Related Experiment Videos
[An case of acute diffuse seleroderma in an infant]
Insights
This case study details a rare infant diffuse scleroderma with unusual features like eosinophilia and lack of visceral lesions, questioning the diagnosis and considering progeria. The infant
Area of Science:
- Pediatric Dermatology
- Rare Diseases
- Infant Pathology
Background:
- Diffuse scleroderma is a rare autoimmune condition affecting connective tissue.
- Infantile-onset scleroderma is exceptionally uncommon, with few documented cases.
- Typical presentations involve skin thickening and potential internal organ involvement.
Abstract:
The authors report a case of diffuse scleroderma in a 15 months old infant. Dermatologic (clinical and pathological) findings are quite typical of the disease. On the other hand, in this case some particularities were observed: the age of the infant (second published case beginning before the age of two); the presence of a durable eosinophilia, the absence of visceral lesions and of biological abnormaliteis (of auto-immune nature specially), the evolution towards athrepsica and death within one year. Thus, because of these particularities, the diagnosis of scleroderma remains questionable and the diagnosis of progeria has been considered. The affection appeared in the course of a hepatitis leaving a hepatic fibrosis without inflammatory signs; no conclusion can be drawn about the relations between the hepatic affection and the fatal dermatologic disease.