CASE REPORT Febrile Ulceronecrotic Mucha-Habermann Disease.

Abstract

Objective: Case report and review of the current literature about febrile ulceronecrotic Mucha-Habermann disease (FUMHD). Methods: Review of our patient's medical records and of the current literature. Results: The FUMHD is a rare and potentially lethal type of pityriasis lichenoides et varioliformis acuta. It is characterized by the sudden onset of ulceronecrotic skin lesions associated with high fever and systemic symptoms. Because of a high case-fatality rate it requires quick and decisive action. Only 40 cases of this severe form of the disease have been reported in the literature to date. We present the case of a 30-year-old male patient with severe FUMHD who was successfully treated in our burn intensive care unit after failed treatment at a dermatological hospital. The patient was treated with topical antiseptics, moisturizers, and artificial skin substitutes, as well as systemic immunosuppressive therapy (glucocorticoids) with which we were able to control the disease activity so that healing of the patient's skin lesions could be achieved. Conclusion: Patients with FUMHD should be treated in a specialized center for severely burned patients. Only such centers can provide the structural and logistical capacities necessary for the treatment of such extensive superficial wounds.

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CASE REPORT
Febrile Ulceronecrotic Mucha-Habermann Disease
Patrick S. Harenberg, MD, Manuel Hrabowski, MD, Henning Ryssel, MD,
Emre Gazyakan, MD, G¨ unter Germann, MD, PhD, Holger Engel, MD,
and Matthias A. Reichenberger, MD
Department of Hand, Plastic, and Reconstructive Surgery Burn Center; BG Trauma Center Lud-
wigshafen, Department of Plastic and Hand Surgery, University of Heidelberg, Ludwig-Guttmann,
Ludwigshafen, Germany
Correspondence: harenberg@uni-hd.de
Published July 16, 2010
Objective: Case report and review of the current literature about febrile ulceronecrotic
Mucha-Habermann disease (FUMHD). Methods: Review of our patient’s medical
records and of the current literature. Results: The FUMHD is a rare and potentially
lethal type of pityriasis lichenoides et varioliformis acuta. It is characterized by the
sudden onset of ulceronecrotic skin lesions associated with high fever and systemic
symptoms. Because of a high case-fatality rate it requires quick and decisive action.
Only 40 cases of this severe form of the disease have been reported in the literature to
date. We present the case of a 30-year-old male patient with severe FUMHD who was
successfullytreatedinourburnintensivecareunitafterfailedtreatmentatadermatolog-
ical hospital. The patient was treated with topical antiseptics, moisturizers, and artificial
skin substitutes, as well as systemic immunosuppressive therapy (glucocorticoids) with
which we were able to control the disease activity so that healing of the patient’s skin
lesions could be achieved. Conclusion: Patients with FUMHD should be treated in a
specialized center for severely burned patients. Only such centers can provide the struc-
tural and logistical capacities necessary for the treatment of such extensive superficial
wounds.
Febrile ulceronecrotic Mucha-Habermann disease (FUMHD) is a rare and potentially
lethal type of pityriasis lichenoides et varioliformis acuta (PLEVA). Characteristics of
FUMHD include a fulminant course of the disease, painful ulceronecrotic erosions, fever,
and severe systemic manifestations. In current literature only 40 cases with an overall case
fatality rate of 20% are described. Until today the knowledge about the etiology of this
disease is only fragmentary, but an association with viral and lymphoproliferative diseases
is being discussed.
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ePlastyVOLUME 10
We are presenting the case of a 30-year-old man who was successfully treated for a
severe case of FUMHD with an affected body surface area of 65%. The treatment took
place in our burn intensive care unit.
CASE REPORT
Eleven months after successful treatment for Hodgkin disease in stage IIIA (chemotherapy,
BEACOPPregimen),thepatientdevelopedacaseofPLEVA/FUMHD.Heinitiallyreceived
dermatologicaltreatmentwithtopicalandsystemicagents(cefuroximaxetil,methothrexate,
prednisolone, aciclovir, moxifloxacin, and doxorubicin). Despite this treatment new skin
lesionsappearedandthepatientwasadmittedtoourburnintensivecareunit.Onadmission,
the patient’s whole integument (with an emphasis on the upper body) was covered with
sharply bounded erythematous papules and plaques. Solitary necroses were visible on the
patient’s abdomen and back. In addition, the patient developed disseminated ulceronecrotic
lesions in the course of his disease.
