Cutaneous S100-negative, CD1a-positive histiocytosis successfully treated with combination therapy of oral methotrexate and corticosteroid.

S100-negative, CD1a-positive histiocytosis is a rare histiocytic disorder characterized by proliferation of histiocytic cells possessing a phenotype of epidermal Langerhans cells except for the lack of S100 expression and Birbeck granules. We report the case of a Japanese man suffering from S100-negative, CD1a-positive histiocytosis. The patient showed numerous smooth erythematous 5-10-mm papules/nodules on most of his body. The key histopathological feature was the presence of dermal infiltrates of non-epidermotropic S100-negative CD1a-positive mononuclear cells. No systemic involvement was detected. Initially bath-psoralen plus ultraviolet A therapy was effective, but the lesions became recalcitrant to this treatment. Methylprednisolone pulse therapy followed by low-dose methotrexate (up to 30 mg/week) in combination with prednisolone (15 mg/day) effectively controlled the skin lesions.

Prognostic indicators in 35 patients with extramammary Paget's disease.

BACKGROUND: Extramammary Paget's disease (EMPD) is an uncommon skin tumor that usually occurs in the genital area. Few studies on EMPD have been conducted. OBJECTIVE: To evaluate the clinical and pathologic features, treatments, recurrence, survival rates, and prognostic factors of EMPD. METHODS: A total of 35 (27 men and 8 women) patients with EMPD was analyzed. The average age of the patients was 73.4 years. RESULT: Twenty nine Of the 35 patients had lesions in the genital area, one in the genital and perianal area, three in the axillary area and two in the perianal area. Eighteen patients had in situ lesions, five had inguinal lymph node metastases and two had distant metastases at the time of diagnosis. Surgical resection was performed in 30 cases and radiation therapy, was administered in three cases. Six patients died of EMPD, and the overall 5 year survival rate was 75%. CONCLUSION: The presence of a nodule on the primary lesion, clinically palpable lymph nodes, the level of tumor invasion, and lymph node metastases were found to be significant prognostic factors in the 35 EMPD cases at our institution.