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Cerebriform plantar hyperplasia: ultrastructural study of two cases
B C Winik1, M C Boente, R A Asial
1Department of Electron microscopy, INSIBIO, CONICET, Universidad Nacional de Tucumán, Tucumán, Argentina. lamenoa@unt.edu.ar
European Journal of Dermatology : EJD
|November 1, 2000
Summary
Histopathological analysis of cerebriform plantar hyperplasia in two mild Proteus syndrome patients revealed unique collagen and elastic fiber changes. These findings aid in better defining this condition and diagnosing Proteus syndrome early.
Area of Science:
- Dermatopathology
- Medical Genetics
Background:
- Proteus syndrome is a rare overgrowth disorder with variable clinical manifestations.
- Cerebriform plantar hyperplasia is a recognized, though not fully understood, feature of Proteus syndrome.
Observation:
- Two patients with mild Proteus syndrome presented with cerebriform plantar hyperplasia.
- Histopathological examination included light and ultrastructural microscopy.
Findings:
- Light microscopy showed increased dermal fibro-adipose tissue and adnexal structures.
- Ultrastructural analysis revealed densely packed collagen fibrils (composite and unraveled) and fragmented elastic fibrils with altered elastin/microfibril ratios.
Implications:
- These histopathological findings help delineate cerebriform plantar hyperplasia.
- The study provides potential clues for the early diagnosis of Proteus syndrome.