title: 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.
authors.affiliation: Dermatology Department, University Hospital of Coimbra, Praceta Mota Pinto, 3000-075, Coimbra, Portugal. ze_carlos_cardoso@yahoo.com.br
authors.forename: J C
authors.initials: JC
authors.lastname: Cardoso
authors.forename: M
authors.initials: M
authors.lastname: Cravo
authors.forename: R
authors.initials: R
authors.lastname: Cardoso
authors.forename: M M
authors.initials: MM
authors.lastname: Brites
authors.forename: J P
authors.initials: JP
authors.lastname: Reis
authors.forename: O
authors.initials: O
authors.lastname: Tellechea
authors.forename: C
authors.initials: C
authors.lastname: Eloy
authors.forename: A
authors.initials: A
authors.lastname: Figueiredo
mesh_terms: D017255:Acitretin / Q000627:therapeutic use*; D000305:Adrenal Cortex Hormones / Q000627:therapeutic use*; D004359:Drug Therapy, Combination; D006119:Groin; D006646:Histiocytosis, Langerhans-Cell / Q000188:drug therapy* / Q000473:pathology; D006801:Humans; D007641:Keratolytic Agents / Q000627:therapeutic use*; D008297:Male; D008875:Middle Aged; D013997:Time Factors; D016896:Treatment Outcome
chemical_list: D000305:Adrenal Cortex Hormones; D007641:Keratolytic Agents; D017255:Acitretin