Schöpf–Schulz–Passarge Syndrome
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Schöpf–Schulz–Passarge syndrome is an
autosomal recessive In genetics, dominance is the phenomenon of one variant (allele) of a gene on a chromosome masking or overriding the Phenotype, effect of a different variant of the same gene on Homologous chromosome, the other copy of the chromosome. The firs ...
condition with punctate symmetric
palmoplantar keratoderma Palmoplantar keratodermas are a heterogeneous group of skin disorders characterized by abnormal thickening (scleroderma) of the stratum corneum of the palms and soles. Autosomal recessive, dominant, X-linked, and acquired forms have all been de ...
, with the keratoderma and fragility of the nails beginning around age 12.Freedberg, et al. (2003). ''Fitzpatrick's Dermatology in General Medicine''. (6th ed.). McGraw-Hill. . In addition to palmoplantar keratoderma, other symptoms include
hypodontia Hypodontia is defined as the developmental absence of one or more teeth excluding the Wisdom tooth, third molars. It is one of the most common dental anomalies, and can have a negative impact on function, and also appearance. It rarely occurs i ...
,
hypotrichosis Hair loss, also known as alopecia or baldness, refers to a loss of hair from part of the head or body. Typically at least the head is involved. The severity of hair loss can vary from a small area to the entire body. Inflammation or scarring ...
, nail dystrophies, and eyelid cysts ( apocrine hidrocystomas). Patients may also develop
syringofibroadenoma Syringofibroadenoma is a cutaneous condition characterized by a hyperkeratotic nodule or plaque involving the extremities. It is considered of eccrine origin. See also * Syringadenoma papilliferum * Skin lesion A skin condition, also known ...
and
squamous cell carcinoma Squamous-cell carcinoma (SCC), also known as epidermoid carcinoma, comprises a number of different types of cancer that begin in squamous cells. These cells form on the surface of the skin, on the lining of hollow organs in the body, and on the ...
s. It was characterized in 1971. It has been associated with WNT10A.


See also

*
Palmoplantar keratoderma Palmoplantar keratodermas are a heterogeneous group of skin disorders characterized by abnormal thickening (scleroderma) of the stratum corneum of the palms and soles. Autosomal recessive, dominant, X-linked, and acquired forms have all been de ...
*
List of cutaneous conditions Many skin conditions affect the human integumentary system—the organ system covering the entire surface of the Human body, body and composed of Human skin, skin, hair, Nail (anatomy), nails, and related muscle and glands. The major function o ...
* List of dental abnormalities associated with cutaneous conditions *
List of cutaneous neoplasms associated with systemic syndromes Many cutaneous neoplasms occur in the setting of systemic syndromes. See also *List of cutaneous conditions *List of contact allergens *List of cutaneous conditions associated with increased risk of nonmelanoma skin cancer *List of cutaneous ...


References


External links

Palmoplantar keratodermas Syndromes {{Cutaneous-condition-stub