Olmsted Syndrome: Rare Occurrence in Four Siblings.

Olmsted syndrome is a very rare and severe cicatrizing keratoderma associated with periorificial lesion. Most cases are sporadic but familial occurrence has been also seen. Till now around 73 cases have been reported and none of the reported cases have 4 siblings affected from this disease. We are reporting cases of 4 siblings of age 30 year female, 26 year female, 20 year male and 10 year male who were born to a third degree consangueinous marriage and presented with palmoplantar keratoderma, periorificial hyperkeratosis, flexion deformity, pseudoainhum and contracture of digits. There was no cardiac involvement. Hence, the diagnosis of Olmsted syndrome was made and all four patients were non responsive to treatment which included topical corticosteroid, topical salicylic acid, systemic isotretinoin, systemic acitretin and oral zinc in child.

Follicular psoriasis causing erythroderma in a child: A rare presentation.

Erythroderma in children is an uncommon, yet striking entity with an incidence of 0.11%. Psoriatic erythroderma accounts for 1.4% of psoriasis cases in children. Follicular psoriasis is an underdiagnosed variant of psoriasis, with only about 15 cases reported till date, characterized by scaly follicular papules on the trunk and extremities. Although two thirds of these reported occurred in adults, cases have been described in children under the age of 10 years. Follicular lesions may present without psoriasis vulgaris elsewhere. We report here a 13-year-old boy who presented with severe erythrodermic psoriasis that started as dark, rough, horny, discrete, follicular papules over knees and elbows, associated with nail and joint involvement. Such a presentation of follicular psoriasis causing erythroderma is uncommonly seen in children and has not yet been reported in literature.

Crusted Piloleiomyoma with mental retardation: a rare association.

Piloleiomyoma is an uncommon benign smooth muscle neoplasm arising from arrector pili muscle. It is clinically defined by the presence of solitary or multiple reddish brown, dome-shaped, smooth papules or nodules, ranging in size from a few millimeters to a centimeter. The patients are otherwise healthy; but mental retardation developing in some patients with multiple Piloleiomyomas has been emerging as an intriguing matter for analysis by the scientists. In this case report, a mentally retarded patient with Piloleiomyoma is described, who, besides the characteristic smooth and dome-shaped lesions on the anterolateral aspect of the dorsum of the right foot, had developed crusting on one of the largest lesions. The histopathological features were consistent with Piloleiomyoma. The occurrence of Piloleiomyoma in a mentally retarded child and its unusual crusted nature has been rarely reported. The association between Piloleiomyoma and mental retardation is further stressed in this case report.