Letter to the Editor| Volume 71, ISSUE 2, P138-140, August 2013

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In vitro reconstruction of epidermis from primary Darier's disease keratinocytes replicates the histopathological phenotype

      Darier disease (DD) is a rare dominant human disorder characterized by warty papules and plaques in seborrheic areas of the skin, which may cause a severe discomfort. Lesions are exacerbated by UV exposure, heat and sweating, and subject to secondary infection. Treatments often remain unsatisfactory. Onset usually occurs during adolescence, with highly variable expressivity. Histological findings in the epidermis reveal suprabasal acantholysis and two abnormal types of keratinocytes. Corps ronds refer to cells with small pycnotic nuclei, perinuclear clear halo and eosinophilic cytoplasm, while grains are compressed cells with elongated nuclei preferentially found in granular and cornified layers (Fig. 1a–c ).
      Figure thumbnail gr1
      Fig. 1Histopathological features of Darier's disease are conserved after epidermis reconstruction with primary keratinocytes derived from lesional skin. (a) Section of Darier's disease (DD) lesion from the patient, showing epidermal hyperplasia, parakeratosis, and suprabasal clefting (asterisks) due to acantholysis (scale bar: 200 μm). (b,c) Higher magnification showing dyskeratosis with corps ronds (black arrowheads) and grains (arrows) (scale bars: 50 μm). (d–g) Perpendicular sections of RHE cultured from primary DD keratinocytes for 11 days at air–liquid interface. Similarities to Darier native epidermis include the presence of grains (d, arrow in e), corps ronds (d, black arrowhead in f), and enlargement of intercellular spaces (acantholysis) (d, asterisks in e and g). Frequent mitoses are seen in the basal layer (d, white arrowhead in f). Hematoxylin-eosin stain (scale bar: 50 μm).
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