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

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 complic...

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Bibliographic Details
Published in:Pediatric dermatology 2024-05, Vol.41 (3), p.518-522
Main Authors: Somasundaram, Arun, Gupta, Ankan, Sathishkumar, Dharshini, Mathew, Lydia, George, Anju, Thomas, Meera
Format: Article
Language:English
Subjects:
Clinical trials
Dapsone
Disease control
Erythromycin
Intravenous administration
Lymphocytes
Methotrexate
Pediatrics
Pityriasis
Pityriasis lichenoides et varioliformis acuta
Recovery (Medical)
Seizures
Skin diseases
Steroid hormones
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Summary: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.
ISSN:0736-8046
1525-1470
DOI:10.1111/pde.15516