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Keratosis palmoplantaris with cystic eyelids, hypodontia, and hypotrichosis. Eccrine tumors with ectodermal dysplasia are part of this medical condition.

Keratosis Palmoplantaris with Cystic Eyelids, Hypodontia and Hypotrichosis; Eccrine Tumors with Ectodermal Dysplasia Syndrome.

Autosomal recessive inheritance

A comprehensive classification of the primary palmoplantar keratodermas was suggested by Stevens et al. in 1996. The term “palmoplantar ectodermal dysplasia” was used where more than a single ectodermal structure was involved to emphasize the generalized nature of the disorder. They further identified a total of 19 subtypes and the Schopf-Schultz-Passarge syndrome was determined to be Type XXIX. Cause unknown. Chromosomal study normal.

Clinical features characterized by a disorder of hair, nail, teeth, and sweat glands in various combinations. The eyelid cysts are considered expansion of the glands of Moll.

Hypotrichosis: fine, sparse head hair; sparse eyelash and eyebrows. Hypodontia: early loss of decidual teeth; few permanent teeth. Eyes: epidermal cysts of eyelids are characteristic. Skin changes: plantar and palmar keratosis; nail dystrophy. Exocrine gland tumor may occur on hands and feet. Affected individuals have normal lifespan and function.

There are no specific anesthesia considerations associated with this condition. Any considerations will be related to the surgical procedure only.

Papillon-Lefèvre Syndrome: An extremely rare genetic disorder that typically becomes apparent during the first 5 years of life. It is characterized by palmar-plantar hyperkeratosis in association with periodontium. The deciduous teeth are lost by age 5 years. Without treatment, most of the permanent teeth may also be lost by approximately age 17 years. Other clinical features include frequent pyogenic skin infections, nail dystrophy, and hyperhidrosis. It is transmitted as an autosomal recessive trait.

Haim Munk Syndrome: Characterized by palmoplantar hyperkeratosis and onychogryphosis. Pes planus, arachnodactyly, and/or acroosteolysis have been reported. Periodontosis may also be present. It is inherited as an autosomal recessive trait. Some researchers believe the disorder may be a variant of Papillon-Lefevre Syndrome.

Meleda Disease (Mal de Meleda): An extremely rare disorder characterized by the slow progressive development of palmoplantar hyperkeratosis. Affected skin may be unusually red and become abnormally thick. Affected children may exhibit abnormalities of the nails, hyperhidrosis with unpleasant odor, and/or lichenoid plaques. In addition, cardiac abnormalities and cardiomegaly have been reported. It is inherited as an autosomal recessive trait.

Sjögren Larsson Syndrome (SLS): A rare inherited disorder characterized by ichthyosis, palmoplantar hyperkeratosis, and ecchymosis. Affected infants may show mental retardation, spastic paraplegia, seizures, and eye abnormalities. It is inherited as an autosomal recessive trait.

Fitzsimmons Syndrome: An extremely rare inherited disorder characterized by slow progressive development of palmoplantar keratosis, mental retardation, and spastic paraplegia. Children may show an abnormally high pes cavus. It is thought to be inherited as an X-linked genetic transmission.

Craifen W, Levy M, Lewis R: Schopf-Schulz-Passarge syndrome with an unusual pattern of inheritance. ...

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