Febrile ulceronecrotic Mucha-Habermann disease (FUMHD) is a rare and severe form of pityriasis lichenoides et varioliformis acuta (PLEVA). PLEVA is characterized by skin lesions that ulcerate, breakdown, form open sores, then form a red-brown crust. FUMHD often begins as PLEVA, but then rapidly and suddenly progresses to large, destructive ulcers. There may be fever and extensive, painful loss of skin tissue as well as secondary infection of the ulcers. Diagnosis of FUMHD is confirmed by biopsy of skin lesions. FUMHD occurs more frequently in children, peaking at age 5 to 10. Males tend to be affected more often than females. While some cases of FUMHD have resolved without therapy, others have resulted in death. Early diagnosis and prompt treatment may help to reduce morbidity and death.

Initial symptoms of FUMHD include red scaly skin legions (papules) that ulcerate, breakdown, form open sores, then a red-brown crust (i.e., PLEVA). In FUMHD the legions suddenly progress to large, destructive ulcers and can be associated with extensive, painful loss of skin tissue. The skin lesions can become infected which may cause pus and a putrid odor. The rate of progression from PLEVA to FUMHD varies among reports but may be days to weeks. Some cases go straight to FUMHD rather than progress from PLEVA. FUMHD is often associated with high fever (up to 104°F) that may be persistant or come and go. Other symptoms may include feeling ill, sore throat, congestion, muscle soreness or pain, joint pain, diarrhea, central nervous system symptoms, abdominal pain, enlarged spleen, arthritis, megaloblastic anemia, interstitial pneumonitis (scarring or thickening of the lungs), lymphocytic (viral) myocarditis, and sepsis. FUMHD can become life threatening.

FUMHD usually begins as PLEVA, but can be distinguished by rapid progression of the skin legions seen in PLEVA to large ulcers with necrotic crusts, bloody blisters (hemorrhagic bullae), and pustules. There may be extensive, painful loss of skin tissue as well as secondary infection of the ulcers, high fever and often other nonspecific symptoms.

In some cases, the symptoms reflect FUMHD from the start, rather than progressing from PLEVA to FUMHD-like symptoms.

The cause of FUMHD is not known (idiopathic). A hypersensitivity to an infectious agent is suggested to be the main cause. Single cases of people with FUMHD and Epstein-Barr virus infection, adenovirus, or cytomegalovirus have been reported, but there has been no consistent finding so far. There is some suggestion that FUMHD may be a type of clonal T-cell disorder. “Clonal” means that all the T-cells were derived from the same cell. T cells are a type of white blood cell (lymphocytes). They make up part of the immune system. T cells help the body fight diseases or harmful substances.

At this time, there is little evidence to suggest that FUMHD is genetic. It is thought to be an acquired condition.

The way in which a person contracts or develops FUMHD is unknown at this time.

• Epidermis - Findings include focal confluent parakeratosis, spongiosis, dyskeratosis, mild to moderate acanthosis, vacuolization of basal layer with necrotic keratino-cytes, occasional intraepidermal vesicles, extensive epidermal necrosis. In advanced disease findings may also include extension of infiltrate into epidermis, invasion of erythrocytes, widespread epidermal necrosis, and nuclear debris in necrotic areas
• Dermis – Swelling, moderately dense lymphohistiocytic perivascular inflammatory infiltrate usually without atypia, extravasation of lymphocytes and erythrocytes with epidermal invasion, subepidermal vesicles in later lesions, dermal sclerosis in older lesions
• Vascular changes – Dilation and engorgement of blood vessels in papillary dermis with endothelial proliferation, vascular congestion, occlusion, dermal hemorrhage, and extravasation of erythrocytes
• Vasculitis – Fibronoid necrosis of vessel walls with leukocytoclassic vasculitis

In the majority of patients, blood tests indicate leukocytosis, anemia, elevated C-reactive protein, and elevated liver enzymes. An association of FUMHD with elevated blood levels of TNF-alpha has also been described.

may include elevated levels of leukocytes, erythrocyte sedimentation, C-reactive protein, lactate dehydrogenase, and liver enzymes, as well as anemia, and hypoproteinemia. Other blood work-up or testing may be done to rule out other explanations for the symptoms (e.g., autoantibody measurements, serology, blood and bacterial skin cultures, urine analysis, serum electrolytes).

There are no specific indications for the use of these or other tests in the diagnosis or treatment of internal organs affected by this disease. As with other serious medical conditions in which internal organs can be affected, these tests may be used to manage FUMHD when internal organs such as the lungs, liver, heart, etc. are involved.

Acyclovir was prescribed in cases where varicella was initially suspected. None of these cases turned out to be associated with herpes simplex or varicella-zoster virus infection. The benefit of acyclovir therapy in people with FUMHD is questionable.

Systemic steroids have been commonly utilized among reported cases (27 of 40 cases), with only one report of a positive effect. Methotrexate has been used in 15 patients. It induced rapid remissions and was successful in cases that did not respond to other therapies. Still four patients died despite methotrexate theapy. It is possible this was due to its late institution.

Debridement and skin grafting was successful in one case, but the patient was left with considerable scaring.

In advanced disease, therapy is also aimed at stabilizing the patient. Intensive care treatment of infection and maintenance of the patient’s general condition is vital.The state of these patients is similar to what is seen in patients with severe burns. Thus, patients with FUMHD may benefit from the same supportive services that burn victims receive.


Treatment with tumor necrosis factor (TNF)-alpha inhibitors (such as infliximab and etanercept) has been suggested as a first-line option in the management of FUMHD because elevated levels of serum TNF-alpha have been reported in this disease However, further studies may be required to establish this approach to treatment.

More detailed information about treatment options for FUMHD can be accessed through the DermNet NZ web site.

Like other FUMHD therapies, the efficacy (benefits) of Methotrexate therapy for treatment of pediatric FUMHD is not known. Because little is known regarding the efficacy of therapies for FUMHD the clinician must use his or her best judgment when deciding on approaches for treatment.

This requires careful monitoring for signs of progressing disease including worsening skin erosions and ulcers. In cases with fatal outcome, death was attributed in a few cases to sepsis, other cases were due to pulmonary thromboembolism, pneumonia, cardiac arrest, sepsis, hypovolemic shock, and massive thrombosis (blood clot) of the superior mesenteric artery. Careful monitoring for early signs of these and other serious complications is required. Click here to view the signs and symptoms of sepsis.

In adults, FUMHD tends to lasts several months with succession of outbreaks until complete healing or transformation to common PLEVA. In the medical literature, all reported deaths due to FUMHD were in adults. Deaths occurred within 8 days to 7 months during follow-up treatment.

The exact incidence or prevalence of FUMHD is not known. Only 42 cases have been reported in the medical literature.