Related Research Articles
Palmoplantar keratodermas are a heterogeneous group of disorders characterized by abnormal thickening of the stratum corneum of the palms and soles.
Sclerodactyly is a localized thickening and tightness of the skin of the fingers or toes that yields a characteristic claw-like appearance and spindle shape of the affected digits, and renders them immobile or of limited mobility. The thickened, discolored patches of skin are called morphea, and may involve connective tissue below the skin, as well as muscle and other tissues. Sclerodactyly is often preceded by months or even years by Raynaud's phenomenon when it is part of systemic scleroderma.
Sézary disease, or Sézary syndrome, is a type of cutaneous T-cell lymphoma that was first described by Albert Sézary. The affected T cells, known as Sézary's cells or Lutzner cells, have pathological quantities of mucopolysaccharides. Sézary disease is sometimes considered a late stage of mycosis fungoides with lymphadenopathy.
Keratoderma is a hornlike skin condition.
Papillon–Lefèvre syndrome (PLS), also known as palmoplantar keratoderma with periodontitis, is an autosomal recessive genetic disorder caused by a deficiency in cathepsin C.
Acrokeratoelastoidosis of Costa or Acrokeratoelastoidosis is a hereditary form of marginal keratoderma, and can be defined as a palmoplantar keratoderma. It is distinguished by tiny, firm pearly or warty papules on the sides of the hands and, occasionally, the feet. It is less common than the hereditary type of marginal keratoderma, keratoelastoidosis marginalis.
Camisa disease is the variant form of Vohwinkel syndrome, characterized by ichthyosis and normal hearing.
Clouston's hidrotic ectodermal dysplasia is a medical condition caused by mutations in a connexin gene, GJB6 or connexin-30, characterized by scalp hair that is wiry, brittle, and pale. It is often associated with patchy alopecia.
Naxos disease is a cutaneous condition characterized by a palmoplantar keratoderma. The prevalence of the syndrome is up to 1 in every 1000 people in the Greek islands.
Corneodermatosseous syndrome is an autosomal dominant condition with onset in infancy, characterized by corneal dystrophy, photophobia, diffuse palmoplantar keratoderma, distal onycholysis, skeletal abnormalities, with brachydactyly, short stature, and medullary narrowing of digits.
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. In addition to palmoplantar keratoderma, other symptoms include hypodontia, hypotrichosis, nail dystrophies, and eyelid cysts. Patients may also develop syringofibroadenoma and squamous cell carcinomas.
Keratoderma climactericum is a skin condition characterized by hyperkeratosis of the palms and soles beginning at about the time of menopause.
Howel–Evans syndrome is an extremely rare condition involving thickening of the skin in the palms of the hands and the soles of the feet (hyperkeratosis). This familial disease is associated with a high lifetime risk of esophageal cancer. For this reason, it is sometimes known as tylosis with oesophageal cancer (TOC).
Woolly hair nevus is a congenital condition in which hair in a circumscribed area of the scalp is kinked or woolly.
Bart–Pumphrey syndrome, also known as palmoplantar keratoderma with knuckle pads and leukonychia and deafness) is a cutaneous condition characterized by hyperkeratoses over the metacarpophalangeal, proximal and distal interphalangeal joints. It was characterized in 1967. It can be associated with GJB2.
Cerebral dysgenesis–neuropathy–ichthyosis–keratoderma syndrome is a neurocutaneous condition caused by mutation in the SNAP29 gene.
Haim–Munk syndrome is a skin disease caused, like Papillon–Lefèvre syndrome, by a mutation in the cathepsin C gene. One of its features is thick curved finger and toenails.
Keratosis linearis with ichthyosis congenita and sclerosing keratoderma syndrome is a rare cutaneous condition characterized by ichthyosis and keratoderma.
References
- 1 2 Rapini, Ronald P.; Bolognia, Jean L.; Jorizzo, Joseph L. (2007). Dermatology: 2-Volume Set. St. Louis: Mosby. ISBN 978-1-4160-2999-1.