Livedoid vasculopathy

Orpha code: 542643OMIM code:

Definition

A rare vascular skin disease characterized by recurrent focal non-inflammatory thrombosis of dermal venulae, predominantly of the lower extremities, resulting in a cutaneous response manifested as pruritus and painful papules and erythematous plaques. The lesions evolve into hemorrhagic vesicles or bullae, which rupture and turn into painful ulcers merging into reticulate, confluent, geometric, and painful ulcerations. During a period of a few months, the ulcerations change to porcelain-white atrophic scars with punctate telangiectasia (so-called atrophie blanche). In active disease, lesions in different stages coexist.

Disease data
Classification

Clinical syndrome

Synonyms
Livedo reticularis with summer ulcerations
Milian atrophie blanche
Segmental hyalinizing vasculitis
Livedo reticularis with summer ulcerations
Milian atrophie blanche
Segmental hyalinizing vasculitis
ORPHA code
542643
OMIM code
-
ICD10 code
L95.0
ICD11 code
EF50

No additional description.

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