Medical expert of the article
New publications
Erythrokeratoderma: causes, symptoms, diagnosis, treatment
Last reviewed: 07.07.2025
All iLive content is medically reviewed or fact checked to ensure as much factual accuracy as possible.
We have strict sourcing guidelines and only link to reputable media sites, academic research institutions and, whenever possible, medically peer reviewed studies. Note that the numbers in parentheses ([1], [2], etc.) are clickable links to these studies.
If you feel that any of our content is inaccurate, out-of-date, or otherwise questionable, please select it and press Ctrl + Enter.
Currently, this group of erythrokeratodermia includes skin keratinization disorders of the hyperkeratosis type and occurring on an erythematous background. However, few dermatologists classify it as an ichthyosis.
Causes and pathogenesis of erythrokeratodermia. The causes of erythrokeratodermia have not been fully studied. According to some scientists, an increase in the content of acid phosphatase in epidermal cells, accumulation of nucleotide triphosphatase and alkaline phosphatase in the papillary layer of the dermis and other factors play a major role in the pathogenesis of erythrokeratodermia. The disease is considered hereditary.
Symptoms. Dermatologists distinguish the following clinical forms of erythrokeratodermia: variable figured; symmetrical progressive (Gottron syndrome); limited symmetrical, progressive with peripheral neuropathy and deafness (Schneider syndrome); rare, atypical, etc.
Erythrokeratoderma symmetrical progressive Gottron often begins in the first years of life. At the beginning (1-3 years) the process actively progresses, and then stops and, possibly, regression of the process occurs. The occurrence of the disease is also described in older and elderly age.
Symptoms of erythrokeratoderma. Symmetrical progressive erythrokeratoderma Gottron is characterized by the presence of hyperkeratotic pinkish-red plaques with a narrow erythematous rim or hyperpigmented edge. The rash is located symmetrically on the skin of the knees and elbows, the back of the hands and feet. Sometimes the rash can be located on other areas of the skin. The skin of the trunk, palms and soles usually remains unaffected. No changes in the nails are noted.
Histopathology. Histological examination reveals hyperkeratosis, local parakeratosis, hypergranulosis, moderate acanthosis, and vascular dilation and lymphocytic infiltrate in the dermis.
Differential diagnosis. The disease should be distinguished from psoriasis, lamellar ichthyosis, etc.
Treatment. Vitamins, antioxidants, retinoids or corticosteroids are prescribed orally. Hormonal and keratolytic agents are prescribed externally. PUVA therapy has shown good results.
[ What do need to examine?
How to examine?