Zahida Rant, Ijaz Hussain, Mansoor Haider.
An Unusual case of Progressive Symmetric Erythrokeratoderma with new unreported features - A Case Report and review of literature.
J Pak Assoc Derma Jan ;11(4):43-7.

We report an unusual case of progressive symmetric erythrokeratoderma in an 8 year-old female with bilaterally symmetrical, erythematous, scaly plaques around orifices, axillae, antecubital fossae and buttocks. There was palmoplantar keratoderma with transgrediens. Additional features included dry and lusterless hair, ectropion and low intelligence. A review of literature is also given.

Case Report: An 8-year-old girl from NWFP presented at the Department of Dermatology, Mayo Hospital Lahore in November 2001. According to her mother she was born after full term and uneventful pregnancy. She was normal at birth, without any Shin lesions. At the age of 7 months, she developed erythematous scaly lesions over her buttocks. Gradually, over the years, other body sites were also involved including face, palms, soles, axillae and arms. These lesions were mildly pruritic and relatively non-migratory in nature, partially responding to keratolytics and oral retinoids. Her milestones were also delayed. A detailed family history revealed that her mother and father were first degree cousins but did not have any skin disease. Our patient was the youngest of four sisters and one brother (Figure 1). One elder sister, 15 years old, had a history of similar skin lesions, appearing at the age of 1 year and healing spontaneously at the age of 14 years. She was also mentally retarded. Her examination revealed no skin lesions but there was atrophy of thenar and hypothenar muscles. Physical examination of the patient revealed multiple, well-demarcated, bilaterally symmetrical, erythematous and scaly plaques on her face, axillae, antecubital fossae and buttocks. Palms and soles showed similar lesions, which also spread to the dorsal surface of hands and feet. The plaque on her face involved part of her forehead, both cheeks, nose and periorbital area and extended down the front of her neck. There was associated ectropion and fissures around the mouth. The lesions on the palms and soles also showed fissuring. The nails of all fingers and toes showed discoloration and Beau`s lines. Hair was dry and lusterless. Mucous membranes and teeth were normal and no other physical or systemic abnormality was detected. Psychiatric evaluation revealed her mental age to be that of a 3-year-old child. Rest of the physical examination was normal.

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