Schopf - Schulz - Passarge - Syndrome

Last Updated: 2019-08-26

Author(s): Anzengruber F., Navarini A.

ICD11: -

Schöpf et al. 1971

Keratosis palmoplantaris with cystic eyelids, hypodontia and hypotrichosis. 
Eccrine tumors with ectodermal dysplasia.  

Palmoplantar keratosis, which is caused by an autosomal recessive mutation in the WNT10A gene (2q35). In addition to cutaneous phenomena, hypodontics and hypotrichosis are also characteristic. Most patients also have eyelid cysts. (OMIM #224750).

Autosomal - recessive mutation of the WNT10A gene.

 

Palmoplantar keratosis.

Hypotrichy.

Hypodenttia.

Eyelid cysts.

Medical history

Clinic.

Genetic analysis.

Dental Consil.

Keratolysis.

Refattening Externa.

Dental and orthodontic checks.

  1. Manchanda N, Anthonappa R, Al-Mulla H, King N. Long-term dental management of a patient with features of Schöpf-Schulz-Passargesyndrome. Spec Care Dentist. 2017 Jul;37(4):204-208.
  2. Painsi C, Aubell K, Wolf P, Hügel R, Lange-Asschenfeldt B. A case of Schöpf-Schulz-Passarge syndrome caused by c.1135C>T WNT10A missense mutation. J Dtsch Dermatol Ges. 2017 Apr;15(4):455-457.