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[Schopf-Schulz-Passarge syndrome: 2 cases].

T Gkolfinopoulos1, S Ingen-Housz-Oro, B Cavelier-Balloy

  • 1Service de Dermatologie I, Hôpital Saint-Louis, 1, avenue Claude Vellefaux, 75475 Paris.

Annales De Dermatologie Et De Venereologie
|March 23, 2002
PubMed
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Schöpf-Schulz-Passarge syndrome is a rare genetic skin disorder characterized by nail defects, palmoplantar keratoderma, and hypodontia. This report details two new cases, highlighting the importance of recognizing this genodermatosis.

Area of Science:

  • Dermatology
  • Genetics
  • Rare Diseases

Background:

  • Schöpf-Schulz-Passarge syndrome is a rare autosomal recessive genodermatosis.
  • It is characterized by hypodontia, palmoplantar keratoderma, eyelid margin cysts, onychodysplasia, and hypotrichosis.

Observation:

  • Two new cases of Schöpf-Schulz-Passarge syndrome are presented.
  • Case 1: A 49-year-old woman with erosive palmoplantar keratoderma, fragile nails, hypodontia, eyelid cysts, and hypotrichosis.
  • Case 2: A 56-year-old man with scaly palmoplantar keratoderma, hypodontia, hair loss, hypoplastic nails, and eyelid cysts. Biopsy confirmed follicular cyst and sweat duct dystrophy.

Findings:

  • Diagnosis of Schöpf-Schulz-Passarge syndrome was confirmed in both patients.
  • No associated cutaneous tumors were observed in these cases.

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  • Differential diagnosis includes other genodermatoses with palmoplantar keratoderma and dental abnormalities.

    • Implications:

    • Early recognition of Schöpf-Schulz-Passarge syndrome is crucial for management.
    • Associated tumors (e.g., eccrine poromas, carcinomas) may develop later in life, necessitating regular follow-up.
    • Biopsy of suspect lesions is recommended for timely diagnosis and management of potential malignancies.