Abstract

Pigmented purpuric dermatoses (PPD) include several skin diseases characterized by multiple petechial hemorrhage as consequence of capillaritis. PPD generally present with red to purple macules that progressively evolve to golden-brown color as the hemosiderin is reabsorbed. These lesions, often asymptomatic or associated with mild pruritus, usually occur on the lower extremities and may be a diagnostic and therapeutic challenge both for general practitioners and specialists in internal medicine or flebology. Clinical presentations include many subtypes that have been described over the years, although histology is usually superimposable. Prompt recognition and patient reassurance on the benign nature of these diseases is crucial. In this comprehensive review, we focused on pathogenesis and clinical pictures.

Highlights

  • Pigmented purpuric dermatoses (PPD) represent a group of cutaneous diseases characterized by petechial hemorrhage as a consequence of capillaritis [1]

  • Based on the clinical appearance, PPD are classified as five major entities: 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, and lichen aureus [3]

  • Pigmented purpuric eruptions on the legs must be differentiated from dermal hemorrhage secondary to venous hypertension, which presents with petechiae superimposed on diffuse hemosiderosis, especially in elderly people with stasis dermatitis

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Summary

Introduction

Pigmented purpuric dermatoses (PPD) represent a group of cutaneous diseases characterized by petechial hemorrhage as a consequence of capillaritis [1]. PPD usually present as remitting-relapsing non-palpable flat purpura with bilateral distribution on the legs of elderly. Adults are more frequently affected, there are numerous reports of childhood PPD in literature [2]. These conditions have no systemic findings, but occasionally lead to a patient evaluation to exclude thrombocytopenia or vasculitis because of the purpuric (usually petechial) nature of the lesions and clinical misdiagnosis. Based on the clinical appearance, PPD are classified as five major entities: progressive pigmentary dermatosis or Schamberg disease (the commonest), pigmented purpuric lichenoid dermatosis of Gougerot and Blum, purpura annularis telangiectodes of Majocchi, eczematid-like purpura of Doucas and Kapetanakis, and lichen aureus [3].

Aetiology and Pathogenesis
Pathology
Schamberg
Purpura Annularis Telangiectodes of Majocchi
Lichen
Lichen Aureus
Eczematid-Like
Other Forms
Differential Diagnosis
Diagnosis
Investigation
Therapy
Conclusions
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