It is a hereditary disorder of the skin and nervous system (neurocutaneous syndrome) which is featured by hard, red plaques which develop in infancy and child hood. After the skin lesions heal the disorder remains dormant for several years, after which the neurological symptoms appear in the form of awkward gait at the older age around 40 years.

Erythrokeratodermia, ataxia, red skin plaques, ataxic gait, hereditary disorder, neurological symptoms, skin lesions, Gottron syndrome, inherited disorder, retinoids emollients, keratolytics.

It is a rare syndrome which is characterized by presence of skin lesions along with the progressive neurological symptoms. It is rare disease. It is also known as Erythrokeratodermia with ataxia, Gottron syndrome.

It is a progressive symmetric Erythrokeratodermia, an inherited disorder which has features like slowly progressive, development of fixed, well-defined plaques of thickened skin with underlying redness. It is an autosomal dominant disorder characterized by reddish plaques, thickened, rough skin which develops during infancy and early childhood and disappears in later life.

It is a hereditary disorder of skin and nervous system. It is characterized by formation of hard, red plaques which develop during infancy and childhood. The kin lesions will heal. The disease will be dormant for many years. After few years the neurological symptoms appear with awkward gait when the individual is around 40 years of age. 

Signs and symptoms

• Red, hard skin plaques
• Awkward gait
• Neurological symptoms
• Red skin plaques
• Gait ataxia
• Reduced reflexes
• Nystagmus

• Dysarthria

• Abnormal walk

Treatment:

Treatment of skin lesions are with retinoids Topical therapy with emollients and keratolytics.