A Comparison of Mohs Micrographic Surgery and Wide Local Excision for Treatment of Dermatofibrosarcoma Protuberans With Long-Term Follow-up: The Mayo Clinic Experience.

BACKGROUND: Dermatofibrosarcoma protuberans (DFSP) is a soft tissue tumor with slow infiltrative growth and local recurrence if inadequately excised. OBJECTIVE: To compare long-term outcomes after Mohs micrographic surgery (MMS) and wide local excision (WLE). MATERIALS AND METHODS: Records of patients with DFSP surgically treated with WLE or MMS from January 1955 through March 2012 were retrospectively reviewed. RESULTS: Mean follow-up for patients treated with MMS (n = 67) and WLE (n = 91) was 4.8 and 5.7 years, respectively. Twenty-eight patients (30.8%) with WLE had recurrence (mean, 4.4 years), whereas only 2 (3.0%) with MMS had recurrence (1.0 and 2.6 years). Recurrence-free survival rates at 1, 5, 10, and 15 years were significantly higher with MMS (p < .001). Mean preoperative lesion sizes were similar (5-6 cm) between the 2 groups, whereas mean (standard deviation) postoperative defect sizes were 10.7 (4.3) cm and 8.8 (5.5) cm for WLE and MMS, respectively (p = .004). Primary closure was used for 73% of MMS cases, whereas WLE more commonly used flaps, grafts, or other closures (52%). Two Mohs layers typically were required for margin control. CONCLUSION: Surgical excision with meticulous histologic evaluation of all surgical margins is needed for DFSP treatment to achieve long-term high cure rates and low morbidity.

Generalized Discoid Lupus Erythematosus as the Presenting Sign of Small Cell Lung Carcinoma.

A 46-year-old woman with a 30 pack-year smoking history presented with a worsening eruption on the left cheek that failed to improve with metronidazole gel. The cutaneous eruption spread to most of her face and did not respond to a brief tapering course of prednisone. During the initial evaluation at our institution, approximately 6 weeks after the onset of the cutaneous eruption, the patient had erythematous, crusted plaques on her face and scalp (Figure 1A); they were also present on the V-area of the anterior aspect of the neck and upper region of the chest, the shoulders, and the arms, with isolated lesions on the trunk and legs. Her oral mucosa had erythematous erosions on the hard palate and gingivae. A review of systems revealed pain and burning of her skin lesions, but no muscle weakness or other systemic clinical manifestations. The differential diagnosis included autoimmune connective tissue disease, pemphigus foliaceous, sarcoidosis, lichen planus, phototoxic drug eruption, and eczema herpeticum.

An update in drug-induced subacute cutaneous lupus erythematosus.

BACKGROUND: It has been over three decades sincethe first report of drug-induced subacute cutaneouslupus erythematosus (DI-SCLE) was described. Withan increasing variety of implicated drugs and thepotential for publication bias, we must consider: 1) hasthere been a change in drugs most often reported inDI-SCLE over time, and, 2) if so, of which drugs shouldclinicians be most suspicious in the setting of possibleDI-SCLE? OBJECTIVE: To determine which drug(s) present thehighest risk for inducing DI-SCLE. METHODS: The PubMed database was queried forreports of DI-SCLE from August, 2009 until May,2016. Cases reported in the English language wereorganized by drug class and compared with theresults of our previous review. RESULTS: From 55 selected publications, 95 qualifiedreports of DI-SCLE were identified. With theexception of a population-based study from Sweden,all other reports of DI-SCLE appeared as case reportsor small case series. Cases associated with protonpump inhibitors relative to all other medicationswere increased by 34.1%. Reports associated withantihypertensive and antifungal medicationsdecreased by 28.9% and 22.4%, respectively duringthis timeframe. The majority of new reports wereassociated with drugs not previously described.Greater than 70% of reports since August, 2009 werefrom European countries. CONCLUSIONS: The number of drugs associated withDI-SCLE is increasing. However, a form of publicationbias has likely contributed to this shift in reporting.There is a need for additional large, populationbasedstudies in this area.

Prognostic impact of fibrosarcomatous transformation in dermatofibrosarcoma protuberans: a cohort study.

