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Familial acral localized late-onset focal dermal elastosis
Diana Camacho1, Salma Machan, Ursula Pielasinski
1Department of Dermatology, Fundación Jiménez Díaz, Universidad Autónoma, Madrid, Spain.
The American Journal of Dermatopathology
|April 7, 2012
Summary
Late-onset focal dermal elastosis, a rare elastic tissue disorder, typically affects the elderly. This report details two sisters with early-onset, familial lesions on their hands, challenging typical presentations.
Area of Science:
- Dermatology
- Genetics
- Histopathology
Background:
- Late-onset focal dermal elastosis (LOFDE) is a rare disorder affecting elastic tissue.
- Characterized by localized elastic fiber accumulation in the dermis, it often mimics pseudoxanthoma elasticum.
- Typically presents in the elderly with yellow papules on the neck and flexural areas.
Observation:
- Two sisters presented with a 20-year history of multiple yellow papules.
- Lesions were predominantly on the dorsum of the hands, with scattered involvement of wrists, thighs, and abdomen.
- This presentation showed an unusually early age of onset and familial clustering.
Findings:
- Histopathologic examination revealed a focal increase in thick, interlacing elastic fibers in the mid and deep reticular dermis.
- The cases highlighted early clinical presentation, familial occurrence, and acral distribution.
- These features distinguish the reported cases from typical LOFDE.
Implications:
- This case series expands the clinical spectrum of LOFDE.
- Highlights the importance of considering familial and early-onset presentations.
- Suggests potential genetic factors influencing elastic tissue disorders.
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