Mucha-Habermann disease
Mucha-Habermann Disease Mucha-Habermann disease, also known as Pityriasis Lichenoides et Varioliformis Acuta (PLEVA), is a rare skin disorder characterized by the sudden eruption of small, scaling papules that can become necrotic and ulcerate. This condition is part of a spectrum of diseases known as pityriasis lichenoides, which also includes the chronic form, Pityriasis Lichenoides Chronica (PLC).
Clinical Presentation[edit | edit source]
Mucha-Habermann disease typically presents with a sudden onset of red, scaly papules that may develop into vesicles or pustules. These lesions can become necrotic, leading to crusted ulcers. The distribution of the rash is often widespread, affecting the trunk and extremities, but sparing the face and mucous membranes.
Symptoms[edit | edit source]
- Papules: Small, red, and scaly.
- Necrosis: Lesions may become necrotic and ulcerate.
- Pruritus: Itching is common but varies in intensity.
- Systemic Symptoms: Fever and malaise can occur, especially in severe cases.
Etiology[edit | edit source]
The exact cause of Mucha-Habermann disease is unknown. It is thought to be an inflammatory reaction, possibly triggered by an infectious agent or an abnormal immune response. Some studies suggest a link to viral infections, such as Epstein-Barr virus, but no definitive causative agent has been identified.
Diagnosis[edit | edit source]
Diagnosis is primarily clinical, supported by histopathological examination of a skin biopsy. The biopsy typically shows a lichenoid tissue reaction with necrotic keratinocytes and a superficial perivascular lymphocytic infiltrate.
Differential Diagnosis[edit | edit source]
Treatment[edit | edit source]
Treatment of Mucha-Habermann disease varies depending on the severity of the condition. Mild cases may resolve spontaneously without treatment, while more severe cases may require intervention.
Therapeutic Options[edit | edit source]
- Topical Corticosteroids: To reduce inflammation and itching.
- Systemic Antibiotics: Such as erythromycin or tetracycline, which may have anti-inflammatory properties.
- Phototherapy: Narrowband UVB or PUVA therapy can be effective.
- Immunosuppressive Agents: In severe cases, medications like methotrexate or cyclosporine may be used.
Prognosis[edit | edit source]
The prognosis for Mucha-Habermann disease is generally good, with many cases resolving spontaneously over weeks to months. However, recurrences are possible, and some patients may experience chronic or relapsing forms of the disease.
See Also[edit | edit source]
External Links[edit | edit source]
- [DermNet NZ: Pityriasis Lichenoides](https://dermnetnz.org/topics/pityriasis-lichenoides/) - [National Organization for Rare Disorders (NORD): Pityriasis Lichenoides](https://rarediseases.org/rare-diseases/pityriasis-lichenoides/)
NIH genetic and rare disease info[edit source]
Mucha-Habermann disease is a rare disease.
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Contributors: Prab R. Tumpati, MD
