Two sisters with familial dyskeratotic comedones.
Familial dyskeratotic comedones is a rare autosomal dominant genodermatosis, which presents with disseminated keratotic papules and comedo-like lesions. The disease usually manifests in childhood. The histology of the lesions is characterized by a crater-like invagination of the epidermis filled with parakeratotic keratin. The epidermis usually shows acantholysis. So far the causative gene defect is unknown. We report on a new family with two affected sisters. The more severely affected woman was treated with oral retinoids without any effect. Afterwards some regions were successfully treated by CO(2) laser-therapy.
Department of Dermatology, University Hospital Goettingen, Germany. Challermann@gmx.de
Christian
C
Hallermann
Hans Peter
HP
Bertsch
D000328:Adult; D007644:Darier Disease / Q000175:diagnosis* / Q000235:genetics / Q000473:pathology; D003937:Diagnosis, Differential; D005121:Extremities; D005145:Face; D005260:Female; D020022:Genetic Predisposition to Disease; D006801:Humans; D035781:Siblings; D013909:Thorax