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NEVO EPITELIALE VERRUCOSO INFIAMMATORIO LINEARE (NEVIL) MULTIPLO CON CHERATODERMIA POROCHERATOTICA PALMOPLANTARE
No full textKeratosis lichenoides chronica is a variant of inflammatory linear verrucous epidermal nevus.
No full text[Eruptive vellus hair cysts: a facial variant]
No full textA case of a 30 year-old man with numerous, pink to whitish, 1-2 mm, cystic lesions, located exclusively on the face and helices in symmetrical distribution is reported. Microscopic examination of serial sections of two biopsies disclosed dermal cystic cavities with vellus hair shafts into the lumen. The cyst wall was connected to rudimentary pilar structures. This picture is typical of eruptive vellus hair cysts (EVHC). Facial variant of this dermatosis is reported only in two cases in the literature. Histogenesis and differential diagnosis are discussed
Sindrome di Rapp-Hodgkin. Espressivita fenotipica diversa in membri della stessa famiglia
No full textA 23-year old woman with narrow nose, maxillary hypoplasia, cleft lip and palate, hypodontia, dystrophic nails and hypohidrosis is described. Her scalp hair was coarse, dry and wiry. Microscopic examination of hair showed longitudinal twisting at irregular intervals, typical of pili torti. Her mother presented only dystrophic nails and hypohidrosis. Rapp-Hodgkin syndrome is an uncommon, autosomal dominant condition characterized by distinctive craniofacial anomalies and cleft lip and palate. In the patients observed, the phenotypical manifestations were different. In fact, the proband showed all the major features of the syndrome, while her mother only onychodysplasia and hypohidrosis. The differential diagnosis between Rapp- Hodgkin syndrome and other forms of ectodermal dysplasia are discussed.
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