Onadmission,theonsetofsepsisandcolonizationoftheskinlesionswithmethicillin-
resistantStaphylococcusaureusandPseudomonasweretheadditionalfactorscomplicating
thiscase.Thephysicalexaminationshowedpainful,disseminated,erythematous,andpapu-
losquamous lesions, partially with hemorrhagic ulcerations (Fig 1).
Figure 1. Skin lesions on admission to the burn ICU.
Initially we conducted topical treatment with Vaseline gauze and a polyhexanide
solution (Lavasept) as well as systemic treatment with 1 mg/kg/d prednisolone. Balanced
fluid substitution was initiated after insertion of a central catheter.
Because microbiological tests (smears) of the patient’s skin lesions revealed a super-
infection with Staphylococcus aureus, Pseudomonas aeruginosa, and enterobacteriaceae a
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calculated systemic antibiotic therapy was initiated. Dressings were changed every second
day. Topical treatment was continued with Suprathel as well as Aquacell. This therapy
regimen was supplemented by dietary and physical therapy as well as psychotherapeutic
attendance.
Withthistreatmentwewereabletocontrolthediseaseandtheskinlesionswerehealed
(Fig 2).
Figure 2. Resultshortlybeforedischargefromourhospital
(5.5 months later).
DISCUSSION
The FUMHD is a rare and potentially lethal type of PLEVA. Characteristics of FUMHD
includeafulminantcourseofthedisease,painfululceronecroticeruptions,fever,andsevere
systemic manifestations.
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The FUMHD was first described by Dagos in 1966 and until today there are only
40 cases documented in international literature.1According to the reports, the majority of
cases are children and young adults (predominantly males, mean age 27.4 years, range 4-82
years). Literature describes the disease’s case-fatality rate at about 20%, with the rate being
0% in children and up to 33.3% in adults.2
Until today the knowledge about the etiology of this disease is only fragmentary,
but an association with viral diseases is being discussed. In these cases an immediate
correlation between management of the underlying disease and prognosis of the FUMHD
is described.3,4
In the early course of the disease differential diagnoses may include chickenpox,
Gianotti-Crosti syndrome, lichen planus, pityriasis rosea, and guttate psoriasis. The
FUMHD either develops spontaneously (as was the case in our patient) or in preexist-
ing pityriasis lichenoides. There is proof that pityriasis lichenoides and PLEVA as well
as lymphomatoid papulomatosis might be a part of the spectrum of benign clonal T-cell
proliferative disorders.5-7
Symptoms include feelings of severe illness, fever, and painful erythematous and
papulosquamous eruptions that gradually shift to hemorrhagic ulcerations. Systemic mani-
festations include diarrhea, abdominal pain, pneumonia, splenomegaly, arthritis, sepsis, or
megaloblastic anemia.1In the majority of patients, blood tests show leukocytosis, anemia,
elevated C-reactive protein, and elevated liver enzymes. An association of FUMHD with
elevated blood levels of TNF-α as been described.8
The FUMHD is a great challenge for the treating physicians. Literature recommends
immunosuppressive monotherapy with methothrexate or cyclosporine or a combination of
one of these with high-dose glucocorticoids. Some authors also described partial success
in the treatment of FUMHD with high-dose immunosuppressive therapy in combination
with virostatic drugs and antibiotics.9,10A preemptive antibiotic therapy for prevention
of infections should always be administered. The extensive loss of skin and soft tissue in
combination with a quick deterioration of the patient’s general condition is similar to the
condition of patients with severe burns. Thus, patients with FUMHD should receive the
same supportive intensive care as the burn victims.
As in the treatment of burn victims we utilized balanced fluid substitution, regular
check of inflammatory parameters, antiseptic topical treatment (Lavasept), nonadhesive
wound dressings (Vaseline gauze), and dietary and physical therapy as well as psychother-
apeutic attendance.
CONCLUSION
The FUMHD is characterized by a high case-fatality rate and requires quick and decisive
action. A search for a focus of a viral or lymphoproliferative disease as well as an early
high-dose immunosuppressive, antibiotic, and topical therapy in a specialized intensive
care unit should have highest priority.
These conditions as well as the existing structural and logistical possibilities that a
center for severely burned patients offers for the treatment of large wounds predestine
patients with FUMHD for treatment in such a center.
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