Widespread skin-limited Langerhans cell histiocytosis: Complete remission with interferon alfa

Association of both Langerhans cell histiocytosis and Erdheim-Chester disease linked to the BRAFV600E mutation

“…IFN-a was consequently the most frequently used therapy because it has also been proposed to treat LCH. [42][43][44][45] However, the efficacy of IFN-a was lower than what has been reported previously for isolated-ECD patients. [46][47][48] These differences cannot be explained by the clinical spectrum of mixed histiocytosis, the dose of IFN-a, or the duration of the follow-up, which were equivalent in our series and in the previous series of patients with isolated ECD.…”

Adult cutaneous Langerhans cell histiocytosis responsive to topical imiquimod

“…1,2 Past treatments for cutaneous LCH have included surgical excision, topical 1,3,4 or systemic steroids, localized radiation therapy, 5 topical nitrogen mustard, [6][7][8][9] phototherapy, 10,11 methotrexate, 12 total skin electron beam therapy, vinblastine, 13 and systemic interferon. 14, 15 We report the first case of adult cutaneous LCH responsive to topical imiquimod.…”

“…1,3,4,7-9 Whereas mild skin involvement responded to topical steroids for 2 out of 3 patients, 1,3,4 more severe and widespread cutaneous forms were responsive to topical mechlorethamine in 3 out of 3 patients, at times resulting in up to two and a half years of remission. [7][8][9] Systemic interferon (IFN) has been effective in the treatment of widespread skin-limited LCH refractory to multiple therapies for 2 out of 2 adult patients, 14,15 resulting in complete remission for up to 29 months in one adult. 14 Imiquimod is a topical immunomodulator believed to function as a potent stimulator of T helper-1 cytokines causing local release of interferon-alfa, 16 IFN gamma, and interleukin-2.…”

Widespread skin-limited adult Langerhans cell histiocytosis: Long-term follow-up with good response to interferon alpha

“…Disseminated multisystem adult LCH has a more benign chronic course; it has a slowly progressive or undulating course in contrast with the childhood type 1–9 . Skin‐limited LCH patients can be subgrouped into four categories according to the course: 1–3 (i) those who resolve spontaneously; (ii) those who respond to therapy and have no recurrence; (iii) those with persistent or recurrent lesions, not responding to therapy but still limited to the skin; and (iv) those who progress to multisystem disease. Our adult case belongs to the third category in which the majority of patients are more than 30 years of age 1,2 .…”

“…Skin‐limited LCH patients can be subgrouped into four categories according to the course: 1–3 (i) those who resolve spontaneously; (ii) those who respond to therapy and have no recurrence; (iii) those with persistent or recurrent lesions, not responding to therapy but still limited to the skin; and (iv) those who progress to multisystem disease. Our adult case belongs to the third category in which the majority of patients are more than 30 years of age 1,2 . There are previous reports of adult, widespread, cutaneous LCH responding to topical carmustine, topical or systemic steroids, or phototherapies but most showed multiple recurrences or only incomplete response 1–3 .…”