Langerhans cell histiocytosis in an adult: good response of cutaneous lesions to acitretin

Abstract

A 57-year-old man presented with a 2-year history of bilateral erosive lesions on the inguinal region, and erythematous, brown and crusted papules over the trunk. Histological examination of one lesion in conjunction with immunohistochemical study and electron microscopy led to the diagnosis of Langerhans' cell histiocytosis. After a thorough examination, the only associated findings were retroperitoneal fibrosis and hypergonadotrophic hypogonadism with a granulomatous testicular infiltrate. The patient was treated with oral acitretin for 1 year (with a topical corticosteroid for the inguinal lesions), resulting in clearing of the cutaneous lesions. He underwent placement of bilateral double-J ureteral catheters and was started on hormone replacement therapy. At follow-up 1 year after treatment with acitretin ceased, the patient remained free of cutaneous lesions and his overall condition, including the retroperitoneal fibrosis, had improved. This case had an uncommon combination of features, with a good response to acitretin.

Langerhans cell histiocytosis first presenting in the skin in adults: frequent association with a second haematological malignancy

“…However, patients presenting with generalized papular lesions and/or ulcerating lesions in the skin folds appear much more resistant to treatment. Many agents including topical and systemic corticosteroids, topical nitrogen mustard, imiquimod, narrowband UVB, PUVA, radiotherapy, thalidomide, methotrexate, retinoids, vinblastine, interferon and cladribine have been tried and have been reported successful in some patients 4–67 . However, no single treatment was effective in all cases.…”

Patient with treatment resistant peristomal and scalp dermatitis

“…Although treatment with acitretin has been shown to be effective in adult onset LCH with skin limited disease, there are no published reports for the use of bexarotene in LCH. 5 …”

Primary Langerhans cell histiocytosis of the vulva: acitretin as a glucocorticoid‐sparing agent

“…To the best of our knowledge, there are reports of successful treatment with isotretinoin 8 and cutaneous LCH treated with acitretin. 9 In the latter, the authors suggested a possible immunomodulatory role for acitretin and paved the way for a new option in the management of this disease. 9 Our case highlights a rare and challenging presentation of restricted LCH treated with acitretin, a still underexplored glucocorticoid-sparing agent in this setting.…”