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Updated: Jun 24, 2026

Recognition of Epidermal Transglutaminase by IgA and Tissue Transglutaminase 2 Antibodies in a Rare Case of Rhesus Dermatitis

Recognition of Epidermal Transglutaminase by IgA and Tissue Transglutaminase 2 Antibodies in a Rare Case of Rhesus Dermatitis

Published on: December 15, 2011

Degos' disease: a rare condition simulating rheumatic diseases.

Ho Yin Chung¹, Nigel J Trendell-Smith, Chi Keung Yeung

¹Department of Medicine, Queen Mary Hospital, The University of Hong Kong, Pok Fu Lam, Hong Kong, China.

Clinical Rheumatology

|March 21, 2009

Summary

Dego's disease, a rare vasculopathy, can mimic rheumatic conditions with skin lesions and organ damage. Early diagnosis is crucial, but even with treatment, complications like bowel infarction can be fatal.

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Recognition of Epidermal Transglutaminase by IgA and Tissue Transglutaminase 2 Antibodies in a Rare Case of Rhesus Dermatitis

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Published on: December 15, 2011

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Area of Science:

Vascular Medicine
Dermatology
Rheumatology

Background:

Dego's disease is a rare thrombo-occlusive vasculopathy.
It presents with skin manifestations and thrombotic complications.
It can mimic rheumatic diseases, often confusing rheumatologists.

Observation:

A middle-aged woman presented with acute bowel infarction, fever, and elevated inflammatory markers.
Clinical suspicion was systemic vasculitis.
Classical skin lesions (papules with central depression and violaceous rim) led to the diagnosis of Dego's disease.

Findings:

Histology revealed wedge-shaped infarction with endothelial proliferation and thrombus-related occlusion.
The patient received aspirin treatment.
Recurrent bowel infarction occurred 1.5 years later.

Implications:

Dego's disease requires careful diagnosis, distinguishing it from rheumatic diseases.
Effective long-term treatment strategies are needed.
The condition carries a significant risk of recurrent thrombosis and mortality.