Pityriasis Rubra Pilaris (PRP) is a rare inflammatory dermatosis, clinically presenting as keratinization disorder, with follicular and palmoplantar hyperkeratosis, in addition to orange-red scaly plaques, accompanied by characteristic islands of healthy skin. Sometimes, conventional treatment proves be ineffective, this work aims present case patient failed first second lines options, respondi...

Sir, Porokeratotic eccrine ostial and dermal duct naevus (PEODDN) is a rare disorder of keratinization with eccrine involvement that histologically presents a cornoid lamella exclusively associated with eccrine ostia and the underlying sweat ducts (1). It is an expression of genetic mosaicism (2) and is characterized by asymptomatic grouped keratotic papules and plaques with a linear distributi...

Darier disease (DD) is an autosomal-dominant skin disorder that is characterized by multiple keratotic papules, loss of epithelial adhesion, and abnormal keratinization. We describe an unusual case of late-onset unilateral segmental DD that follows the lines of Blaschko. Our patient did not exhibit other classic findings of DD. Our case and review of the literature suggest that lesions previous...

Porokeratosis is a disorder of epidermal keratinization characterized by annular plaques with an atrophic center and hyperkeratotic edges, and includes a heterogeneous group of disorders that are mostly inherited in an autosomal dominant form. This report describes a 5 year-old female patient, with porokeratosis of Mibelli confirmed histopathologically. The rarity of this disorder, its clinical...

INTRODUCTION Pityriasis rubra pilaris is an uncommon inflammatory and hyperproliferative dermatosis of juvenile or adult onset. The etiology of the disease is still unknown. CASE PRESENTATION We present the cases of two Caucasian men aged 53 and 48 who presented with pityriasis rubra pillaris type 1; both patients also exhibited an abnormal immunological profile. CONCLUSION Pityriasis rubra...

Porokeratosis is a disorder of epidermal keratinization characterized by the presence of annular hyperkeratotic plaques. Its etiopathogenesis is not yet fully understood, but a relationship with immunosuppression has been reported. Dermoscopic examination revealed a classic yellowish-white ring-like structure that resembled "volcanic crater contour" - the so-called cornoid lamella. We describe ...

Bullous ichthyosiform erythroderma (BIE) is a rare disorder of keratinization (mutations in either keratin 1 or 10). It typically presents with fragile skin, which gives way to gradual evolutionof hyperkeratosis. Flaccid blisters, peeling, and superficial erosions at sites of minor trauma or friction are apparent within the first few hours of life. Yellow-brown, waxy, ridged or corrugated...

Ichthyosis hystrix of Curth-Macklin ( IHCM) is an extremely rare keratinization disorder, with verrucous to hystrixlike hyperkeratotic plaques of varying extent. The diagnosis is based on a distinctive pattern of epidermolytic hyperkeratosis and the presence of binucleate cells under light microscopy, as well as unique electron microscopic findings of continuous perinuclear tonofibril shells in...

We report the case of a patient diagnosed with genitogluteal porokeratosis, a disorder of epidermal keratinization. The location described is extremely rare and very often late diagnosed or even misdiagnosed. Histopathology showed a typical cornoid lamella of great value to support this diagnosis. The importance of awareness of this entity by the specialist is emphasized as a differential diagn...