Years published: 1990, 1995, 2003, 2009, 2012, 2015, 2018, 2023
NORD gratefully acknowledges Amy S. Paller, MD, Walter J. Hamlin Professor and Chair of Dermatology, Professor of Pediatrics, Northwestern University, Feinberg School of Medicine, for assistance in the preparation of this report.
Mucha-Habermann disease, also known as pityriasis lichenoides et varioliformis acuta or PLEVA, is a rare skin disorder. The lesions most often appear on the trunk, arms and legs. Lesions tend to develop in small groups. Mucha-Habermann disease most often affects children or young adults. A more severe type of this disorder, known as febrile ulceronecrotic Mucha-Habermann disease, can cause life-threatening complications. The exact cause of Mucha-Habermann disease is unknown, but it may be an immune response to viruses and other triggers. Mucha-Habermannn is considered to be the acute end of a spectrum of skin disease known as pityriasis lichenoides. The more chronic end is known as pityriasis lichenoides chronica or chronic parapsoriasis. In some cases, the term Mucha-Habermann disease may be used to denote the entire spectrum.
The onset of Mucha-Habermann disease is usually sudden and is marked by the development of a recurrent rash, consisting of round, elevated lesions (papules or plaques) that may be itchy and burning. These lesions are usually reddish-purple to reddish-brown and may progress to develop a black-purple crust, representing tissue death (necrosis) and bleeding (hemorrhage). The lesions eventually blister, often causing scarring or temporary discoloration upon healing.
Although the trunk, arms and legs are most often affected by Mucha-Habermann disease, any part of the body may potentially develop skin lesions, including the scalp and the mouth. Lesions may number only a few to hundreds. Lesions may resolve without treatment in a few weeks but persist or recur on and off for years.
Most affected individuals do not have any symptoms other than the skin findings. However, some individuals may have headaches, fever, joint pain (arthralgia) and a general feeling or poor health (malaise). In some people, swelling of nearby lymph nodes (lymphadenopathy) may also occur.
Febrile Ulceronecrotic Mucha-Haberman Disease (FUMHD)
FUMHD is a rare, severe type of Mucha-Habermann disease characterized by the rapid development of numerous black or necrotic bumps (papules) on the skin. These lesions may grow and spread rapidly, eventually combining (coalescing) into extremely painful ulcers and blisters. These lesions tend to be larger than those associated with the more common form of Mucha-Habermann disease. They may bleed, often scar upon healing and may become infected.
FUMHD is associated with additional symptoms, including a high fever, joint pain (arthritis), gastrointestinal abnormalities (e.g., diarrhea, sore throat, and abdominal pain), enlargement of the spleen, inflammation of the lungs (interstitial pneumonitis) and central nervous system abnormalities. FUMHD occurs more often in children than adults, but progression to life-threatening complications such as infection of the blood (sepsis) occurs occasionally and more often in adults than in children.
FUMHD usually lasts several months before resolving on its own but recurs on and off for several years. Eventually, FUMHD may transform into the less severe form of Mucha-Habermann disease.
The exact cause of Mucha-Habermann disease is unknown. Mucha-Habermann disease is part of the spectrum of pityriasis lichenoides, a benign group of disorders. Within this spectrum is also pityriasis lichenoides chronica, in which the lesions are more persistent and are characterized as pink scaling round patches on the trunk and extremities. Researchers have speculated that pityriasis lichenoides occurs because of an exaggerated, inflammatory reaction (hypersensitivity) of the body to an infectious agent. A range of causes have been suspected, including several reports of occurrence after infection or vaccination with SARS-CoV-2.
Some researchers have suggested that Mucha-Habermann disease is a benign, self-healing lymphoproliferative disorder. Lymphoproliferative disorders are characterized by the overproduction of certain white blood cells called lymphocytes. These cells often accumulate in structures and tissues of the body, potentially damaging them.
Mucha-Habermann disease affects males more often than females. The disorder is most common in children and young adults but can affect people of any age including newborns (with lesions present at birth) and the elderly. The incidence of Mucha-Habermann disease is unknown.
A diagnosis of Mucha-Habermann disease is made based upon a thorough clinical evaluation, detailed patient history, identification of characteristic skin lesions and, if needed for confirmation, microscopic examination (biopsy) of affected skin tissue.
Mucha-Habermann disease usually resolves on its own within several weeks to several months. However, several therapies have been used to treat the condition. Oral antibiotics can help to clear lesions in about 50% of affected individuals, particularly erythromycin in children and a tetracycline derivative in adults. Exposure to ultraviolet light is the most effective therapy, particularly if the pityriasis lichenoides is persistent. While individuals can show considerable improvement from summer sunlight, phototherapy (light treatments) with narrow band ultraviolet light is an alternative for more controlled light delivery and during months that are not sunny. PUVA (psoralens and ultraviolet A light) is less commonly used, given its greater associated risk of the development of skin cancer and accelerated skin aging. Topical corticosteroids and systemic antihistamines have been used to ease pruritus, but do not clear the lesions. Methotrexate or dapsone may be necessary in severe cases.
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