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Febrile Ulceronecrotic Mucha-Habermann disease


Other Names for this Disease

  • FUMHD
  • Ulceronecrotic Mucha-Habermann disease
  • Variant of Mucha-Habermann disease
See Disclaimer regarding information on this site. Some links on this page may take you to organizations outside of the National Institutes of Health.

Your Question

How does a person contract FUMHD? How is a person tested for FUMHD? If it is genetic, can other children of the same parents be tested for it? How can doctors ensure they have not wrongly diagnosed someone that has FUMHD (in my loved one FUMHD was confused with chicken pox)? At what point does the disease stop being PLEVA and become FUMHD? What are the signs and symptoms that someone has FUMHD? How do doctors determine whether or not the treatments being administered for FUMD are working? At what point should Methotrexate be used to treat FUMHD? When should MRI's and X-rays be used to look for disease affecting the interior?

Our Answer

We have identified the following information that we hope you find helpful. If you still have questions, please contact us.

What is febrile ulceronecrotic Mucha-Habermann disease (FUMHD)?

Febrile ulceronecrotic Mucha-Habermann disease (FUMHD) is a rare and severe form of pityriasis lichenoides et varioliformis acuta (PLEVA).[1][2][3][4][5][6] 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.[6] Males tend to be affected more often than females.[2][6] 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.
Last updated: 9/7/2012

How rare is febrile ulceronecrotic Mucha-Habermann disease?

The exact incidence or prevalence of FUMHD is not known. Only 40 cases have been reported in the medical literature.[7]
Last updated: 7/31/2009

Is febrile ulceronecrotic Mucha-Habermann disease genetic?

At this time, there is little evidence to suggest that FUMHD is genetic. It is thought to be an acquired condition.[1][2]
Last updated: 7/31/2009

What causes febrile ulceronecrotic Mucha-Habermann disease?

The cause of FUMHD is not known (idiopathic).[1][2] A hypersensitivity to an infectious agent is suggested to be the main cause.[1][2][7] 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.[2][7] There is some suggestion that FUMHD may be a type of clonal T-cell disorder.[1][2][7] “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.
Last updated: 7/31/2009

How does a person contract febrile ulceronecrotic Mucha-Habermann disease?

The way in which a person contracts or develops FUMHD is unknown at this time.
Last updated: 7/31/2009

What are the signs and symptoms of febrile ulceronecrotic Mucha-Habermann disease?

Initial symptoms of FUMHD include red scaly skin legions (papules) and papulonecrotic lesions often around 2- to 5-mm in diameter (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 legions can become infected which may cause pus and a putrid odor.[2] The rate of progression from PLEVA to FUMHD varies among reports but may be days to weeks.[2]  Some cases go straight to FUMHD rather than progress from PLEVA.[7] 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.[6][1][2][3][7] FUMHD can become life threatening.
Last updated: 7/31/2009

At what point does the disease stop being PLEVA and become febrile ulceronecrotic Mucha-Habermann disease?

PLEVA is characterized by 2- to 5-mm diameter erythematous papules with a fine scale on top.[6] As the scale thickens it loosens around it’s edges but remains attached in the center. The center of the papule often becomes fluid filled or pussy and the tissue begins to die, becomes ulcerated, with overlying red-brown crusts.  Symptoms may include burning and itchiness. Successive crops of lesions can last indefinitely, from a few weeks to months to years.[6] As the legions heal they may leave scars and cause skin discoloration.

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.[6]

In some cases, the symptoms reflect FUMHD from the start, rather than progress from PLEVA to FUMHD like symptoms.[7]
Last updated: 10/22/2010

How is febrile ulceronecrotic Mucha-Habermann disease definitively diagnosed?

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[6]
  • 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[6]
  • Vascular changes – Dilation and engorgement of blood vessels in papillary dermis with endothelial proliferation, vascular congestion, occlusion, dermal hemorrhage, and extravasation of erythrocytes[6]
  • Vasculitis – Fibronoid necrosis of vessel walls with leukocytoclassic vasculitis[6]
Last updated: 7/31/2009

What types of tests might be needed for diagnosis of febrile ulceronecrotic Mucha-Habermann disease?

Skin biopsy is important for confirmation of diagnosis of FUMHD. Laboratory abnormalities may include elevated levels of leukocytes, erythrocyte sedimentation, C-reactive protein, lactate dehydrogenase, and liver enzymes, as well as anemia, and hypoproteinemia.[6] 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…).
Last updated: 7/31/2009

At what point should MRI’s, X-rays or other tests be used to look for disease affecting the interior?

