[Malignant atrophic papulosis (Köhlmeier-Degos): diagnosis, therapy and course].

[malignant atrophic papulosis]

A 71 -year -old male patient presented with malignant atrophic papulosis (Köhlmeier-Degos disease ). He developed multiple distinctive cutaneous lesions . The histopathological findings showed an obliterated arteriole with a wedge-shaped area of ulcerated and necrobiotic dermis . Laboratory tests were within normal limits or not specific . Although the involvement of the gastrointestinal tract and other organs has been noted in approximately 60 % of the reported cases , in this patient so far the skin seems to be the only involved site . There is no proven effective therapy for Köhlmeier-Degos disease . In our case treatment with pentoxifylline and aspirin led to healing of all skin lesions within 3 months . One year after beginning treatment , the patient showed neither new cutaneous lesions , nor any signs of systemic involvement . The combination of pentoxifylline and aspirin should be considered when planning treatment strategies for malignant atrophic papulosis .

Diseases
Validation

Diseases presenting "skin lesions" symptom

child syndrome

cowden syndrome

cutaneous mastocytosis

cystinuria

dystrophic epidermolysis bullosa

epidermolysis bullosa simplex

erdheim-chester disease

erythropoietic protoporphyria

familial mediterranean fever

focal myositis

gm1 gangliosidosis

heparin-induced thrombocytopenia

hirschsprung disease

junctional epidermolysis bullosa

lamellar ichthyosis

liposarcoma

lymphangioleiomyomatosis

malignant atrophic papulosis

oligodontia

omenn syndrome

papillon-lefèvre syndrome

primary effusion lymphoma

proteus syndrome

severe combined immunodeficiency

sneddon syndrome

waldenström macroglobulinemia

werner syndrome

wiskott-aldrich syndrome

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