Febrile Ulceronecrotic Mucha-Habermann Disease: A Case Report and Review of the Literature

Abstract

A 32-year-old male with febrile ulceronecrotic Mucha-Habermann disease (FUMHD) responsive to methotrexate is reported. This is a severe variant of pityriasis lichenoides et varioliformis acuta characterized by the acute onset of a widespread ulceronecrotic cutaneous eruption together with high fever and systemic involvement. To our knowledge, only 13 patients with FUMHD have been reported to date.

Febrile ulceronecrotic Mucha-Habermann disease with clonality: A cutaneous T-cell lymphoma entity?

“…7 Here, we report that two patients with PLEVA developed a fulminant course of the disease with widespread erosions and ulcerations, as first described by Degos et al 8 A review of the literature reveals 23 cases of FUMHD. 1,2,[8][9][10][11][12][13][14][15][16][17][18][19][20][21][22][23] Including our cases, the lethality of FUMHD is approximately 24% (6 of 25 patients). Nineteen patients recovered using a variety of therapeutic regimens such as systemic corticosteroids, 2,9-16,18-21 dimethylsulfone (DDS), 18,21 MTX, 1-3,13,21 antibiotics, 1,2,8,10,11,13-21 PUVA, 2,11 acyclovir, 16,17 and debridement and skin grafting.…”

Pityriasis lichenoides and its subtypes

“…The ulcers often heal with hypopigmentation and atrophic scars. Systemic manifestations include high fever, sore throat, 43 diarrhea, 43 central nervous system symptoms, 9 abdominal pain, 9,23 interstitial pneumonitis, 22 splenomegaly, 25 arthritis, 27 sepsis, 27 megaloblastic anemia, 30 conjunctival ulcers, 34 and death. To date, there have been 6 reported fatalities, which have been limited to adults.…”

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

“…Immunosuppressive monotherapy with methotrexate [6,8,11,13,15,19,25,28,29,36,40] or cyclosporine [31,35] or a combination of one of these with a high dose of glucocorticoids [6,8,11,13,25,28,29,36,41,43] has been used in -several cases. Antibiotics like erythro-mycin, acyclovir, ultraviolet photother-apy (PUVA) [11,19], IVIG [5,28,36,41,] photochemotherapy [41], 4,4-diaminodiphenyl sulfone [7,8,10,23,] and debridement with skin grafting [23] have also been used to treat FUMHD.…”

“…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].…”