Degos' disease with delayed involvement of the gastrointestinal tract.
A 75-year-old woman presented with pink papules over the trunk and limbs. Over the next few weeks, the papules became umbilicated and then developed into porcelain-white lesions with a rose or violaceous rim. A biopsy of one of these lesions revealed superficial and deep dermal lymphohistiocytic inflammation, mainly perivascular with a central zone of infarction consistent with Degos' disease. Three years later, she presented with increasing abdominal pain, anaemia, raised inflammatory markers and an abdominal bruit. Angiography showed splanchnic vessel stenosis possibly secondary to vasculitis and she underwent successful balloon dilatation. Treatment with prednisolone was commenced. There has been no recurrence of lesions in the 12 months since the balloon dilatation.
Subramaniam
Kavitha
K
Cutaneous Pathology, Nedlands, Western Australia, Australia.
Debinski
Henry
H
Heenan
Peter
P
D000368:Aged; D002404:Catheterization; D012809:Colon, Sigmoid / Q000098:blood supply / Q000473:pathology*; D003251:Constriction, Pathologic; D016099:Endoscopy, Gastrointestinal; D005260:Female; D006801:Humans; D054853:Malignant Atrophic Papulosis / Q000150:complications / Q000175:diagnosis*; D012810:Sigmoid Diseases / Q000209:etiology* / Q000628:therapy; D012867:Skin / Q000473:pathology*; D013152:Splanchnic Circulation; D013276:Stomach Ulcer / Q000209:etiology* / Q000628:therapy