title: Febrile ulceronecrotic Mucha-Habermann disease: treatment with infliximab and intravenous immunoglobulins and review of the literature.
abstract: Febrile ulceronecrotic Mucha-Habermann disease (FUMHD) is a rare subtype of pityriasis lichenoides et varioliformis acuta, characterized by an acute onset of ulceronecrotic papules, rapidly coalescing into large ulcers with necrotic crusts, associated with high fever and severe systemic symptoms. We report a case of a 65-year-old woman with a resistant form of FUMHD successfully treated with a tumor necrosis factor-α (TNFα) inhibitor (infliximab). After 1 year of treatment, because of the recurrence of lesions and -occurrence of severe sepsis, we decided to change the therapeutic procedure by introducing intravenous immunoglobulin witch induced a spectacular improvement. Only few cases of FUMHD treated with intravenous immunoglobulin have been reported to date. In our case, we describe the first utilization of TNFα inhibitors in the treatment of FUMHD: TNFα inhibitors may be useful, particularly in resistant cases. Further reports are required to confirm this potential therapeutic option.
authors: Department of Dermatology, Amiens University Medical Centre, South Hospital, and University Picardie Jules Verne, Amiens, France. liliameziane@yahoo.fr
authors: L
authors: L
authors: Meziane
authors: A
authors: A
authors: Caudron
authors: F
authors: F
authors: Dhaille
authors: M
authors: M
authors: Jourdan
authors: A
authors: A
authors: Dadban
authors: C
authors: C
authors: Lok
authors: G
authors: G
authors: Chaby
mesh_terms: D000368:Aged; D000911:Antibodies, Monoclonal / Q000627:therapeutic use*; D003879:Dermatologic Agents / Q000627:therapeutic use*; D004359:Drug Therapy, Combination; D005260:Female; D006561:Herpes Simplex / Q000188:drug therapy*; D006801:Humans; D016756:Immunoglobulins, Intravenous / Q000627:therapeutic use*; D000069285:Infliximab; D017514:Pityriasis Lichenoides / Q000188:drug therapy*; D013997:Time Factors; D016896:Treatment Outcome; D014409:Tumor Necrosis Factor-alpha / Q000037:antagonists & inhibitors
chemical_list: D000911:Antibodies, Monoclonal; D003879:Dermatologic Agents; D016756:Immunoglobulins, Intravenous; D014409:Tumor Necrosis Factor-alpha; D000069285:Infliximab