Genital porokeratosis is a rare form of porokeratosis which can occur in two ways : in isolated form or as a part of disseminated porokeratosis. The former one is much rarer and less than 50 such cases of isolated involvement of genitalia in porokeratosis has been reported so far. Being a inherited disorder of keratinization, usual mode of heritance is autosomal dominant. The aim of present rep...

Porokeratosis is a disorder of keratinization showing a well-defined lesion with a hyperkeratotic ridge on the border that contains the coronoid lamella. We report familial (autosomal dominant with reduced penetrance) disseminated plaque type (Mibelli's type) porokeratosis in a father and son. In the father, there were multiple horns and a large squamous cell carcinoma in a large lesion over th...

Sir, Abnormal keratinization of the distal part of the hair follicle can prevent emergence of the growing hair from the follicle and lead to spiral growth within a hyperkeratotic papule, socalled ``rolled hairs'' (1). Rolled hairs have been reported in patients with keratosis pilaris (1), neurodermatitis (1), palmoplantar keratoderma (2), and hypertrichosis (3). The disorder has also been assoc...

Darier disease (DD) is a rare dominantly inherited genodermatosis characterized by loss of intercellular adhesion (acantholysis) and abnormal keratinization. DD is often difficult to manage. Numerous treatments have reportedly been used for the treatment of DD, with limited success. Systemic retinoids are considered the drug of choice for treating DD. However, their use is limited by potential ...

We present a 32-year-old white patient with congenital reticular ichthyosiform erythroderma, also known as ichthyosis variegata. The patient had typical clinical features with areas of normal appearing skin surrounded by erythematous hyperkeratotic patches. The smaller ‘‘confetti-like’’ patches were mostly present on the trunk, while the larger areas forming a reticular pattern predominated on ...

Porokeratotic eccrine and hair follicle nevus is a very rare non-hereditary disorder of keratinization with eccrine and hair follicle involvement with only 9 cases described in the literature. In 2009 the term porokeratotic anexial ostial nevus was proposed to comprehend porokeratotic eccrine and hair follicle nevus and a related and more common process without follicular involvement: porokerat...

Porokeratosis represents a group of disorders of epidermal keratinization that are characterized by one or more annular plaques surrounded by a histologically distinctive hyperkeratotic ridge-like border called the cornoid lamella. Many studies showed that organ transplantation and immunosuppression were associated in a significant number of cases. Furthermore, an association with squamous cell...

Porokeratosis is a specific disorder of keratinization that has five major clinical types and shows a characteristic cornoid lamella on histopathology. Giant porokeratosis is considered to be a morphological variant of porokeratosis of mibelli. Malignant degeneration has been described in all forms of porokeratosis but highest risk is associated with linear and giant porokeratosis . We report a...

Porokeratosis is a specific keratinization disorder that manifests clinically as well-demarcated annular or linear keratotic plaques of various sizes and forms and with distinguished histology showing cornoid lamella, which is a column of closely packed parakeratotic cells extending through the stratum corneum. Nail changes secondary to porokeratotic lesions involving digits are quite uncommon ...