Progressive symmetric erythrokeratodermia
Progressive symmetric erythrokeratodermia
Progressive symmetric erythrokeratodermia (PSEK) is a rare genetic disorder that affects the skin. It is characterized by symmetric, fixed erythema and hyperkeratosis that are usually present at birth or appear in the first years of life.
Pronunciation
Progressive symmetric erythrokeratodermia is pronounced as pro-gres-sive sym-met-ric ery-thro-ker-a-to-der-mia.
Etymology
The term "Progressive symmetric erythrokeratodermia" is derived from the Greek words "pro" meaning forward, "symmetria" meaning balance, "erythros" meaning red, "keras" meaning horn, and "derma" meaning skin. This term describes the progressive, symmetric redness and hardening of the skin that characterizes this condition.
Symptoms
The main symptoms of PSEK include erythema (redness of the skin) and hyperkeratosis (thickening of the skin). These symptoms are usually symmetric and can affect any part of the body, but they are most commonly seen on the limbs, buttocks, and trunk.
Causes
PSEK is a genetic disorder, which means it is caused by changes (mutations) in genes. The specific genes involved in PSEK are not yet fully understood, but it is believed to be an autosomal dominant condition, which means one copy of the altered gene in each cell is sufficient to cause the disorder.
Treatment
There is currently no cure for PSEK, but treatments can help manage the symptoms. These may include topical treatments such as emollients, keratolytics, and retinoids, as well as systemic treatments such as retinoids and immunosuppressants.
Related Terms
External links
- Medical encyclopedia article on Progressive symmetric erythrokeratodermia
- Wikipedia's article - Progressive symmetric erythrokeratodermia
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