Linear porokeratosis

A Review of Therapeutic Options for Linear Porokeratosis

“…Porokeratosis is a chronic, progressive cutaneous disorder of clonal keratinization that demonstrates sharply demarcated, hyperkeratotic, annular lesions with central atrophy and a characteristic raised keratotic edge corresponding histologically to a parakeratotic column within the stratum corneum, known as a coronoid lamella. 1 Among the different clinical variations of porokeratosis, Linear Porokeratosis (LP) is possibly the least prevalent, but it has major clinical consequences due to its propensity for malignant transformation and influence on patients' quality of life. 1,2 LP demonstrates heterogeneous clinical features of mosaicism with unilateral blaschkoid segmental linear plaques on the trunk and extremities appearing congenitally or arising in infancy or childhood.…”

“…1 Among the different clinical variations of porokeratosis, Linear Porokeratosis (LP) is possibly the least prevalent, but it has major clinical consequences due to its propensity for malignant transformation and influence on patients' quality of life. 1,2 LP demonstrates heterogeneous clinical features of mosaicism with unilateral blaschkoid segmental linear plaques on the trunk and extremities appearing congenitally or arising in infancy or childhood. 1,3 A combination of heterozygous germline and second-hit postzygotic somatic (in utero) loss-of-function mutations in mevalonate pathway genes (PMKV, MVK, MVD) have been implicated.…”

“…1,2 LP demonstrates heterogeneous clinical features of mosaicism with unilateral blaschkoid segmental linear plaques on the trunk and extremities appearing congenitally or arising in infancy or childhood. 1,3 A combination of heterozygous germline and second-hit postzygotic somatic (in utero) loss-of-function mutations in mevalonate pathway genes (PMKV, MVK, MVD) have been implicated. 4 Familial (autosomal dominant) inheritance or de novo mutations are possible.…”

“…8 The purpose of this paper is to discuss the current treatment options for LP, which include topical, cryotherapy, photodynamic, laser, surgical, and systemic therapy. 1 These treatments are critical in controlling LP, lowering the risk of malignant transformation, and enhancing patients' quality of life. 1,2,6 Despite the disease's rarity, researching and enhancing these therapy choices is critical given the disease's potential morbidity and mortality, as well as the enormous impact it can have on patients' lives.…”

“…1 These treatments are critical in controlling LP, lowering the risk of malignant transformation, and enhancing patients' quality of life. 1,2,6 Despite the disease's rarity, researching and enhancing these therapy choices is critical given the disease's potential morbidity and mortality, as well as the enormous impact it can have on patients' lives. 1,10 Although no randomized-controlled clinical trials have been performed for LP, several treatment options have been reported.…”