CONGENITAL lack of lashes and eyebrows, whether total or partial, is seldom encountered. Associated at times with other developmental defects, mostly ectodermal, it may appear in conjunction with general alopecia, faulty dentition, and/or cataract formation (Duke-Elder, 1952). In the so-called Ullrich-Dohna syndrome, it has been seen in combination with multiple ocular malformations, strabismus...

Tuen Mun Social Hygiene Clinic, 9/F, Tuen Mun Hospital, Ambulatory Care Centre, Ching Chung Koon Road, Tuen Mun, New Territories, Hong Kong Darier's disease (Keratosis follicularis) is an uncommon autosomal dominant genodermatosis. Patients present with hyperkeratotic papules in seborrhoeic areas with nail abnormalities. We describe a young gentleman with this disorder for more than three years...

Comedonal Darier’s disease is an extremely rare variant demonstrating unique clinical and histopathological findings; however, it is commonly misdiagnosed. Herein, we report a case of comedonal Darier’s disease and discuss its different diagnostic and therapeutic challenges.

Darier's disease, also known as keratosis follicularis or dyskeratosis follicularis, is a rare disorder of keratinization. It is an autosomal dominant genodermatosis with high penetrance and variable expressivity. Its manifestation appears as hyperkeratotic papules, primarily affecting seborrheic areas on the head, neck, and thorax and less frequently on the oral mucosa. When oral manifestation...

Two cases of borderline-tuberculoid leprosy which developed keratosis spinulosa over the anaesthetic areas alone during type I lepra reactions are described. Both patients only developed spiny papules during the period of reaction and subsided with control of the reaction. The probable mechanism of this peculiar phenomenon might be due to the generation of epidermal growth factors by local T ce...

Darier disease, also known as keratosis follicularis or dyskeratosis follicularis, is a rare autosomal dominant genodermatosis. It is clinically manifested by hyperkeratotic papules primarily affecting seborrheic areas on the head, neck and thorax, with less frequent involvement of the oral mucosa. When oral manifestations are present, they primarily affect the palatal and alveolar mucosa, are ...

Darier's disease, also known as keratosis follicularis or dyskeratosis follicularis, is a rare disorder of keratinization. It is an autosomal dominant genodermatosis with high penetrance and variable expressivity. Its manifestation appears as hyperkeratotic papules primarily affecting seborrheic areas on the head, neck, thorax, and less frequently the oral mucosa. When oral manifestations are p...

Keratosis linearis with ichthyosis congenita and keratoderma (KLICK) is an autosomal recessive skin disorder associated with a single-nucleotide deletion in the 5'untranslated region of the proteasome maturation protein (POMP) gene. The deletion causes a relative switch in transcription start sites for POMP, predicted to decrease levels of POMP protein in terminally differentiated keratinocytes...