Febrile ulceronecrotic Mucha-Habermann's disease

Abstract

A patient with febrile ulceronecrotic Mucha-Habermann's disease manifested the characteristic features of this entity. These include a polymorphous eruption with histopathologic findings of Mucha-Habermann's disease, large ulceronecrotic skin lesions, intermittent high fever, and constitutional symptoms. The patient was unique in that he also had malabsorption and eosinophilia. This disease may represent a hypersensitivity reaction. To our knowledge, there are five previous cases of febrile ulceronecrotic Mucha-Habermann's disease reported in the world literature.

Medullary CD30+ T Cell Lymphoma with Eosinophilia and Hyper-IgE Supervening during the Relentless Course of Pityriasis lichenoides

“…A similar observation has previously been reported [7]. It was speculated that pityriasis lichenoides might result from hypersensitivity reactions to infectious agents, neoplasia and drugs [3,[8][9][10]. When lesions of acute pityriasis lichenoides developed in our patient, a marked increase in circulating IgE was yielded in association with a strong positivity for a series of antigens.…”

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

“…95 Studies by Hayashi 96 and Hayashi and Kawada 97 also demonstrated immunofluorescence in lesions of pityriasis lichenoides, as did Warshauer et al in a specimen of PLEVA. 23 Other investigators have not been able to detect significant amounts of immune complexes in pityriasis lichenoides. 98-100 Furthermore, Nieboer and Kalsbeek 99 cited the fact that granular IgM/C3 deposits were found in normal sun-exposed skin of more than 50% of healthy persons in a study performed by Baart de la Faille-Kuyper er al, 101 suggesting that controlled studies are needed to validate this hypothesis.…”

“…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.…”