress suture, and the fully buried horizontal mattress suture described by Alam and Goldberg.

Localized lymphomatoid papulosis

Ya-Jing Hsu, Lin-Hui Su, Yu-Ling Hsu, Tsung-Hua Tsai, Cheng-Hsiang Hsiao — 2010-02-01

To the Editor: Lymphomatoid papulosis (LyP) is a chronic, recurrent, self-healing papulonecrotic or papulonodular cutaneous disorder with histologic characteristics suggestive of a CD30+ lymphoma. It is regarded as indolent cutaneous T-cell lymphoma in the World Health Organization–European Organization for Research and Treatment of Cancer classification for cutaneous lymphomas. Lesions of LyP are typically scattered on the trunk and extremities in a generalized pattern, but a localized variant or a regional distribution may be encountered. We report a case of LyP with an unusual localized presentation.

Pigmented Bowen disease of the palm: An atypical case diagnosed by dermoscopy

2010-02-01

To the Editor: Bowen disease (BD) is a squamous cell carcinoma in situ that typically presents as a scaly erythematous plaque. On rare occasions, the lesion is pigmented and may resemble seborrheic keratosis, actinic keratosis, basal cell carcinoma, atypical nevus, or melanoma. We report an exceptional case of pigmented BD of the palm clinically suspicious for melanoma, whose correct diagnosis was suggested by dermoscopy.

Multiple palisaded encapsulated neuromas in a child without other associated abnormalities

Rachael L. Moore, Clifton R. White — 2010-02-01

To the Editor: Palisaded encapsulated neuroma (PEN) is a benign, typically solitary neural neoplasm seen in adults. Multiple mucosal neuromas are seen in multiple endocrine neoplasia (MEN) 2b syndrome. Rarely, multiple cutaneous PENs have been reported in adult patients with and without features of MEN2b. We report a case of 7-year-old white female who presented to our clinic with a 3-year history of three 3- to 4-mm, skin-colored papules on her nose and one on each foot (). The papules were asymptomatic and there was no history of trauma. A biopsy of the lesion on the right foot revealed a well circumscribed proliferation of cells with delicate spindled nuclei in a loose myxoid stroma in the superficial dermis, findings that are characteristic of PEN ().

Bullous lesions with scarring and mutilation

David Roffwarg, Allison Brown, Marcie Alisangco, Loretta S. Davis, Greg Simpson — 2010-02-01

A 52-year-old white male presented with worsening blistering skin lesions over the past 2 weeks and reported a history of similar symptoms since shortly after birth. The physical examination was notable for vesicles, bullae, scarring, and mutilation of the face, hands, and forearms, while the upper arms, trunk, and lower extremities appeared normal ( and ). Laboratory values were remarkable for low hemoglobin and iron, and markedly elevated urine porphyrins. The patient mentioned that a distant family member has a similar skin condition.

Iotaderma #193

Robert I. Rudolph — 2010-02-01

January iotaderma (#192)

Robert I. Rudolph — 2010-02-01

Dermatology Calendar

2010-02-01

Editorial Board

2010-02-01

Information for Readers

2010-02-01

Instructions for Category 1 CME Credit

2010-02-01

Journal of the American Academy of Dermatology

Alopecia areata update: Part I. Clinical picture, histopathology, and pathogenesis

CME examination

Answers to CME examination

Announcing a New Online E-Blue Feature

Alopecia areata update: Part II. Treatment

CME examination

Answers to CME examination

American Board of Dermatology Examination Dates

Infliximab therapy for patients with moderate to severe hidradenitis suppurativa: A randomized, double-blind, placebo-controlled crossover trial

The relation between the amount of sunscreen applied and the sun protection factor in Asian skin

Standards for genital protection in phototherapy units

A population-based study of cutaneous melanoma in Alberta, Canada (1993-2002)

Cheilitis glandularis: A clinicopathological study in 22 patients

Efficacy of imipenem therapy for Nocardia actinomycetomas refractory to sulfonamides

Clinical usefulness of variable-frequency ultrasound in localized lesions of the skin

How informative are dermatopathology requisition forms completed by dermatologists? A review of the clinical information provided for 100 consecutive melanocytic lesions

Ultrasound tightening of facial and neck skin: A rater-blinded prospective cohort study

Perineural invasion progressing to leptomeningeal carcinomatosis: Is the absence of peripheral nerves an important sign?

Trichoadenoma of Nikolowski is a distinct neoplasm within the spectrum of follicular tumors

Analysis of alterations adjacent to invasive squamous cell carcinoma of the penis and their relationship with associated carcinoma

Psoriasis in patients with HIV infection: From the Medical Board of the National Psoriasis Foundation

The National Institute of Allergy and Infectious Diseases to Seek Public Comment on Food Allergy Practice Guidelines

Primary cutaneous aggressive epidermotropic CD8+ T-cell lymphoma

Juvenile temporal arteritis

Minocycline-induced drug reaction with eosinophilia and systemic symptoms (DRESS) syndrome with persistent myocarditis

Erratum

Change of Address

Cutaneous B-cell lymphoma with histologic features of mycosis fungoides

Congenital pigment synthesizing melanoma of the scalp

A child with coexistent juvenile xanthogranuloma and Langerhans cell histiocytosis

Synovitis-acne-pustulosis-hyperostosis-osteitis (SAPHO) syndrome complicated by seven pulmonary emboli in a 15-year old patient

Tissue gadolinium deposition and fibrosis mimicking nephrogenic systemic fibrosis (NSF)–subclinical nephrogenic systemic fibrosis?

Rapid improvement of nephrogenic systemic fibrosis with rapamycin therapy: Possible role of phospho-70-ribosomal-S6 kinase

Botox and Dysport: Is there a dose conversion ratio in dermatology and aesthetic medicine?

Conflict of interest?

A comparison of sunburn protection of high–sun protection factor (SPF) sunscreens: SPF 85 sunscreen is significantly more protective than SPF 50

Thyroid autoimmunity and pemphigus vulgaris: Is there a significant association?

The set-back buried dermal suture: An alternative to the buried vertical mattress for layered wound closure

Localized lymphomatoid papulosis

Pigmented Bowen disease of the palm: An atypical case diagnosed by dermoscopy

Multiple palisaded encapsulated neuromas in a child without other associated abnormalities

Bullous lesions with scarring and mutilation

Iotaderma #193

January iotaderma (#192)

Dermatology Calendar

Editorial Board

Table of Contents

Information for Readers

Instructions for Category 1 CME Credit