EF40.0

ICD-11 MMS 2026-01

Capillaritis

Description

Capillaritis results from extravasation of red blood cells from leaky capillaries into the dermis and manifests initially as a finely stippled pink to purple purpura, most commonly affecting the lower limbs. As iron is released and converted into haemosiderin, the skin stains gold or brown. Various patterns of capillaritis have been described and given separate names depending on the distribution, time course, extent and degree of pigmentation and presence or otherwise of epidermal thickening. The underlying processes involved in all these variants are very similar and of unknown cause. Histology may show mild inflammation around capillaries but no vasculitis.

Index terms

  • Capillaritis
  • Pigmented purpura
  • Purpura simplex
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  • Schamberg capillaritis
  • Schamberg disease
  • pigmented purpuric dermatosis
  • progressive pigmentary dermatosis
  • progressive pigmented purpura
  • progressive pigmented purpura of Schamberg
  • Majocchi capillaritis
  • Majocchi disease
  • Majocchi pigmented purpuric dermatosis
  • Majocchi purpura
  • Purpura annularis telangiectodes of Majocchi
  • Lichenoid capillaritis
  • Gougerot-Blum trisymptome
  • Lichenoid purpuric dermatitis
  • Pigmented purpuric lichenoid dermatitis of Gougerot and Blum
  • Itching purpura
  • Eczematid-like purpura
  • Lichen aureus
  • Drug-induced capillaritis
  • Pigmented purpura due to drug
  • medicament-induced capillaritis

Inclusions

  • Pigmented purpura

Excludes

  • CB04.4 – Pulmonary capillaritis

Sibling codes

  • EF40.1 – Vasculitis affecting small cutaneous blood vessels
  • EF40.2 – Localised cutaneous vasculitis
  • EF40.Z – Cutaneous vasculitis unspecified

Foundation entity

Capillaritis – http://id.who.int/icd/entity/280360139

Allowed postcoordination axes

Part of: EF40: Vasculitis or capillaritis involving the skin › 14: Diseases of the skin