ABSTRACT
We present a case report of two brothers suffering from Sjogren-Larsson syndrome, who were born to consanguineous parents. Sjogren-Larsson syndrome is one of the congenital icthyoses with an autosomal recessive inheritance. It is characterized by the combination of congenital ichthyosis with spastic diplegia, moderate mental retardation and retinopathy. Defects in essential fatty acid metabolism have been attributed to as the cause
Subject(s)
Humans , Male , ConsanguinityABSTRACT
Small vessel vasculitis in a dermatologic patient may present with clinically indeterminate lesions such as palpable purpura, plaques, nodules or ulcers. In this study, it was aimed to determine the cause of such lesions by correlating clinical with histopathological features to formulate a final diagnosis. This was a study of the descriptive type. Patients of both genders aged between 30-60 years presenting with palpable purpura, plaques, nodules and ulcers of less than 6 months' duration which did not fit into any other disease entity were selected for skin biopsy. The findings on histopathology were correlated with the clinical features for the final diagnosis. A total of 18 patients were evaluated histologically. Six [33.3%] patients were diagnosed as various types of small vessel vasculitis. Five [27.7%] patients were found to be insect bite reactions. Three [16.7%] patients were diagnosed as urticarial vasculitis. There were 2[11.1%] cases of pyoderma gangrenosum and 1[5.55%] each of erythema elevatum diutinum and lupus profundus. In clinically indeterminate cutaneous vasculitic lesions which do not resolve spontaneously, correlation of clinical with histopathological features aids in reaching the diagnosis and prognosis for better patient outcome