What are the other Names for this Condition? (Also known as/Synonyms)

• FUMHD (Febrile Ulceronecrotic Mucha-Habermann Disease)
• Ulceronecrotic Mucha-Habermann Disease
• Variant of Mucha-Habermann Disease

What is Febrile Ulceronecrotic Mucha-Habermann Disease? (Definition/Background Information)

• 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
• Febrile Ulceronecrotic Mucha-Habermann Disease occurs more frequently in children, peaking at age 5 to 10. Males tend to be affected more often than females. There may be fever and extensive, painful loss of skin tissue as well as secondary infection of the ulcers
• Diagnosis of Febrile Ulceronecrotic Mucha-Habermann Disease is confirmed by biopsy of skin lesions

(Source: Febrile Ulceronecrotic Mucha-Habermann Disease; Genetic and Rare Diseases Information Center (GARD) of National Center for Advancing Translational Sciences (NCATS), USA.)

Who gets Febrile Ulceronecrotic Mucha-Habermann Disease? (Age and Sex Distribution)

• Febrile Ulceronecrotic Mucha-Habermann Disease is a very rare skin disorder. The exact prevalence of the condition is not known. So far, fewer than 50 cases have been reported in the medical literature
• The presentation of symptoms may occur at any age, but children are more commonly affected than adults
• Although the condition can occur in both genders, males exhibit a higher susceptibility to the disorder than females
• Worldwide, individuals of all racial and ethnic groups may be affected

What are the Risk Factors for Febrile Ulceronecrotic Mucha-Habermann Disease? (Predisposing Factors)

• Currently, no risk factors have been clearly identified for Febrile Ulceronecrotic Mucha-Habermann Disease

It is important to note that having a risk factor does not mean that one will get the condition. A risk factor increases one’s chances of getting a condition compared to an individual without the risk factors. Some risk factors are more important than others.

Also, not having a risk factor does not mean that an individual will not get the condition. It is always important to discuss the effect of risk factors with your healthcare provider.

What are the Causes of Febrile Ulceronecrotic Mucha-Habermann Disease? (Etiology)

The cause of Ulceronecrotic Mucha-Habermann Disease 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

(Source: Febrile Ulceronecrotic Mucha-Habermann Disease; Genetic and Rare Diseases Information Center (GARD) of National Center for Advancing Translational Sciences (NCATS), USA.)

What are the Signs and Symptoms of Febrile Ulceronecrotic Mucha-Habermann Disease?

The signs and symptoms of Febrile Ulceronecrotic Mucha-Habermann Disease may include the following:

• Red scaly skin lesions (papules) that ulcerate, breakdown, form open sores, then a red-brown crust (i.e., PLEVA)
• Sudden progression to large and destructive ulcers, which can be associated with extensive, painful loss of skin tissue
• Infection of skin lesions, which may cause pus and a putrid odor
• High fever (up to 104°F), that may be persistent or come and go
• A feeling of illness
• Sore throat
• Congestion
• Abdominal pain
• Enlarged spleen 
• Diarrhea
• Central nervous system symptoms
• Muscle soreness or pain
• Joint pain
• Arthritis
• Megaloblastic anemia
• Interstitial pneumonitis (scarring or thickening of the lungs)
• Lymphocytic (viral) myocarditis 
• Sepsis

(Source: Febrile Ulceronecrotic Mucha-Habermann Disease; Genetic and Rare Diseases Information Center (GARD) of National Center for Advancing Translational Sciences (NCATS), USA.)

How is Febrile Ulceronecrotic Mucha-Habermann Disease Diagnosed?

Febrile Ulceronecrotic Mucha-Habermann Disease is diagnosed on the basis of the following:

• Complete physical examination
• Thorough personal and family medical history evaluation
• Assessment of signs and symptoms
• Laboratory tests, which may include tests to assess:
  • Complete blood count
  • Levels of C-reactive protein
  • Levels of TNF-alpha
  • Liver enzymes
• Imaging studies
• Skin biopsy studies

Many clinical conditions may have similar signs and symptoms. Your healthcare provider may perform additional tests to rule out other clinical conditions to arrive at a definitive diagnosis.

What are the possible Complications of Febrile Ulceronecrotic Mucha-Habermann Disease?

The complications of Febrile Ulceronecrotic Mucha-Habermann Disease may include:

• Severe pain
• Scarring
• Breathing difficulties due to scarring of lung tissue
• Infection of lesions spreading to blood (sepsis)

Complications may occur with or without treatment, and in some cases, due to treatment also.

How is Febrile Ulceronecrotic Mucha-Habermann Disease Treated?

The treatment for Febrile Ulceronecrotic Mucha-Habermann Disease may include one or more of the following methods:

• Administration of steroids
• Anti-inflammatory medications such as dapsone
• Immune-suppressing agents such as cyclosporine
• Debridement and skin grafting
• Psoralen (medication used to treat psoriasis) in combination with ultraviolet A (PUVA)
• Ultraviolet B treatment
• Anti-viral agents (such as acyclovir) 
• Antibiotics for infections
• Chemotherapeutic agents such as methotrexate
• Tumor necrosis factor-alpha (TNF-alpha) inhibitors

How can Febrile Ulceronecrotic Mucha-Habermann Disease be Prevented?

• The cause of Febrile Ulceronecrotic Mucha-Habermann Disease is not known, therefore no effective methods or guidelines for its prevention are available
• Active research is currently being performed to explore the possibilities for treatment and prevention of inherited and acquired genetic disorders
• Regular medical screening at periodic intervals with tests and physical examinations are recommended

What is the Prognosis of Febrile Ulceronecrotic Mucha-Habermann Disease? (Outcomes/Resolutions)

The prognosis of Febrile Ulceronecrotic Mucha-Habermann Disease varies between adults and children.

• In children, the condition may resolve by itself or be effectively treated with aggressive steroids and/or antibiotics therapy. Even though the disease progression is rapid in children, the prognosis is better
• In adults, the condition may linger for several months. Approximately, 20% of affected adults are reported to succumb to FUMHD. Deaths are known to occur anywhere between 8 days to 7 months
• An early diagnosis and timely treatment may help avoid complications and a consequent poor prognosis

Additional and Relevant Useful Information for Febrile Ulceronecrotic Mucha-Habermann Disease:

The following DoveMed website link is a useful resource for additional information:

http://www.dovemed.com/diseases-conditions/rare-disorders/