Schöpf-Schulz-Passarge syndrome (SSPS; OMIM 224750) is an autosomal recessive inherited form of ectodermal dysplasia characterized by eyelid cysts (apocrine hidrocystomas), palmoplantar keratoderma, hypodontia, hyperhidrosis, hypotrichosis and onychodystrophy, as well as other, often variable, ectodermal developmental anomalies [ 1 Schöpf E. Schulz H.J. Passarge E. Syndrome of cystic eyelids, palmo-plantar keratosis, hypodontia and hypotrichosis as a possible autosomal recessive trait. Birth Defects Orig Art Ser. 1971; 7: 219-221 PubMed Google Scholar , 2 Castori M. Ruggieri S. Giamnett L. Annessi G. Zambruno G. Schöpf-Schultz-Passarge syndrome; further definition of the phenotype and genetic considerations. Acta Derm Venereol. 2008; 88: 607-612 PubMed Google Scholar ]. SSPS shows clinical overlap with odonto-onycho-dermal dysplasia (OODD; OMIM 277980), but the eyelid cysts are a typical feature of SSPS [ [1] Schöpf E. Schulz H.J. Passarge E. Syndrome of cystic eyelids, palmo-plantar keratosis, hypodontia and hypotrichosis as a possible autosomal recessive trait. Birth Defects Orig Art Ser. 1971; 7: 219-221 PubMed Google Scholar ].