Syn .: Papulosis maligna atrophicans, Köhlmeier-Degos syndrome, Degos syndrome, thrombangitis cutaneointestinalis disseminata, lethal enterocutaneous syndrome.
Def .: Idiopathic vascular occlusive disease accompanied by papules transforming into white scars, often with lesions of the gastrointestinal vessels, CNS and other organs.
Epid .: Extremely rare disease, most often affects young and middle-aged people, especially women.
Etiol .: It is unknown. Vasculitis or a hypercoagulable state was suspected as the cause, possibly related to the antiphospholipid syndrome. Recently, cases of this disease have been described in the course of HIV / AIDS.
Pat: The basis is numerous infarctions in the skin and internal organs associated with inflammation and thrombosis of small and medium-sized arteries.
Loc .: Torso, belly.
Clinical: The skin has asymptomatic, diffuse, pale pink papules, 3-5 mm in size, which transform into white porcelain scars after a few days. Some of the papules are necrotic with ulceration, followed by atrophic scars surrounded by an erythematous rim. General symptoms are related to infarcts in the small intestine, which can lead to death due to its perforation or peritonitis. Neurological symptoms depend on the location and size of the ischemic necrosis foci. Some patients do not have any organ changes (cutaneous form of the disease).
DL: A skin biopsy reveals an inverted cone-shaped ischemic necrosis that heals by fibrosis and mucin deposition. The features of lymphocytic vasculitis occur early in the lesion.
DI: Direct immunofluorescence testing is negative.
DR: Systemic lupus erythematosus, pityriasis lichenoides et varioliformis (PLEVA).
Healing: Anticoagulants, corticosteroids, surgical interventions are ineffective.
Year: Good when skin only changes; in the case of organ lesions it is unsuccessful
Lit .: [1] Wolnik-Trzeciak G., Trzeciak P., Głośnicka M. et al. Systemic fatal systemic degos disease – case report. Dermatol Klin 2007, 9; 108-11. [2] Vicktor C., Schultz-Ehrenburg U .: Papulosis maligna atrophicans (Köhlmeier-Degos). Hautarzt 2001, 52; 734.
Source: A. Kaszuba, Z. Adamski: “Lexicon of dermatology”; XNUMXst edition, Czelej Publishing House