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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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[Progressive pigmented purpuric dermatosis in young children]
A-L Hérissé1, C Chiaverini1, F Boralevi2
1Service de dermatologie, CHU l'Archet 2, 151, route Saint-Antoine-de-Ginestière, 06200 Nice, France; Hôpital pédiatrique, CHU Lenval, 57, avenue de la Californie, 06200 Nice, France.
Annales De Dermatologie Et De Venereologie
|May 20, 2014
Summary
Schamberg's disease, a rare condition causing purpura, can affect young children. This study highlights five pediatric cases, suggesting the diagnosis is not exceptional despite its rarity in this age group.
Area of Science:
- Pediatric Dermatology
- Vascular Disorders
- Capillaritis
Background:
- Pigmented purpuric dermatosis, also known as Schamberg's disease, is a chronic condition characterized by macular purpura and capillaritis.
- Typically affecting young adult males and adolescents, it commonly presents on the lower limbs.
Observation:
- This report details five pediatric cases of generalized Schamberg's disease, with patients ranging from 13 months to 5 years old.
- All presented with asymptomatic, widespread macular purpura. Skin biopsies in four cases confirmed characteristic findings.
- Coagulation tests and complete blood counts were normal in all patients, ruling out other bleeding disorders.
Findings:
- The clinical course was chronic, marked by intermittent periods of improvement and worsening.
- No specific treatment was administered to the patients.
- Diagnosis can be made based on clinical presentation, patient history, and normal hematological parameters.
Implications:
- Schamberg's disease is less common in children but should be considered in the differential diagnosis of purpuric rashes.
- The condition typically follows a chronic, self-limiting course without intervention.
- Further research may explore potential therapeutic options for persistent cases.
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