Febrile ulceronecrotic Mucha-Habermann disease (pityriasis lichenoides et varioliformis acuta fulminans) associated with parvovirus infection

• Published in: The American journal of dermatopathology Vol. 35; no. 4; pp. 503 - 506
• Main Authors: Nanda, Arti, Alshalfan, Faisal, Al-Otaibi, Mohammad, Al-Sabah, Humoud, Rajy, Jihan M
• Format: Journal Article
• Language: English
• Published: United States 01-06-2013
• Subjects: Biopsy; Child; Combined Modality Therapy; Glucocorticoids - therapeutic use; Humans; Male; Parvoviridae Infections - complications; Parvoviridae Infections - diagnosis; Parvoviridae Infections - immunology; Parvoviridae Infections - virology; Pityriasis Lichenoides - diagnosis; Pityriasis Lichenoides - etiology; Pityriasis Lichenoides - immunology; Pityriasis Lichenoides - therapy; Pityriasis Lichenoides - virology; Prednisolone - therapeutic use; Skin - immunology; Skin - pathology; Treatment Outcome; Ultraviolet Therapy
• ISSN: 0193-1091; 1533-0311
• DOI: 10.1097/DAD.0b013e3182770626

Febrile ulceronecrotic Mucha-Habermann disease is a rare fulminant variant of pityriasis lichenoides et varioliformis acuta, characterized by a rapidly progressive course with predominant ulceronecrotic lesions associated with fever and systemic manifestations. It carries a great morbidity and is potentially fatal. The exact pathogenesis is not clear, and it has been proposed to be the result of hypersensitivity reaction to an infection. We report a patient with febrile ulceronecrotic Mucha-Habermann disease in a 12-year-old boy in whom the condition was most likely precipitated by parvovirus infection, and he showed a favorable response to a combination of prednisolone with narrow band ultraviolet B (NB-UVB) phototherapy.