Schamberg's disease pigmentation disorder

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Schamberg's Disease

Schamberg's Disease, also known as progressive pigmented purpuric dermatitis, is a chronic skin condition characterized by the appearance of reddish-brown patches caused by leaky blood vessels. This condition is named after Dr. Jay Frank Schamberg, who first described it in 1901.

Clinical Presentation[edit | edit source]

Patients with Schamberg's Disease typically present with asymptomatic, orange-brown patches on the skin, often with a "cayenne pepper" appearance due to pinpoint petechiae. These patches are most commonly found on the lower extremities but can occur anywhere on the body. The condition is more prevalent in males and can occur at any age, though it is more common in adults.

Pathophysiology[edit | edit source]

The exact cause of Schamberg's Disease is not well understood, but it is believed to be a form of capillaritis, where small blood vessels (capillaries) become inflamed and leak red blood cells into the skin. The breakdown of these red blood cells releases hemosiderin, a brown pigment, which deposits in the skin and causes the characteristic discoloration.

Diagnosis[edit | edit source]

Diagnosis of Schamberg's Disease is primarily clinical, based on the appearance of the skin lesions. A skin biopsy can be performed to confirm the diagnosis, which will show a perivascular lymphocytic infiltrate and hemosiderin deposition in the dermis.

Treatment[edit | edit source]

There is no cure for Schamberg's Disease, and treatment is aimed at managing symptoms and improving the appearance of the skin. Topical corticosteroids may be used to reduce inflammation. In some cases, oral medications such as pentoxifylline or ascorbic acid have been used with varying success. Patients are advised to avoid trauma to the affected areas and to use emollients to maintain skin hydration.

Prognosis[edit | edit source]

Schamberg's Disease is a benign condition and does not lead to serious health problems. However, it can be chronic and persistent, with lesions lasting for months to years. The condition may improve spontaneously over time.

Also see[edit | edit source]


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Contributors: Prab R. Tumpati, MD