Majocchi-Schamberg disease
Majocchi-Schamberg Disease
Majocchi-Schamberg disease, also known as Schamberg's disease or progressive pigmented purpuric dermatitis, is a chronic skin condition characterized by the appearance of purpuric and pigmented patches on the skin. It is a type of capillaritis, which involves inflammation of the small blood vessels (capillaries) in the skin.
Clinical Presentation[edit | edit source]
Majocchi-Schamberg disease typically presents as reddish-brown patches on the skin, often with a "cayenne pepper" appearance due to the presence of pinpoint petechiae. These patches are usually asymptomatic, but some patients may experience mild itching. The lesions are most commonly found on the lower extremities, particularly the legs, but can also appear on the arms and trunk.
Pathophysiology[edit | edit source]
The exact cause of Majocchi-Schamberg disease is not well understood, but it is believed to be related to an immune-mediated process that leads to damage of the capillaries in the skin. This results in leakage of red blood cells into the surrounding tissue, causing the characteristic purpuric and pigmented appearance.
Diagnosis[edit | edit source]
Diagnosis of Majocchi-Schamberg disease is primarily clinical, based on the appearance of the skin lesions. A skin biopsy may be performed to confirm the diagnosis and rule out other conditions. Histopathological examination typically shows a perivascular lymphocytic infiltrate and hemosiderin deposition.
Treatment[edit | edit source]
There is no specific cure for Majocchi-Schamberg disease, and treatment is generally aimed at managing symptoms and improving the appearance of the skin. Topical corticosteroids may be used to reduce inflammation and itching. In some cases, oral medications such as pentoxifylline or ascorbic acid may be prescribed to improve capillary function.
Prognosis[edit | edit source]
Majocchi-Schamberg disease is a chronic condition that may persist for many years. While it is not life-threatening, it can be cosmetically distressing for patients. The disease may have periods of exacerbation and remission.
Also see[edit | edit source]
Dermatology and Rheumatologic diseases A - Z
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