About mucha habermann disease

What is mucha habermann disease?

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 and the arms and legs. Lesions tend to develop in small groups. Mucha-Habermann disease most often affects children or young adults. A more severe variant of this disorder, known as febrile ulceronecrotic Mucha-Habermann disease, can cause life-threatening complications in adults. The exact cause of Mucha-Habermann disease is unknown.

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. In some cases, the term Mucha-Habermann disease may be used to denote the entire spectrum.



What are the symptoms for mucha habermann disease?

Pruritis symptom was found in the mucha habermann disease condition

The onset of Mucha-Habermann disease is usually sudden and is marked by the development of a recurrent Rash consisting of rounded, elevated Lesions (papules or macules) that may be Itchy and burning. These Lesions are usually reddish-purple to reddish-brown and may progress to develop a ish-brown crust, tissue death (necrosis) and bleeding (hemorrhaging). The Lesions eventually blister, often causing scarring or temporary discoloration upon healing.

Although the trunk and the arms and legs are most often affected by Mucha-Habermann disease, any part of the body may potentially develop skin lesions. Lesions may number only a few to more than one hundred. Lesions may resolve without treatment in a few weeks, but may recur on and off for years.

In most cases, affected individuals do not have any symptoms in addition to the skin findings. However, some individuals may have headaches, fever, Joint Pain (arthralgia), and a general feeling or poor health (malaise). In some cases, Swelling of nearby lymph nodes (lymphadenopathy) may also occur.

Febrile Ulceronecrotic Mucha-Haberman Disease (FUMHD)

FUMHD is a rare, severe variant of Mucha-Habermann disease characterized by the rapid development of numerous 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. However, in adults some cases have progressed to cause life-threatening complications such as infection of the blood (sepsis). Life-threatening complications have not been reported in children with FUMHD.

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.



What are the causes for 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. However, no causative infectious agent has been identified.

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.



What are the treatments for mucha habermann disease?

Mucha-Habermann disease usually resolves on its own within several weeks to several months. However, in some cases various therapies to treat condition may be used. 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 eruption. Methotrexate or dapsone may be necessary in severe cases.



What are the risk factors for mucha habermann disease?

Mucha Habermann disease is a rare skin disorder, which is a part of a spectrum of disorders called Pityriasis Lichenoides Et Varioliformis Acula.
Higher patient age, sepsis, systemic and mucosal involvement, and literature data of 119 FUMHD case reports, were four key unfavorable risk variables relating to a death result.

Risk factors

  • The affected persons develop hypersensitivity to infectious agents and allergens. Therefore, exposure to pollutants and irritants increases the area of skin where lesions expand.
  • The disease is more incident in spring and autumn.
  • Microbial infections promote the progression of the disease wherein the lesions grow deeper into the systemic level.
  • Exposure to heat and friction worsens the condition. However, exposure to sunlight poses no risks. On the contrary, UV light is used to treat the disease.
  • The self-resolutive capacity is low in elderly person, indicating age is a risk factor.
  • The presence of wound and consequent increase in immune activity poses risks of progression.
  • An early transfer to the appropriate pediatric or adult critical care unit may be beneficial for patients who are both pediatric and adult and have a FUMHD mortality risk score of less than 10.


Symptoms
Lesions,Itching and burning around the lesions,Pruritis,Headaches,Fever,Joint pain,Malaise,Swollen lymph nodes,Blisters
Conditions
Arthritis,Interstitial pneumonitis,Pruritis
Drugs
Oral antibiotics: erythromycin for children and tetracycline derivatives for adults,Topical corticosteroids and antihistamines to ease pruritis,Methotrexate or dapsone in severe cases



Is there a cure/medications for mucha habermann disease?

Usually, Mucha-Habermann's disease goes away on its own in a few weeks to a few months. However, different therapies may be utilized to treat a problem in some circumstances.

  • About 50% of those with lesions can benefit from oral antibiotics, especially erythromycin in children and a tetracycline derivative in adults.
  • The best treatment, especially for persistent pityriasis lichenoides, is exposure to ultraviolet radiation.
  • Although summer sunlight can significantly enhance a person's condition, narrow-band ultraviolet light phototherapy (light therapies) is an alternative for more controlled light delivery throughout the winter and fall.
  • Psoralens and ultraviolet A light, often known as PUVA, are less frequently used because of the increased risk of skin cancer and early aging that they are connected with.
  • Antihistamines taken orally and topically have been used to reduce pruritus, but they do not remove the eruption.
  • In extreme situations, methotrexate or dapsone may be required.
  • Low doses of oral methotrexate were used to treat six patients with Mucha-Habermann illness.
  • All displayed quick clearance while undergoing methotrexate therapy, although recurrences were frequent when the medication was stopped.


Symptoms
Lesions,Itching and burning around the lesions,Pruritis,Headaches,Fever,Joint pain,Malaise,Swollen lymph nodes,Blisters
Conditions
Arthritis,Interstitial pneumonitis,Pruritis
Drugs
Oral antibiotics: erythromycin for children and tetracycline derivatives for adults,Topical corticosteroids and antihistamines to ease pruritis,Methotrexate or dapsone in severe cases



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