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Febrile ulceronecrotic Mucha-Habermann disease
J L López-Estebaranz1, F Vanaclocha, R Gil
1Department of Dermatology, 12 de Octubre Hospital, Madrid, Spain.
Journal of the American Academy of Dermatology
|November 1, 1993
Summary
Febrile ulceronecrotic Mucha-Habermann disease is a severe pityriasis lichenoides et varioliformis acuta (PLEVA) variant. This case report details its presentation, leukocytoclastic vasculitis, and successful treatment with PUVA and methotrexate.
Area of Science:
- Dermatology
- Pathology
- Immunology
Background:
- Pityriasis lichenoides et varioliformis acuta (PLEVA) is a rare skin condition.
- Febrile ulceronecrotic Mucha-Habermann disease (FUMHD) represents a severe, systemic variant of PLEVA.
Observation:
- An 18-year-old male presented with diffuse ulcerations and high fever, indicative of FUMHD.
- The condition followed a typical PLEVA presentation.
- Histopathology revealed leukocytoclastic vasculitis alongside characteristic PLEVA features.
Findings:
- Laboratory results showed elevated erythrocyte sedimentation rate, leukocytosis, and mild liver enzyme elevation.
- T cell receptor gene analysis of skin and blood yielded normal results.
- No systemic organ involvement was identified in this patient.
Implications:
- This case highlights the importance of recognizing FUMHD as a distinct entity.
- Successful treatment with PUVA and methotrexate suggests therapeutic options.
- Further research into the pathogenesis and management of FUMHD is warranted.