Description of the disease * EnglishPolish Pobierz sekcję do PDF 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 *Soruce Extended description of the disease Pobierz sekcję do PDF No additional description. Orphanet - interntowa baza danych dotyczących rzadkich chorób i sierochych leków. ©INSERM 1999 - Dostępna na stronie www.orphanet.pl