ABSTRACT
Darier's disease is an uncommon genodermatosis, characterized by the symmetric eruption of keratotic papules, focal loss of epithelial adhesion and abnormal keratinization. A unilateral, or localized variant has been identified as a mosaic form, involving linear or 'zosteriform' epidermal lesions with clinical and histological features of Darier's disease. Some suggest that unilateral lesions should be classified as acantholytic dyskeratotic epidermal nevi, rather than localized Darier disease. A 17-year-old male presented with unilateral zosteriform distribution of brownish hyperkeratotic papules with ipsilateral v-notching of the thumb nail. A biopsy specimen revealed acantholysis, suprabasal cleft and multiple dyskeratotic cells. We report a case of unilateral Darier's disease which further supports unilateral dermatologic manifestation as a variant of Darier's disease.
Subject(s)
Adolescent , Humans , Male , Acantholysis , Biopsy , Darier Disease , Keratins , Nails , ThumbABSTRACT
Acantholytic dyskeratosis is a regular histologic feature in Darier's disease, transient acantholytic dermatosis and warty dyskeratoma. It is also occasionally observed as an incidental histological finding in linear epidermal nevus. We report a case of acantholytic dyskeratotic epidermal nevus in an 11-month-old boy. The patient showed unilateral, brown colored verrucous plaque on the right shin. Histological finding showed hyperkeratosis, irregular acanthosis & focal acantholytic dyskeratosis.
ABSTRACT
We report a case of acantholytic dyskeratotic epidermal nevus in a 28-year-old female. The patient showed unilateral, asymptomatic, grouped, 2 mm sized, brownish, follicular ant non-follicular, keratotic papules disposed along Blaschko´s lines on the right side of th back, flank and abdomen. She had had this condition for 4 years. The biopsy specimen obtaine, from the abdomen showed hyperkeratosis, parakeratosis, acanthosis, acantholytic suprabasal clefts, corps ronds, and grains. Although cryotherapy with liquid nitrogen was per formed on half of the lesions, the whole skin lesions healed with focal hypertrophic scars 4 weeks later. There was no recurrence after a 12 months follow-up period. To our knowledge this is the first reported case of acantholytic dyskeratotic epidermal nevus in Korean dermatologi, literature.