Pigmented purpuric dermatosis

Alternate names

Schamberg disease; Familial pigmented purpuric eruption; Schamberg purpura; Pigmented purpura; Progressive pigmented purpura; Pigmented purpuric eruption

Definition

Pigmented purpuric dermatosis is a chronic condition characterized by reddish-brown skin lesions caused by leaky capillaries.

Epidemiology

• Pigmented purpuric dermatoses are rare.
• There is not a racial predilection; although, they appear slightly more often in males.
• Children can also be affected.[1].

Cause

• The cause of pigmented purpuric dermatosis is unknown.
• Occasionally, it occurs as a reaction to a medication, food additive, viral infection or following exercise.
• In rare cases, there appears to be a genetic component.

Signs and symptoms

• The main features of pigmented purpuric dermatosis (PPD) are petechiae (tiny red spots due to broken blood vessels) or purpura (purple-colored spots or patches due to broken blood vessels), and yellow to brown pigmented patches.
• The most common sites are the legs, although lesions also may develop in other areas.
• Infrequently, PPD manifests as a generalized rash.
• The palms of the hands, soles of the feet, genitalia, and mucosa typically are not affected.
• Depending on the type of PPD a person has, additional signs and symptoms may include red-brown patches, plaques, linear lesions, itching, and/or other features.
• In general, PPD does not cause serious health problems but is a chronic condition.
• Signs and symptoms may persist, wax and wane, or slowly progress, and may go away over a period of months to years.

Diagnosis

• Pigmented purpuric dermatoses are largely diagnosed based on the clinical presentation.
• However, with atypical presentations or to rule out cutaneous vasculitis, dermatitis, or cutaneous t-cell lymphoma, skin biopsy by punch technique can be helpful. CBC and coagulation studies can be performed to rule out thrombocytopenia or clotting disorders.
• Medication history should also be obtained to screen for drug hypersensitivity as a cause.
• To rule out concomitant allergic contact dermatitis, patch testing can be performed.[2].

Treatment

• If the condition is mild, treatment may not be necessary.
• For those who suffer from itching, topical steroids and antihistamines may be useful.
• Compression stockings may be used if the lower legs are affected.
• Additional treatments that have been successful in limited studies include pentoxifylline, aminaphtone, photochemotherapy (PUVA), griseofulvin, oral cyclosporin, and ascorbic acid with rutoside.

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NIH genetic and rare disease info

• NIH info on Pigmented purpuric dermatosis

Pigmented purpuric dermatosis is a rare disease.

Pigmented purpuric dermatosis Resources
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