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Medical Genetics, Molecular Medicine Department, “Sapienza” University of Rome, San Camillo Forlanini Hospital, Rome, ItalyMedical Genetics, Experimental Medicine Department, “Sapienza” University of Rome, Rome, Italy
Corresponding author at: Research Unit for Molecular Physiology (URPHYM), Cell and Tissue Laboratory, NARILIS, University of Namur (FUNDP), 61 Rue de Bruxelles, B-5000 Namur, Belgium. Tel.: +32 81 72 42 57; fax: +32 81 72 42 61.
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 ).
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).
Immortalized keratinocytes derived from patients with epidermolytic ichthyosis reproduce the disease phenotype: a useful in vitro model for testing new treatments.