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.
Last updated: 7/31/2009

How might pediatric FUMHD be distinguished from chicken pox or other conditions?

Distinguishing FUMHD from other conditions requires a good understanding of the characteristics of both conditions in question. In children, FUMHD is often initially confused with chicken pox (varicella). Chicken pox can be distinguished from FUMHD by a physician based up the following features:[6]

  • Tzanck-positive clear vesicles
  • Constitutional symptoms
  • Common involvement of mucous membranes and face (although a case of oral involvement of FUMHD has been reported)
  • Extensive vesiculation in infiltrate
  • Presence of balloon cells
  • Presence of multinucleate giant cells
Last updated: 8/7/2009

How is febrile ulceronecrotic Mucha-Habermann disease (FUMHD) treated?

It is important that FUMHD is diagnosed and treated as soon as possible.[1] 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.[1][2][3][6][7]

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.[2]

Systemic steroids have been commonly utilized among reported cases (27 of 40 cases), with only one report of a positive effect.[7] 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.[7]

Debridement and skin grafting was successful in one case, but the patient was left with considerable scaring.[1]

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.[1][7]

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[7][8] 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.
Last updated: 9/7/2012

At what point should Methotrexate be used to treat pediatric FUMHD?

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.
Last updated: 7/31/2009

How do doctors determine whether or not the treatments for FUMHD are working?

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.[7] Careful monitoring for early signs of these and other serious complications is required. Click here to view the signs and symptoms of sepsis.
Last updated: 7/31/2009

What is the typical prognosis for people with this condition?

FUMHD is a very aggressive disorder. The reported mortality rate is 20%.[7] 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. Some cases in children have resolved spontaneously, others resolved with aggressive therapy and antibiotics.[9] Once the condition resolves, children may be left with some residual scaring and skin discoloration,[2] however healing without scaring has been reported.[2]

In adults, FUMHD tends to lasts several months with succession of outbreaks until complete healing or transformation to common PLEVA.[2] In the medical literature, all reported deaths due to FUMHD were in adults.[1] Deaths occurred within 8 days to 7 months during follow-up treatment.[3]
Last updated: 7/31/2009

References
  • Aytekin S, Balci G, Duzgun OY. Febrile ulceronecrotic Mucha-Habermann disease: a case report and a review of the literature. Dermatol Online J. 2005 Dec 1;
  • Yang CC, Lee JY, Chen W. Febrile ulceronecrotic Mucha-Habermann disease with extensive skin necrosis in intertriginous areas. Eur J Dermatol. 2003 Sep-Oct;
  • Miyamoto T, Takayama N, Kitada S, Hagari Y, Mihara M. Febrile ulceronecrotic Mucha-Habermann disease: a case report and a review of the literature. J Clin Pathol. 2003 Oct;
  • Hoghton MA, Ellis JP, Hayes MJ. Febrile ulceronecrotic Mucha Habermann disease: a fatality. J R Soc Med. 1989 Aug;
  • Miller ML. Miscellaneous Conditions Associated with Arthritis. In: Kliegman. Nelson Textbook of Pediatrics, 18th ed. Philadelphia, PA: Saunders; 2007;
  • Bowers S, Warshaw EM. Pityriasis lichenoides and its subtypes. J Am Acad Dermatol. 2006;
  • Sotiriou E, Patsatsi A, Tsorova C, Lazaridou E, Sotiriadis D. Febrile ulceronecrotic Mucha-Habermann disease: a case report and review of the literature. Acta Derm Venereol. 2008;
  • Tsianakas A, Hoeger PH. Transition of pityriasis lichenoides et varioliformis acuta to febrile ulceronecrotic Mucha-Habermann disease is associated with elevated serum tumour necrosis factor-alpha. Br J Dermatol. 2005 Apr;
  • Cozzzio A. Et al.,. Febrile ulceronecrotic Mucha-Habermann disease with clonality: A cutaneous T-cell lymphoma entity?. Am Acd Dermatol. 2004;
Other Names for this Disease
  • FUMHD
  • Ulceronecrotic Mucha-Habermann disease
  • Variant of Mucha-Habermann disease
See Disclaimer regarding information on this site. Some links on this page may take you to organizations outside of the National Institutes of Health.