Febrile ulceronecrotic mucha-habermann disease: A case report and a review of the literature
Sema Aytekin, Gokcen Balci, and Ozgur Yagdiran Duzgun
Dermatology Online Journal 11 (3): 31

Dicle University, School of Medicine, Department of Dermatology, Diyarbakir, Turkey. semaaytekin@yahoo.com

Abstract

We report a case of febrile ulceronecrotic Mucha-Habermann disease (FUMHD) in a 27-year-old woman. After 20 days of a mild eruption, extensive polymorphous, papular and ulcerohemorrhagic skin lesions gradually developed, associated with intermittent high temperature, and constitutional symptoms. The initial treatment with acyclovir was not successful, the skin lesions still progressed distally and individual lesions evolved from necrotic papules and bullae to erosions and ulcers. Skin biopsies showed the typical histopathological changes of PLEVA. The patient was treated with systemic prednisolone but dosage was limited in order to avoid sepsis. Despite corticosteroid therapy and supportive therapy, the fulminating course led to death. Including this present case, only 31 cases of FUMHD have been reported in English literature. Our case is the second report from Turkey.



Introduction

Pityriasis lichenoides et varioliformis acuta (PLEVA), also known as Mucha-Habermann disease, is an uncommon, idiopathic, acquired dermatosis characterized by erythematous, scaly papules often accompanied by hemorrhagic and papulonecrotic lesions. Febrile ulceronecrotic variant of PLEVA, also termed febrile ulceronecrotic Mucha-Habermann disease (FUMHD), was first described by Degos in 1966 [1]. FUMHD characterized by the acute onset of large, more destructive, coalescent ulceronecrotic skin lesions associated with high fever and other systemic symptoms.

Including this case, only 31 cases of FUMHD have been reported in English literature [2-23]. Our case is the second report from Turkey.


Clinical synopsis

A 27-year-old healthy female gradually developed widespread polymorphous, papular and ulcerohemorhagic skin lesions and intermittent high fever, malaise, and myalgia of 20-days duration. She denied taking any medications before the onset of the eruption. On the initial examination, it was seen that her body temperature was 39.5° C, and there were many red-to-dark erythematous papules and bullae varying in size from 3 to 15 mm associated with modest pruritus (Fig. 1). After a few days, these scattered erythematous plaques than showed central necrosis (Fig. 2).

The routine laboratory findings revealed hemoglobin at 10.5 g/dL, an elevated erythrocyte sedimentation rate (80 mm/h), and raised C-reactive protein (197 mg/L). The results of other investigations, including autoantibody measurements, serology (HIV, varicella, herpes simplex virus, hepatitis B and C) and blood and bacterial skin cultures were normal or negative.

The biopsy of a cutaneous lesion on his leg showed acanthosis, hyperkeratosis, exocytosis of lymphocytes, and subcorneal small necrotic bullous lesions (Fig. 3). These clinical and histological findings suggested a diagnosis of FUMHD.

The initial treatment with acyclovir was unsuccessful and the skin lesions progressed distally and individual lesions evolved from necrotic papules and bullae to erosions and ulcers. Systemic prednisolone (80mg/day) was then used. In association with the spread of the generalized skin ulcers (Fig.4), hyponatremia, hypercalcaemia, hypoprotenemia, and sepsis developed. High dose corticosteroids were avoided to prevent sepsis. The patient received supportive care including high-calorie parenteral therapy, appropriate antibiotics, fresh frozen plasma, and human immunoglobulin. But the therapy did not lead to clinical stabilization. In this patient, despite the corticosteroid therapy and supportive therapy, the fulminating course led to death.


Figure 1 Figure 2
Figure 1. Reddish macules, papules and vesicles over the ankle.
Figure 2. Erythematous plaques with central necrosis over the leg.

Figure 3 Figure 4
Figure 3. Acanthosis and hyperkeratosis, exocytosis of lymphocytes and subcorneal small necrotic bullous (HEX20 )
Figure 4. Ulcerations covered more than 80% of total body surface.

Discussion

Febrile ulceronecrotic Mucha-Habermann disease involves a sudden, severe flare characterized by innumerable coalescent necrotic ulcerations associated with high fever. The etiology of PLEVA or FUMHD remains unclear. Hypersensitive reaction to some kind of infectious agent is suggested as the main cause according to some authors [1, 2, 3, 4]. Associated symptoms include fever (up to 40° C), malaise, myalgia, arthralgia, gastrointestinal and central nervous system symptoms, interstitial pneumonitis, lymphocytic myocarditis, and death [5, 6, 7].

