Febrile Ulceronecrotic Mucha‐Habermann Disease (Pityriasis Lichenoides et Varioliformis Acuta Fulminans) Presenting as Stevens‐Johnson Syndrome

Abstract

We present two pediatric patients with pityriasis lichenoides et varioliformis acuta fulminans whose admitting diagnosis was Stevens Johnson Syndrome. The patients were successfully treated with methotrexate and prednisone. These cases highlight the importance of early recognition and treatment of this disease to prevent further morbidity and a potentially fatal prognosis.

Febrile ulceronecrotic Mucha–Habermann disease: proposed diagnostic criteria and therapeutic evaluation

“…Many laboratory abnormalities have been detected in different cases of FUMHD such as increased leukocytic count, elevated erythrocyte sedimentation rate and C‐reactive protein, anemia, mild hypergammaglobulinemia, and hypoproteinemia, hypoalbuminemia, hypocalcemia, eosinophilia, lymphopenia, and positive skin and blood cultures. However, no specific laboratory tests that help to establish the diagnosis of FUMHD have been described in all of the reported cases, adding to the difficulty in diagnosis of this very rare entity …”

“…Clinically, FUMHD differs from classic PLEVA by the abrupt onset, predominance of large coalescent ulceronecrotic skin lesions, involvement of oral and genital mucosae, and association with high fever and other systemic symptoms …”

“…One of the major problems in the diagnosis of FUMHD is its extreme rarity, which leads to unfamiliarity among dermatologists, resulting in missing cases. In addition, the very wide age range, presentation by fever, and association with systemic manifestations make patients seek medical advice at different specialties, thus decreasing the experience needed for better awareness of this rare entity. Moreover, the clinical overlapping with PLEVA, by which most of the cases present at first, leads to underdiagnosis and consequently missing of cases due to imprecise follow‐up .…”

“…in 1966, about 65 cases of FUMHD have been reported; however, no definite diagnostic criteria have been established, and an optimum treatment is not yet determined. Herein, we review the different aspects of FUMHD, suggesting two sets of diagnostic criteria and evaluating the different diagnostic and therapeutic challenges associated with this rare entity …”

Febrile Ulceronecrotic Mucha-Habermann Disease: Treatment with Infliximab and Intravenous Immunoglobulins and Review of the Literature

“…A febrile ulceronecrotic severe variant of PLEVA, called febrile ulceronecrotic Mucha-Habermann disease (FUMHD) is more destructive and associated to large coalescent skin necrosis with a rapid progression [1]. 48 cases of PLEVA fulminans have been reported to date in the literature with 9 lethal forms (20%) (table 1) [1,2,3,4,5,6,7,8,9,10,11,12,13,14,15,16,17,18,19,20,21,22,23,24,25,26,27,28,29,30,31,32,33,34,35,36,37,38,39,40,41,42,43,44]. Oral, genital and conjunctival mucosa can be affected [2,6,9,12,13].…”