She attempted high-potency topical corticosteroids, topical tacrolimus, and a combination of amitriptyline, ketamine, and lidocaine patch 5% without relief. A 12-week course of topical imiquimod 5% cream to the inframammary folds was discontinued due to progression of cutaneous disease. She experienced significant irritation from brachytherapy to her vulvar lesions.
She was prescribed topical nitrogen mustard, or mechlorethamine 0.016% gel, to the inframammary folds daily. At 6 months’ follow-up, she had complete response and clearance of disease on the inframammary folds with post-inflammatory hyperpigmentation (PIH; Figure 3). Despite complete response on topical nitrogen mustard, topical therapies were eventually discontinued due to patient’s intolerance of all topical applications including petrolatum. She is being treated with hydroxyurea for her CMML.
Case 2
An 85-year-old woman presented in dermatologic consultation for a pruritic eruption of 9 years’ duration. Physical examination showed crusted red-brown papules on her bilateral axillae (Figure 4), inframammary folds, inguinal folds, and left vulva.
A punch biopsy from the left popliteal fossa showed a dense superficial dermal polymorphous infiltrate composed of small lymphocytes, histiocytes, and numerous eosinophils. Immunohistochemical studies showed positivity for CD1a and CD4, and negative for langerin, S100, and CD68. Evaluation for systemic disease including brain MRI was negative. The final diagnosis was of LCH, limited to cutaneous involvement. She was prescribed topical corticosteroids and topical imiquimod 5% cream without relief. She was initiated on topical nitrogen mustard therapy, or mechlorethamine 0.016% gel daily to affected lesions. She experienced complete response; all large nodules in her bilateral axilla and inguinal folds resolved completely with mild PIH (Figure 5). At 2 years’ follow-up, patient’s cutaneous LCH remained in remission. She was eventually maintained on triamcinolone 0.01% cream to her axilla and inframammary folds as needed for itch.
DISCUSSION
Topical nitrogen mustard therapy, an alkylating agent that induces breaks in DNA, has traditionally been used for treatment of limited-stage mycosis fungoides and cutaneous T-cell lymphoma.1 There is a paucity of data to guide management of adult patients with unisystem LCH, particularly disease limited to the skin.1,3 While systemic treatments are more varied, options for topical therapies typically include topical corticosteroids or topical imiquimod.1,5,7 The data on topical nitrogen mustard as treatment for primary cutaneous LCH in adults is limited to 5 known cases in the literature.8-11 In these cases, patients had clearance within an average of 2 to 3 weeks. Therapy was well-tolerated, with limited short-term side effects including hyperpigmentation and mild to moderate allergic contact dermatitis. 8-11
Our two cases support topical nitrogen mustard as a possible treatment for primary cutaneous LCH recalcitrant to other treatment modalities, inducing complete response of lesions in a rapid manner. In our two cases, patients experienced com-
Our two cases support topical nitrogen mustard as a possible treatment for primary cutaneous LCH recalcitrant to other treatment modalities, inducing complete response of lesions in a rapid manner. In our two cases, patients experienced com-
