Darier's follicular dyskeratosis (synonym: Dariers disease, DarierWhites disease) is a rare genetic disease with an autosomal dominant type of inheritance, which belongs to the group acantholytic dermatoses and characterized by violation keratinization processes lesions skin, nails, mucous membranes oral cavity genitals. caused mutation in ATP2A2 gene. This disrupts operation SERCA2 pump leads ...

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...

Volume 84, Number 9 www.aafp.org/afp American Family Physician 1035 A 51-year-old man developed a mild fever and several serous vesicles on an erythematous base on his trunk (Figures 1 and 2) three months after having a renal transplant. During the following 10 days, similar lesions developed on his neck, upper arms, lower back, and inner thigh. The patient had the transplant because of end-sta...

Pemphigus vegetans is a rare variant of deep acantholytic pemphigus which usually presents with vesiculobullous rash and vegetative plaques on the folds. We report a case of pemphigus vegetans patient who presented with rashes on tips of fingers and toes resembling paronychia and onychomadesis that misled the diagnosis for months. The final diagnosis of Hallopeau-type pemphigus vegetans was mad...

BACKGROUND IgA pemphigus is a rare autoimmune vesiculo-pustular skin disease. Only approximately 70 cases have been reported to date. We report a case of IgA pemphigus with IgA antibodies to desmoglein 1 (Dsg1) and desmoglein 3 (Dsg3). CASE REPORT We report the case of an 60-year-old man with intraepidermal neutrophilic IgA pemphigus with IgA antibodies to Dsg1 and Dsg3. Histologic examinatio...

A case of so-called pseudoglandular (adenoid, acantholytic) squamous cell carcinoma (SCC) of the penis occurring in a 60-year-old man is described. The tumor showed, in addition to the pattern of conventional moderately to poorly differentiated SCC, a component of tubular-appearing pseudoglandular SCC. No precancerous dysplastic lesion was found near the lesion. Immunohistochemically, the tumor...