Lowe, Garrett C.

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.

AbstractBackgroundThe incidence of melanoma has been rising in the United States, with conflicting evidence regarding trends in children.MethodsWe 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.ResultsThe 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.ConclusionThe 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.

AbstractIn 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.

AbstractLangerhans 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.