Several treatment modalities of FUMHD have been described and it is difficult to evaluate the individual efficacy because of the small number of patients and the combination therapy in most cases. According to Yang, the initial combination use of high-dose corticosteroids rapidly brings down the inflammatory component of the FUMHD, and prolonged treatment with erythromycin can maintain the therapeutic effect [19]. Other reports suggest the use of methotrexate, PUVA photochemotherapy, antibiotics, acyclovir, 4,4diaminodiphenyl sulfone, and debridement and skin grafting to treat FUMHD [1,5, 6, 7, 8, 9, 10, 11, 12, 13, 14, 15, 17, 18, 19].

Large skin ulcers can develop over the trunk and intertriginous areas [8, 16, 20, 21]. Intensive care and maintenance of the patient's general condition are required especially for the treatment of adult FUMHD. Early debridement of the necrotic tissue and enhancement of granulation and reepithelialization using whirl bath and skin grafting are recommended [16, 20, 21]. Yanaba and his colleagues performed skin grafting with a combination of cultured epidermal autograft and meshed allograft of cadaver skin on a patient with FUMHD. They used meshed cadaver allograft to prevent the loss of fluids and bacterial infection for a few weeks before spontaneous reepithelialization and propagation of epidermal autografts were started. However, they reported that their patient had severe extensive hypertrophic scars, contractures of joints, and generalized muscular atrophy requiring rehabilitation [16].

Reported cases of FUMHD generally involved children or young adults [6, 8, 12, 15, 20, 21]. More recent cases involving older patients are reported [7, 17, 22]. Although FUMHD can occur in both adults and children, the presentations may differ. Prognosis is better for children, no child fatalities have been seen; fatal outcome is reported for adults. Adult type of FUMHD has a malignant potential that may be related to T-cell clonality. Cutaneous lesions of PLEVA are clinically similar to lymphomatoid papulosis, and some authors have suggested that they are part of the same spectrum of lymphoproliferative disorders [17, 22, 23]. Dereure and colleagues report that 13 of 20 PLEVA biopsy specimens revealed the presence of a dominant T-cell clone. They suggest that PLEVA is part of the spectrum of clonal T-cell cutaneous lymphoproliferative disorders. T-cell clonalitiy in FUMHD is a prognostic factor for unfavorable outcome [24].

FUMHD is a rare and severe variant of PLEVA that can lead to death in adults. The severity of the disease led to death in seven cases out of 31 reported cases. It is important to make a diagnosis in the early stage and to start a treatment in the extensive care unit as soon as possible.

References

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11. Lowe NJ. Pityriasis lichenoides et varioliformis acuta and suspected cardiomyopathy. J R Nav Med Serv 1975;61:85-87.

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16. Yanaba K, Ito M, Sasaki H, Inoue M, Nobeyama Y, Yonemoto H, Ishiji T,. Tanaka H, Kamide R, Niimura M. A case of febrile ulceronecrotic Mucha-Habermann disease requiring debridement of necrotic skin and epidermal autograft. Br J Dermatol 2002;147(6): 1249-1253.

17. Miyamoto T, N Takayama, S Kitada, Y Hagari and M Mihara. Febrile ulceronecrotic MuchaÐHabermann disease: a case report and a review of the literature. Journal of Clinical Pathology 2003;56:795-797.

18. Ito N, Ohshima A, Hashizume H, Takigawa M, Tokura Y. Febrile ulceronecrotic Mucha-Habermann's disease managed with methylprednisolone semipulse and subsequent methotrexate therapies. J Am Acad Dermatol. 2003;49(6):1142-1148.

19. Yang CC, Lee JY, Chen W. Febrile ulceronecrotic Mucha-Habermann disease with extensive skin necrosis in intertriginous areas. Eur J Dermatol 2003;13(5):493-496.

20. Chao-Chun Yang, Julia Yu-Yun Lee, WenChieh Chen. Febrile ulceronecrotic Mucha-Habermann disease with extensive skin necrosis in intertriginous areas. Eur J Dermatol 2003;13(5):493-6.

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22. Cozzio A, Hafner J, Kempf W, Haffner A, Palmedo G, Michaelis S, Gilliet M, Zimmermann D, Burg G. Febrile ulceronecrotic Mucha-Habermann disease with clonality: a cutaneous T-cell lymphoma entity? J Am Acad Dermatol. 2004; 51(6):1014-7.

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24. Dereure O, Levi E, Kadin ME. T-Cell Clonality in Pityriasis Lichenoides et Varioliformis Acuta A Heteroduplex Analysis of 20 Cases. Arch Dermatol 2000;136:1483-1486.

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Title:

Febrile ulceronecrotic Mucha-Habermann disease: A case report and a review of the literature

Journal Issue:

Dermatology Online Journal, 11(3)

Author:

Aytekin, Sema;
Balci, Gokcen;
Duzgun, Ozgur Yagdiran

Publication Date:

2005

Publication Info:

Dermatology Online Journal, UC Davis

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