BACKGROUND: Dermatofibrosarcoma protuberans (DFSP) is a rare, low-grade cutaneous malignancy that sometimes transforms into a high-grade fibrosarcomatous variant (DFSP-FS). Limited data compare clinical features and biological behavior of these 2 entities. OBJECTIVE: We sought to compare clinical features and biological behavior of DFSP and DFSP-FS. METHODS: This was a retrospective cohort study of ambulatory patients with DFSP or DFSP-FS treated between January 1955 and March 2012 in the dermatology department of a tertiary care academic medical center. RESULTS: Of 188 patients, 171 (91%) had DFSP and 17 (9%) had DFSP-FS. Recurrence-free survival differed significantly between the groups over time (P = .002). The 1-year and 5-year recurrence-free survival was 94% and 86%, respectively, for DFSP, vs 86% and 42%, respectively, for DFSP-FS. Metastatic disease occurred in no patients with DFSP and in 18% (3 of 17) with DFSP-FS (P < .001). There were no statistically significant differences in age at diagnosis, sex, race, symptomatology, maximum tumor size, muscle/bone invasion, or duration of tumor before diagnosis. LIMITATIONS: The retrospective nature of study was a limitation. CONCLUSIONS: DFSP-FS exhibits more aggressive behavior than DFSP, with lower recurrence-free survival and greater metastatic potential. Their similar clinical presentation mandates histopathological differentiation for prognosis.

Incidence of Melanoma in Children: A Population-Based Study in Olmsted County, Minnesota.

BACKGROUND: The incidence of melanoma has been rising in the United States, with conflicting evidence regarding trends in children. METHODS: We identified patients 0 to 17 years old with a diagnosis of melanoma from January 1, 1970, through December 31, 2010, in Olmsted County, Minnesota. Information on survival and demographic characteristics was abstracted, and estimates of true incidence were calculated. RESULTS: The estimated true incidence of melanoma in children from 1970 to 2010 was found to be 0.62 per 100,000 girls and 0.45 per 100,000 boys. The incidence of melanoma in this population did not increase with time after adjusting for age and sex. Only one case of metastatic disease (lymph node) was identified. Girls were more commonly affected and the mean age of disease onset was 14 years. Five of the seven melanomas in this population arose in association with a nevus, and none involved the trunk. Overall and disease-specific survival rates were not calculated because all patients studied were alive at the last follow-up. CONCLUSION: The estimated true incidence rates of pediatric melanoma from our population-based study in Olmsted County, Minnesota, appear stable. This finding is in contrast to our prior research showing rapidly increasing incidence rates of melanoma in young and middle-aged adults from the same population.

Narrowband ultraviolet B light in Langerhans cell histiocytosis: a case report.

Langerhans cell histiocytosis (LCH) (previously called eosinophilic granuloma, Hand-Schüller-Christian syndrome, Letterer-Siwe disease, and Hashimoto-Pritzker disease) is a rare, heterogeneous disorder with highly variable presentation. LCH commonly affects the skin, as well as internal organs. Because the skin lesions appear benign, and LCH is unfamiliar to most physicians, diagnosis is often delayed. Treatment is controversial, with further clinical study needed. For persons with extensive, skin-limited disease, the existing topical therapies are impractical. We present a child with cutaneous LCH lesions that responded to ultraviolet light phototherapy with minimal adverse effects or patient discomfort.

Cutaneous manifestations of immunoglobulin G4-related disease: what dermatologists need to know.

In this review, we summarize the recent literature and use case examples to reach diagnostic criteria for the diagnosis of immunoglobulin G4 (IgG4)-related diseases that may be of relevance to the practicing dermatologist.

Increasing incidence of melanoma among middle-aged adults: an epidemiologic study in Olmsted County, Minnesota.

OBJECTIVE: To identify changes in the incidence of cutaneous melanoma over time in the fastest-growing segment of the US population-middle-aged adults. PATIENTS AND METHODS: By using the Rochester Epidemiology Project resource, we identified patients aged 40 to 60 years who had a first lifetime diagnosis of melanoma between January 1, 1970, and December 31, 2009, in Olmsted County, Minnesota. The incidence of melanoma and overall and disease-specific survival rates were compared by age, sex, year of diagnosis, and stage of disease. RESULTS: Between 1970 and 2009, age- and sex-adjusted incidence increased significantly over time (P<.001) from 7.9 to 60.0 per 100,000 person-years, with a 24-fold increase in women and a 4.5-fold increase in men. Although not significant (P=.06), the incidence of melanoma increased with age. Overall and disease-specific survival improved over time, with hazard ratios of 0.94 (P<.001) and 0.93 (P<.001) for each 1-year increase in the year of diagnosis, respectively. Each 1-year increase in the age at diagnosis was associated with an increased risk of death from any cause (hazard ratio, 1.07; P=.01) but was not significantly associated with disease-specific death (P=.98). Sex was not significantly associated with death from any cause (P=.81) or death from disease (P=.23). No patient with malignant melanoma in situ died from disease. Patients with stage II, III, and IV disease were more than 14 times more likely to die from disease than were patients with stage 0 or I disease (P<.001). CONCLUSION: The incidence of cutaneous melanoma among middle-aged adults increased over the past 4 decades, especially in middle-aged women, whereas mortality decreased.