ABSTRACT
Porokeratosis is a keratinization disorder with unclear etiopathogenesis, varied clinical presentation and characteristic histopathology, and is usually unresponsive to current therapeutic options. Until now, it was considered to be a clonal disorder with immunity, ultra violet radiation and other factors playing important roles in etiopathogenesis. It is now known that abnormalities in the mevalonate pathway are responsible for this clonal keratinization abnormality. New variants of porokeratosis like eruptive bullous, pruriginous, lichen planus like, follicular variants and porokeratoma have been described. While the cornoid lamella is the classical histopathologic feature, dermoscopy and reflectance confocal microscopy make the diagnosis clearer. Development of malignancy in a few variants is a concern. Linear, disseminated superficial actinic and giant lesions are most prone to developing malignancies. Bowen's disease, squamous cell carcinoma, basal cell carcinoma and even melanoma have been reported in cases of long-standing porokeratosis. Newer modalities of therapy such as photodynamic therapy, ingenol mebutate and HMGCoA inhibitors may play a role in the future.
Subject(s)
Bowen's Disease , Carcinoma, Squamous Cell , Porokeratosis , Skin Neoplasms , Humans , Porokeratosis/diagnosis , Porokeratosis/therapy , Skin Neoplasms/diagnosis , Skin Neoplasms/therapy , Ultraviolet RaysSubject(s)
Porokeratosis/diagnosis , Porokeratosis/pathology , Buttocks , Humans , Male , Middle AgedSubject(s)
Mouth Mucosa/pathology , Porokeratosis/diagnosis , Adult , Humans , Male , Porokeratosis/geneticsSubject(s)
Dermatitis/pathology , Diagnostic Errors , Epidermis/pathology , Porokeratosis/pathology , Adult , Aged , Biopsy, Needle , Child , Dermatitis/diagnosis , Humans , Immunohistochemistry , Male , Porokeratosis/diagnosis , Rare Diseases , Sampling StudiesSubject(s)
Porokeratosis/pathology , Adolescent , Arm , Humans , Hyperpigmentation/complications , Male , Porokeratosis/complications , Porokeratosis/diagnosis , Shoulder , ThoraxSubject(s)
Porokeratosis/diagnosis , Porokeratosis/genetics , Humans , Male , Middle Aged , PedigreeABSTRACT
Porokeratotic eccrine ostial and dermal duct nevus (PEODDN) is a rare variant of porokeratosis with characteristic histological feature of cornoid lamella involving the acrosyringium. We report a classic case of a 20-year-old male, who clinically presented to us with keratotic papules and plaque with pits, few having comedo like plugs, on right palm and sole since 1 year of age. A punch biopsy from palm was diagnostic as well as confirmatory showing cornoid lamella involving an eccrine duct which is the characteristic histopathological feature of PEODDN.
Subject(s)
Eccrine Glands/pathology , Nevus, Intradermal/diagnosis , Porokeratosis/diagnosis , Biopsy, Needle , Humans , Male , Nevus, Intradermal/complications , Porokeratosis/complications , Young AdultSubject(s)
Lichen Planus/complications , Lichen Planus/diagnosis , Porokeratosis/complications , Porokeratosis/diagnosis , Adult , Humans , MaleABSTRACT
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 the perianal region that reached up to the squamo-columnar junction of the anal mucosa and even invaded the anal sphincteric muscles. Disseminated lesions of the Mibelli's type, development of horns, and malignancy in this unusual location have not been previously reported.