Tranebjaerg Svejgaard syndrome

Overview

Tranebjaerg-Svejgaard syndrome: A rare syndrome characterized mainly by mental retardation, seizures and a skin disorder.

Symptoms

The list of signs and symptoms mentioned in various sources for Tranebjaerg-Svejgaard syndrome includes the 16 symptoms listed below: Mental retardation Seizures Psoriasis Dry skin Speech defect Weak eye muscles Poor muscle tone Mouth held open Anteverted nostrils Ataxia Large mouth Large tongue Curved spine Thick lips Respiratory distress Protruding jaw Note that Tranebjaerg-Svejgaard syndrome symptoms usually refers to various symptoms known to a patient, but the phrase Tranebjaerg-Svejgaard syndrome signs may refer to those signs only noticable by a doctor.

Causes

The tendency to develop psoriasis is genetically determined. Researchers have discovered a significantly higher-than-normal incidence of certain human leukocyte antigens (HLA) in families with psoriasis, suggesting a possible immune disorder. Onset of the disease is also influenced by environmental factors. Trauma can trigger the isomorphic effect or Koebner’s phenomenon, in which lesions develop at sites of injury. Infections, especially those resulting from beta-hemolytic streptococci, may cause a flare-up of guttate (drop-shaped) lesions. Other contributing factors include pregnancy, endocrine changes, climate (cold weather tends to exacerbate psoriasis), and emotional stress. Generally, a skin cell takes 14 days to move from the basal layer to the stratum corneum, where after 14 days of normal wear and tear, it’s sloughed off. The life cycle of a normal skin cell is 28 days, compared to only 4 days for a psoriatic skin cell. This markedly shortened cycle doesn’t allow time for the cell to mature. Consequently, the stratum corneum becomes thick and flaky, producing the cardinal manifestations of psoriasis.