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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.
Kindler syndrome is a rare congenital disease of the skin caused by a mutation in the KIND1 gene.
Vorinostat (rINN), also known as suberoylanilide hydroxamic acid, is a member of a larger class of compounds that inhibit histone deacetylases (HDAC). Histone deacetylase inhibitors (HDI) have a broad spectrum of epigenetic activities.
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.
Trichilemmoma is a benign cutaneous neoplasm that shows differentiation toward cells of the outer root sheath. The lesion is often seen in the face and neck region. Multifocal occurrence is associated with Cowden syndrome, in which hamartomatous intestinal polyposis is seen in conjunction with multiple tricholemmoma lesions.
A trichodiscoma is a cutaneous condition, a benign, usually skin-colored tumor most often affecting the face and upper trunk.
SAHA syndrome, is a medical syndrome characterized by seborrhoea, acne, hirsutism and alopecia, and was first described in 1982. It is frequently associated with polycystic ovary syndrome, cystic mastitis, obesity, and infertility.
Wende–Bauckus syndrome is a cutaneous condition characterized by tiny white macules on the trunk with confluence within flexures.
Hystrix-like ichthyosis–deafness syndrome is a cutaneous condition characterized by a keratoderma.
Arthrogryposis–renal dysfunction–cholestasis syndrome is a cutaneous condition caused by a mutation in the VPS33B gene.
Neonatal ichthyosis–sclerosing cholangitis syndrome is a cutaneous condition which is characterized by hypotrichosis of the scalp, alopecia, ichthyosis and sclerosing cholangitis. Only 5 cases from 3 families worldwide have been described in medical literature. It caused by mutations in the Claudin 1 gene.
Laryngo-onycho-cutaneous syndrome is a rare epithelial disorder inherited in an autosomal recessive fashion. It is characterized by abnormalities in the larynx, nails, and skin ("cutaneous"). The disorder is only found in Punjabi Muslims and only a few cases have been reported.
Persistent adrenarche syndrome is a cutaneous condition seen typically in thin young women who report great psychological and physical stress in their lives.
Pseudomonas hot-foot syndrome is a self-limited cutaneous condition that occurs on the plantar surface of children after swimming in pool water that has high concentrations of P. aeruginosa. The condition typically presents as plantar purple-red nodules.
Autosomal dominant multiple pterygium syndrome is a cutaneous condition inherited in an autosomal dominant fashion.
Limb–mammary syndrome is a cutaneous condition characterized by p63 mutations.
Excess ovarian androgen release syndrome is a cutaneous condition usually seen in young women between the ages of 16 and 20.
The HAIR-AN syndrome is a rare subtype of polycystic ovary syndrome (PCOS) characterized by hyperandrogenism (HA), insulin resistance (IR) and acanthosis nigricans (AN). The symptoms of the HAIR-AN syndrome are largely due to severe insulin resistance, which can be secondary to blocking antibodies against the insulin receptor or genetically absent/reduced insulin receptor number/function. Insulin resistance leads to hyperinsulinemia which, in turn, leads to an excess production of androgen hormones by the ovaries. High levels of androgen hormones (hyperandrogenism) in females causes excessive hair growth, acne and irregular menstruation. Patients with both underlying mechanisms of insulin resistance may have more severe hyperandrogenism. Insulin resistance is also associated with diabetes, heart disease and excessive darkening of the skin
References
- ↑ Rapini, Ronald P.; Bolognia, Jean L.; Jorizzo, Joseph L. (2007). Dermatology: 2-Volume Set. St. Louis: Mosby. ISBN 978-1-4160-2999-1.
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