Pigmented Purpura

Pigmented Purpura

 

SYNONYMS

  1. Capillaritis
  2. Purpura pigmentosa chronica
  3. Majocchi-Schamberg disease

VARIANTS:

  1. Progressive pigmentary dermatosis of Schamberg; Schamberg’s disease
  2. Purpura annularis telangiectodes of Majocchi; Majocchi’s disease
  3. Pigmented purpuric lichenoid dermatitis of Gougerot and Blum
  4. Eczematid like purpura of Doucas and Kapetanakis
  5. Lichen aureus

KEY FEATURES

  1. Clustered petechial hemorrhage
  2. Often a background of yellow brown discoloration due to hemosiderin deposition
  3. Location and pattern depends on particular variant

Chronic pigmented purpuras represent a group of diseases characterized by petechial hemorrhage thought to be secondary to capillaritis.  These diseases have no systemic findings, but they occasionally lead to a work up for thrombocytopenia or vasculitis because of the purpuric (usually petechial) nature of the lesions and clinical misdiagnosis. 

PATHOGENESIS

These diseases all represent minimal inflammation and hemorrhage of superficial papillary dermal vessels, usually the capillaries.  The reason for this inflammation is unknown.

CLINICAL FEATURES

The most common syndrome is Schamberg’s disease, seen from childhood on most typically seen on the lower legs in older men.  Lesions consist of oval to irregular patches that are yellow brown in color with superimposed pinpoint cayenne pepper macules. Over time, the lesions may fade or become darker brown in color.

Purpura annularis telangiectodes (Majocchi’s disease) is seen most often in adolescents or young adults, especially women.  Plaques are usually 1-3 cm in diameter and are annular, with telangiectasias and cayenne pepper petechiae in the border.  Usually asymptomatic, the lesions can persist for years and may slowly extend.

Pigmented purpuric dermatosis of Gougerot and Blum is rare, typically affecting men between 40 and 60 years of age.  Lesions similar to those of Schamberg’s purpura are usually seen, but in addition lichenoid papules are seen.  This is chronic but usually symptomatic disease.

Lichen aureus is rare, and usually consists of a solitary lesion on the lower extremity, though a zosteriform pattern has been reported.  Chronic rust to purple colored, occasionally golden, patches are seen and are symptomatic.  In some patients, the lesion overlies a perforator vein.

PATHOLOGY

These diseases share a histologic picture of red cell extravasion, endothelial cell swelling, a perivascular lymphocytic infiltrate and hemosiderin containing macrophages.  

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