Skin-nontumor / Clinical Dermatology - Malignant atrophic papulosis

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Other dermatoses

Malignant atrophic papulosis

Reviewer: Mowafak Hamodat, MB.CH.B, MSc., FRCPC, Eastern Health, St. Johns, Canada (see Reviewers page)
Revised: 1 August 2011, last major update August 2011
Copyright: (c) 2002-2011, PathologyOutlines.com, Inc.

Definition
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● Intimal proliferation of deep-seated arterioles which causes infarcts
● Dego's disease: variant causing a lethal syndrome, involving small bowel, heart, lungs, kidney, bladder and liver
● Also benign and familiar variants

Clinical description
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● Discrete small patches composed of a central zone with a depressed white, porcelain like appearance and a fine scale surrounded by a narrow red or violaceous rim associated with a fine telangiectasia
● Skin lesions affect the trunk and proximal extremity

Clinical images
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Various images

Micro description
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● Epidermis is hyperkeratotic and atrophic
● Wedge shaped dermal infarct with the base parallel to the surface epithelium
● Pale in color, relatively acellular
● Mucin deposition common
● Older lesions are frequently ulcerated
● Vessels adjacent to and deep to the infarct show a perivascular lymphocytic infiltrate
● Endovasculitis in dermal vessels with endothelial cell hyperplasia and complicated by thrombosis
● Panniculitis mimicking lupus erythematosus profundus has been described
● Microscopic examination of bowel reveals transmural intestinal inflammation with ulceration and hemorrhage

Differential diagnosis
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● SLE, systemic sclerosis, rheumatoid arthritis, dermatomyositis, Crohn’s disease

Additional references
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● eMedicine

End of Skin-nontumor / Clinical Dermatology > Other dermatoses > Malignant atrophic papulosis

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