title: Febrile ulceronecrotic Mucha-Habermann's disease managed with methylprednisolone semipulse and subsequent methotrexate therapies.
abstract: Febrile ulceronecrotic Mucha-Habermann's disease is an unusual severe form of pityriasis lichenoides et varioliformis acuta characterized by abrupt onset of ulceronecrotic eruption associated with a high fever and systemic symptoms. To our knowledge, 19 cases of this disease have been reported in the literature, and 4 of them were fatal. We report the case of a 12-year-old boy with this disorder who had abdominal pain, hypoproteinemia, and anemia. Although these associated symptoms are considered life-threatening factors according to reported cases, our patient was successfully treated with methylprednisolone semipulse and subsequent methotrexate therapies. From a review of the literature and the present case, we propose that when patients have these systemic symptoms, therapeutic choices include methotrexate, high-dose corticosteroids, and 4,4-diamino-diphenyl-sulfone, which may depress early development of this disease.
authors.affiliation: Department of Dermatology, Hamamatsu University School of Medicine, Hamamatsu, Japan.
authors.forename: Natsuho
authors.initials: N
authors.lastname: Ito
authors.forename: Akihiro
authors.initials: A
authors.lastname: Ohshima
authors.forename: Hideo
authors.initials: H
authors.lastname: Hashizume
authors.forename: Masahiro
authors.initials: M
authors.lastname: Takigawa
authors.forename: Yoshiki
authors.initials: Y
authors.lastname: Tokura
mesh_terms: D002648:Child; D005334:Fever; D006801:Humans; D008297:Male; D008727:Methotrexate / Q000008:administration & dosage*; D008775:Methylprednisolone / Q000008:administration & dosage*; D017514:Pityriasis Lichenoides / Q000188:drug therapy* / Q000473:pathology; D020551:Pulse Therapy, Drug
chemical_list: D008775:Methylprednisolone; D008727:Methotrexate