Sjögren‐Larsson syndrome: a dermato‐histopathological study

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Abstract
Thirty‐six Swedish patients with the recessively inherited Sjögren‐Larsson syndrome (SLS) were examined by histopathologic and, in some cases, morphometric techniques. Skin biopsy specimens were taken on the volar side of the right forearm below the cubital fossa. The histopathological picture was characterized by a moderate to pronounced hyperkeratosis that could include follicular hyperkeratosis The stratum granulosum was thickened. There was acanthosis and usually some papillomatosis. These observations were in accordance with the morphometric measurements. There was often a slight, chronic infiltrate in the upper dermis. The ichthyosis in SLS can be included in the recessively inherited, congenital group with a seemingly lower tendency to parakeratosis.