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Inflammatory familial palmoplantar keratoderma: Greither's disease?
M Beylot-Barry1, A Taïeb, J E Surlève-Bazeille
1Service de Dermatologie, Hôpital des Enfants, Bordeaux, France.
Dermatology (Basel, Switzerland)
|January 1, 1992
Summary
This study reports a rare case of familial palmoplantar inflammatory keratoderma, a condition inherited in an autosomal dominant pattern. Findings suggest Greither's disease, characterized by specific ultrastructural changes in the skin's stratum corneum.
Area of Science:
- Dermatology
- Genetics
- Cell Biology
Background:
- Familial palmoplantar inflammatory keratoderma is a rare inherited skin condition.
- Greither's disease is a specific diagnosis associated with this keratoderma, characterized by vasomotor issues and hyperhidrosis.
- Understanding the genetic and cellular basis of such conditions is crucial for diagnosis and management.
Observation:
- A case presented with palmoplantar inflammatory keratoderma and autosomal dominant inheritance.
- Clinical features included vasomotor disturbances and hyperhidrosis, aligning with Greither's disease.
- Light microscopy of skin samples was non-specific.
Findings:
- Electron microscopy revealed aggregated tonofilaments around the nucleus, but no true clumps.
- An increased number of desmosomes were observed.
- Cell-cell junctions exhibited an imbricated pattern, particularly evident in the stratum corneum.
Implications:
- The findings contribute to the understanding of Greither's disease pathophysiology.
- This case highlights the importance of electron microscopy in diagnosing rare dermatological conditions.
- Further research into the genetic mutations underlying this keratoderma may reveal therapeutic targets.