Return to course: Palmoplantar Keratodermas
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Odonto-onycho-dermal dysplasia (Schöpf– Schulz–Passarge Syndrome)
Epidemiology
Wingless integrated family 10A gene mutation
Autosomal recessive inheritance
Pathogenesis
Impaired ectodermal “appendage” development
Clinical Features
Diffuse palmoplantar keratoderma, facial telangiectasia / reticulate erythema, dental defects, nail dystrophy, hypotrichosis
Schöpf– Schulz–Passarge syndrome
Eyelid hidrocystomas, adnexal tumours
Diagnosis & Differential
Pathology:
Eccrine syringofibroadenomas
Treatment
Unknown