Schöpf–Schulz–Passarge syndrome explained

Schöpf–Schulz–Passarge syndrome
Synonyms:	Eyelid cysts, Palmoplantar keratoderma, Hypodontia, and Hypotrichosis

Schöpf–Schulz–Passarge syndrome is an autosomal recessive condition with punctate symmetric palmoplantar keratoderma, with the keratoderma and fragility of the nails beginning around age 12.^{[1] [2]} In addition to palmoplantar keratoderma, other symptoms include hypodontia, hypotrichosis, nail dystrophies, and eyelid cysts (apocrine hidrocystomas). Patients may also develop syringofibroadenoma and squamous cell carcinomas.^[3]

It was characterized in 1971.^[4]

It has been associated with WNT10A.^[5]

See also

• Palmoplantar keratoderma
• List of cutaneous conditions
• List of dental abnormalities associated with cutaneous conditions
• List of cutaneous neoplasms associated with systemic syndromes

Notes and References

1. Freedberg, et al. (2003). Fitzpatrick's Dermatology in General Medicine. (6th ed.). McGraw-Hill. .
2. Book: Rapini, Ronald P. . Bolognia, Jean L. . Jorizzo, Joseph L. . Dermatology: 2-Volume Set . Mosby . St. Louis . 2007 . 778 . 978-1-4160-2999-1 .
3. Book: Calonje, Eduardo. Mckee's Pathology of the Skin: With Clinical Correlations. Elsevier/Saunders. 2012.
4. Schöpf E, Schulz HJ, Passarge E . Syndrome of cystic eyelids, palmo-plantar keratosis, hypodontia and hypotrichosis as a possible autosomal recessive trait . Birth Defects Orig. Artic. Ser. . 7 . 8 . 219–21 . June 1971 . 4281327 .
5. Bohring A, Stamm T, Spaich C, etal . WNT10A mutations are a frequent cause of a broad spectrum of ectodermal dysplasias with sex-biased manifestation pattern in heterozygotes . Am. J. Hum. Genet. . 85 . 1 . 97–105 . July 2009 . 19559398 . 2706962 . 10.1016/j.ajhg.2009.06.001 .