Pigmented Purpuric Dermatosis

Updated: Aug 27, 2024
  • Author: Darius Mehregan, MD; Chief Editor: Dirk M Elston, MD  more...
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Overview

Practice Essentials

Pigmented purpuric dermatoses are a group of chronic cutaneous diseases of mostly unknown etiology, characterized by multiple petechial hemorrhage due to capillaritis. The presentation includes red to purple macules that evolve to golden-brown color with reabsorbtion of the hemosiderin. Lesions usually occur bilaterally on the lower limbs but may also involve the thighs, buttocks, trunk, and arms.  Lesions are frequently asymptomatic although they may be mildly pruritic. [1]

A number of clinical patterns of pigmented purpuric dermatoses or capillaritis are recognized that may represent different presentations of the same disorder; however, this generally does not influence the treatment or the prognosis. They all show a similar histologic appearance. Based on the clinical appearance, pigmented purpuric dermatoses are classified as follows [1]

  • Progressive pigmentary dermatosis or Schamberg disease
  • Pigmented purpuric lichenoid dermatosis of Gougerot and Blum
  • Purpura annularis telangiectodes of Majocchi
  • Eczematid-like purpura of Doucas and Kapetanakis
  • Lichen aureus
  • Unilateral linear pigmented purpura

Schamberg disease is the most common subtype with a symptomatic varient, itching purpura of Loewenthal, characterized by abrupt onset and severe itching. Ezematid-like purpura is also considered to be a symptomatic variant of Schamberg disease. [1]

Granulomatous pigmented purpuric dermatosis (GPPD) is a rare subtype. It is distinguished from other subtypes by inflammatory infiltrate that can extend to the mid or deep dermis and the admixed lymphocytic infiltrate is mainly composed of CD8+ T-cells. [2]

 

Pathophysiology

The etiology is unknown. Several cofactors have been reported that appear to influence disease presentation, including hypertension, diabetes mellitus, venous stasis, strenuous exercise, gravitational dependency, capillary fragility, focal infections, and chemical ingestion. [3]   Alcohol use may be related to the development of Schamberg disease, especially is cases resulting in liver disease. [1]

Histologically, a perivascular T-cell lymphocytic infiltrate is centered on the superficial small blood vessels of the skin, which show signs of endothelial cell swelling and narrowing of the lumen. Extravasation of red blood cells with marked hemosiderin deposition in macrophages is also found, and a rare granulomatous variant of chronic pigmented dermatosis has been reported. [4]

Early onset disease may sometimes be associated with platelet-storage defects. [5]

Etiology

The cause of pigmented purpuric dermatoses is unknown. Rare familial cases of Schamberg disease and Majocchi disease have been reported in the literature, implying a genetic cause in a minority of patients.

Epidemiology

Frequency

United States

Pigmented purpuric dermatoses are common.

International

During a 10-month period, the author's United Kingdom hospital-based dermatology practice, which serves a population of 300,000 persons, identified only 10 such cases. Five cases were diagnosed as having lichen aureus, and the remainder had more extensive capillaritis.

Race

Persons of any race can be affected by pigmented purpuric dermatoses.

Sex

Pigmented purpuric dermatoses usually occur more frequently in men than in women. However, purpura annularis telangiectodes of Majocchi is seen more frequently in women.

Age

Schamberg disease may occur in persons of any age.

Itching purpura and the dermatosis of Gougerot and Blum mainly affect middle-aged men.

Lichen aureus and Majocchi disease are predominantly diseases of children or young adults.

Prognosis

Many lesions persist or extend with time. Most eventually resolve spontaneously. Typically, the condition is asymptomatic, but pruritus may sometimes be a prominent feature in some cases, especially in patients with itching purpura or eczematidlike purpura of Doucas and Kapetanakis. These diseases have no systemic findings.

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