Febrile Ulceronecrotic Mucha-Habermann disease

Alternate names

FUMHD; Ulceronecrotic Mucha-Habermann disease; Variant of Mucha-Habermann disease

Definition

• 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.

Epidemiology

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

Cause

• 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.

Signs and symptoms

• 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.

Diagnosis

FUMHD is diagnosed based upon the clinical symptoms in the patient, with confirmation by skin biopsy. Skin biopsy findings suggestive of FUMHD are outlined below. Because this information is technical

we recommend that you review it with a health care provider: 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.

Treatment

• It is important that FUMHD is diagnosed and treated as soon as possible.
• While a number of treatments have been tried, it is hard to asses the benefit of the therapies because there are so few cases of FUMHD and among reported cases the treatment approach may vary. The case reports describe treatment with systemic steroids, methotrexate, antibiotics, dapsone, cyclosporine, psoralen and ultraviolet A (PUVA), ultraviolet B (UVB), unspecified ultraviolet receptor, acyclovir, immunoglobulins, and 4,4-diaminodiphenylsulphone (DDS).
• Again the efficacy of these therapies are not known.
• 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.

Prognosis

• FUMHD is a very aggressive disorder. The reported mortality rate is 20%.
• All deaths reported in the medical literature have been in adults.
• In children the progression from PLEVA to FUMHD tends to be quicker, yet children also tend to have a better prognosis than adults.

NIH genetic and rare disease info

• NIH info on Febrile Ulceronecrotic Mucha-Habermann disease

Febrile Ulceronecrotic Mucha-Habermann disease is a rare disease.

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