Linear Porokeratosis in Two Families with Disseminated Superficial Actinic Porokeratosis

Abstract

Two case reports of linear porokeratosis occurring in individuals or families with disseminated, superficial, actinic porokeratosis (DSAP) are presented. Linear porokeratosis and DSAP may be different expressions of one dominantly inherited condition. We reviewed the clinical features of linear porokeratosis and its association with other forms of porokeratosis. The linear form has a potential for carcinomatous change.

Co‐Existence of Variants of Porokeratosis: A Case Report and a Review of the Literature

“…A third variant was observed in another two patients along with LP and DSAP, which were PM and PP (4,10). In family members of these patients, the only variant reported was DSAP (3,6,12).…”

“…Welton first described an association of LP and DSAP in a woman along with DSAP in her three sisters (3). A total of only 12 cases of the coexistence of histologically confirmed variants of porokeratosis have been described in the English literature 307 (3)(4)(5)(6)(7)(8)(9)(10)(11)(12)(13) in the last 30 years ( Table 2). A few interesting observations can be made from a review of these reports.…”

Segmental forms of autosomal dominant skin disorders: Different types of severity reflect different states of zygosity

“…Such cases have been reported, for example, in neurofibromatosis type 1 [Boltshauser et al, 19891, nevoid basal cell carcinoma syndrome [Shelley et al, 1969;Camisa et al, 19851 and epidermolytic hyperkeratosis of Brocq [Nazzaro et al, 1990;Paller et al, 19941. However, sometimes the degree of involvement of the confined area is far more pronounced and, notably, the segmental lesions are superimposed on a milder, diffuse manifestation of the same phenotype. Such dichotomous forms of expression have been described in cases showing a linear arrangement of neurofibromatosis [Archer et al, 19881, cutaneous leiomyomatosis [Rudner et al, 1964;Mezzadra, 1965;Berendes et al, 19711, epidermolytic hyperkeratosis of Brocq [Hadlich and Linse, 19891, or porokeratosis, a disorder characterized by circular skin lesions showing central atrophy and a tiny keratotic ridge [Moreland and Wyre, 1981;Commens and Shumack, 1987;Happle, 19911. To explain this phenomenon that has so far not attracted much attention, the following rule may be considered.…”

Disseminated superficial actinic porokeratosis co-existing with linear and verrucous porokeratosis in an elderly woman: Update on the genetics and clinical expression of porokeratosis

“…[7][8][9][10][11][12][13][14][15][16][17][18] When an individual has isolated linear porokeratosis, often there are family members with DSAP. 11,19 Our patient had DSAP on the distal extremities, linear porokeratosis on the lateral thighs, and verrucous porokeratosis on the buttocks and mons pubis.…”