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Gangopadhyay A. Erythrokeratoderma variabilis. Indian J Dermatol Venereol Leprol 2002;68:232
AbstractA case of erythrokeratoderma variabilis with classical clinical and histological features is being presented here.
Erythrokeratoderma variabilis (EKV), included in ′Less common Ichthyosis′ group, is a rare disorder of keratinisation. If was first described by Mendes da Costa in 1925. It usually mainfests at birth or during first year of life. There are two clinical subtypes of EKV. One type is characterised by generalised, persistent hyperkeratosis while in the other type the involvement is localised, symmetrically distributed and sharply demarcated. Here is the report of first clinical subtype of EKV showing generalised involvement.
A young girl of 8 years presented with extensive thickening of the skin since childhood. According to the parents statement the lesions started appearing at the age of 6 months as finely scaly erythematous patches which gradually increased in size and took the shapes of different geographical figures. Some of the patches regressed spontaneously and some persisted for long time. These erythematous patches appeared mainly over the limbs, buttock and abdomen over the years. Many hyperkeratotic plaques of different sizes started appearing independently of the erythematous patches, over abdomen, buttock and limbs during the course of the disease. These plaques gradually enlarged in size with polycyclic borders which gradually coalesced to form diffuse hyperkerafosis. There was no triggering factor identified. The condition worsened in winter due to xerois. Detailed history revealed no other member of the family tree was involved.
On examination there was diffuse dark brown hyperkeratosis involving all the four limbs buttock, back, anterior abdominal wall, and lateral aspects of the chest wall. The anterior and posterior of the chest wall and face were surprisingly spared. There was palmoplantar keratoderma. Mucous membrane, hair and nails were normal. There was no other abnormality in physical examination, nor there any neurological defect. Punch biopsy from abdominal wall showed rather non-specific changes. There was hyperkeratosis, hypergranulosis and mild acanthosis.
EKV is a rare gentic disorder and has got autosomal dominant mode of inheritance. A close genetic linkage has been found between EKV and RH locus on the short arm of chromosome. The lesions have two morphological features-erythema and hyperkeratosis in both subtypes. The erythema changes its pattern within minutes, hours, or days. The commonest sites involved are the face, buttock and limbs. Palmoplantar keratoderma has been reported in some patients. This particular case reported herein had palmoplantar keratoderma along with diffuse hyperkeratosis. This particular case has to be differentiated from progressive symmetric erythrokeratoderma (PSEK). Unlike PSEK, the lesions in EKV fluctuate in configuration and extent, involve abdomen and thorax, and may show seasonal variations. Treatment of EKV is symptomatic with emollients. Topical retinoic acid and oral retinoid may give variable results.
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