Mucha-Habermann Disease - Causes, Symptoms, Diagnosis, and Management

Introduction:

Mucha Habermann disease is considered the acute end of a spectrum of skin diseases. This is known as pityriasis lichenoides, with its more chronic end known as pityriasis lichenoides chronica. Sometimes, it may denote the entire spectrum term as Mucha-Habermann disease. Mucha Haberman disease, also known as pityriasis lichenoid et varioliformis acute or PLEVA, is a rare skin disorder. These lesions most often appear on the trunk, the arms, and the legs. Lesions tend to develop in small groups. The Mucha-Habermann disease most often affects children or young adults. A more severe form of this disorder is known as febrile ulceronecrotic Mucha Habermann disease, which can cause life-threatening complications in adults.

What Are the Other Names for This Condition?

• FUMHD (febrile ulceronecrotic Mucha Habermann disease).

• Ulceronecrotic Mucha Habermann disease.

• A variant of Mucha Habermann disease.

What Is Febrile Ulceronecrotic Mucha-Habermann Disease?

Febrile ulceronecrotic Mucha-Habermann is a skin disease that is a rare and severe form of the pityriasis lichenoides et varioliformis acuta (PLEVA). PLEVA is characterized by skin lesions that ulcerate, break down, form open sores, then form a red-brown crust. FUMHD often begins as PLEVA but rapidly and suddenly progresses to large or destructive ulcers. Febrile ulceronecrotic Mucha-Habermann is a disease that occurs more frequently in children, peaking at ages five to ten. Males tend to be more often affected than females. It may be with fever and extensive and painful loss of skin tissues, as well as a secondary infection of the ulcers. Biopsy of skin lesions is the confirmatory diagnosis done for febrile ulceronecrotic Mucha-Habermann Disease.

Who Gets Febrile Ulceronecrotic Mucha-Habermann Disease?

Febrile ulceronecrotic Mucha-Habermann disease is a rare skin disorder. The exact cause of this disease is unknown. 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 are more susceptible to the disorder than females. Individuals of all ethnic and racial groups may be affected.

What Are the Causes of Febrile Ulceronecrotic Mucha- Habermann Disease?

The cause of ulceronecrotic Mucha Habermann's disease is not known.

• Hypersensitivity to infectious agents is suggested to be the leading cause.

• Single cases of people with febrile ulceronecrotic Mucha Habermann and Epstein Barr virus infection, adenoviruses, or cytomegalovirus have been reported, but there have been no consistent findings.

• There are some suggestions that febrile ulceronecrotic Mucha Habermann disease 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 the type of white blood cell (lymphocytes) that makes a part of the immune system.

What Are the Risk Factors for Febrile Ulceronecrotic Mucha Habermann Disease?

Currently, no risk factors have been identified for febrile ulceronecrotic Mucha- Habermann disease. However, it is essential to note that one will get the condition based on the risk factor that increases one's chances of the severity of the disease. Some risk factors are more critical than others. Also, not having a risk does not mean an individual will not get this condition. Discussing the effect of risk factors with healthcare providers is always essential.

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:

1. Red scaly skin lesions (papules) that ulcerate, break down, form open sores, then a red-brown crust (that is PLEVA).

2. The sudden onset is only marked by the development of a recurrent rash consisting of rounded and elevated lesions (papules or macules) that may be itching and burning on the lesion site.

3. These lesions are mostly reddish-purple to reddish-brown and progress to develop as a blackish-brown crust type, causing tissue death (necrosis) and bleeding (hemorrhagic).

4. Sudden progression to significant and destructive ulcers can be associated with the extensive, painful loss of skin tissue.

5. The rash consists of rounded or elevated lesions (papules and macules) that may be itchy and burning.

6. The lesions eventually blister, causing scarring or temporary discoloration upon healing.

7. Infection of skin lesions, which may cause pus and putrid odor.

8. High fever that may be persistent or come and go.

9. A feeling of illness.

10. Sore throat.

11. Congestion.

12. Enlarged spleen.

13. Diarrhea.

14. Central nervous system symptoms.

15. Muscle soreness or pain.

16. Joint pain.

17. Arthritis.

18. Lymphocytic (viral) myocarditis.

19. Sepsis.

20. Interstitial pneumonitis (scarring or thickening of the lungs).

How Is Mucha-Habermann Disease Been Diagnosed?

Diagnosis of Mucha-Habermann disease is based on the following:

• Thorough clinical evaluation.

• A detailed patient history.

• Identification of characteristic skin lesions.

• Needed microscopic examination (biopsy) of affected skin tissue.

How Is Febrile Ulcerative Mucha- Habermann Disease Treated?

Treatment needed for febrile ulceronecrotic Mucha-Habermann disease may include one or more of the following methods:

1. Administration of steroids.

2. Anti-inflammatory medications such as Dapsone.

3. Debridement and skin grafting.

4. Immune-suppressing agents such as Cyclosporine.

5. Chemotherapeutic agents.

6. Psoralen (medications used to treat psoriasis) in combination with ultraviolet A (PUVA).

7. Antibiotics for infections.

8. Ultraviolet B treatment.

9. Antiviral agents (such as Acyclovir).

10. Tumor necrosis factor-alpha (TNF-alpha) inhibitors.

What Are the Possible Complications of Febrile Ulceronecrotic Mucha-Habermann Disease?

Complications for febrile ulceronecrotic Mucha-Habermann disease may include:

• Severe pain.

• Breathing difficulties due to scarring of the lung tissue.

• Infections of lesions spreading to blood (sepsis).

How Can Febrile Ulceronecrotic Mucha-Habermann Disease Be Prevented?

The cause of the Mucha-Habermann disease is not known. Therefore, effective methods or guidelines for its prevention are available. Active research is performed to explore the possibilities for treating and preventing inherited and acquired genetic disorders. Regular medical screening with tests and physical examinations is recommended.

What Is the Prognosis of Febrile Ulceronecrotic Mucha Habermann Disease?

The prognosis of this febrile ulceronecrotic Mucha-Habermann disease varies between adults and children. The condition may resolve or be effectively treated with steroids or antibiotic therapy in children. Even though the disease progression is rapidly seen in children, the prognosis is also better. However, the condition may linger for several months in adults. Approximately 20 % of affected adults are reported. Deaths may occur anywhere between eight days to seven months.

Conclusion:

Mucha Habermann's disease usually resolves within several weeks to seven months. However, an early diagnosis of the disease and time management may help to avoid a poor prognosis.