Febrile Ulceronecrotic Mucha-Habermann disease

What causes febrile ulceronecrotic Mucha-Habermann disease?

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.

Last updated on 05-01-20

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
  • 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.

Last updated on 05-01-20

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. 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 on 05-01-20

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 on 05-01-20

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.

Last updated on 05-01-20

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 on 05-01-20

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. 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. As the lesions 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.

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

Last updated on 05-01-20

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. 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 on 05-01-20

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). 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.

Last updated on 05-01-20

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:

  • 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 on 05-01-20

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 on 05-01-20

What is the typical prognosis for people with this condition?

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. Some cases in children have resolved spontaneously, others resolved with aggressive therapy and antibiotics. Once the condition resolves, children may be left with some residual scaring and skin discoloration, however healing without scaring has been reported.

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.

Last updated on 05-01-20

How rare is febrile ulceronecrotic Mucha-Habermann disease?

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

Last updated on 05-01-20

Where can I learn about new treatment options for febrile ulceronecrotic Mucha-Habermann (FUMHD) disease?

You can find relevant articles on treatment of FUMHD through PubMed, a searchable database of biomedical journal articles. Although not all of the articles are available for free online, most articles listed in PubMed have a summary available. To obtain the full article, contact a medical/university library or your local library for interlibrary loan. You can also order articles online through the publisher’s Web site. Using "febrile ulceronecrotic Mucha-Habermann disease AND treatment" as your search term should help you locate articles. Use the advanced search feature to narrow your search results. Click here to view a search.
http://www.ncbi.nlm.nih.gov/PubMed

The National Library of Medicine (NLM) Web site has a page for locating libraries in your area that can provide direct access to these journals (print or online). The Web page also describes how you can get these articles through interlibrary loan and Loansome Doc (an NLM document-ordering service). You can access this page at the following link http://nnlm.gov/members/. You can also contact the NLM toll-free at 888-346-3656 to locate libraries in your area.

Last updated on 05-01-20

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

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.

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

Last updated on 05-01-20

In-Depth Information

Medscape Reference PLEVA

Medscape Reference provides information on PLEVA which includes information on FUMHD. You may need to register to view the article, but registration is free.

Last updated on 04-27-20

Selected Full-Text Journal Articles

Article - FUMHD 1

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;11(3):31.

Last updated on 04-27-20

Auster et al Much-Haberman

Auster BI, Santa Cruz DJ, Eisen AZ. Febrile Ulceronecrotic Mucha-Habermann's Disease with Interstitial Pneumonitis. Journal of Cutaneous Pathology. 2006:6(1);66-76.

Last updated on 04-27-20

article - FUMHD 3

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;56(10):795-7.

Last updated on 04-27-20

article - FUMHD 2

Yang CC, Lee JY, Chen W. Febrile ulceronecrotic Mucha-Habermann disease with extensive skin necrosis in intertriginous areas. Eur J Dermatol. 2003 Sep-Oct;13(5):493-6.

Last updated on 04-27-20

Where To Start

The doctor's doctor - PLEVA

The medical website TheDoctorsDoctor also provides information on PLEVA and FUMHD. Click on the link above to view the information page.

Last updated on 04-27-20

Name: American Academy of Dermatology 1445 New York Ave, NW Suite 800
Washington, DC, 20005, United States
Toll Free: 888-462-DERM (3376) Fax : 847-240-1859 Email: https://www.aad.org/Forms/ContactUs/Default.aspx Url: https://www.aad.org/
Name: British Association of Dermatologists 19 Fitzroy Square London, UK W1T 6EH
United Kingdom
Phone: 0207 383 0266 Fax : 0207 388 5263 Email: admin@bad.org.uk Url: http://www.bad.org.uk
Name: American Osteopathic College of Dermatology P.O. Box 7525
Kirksville, MO, 63501 , United States
Phone: 660-665-2184 Toll Free: 800-449-2623 Fax : 660-627-2623 Email: dermatology@aocd.org Url: http://www.aocd.org/
Name: American Autoimmune Related Diseases Association (AARDA) 22100 Gratiot Avenue
Eastpointe, MI, 48021, United States
Phone: 586-776-3900 Toll Free: 800-598-4668 Fax : 586-776-3903 Email: aarda@aarda.org Url: https://www.aarda.org/

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