There are a lack of guidelines and consensus in the management of cutaneous LCH.3 Current reported therapies include topical corticosteroids, topical imiquimod, radiation therapy, methotrexate, hydroxyurea, phototherapy, thalidomide, or vinblastine.1,4-7 Topical nitrogen mustard therapy, or mechlorethamine, is a treatment with positive responses in six known pediatric cases5 and five known adult cases of cutaneous LCH without systemic involvement.5,8-11 It has also shown positive response in larger pediatric cohort studies of multisystem LCH.5,6 We present two adults with skin-limited LCH with complete response after treatment with topical nitrogen mustard.
Report of Cases
A 75-year-old woman presented in dermatologic consultation with a painful, pruritic eruption of 8 years’ duration. Physical examination showed scattered, red-brown crusted papules without ulcerations in her inguinal folds and vulva, extending onto the perineum and labia. There were also scattered papules on a background of macular erythema on the bilateral inframammary folds (Figure 1). The patient did not have systemic symptoms at diagnosis but was diagnosed at subsequent follow-up with chronic myelomonocytic leukemia (CMML) and immune thrombocytopenic purpura.
A punch biopsy from her vulvar area found focal parakeratosis overlying a superficial dermal proliferation of medium-sized discohesive cells, positive for CD1a, langerin, S100, and cyclin D1, and negative for CD123 (Figure 2). She was positive for the BRAFV600E mutation. Positron emission tomography, pituitary MRI, and bone survey did not show multisystem involvement. The final diagnosis was of LCH, limited to cutaneous involvement.