Biology & Medicine Case Reports

Erythrokeratoderma Review Articles

Skin biopsy might be performed for histology yet there are no different peculiarities. Hereditary directing ought to be offered to influenced people and their groups of childbearing age. There is no particular or therapeutic treatment. Minimizing temperature changes and mechanical erosion is vital. Symptomatic change might be acquired by Emollients, Keratolytics, for example, urea, salicylic corrosive or alpha hydroxy acids, Topical steroids Topical retinoid, Oral retinoid, for example, acitretin or isotretinoin. These slender down the plaques and diminish scaling however the redness holds on. Though majority of genetic analyzes in affected individuals and transgenic mice have found mutations in gene for loricrin as the underlying defect, a recent report demonstrated connexin mutations in a PSEK patient.[Loricrin is the major structural component of the cornified cell envelope while connexins are the protein units of gap junctions distributed in skin, nervous system, internal ear, cornea, and lens. Due to unavailability of genetic testing in our institute, exact mutation in our patient could not be elucidated. Associated neurological abnormalities have been described recently. We hypothesize that unusual association of ocular and neurological complaints in our patient could be due to underlying mutation of gap junction protein, connexin. Clinically characterized by well-demarcated erythematous and hyperkeratotic plaques that are distributed with an almost perfect symmetry on the head,

There are two major subtypes of EKV: (1) EK variabilis (Mendes da Costa)and (2) EK progressiva symmetrica (Gottron).The classical EKV initially described by Mendes da Costa is characterized by two types of skin lesions: (a) figurate hyperkeratosis plaques and (b) transient erythematous areas. These subtypes are independent, and their shapes and distribution can be changeable at any time. The lesions have propensity to locate on the distal extremities, buttocks, and trunk. EKV usually presents at birth or during infancy. The case one had developed the lesions since age 5, and the case two had developed since age 2. It presents as symmetrical plaques on the limbs, buttocks, and face during early childhood. The plaques progress in childhood and frequently stabilize in adolescent age. It is autosomal dominant disease, often with incomplete penetrance. There is considerable similarity between PSEK and EKV due to the presence of a symmetrically distributed, fixed or very slowly progressive erythematosus, scaly plaques. PSEK differs in the absence of migratory erythematous lesions and in greater incidence of palmoplantar keratoderma.A distinctive feature of EKV from PSEK is the lack of facial involvement. extremities, and buttocks. Onset is usually in early childhood, which progresses over the next few years and then remains stable over time with morphology, color, and site remaining constant. Rarely, they may partially regress after puberty. Patients are usually mentally and physically unaffected. Differential diagnoses include EKV, psoriasis, and pityriasis rubra pilaris