Porokeratosis is a clonal growth of keratinocytes which differentiate unusually but not completely neoplastic in type. The various types of porokeratosis are characterized by a thin pillar of parakeratosis, the cornoid lamella, the active border of injury.Porokeratosis is listed as a excellent disease by Orphanet and the Office of Unique Diseases (ORD) of the National Institute of Fitness.Mibelli described the simple form that bears his name (1893). The diagnosis is habitual by histopathological examination. It may be difficult by development of cutaneous malignancies, like squamous container carcinoma (most common in undeviating variant) .Etiopathogenesis of porokeratosis include Ultraviolet light exposure, far-reaching radiation medicine, immunosuppression, transplant procedures, immunodeficiency syndromes, never-ending renal failure, chronic liver ailment, infections, hematological malignancies including lymphomas, Immunological disorder infection and hepatitis C bacterium infection . Recent hypotheses suggest that abnormalities in the mevalonate road are responsible for the pathogenesis of porokeratosis. Irregularities in the genes MVD (mevalonate decarboxylase), mevalonate kinase, PMVK (phosphomevalonate kinase), FDPS (farnesyl diphosphate synthase) and solute carrier offspring 17 member 9 (SLC17A9) have been raise to be pathogenic in porokeratosis .This case report addresses a excellent case of giant porokeratosis of Mibelli showing features of pseudoepitheliomatous changes lifting suspicion of future malignant shift possibility that was medicated with surgical excision of wound coupled with systemic and local therapies in spite of the patient was lost to follow-up.
Author(s) Details:
Sushantika,
Department
of Dermatology, AIIMS Rishikesh, India.
Please see the link here: https://stm.bookpi.org/ACMMR-V11/article/view/12888
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