Rapid recovery in a child with febrile ulceronecrotic Mucha-Habermann disease following intravenous immunoglobulin administration.

Autor: Somasundaram A; Department of Dermatology, Venereology & Leprosy, Christian Medical College and Hospital, Vellore, India., Gupta A; Department of Dermatology, Venereology & Leprosy, Christian Medical College and Hospital, Vellore, India., Sathishkumar D; Department of Dermatology, Venereology & Leprosy, Christian Medical College and Hospital, Vellore, India., Mathew L; Department of Dermatology, Venereology & Leprosy, Christian Medical College and Hospital, Vellore, India., George A; Department of Dermatology, Venereology & Leprosy, Christian Medical College and Hospital, Vellore, India., Thomas M; Department of Pathology, Christian Medical College and Hospital, Vellore, India.
Jazyk: angličtina
Zdroj: Pediatric dermatology [Pediatr Dermatol] 2024 May-Jun; Vol. 41 (3), pp. 518-522. Date of Electronic Publication: 2024 Jan 17.
DOI: 10.1111/pde.15516
Abstrakt: Febrile ulceronecrotic Mucha-Habermann disease (FUMHD), a lymphocyte-mediated inflammatory skin disorder, is considered a severe variant of pityriasis lichenoides et varioliformis acuta that can lead to a fatal outcome if not managed in a timely fashion. Children with FUMHD can have systemic complications involving various organs. The scarcity of reported cases and the absence of well-designed studies or randomized clinical trials to evaluate different therapeutic modalities pose a major challenge in treating this potentially life-threatening disorder. We report a five-year-old child with FUMHD and seizures treated unsuccessfully with a combination of systemic steroids, methotrexate, dapsone, and oral erythromycin, who improved rapidly and achieved disease control with just a single infusion of low-dose intravenous immunoglobulin.
(© 2024 Wiley Periodicals LLC.)
Databáze: MEDLINE