A Diagnostically Challenging Case of Febrile Ulceronecrotic Mucha-Habermann Disease in an Adult Female Successfully Treated with Methotrexate and Cyclosporine

Abstract

Febrile ulceronecrotic Mucha-Habermann disease (FUMHD) is a rare and severe variant of pityriasis lichenoides et varioliformis acuta (PLEVA) characterized by intermittent pyrexia, acute onset of generalized ulceronecrotic lesions, and histopathology suggestive of PLEVA. Prompt diagnosis and treatment are necessary to halt the progression of this potentially fatal disease; however, the widely variable presentation of FUMHD in addition to its rarity poses a diagnostic challenge. We report the case of a previously healthy 43-year-old woman who presented to the emergency department with 1 month of generalized rash and intermittent fevers. Her only reported new exposure were elective intravenous vitamin infusions received at a medi-spa 1 week prior to onset of lesions. Initial evaluations were inconclusive, and confluent ulceronecrotic, hemorrhagic lesions appeared on approximately 90% of her body despite steroid, antibiotic, and cyclosporine therapy. Repeat histopathology was consistent with PLEVA, and in the context of her clinical presentation she was diagnosed with FUMHD. The patient rapidly attained remission with methotrexate therapy but sustained residual scarring.

Keywords: Cyclosporine; Febrile ulceronecrotic Mucha-Habermann disease; Methotrexate; Pityriasis lichenoides et varioliformis acuta; Pityriasis lichenoides with ulceronecrosis and hyperthermia.

Fig. 1

a Severe, extensive, crusted papules and papulovesicles, hemorrhagic bullae, weeping erosions, and collarettes of scale on the patient's back. b Violaceous papules, purpuric, umbilicated, hemorrhagic bullae, and numerous scalloped confluent ulcerations on the patient's right leg.

Fig. 2

Generalized vesicles and eroded papules with central necrotic crust, coalescing into plaques covering approximately 90% of the body surface area.

Fig. 3

Punch biopsy showing interface vesicular dermatitis with epidermal necrosis. The results are compatible with pityriasis lichenoides et varioliformis acuta. H&E, original magnification 40× (a) and 200× (b).