Scaly Atrophic Lesions Both Scattered and in Linear Arrays

Abstract

A 30-year-old woman presented with scattered, subtle red spots on her right upper extremity that had developed over the previous year. These spots were not tender or pruritic and exhibited no tendency for spontaneous resolution.This patient was particularly concerned that these lesions might continue to develop, because she had a far more extensive eruption in linear groups confined to the left side of her body. This latter eruption developed at age 6 months and had remained stable since. It was asymptomatic, except for its cosmetic implications. Besides the eruptions, she was in good health.The physical examination was notable for scattered erythematous, slightly atrophic maculae confined to the sun-exposed right upper extremity (Fig 1). A faint keratotic rim was visible around portions of the lesions. The left side of the trunk and the left upper and lower extremities had far more extensive linear groups of more prominent lesions (Figs 2 and 3). The keratotic rim was more prominent on these lesions. The lesions on the trunk exhibited a zosteriform pattern; nearly the entire length of the upper and lower extremities was covered with lesions.A biopsy specimen was obtained from one of the two lesions on the right arm (Fig 4). Biopsy specimens were also obtained from the linear lesions of the left upper extremity (Fig 5).

Porokeratoses—A Comprehensive Review on the Genetics and Metabolomics, Imaging Methods and Management of Common Clinical Variants

“…Of note, PMVK and MVK mutations were demonstrated to be linked to Th17-dependent responses in LP [403]. PMVK and MVD have also been targeted in DSAP, which confirms the common genetic background of LP and DSAP, and there are multiple cases in the literature where both disorders coexist [5,7,9,[45][46][47][48][49][50][51][52][53][54][55][56][57]. The timing of a second-hit mutation may play a vital role in the presentation of the lesions, whether they appear in childhood (LP) or later on (DSAP) [401].…”

“…DSAP occasionally coexists with LP as a form of type 2 segmental involvement [1,45,46]. Common coexistence with LP (multiple cases) [5,7,9,[45][46][47][48][49][50][51][52][53][54][55][56][57] and occasional coexistence with VP (5 cases) [9,[58][59][60][61], PM [62] and PPPD [46] was also reported. Such cases are likely caused by second-hit mutations in the monoallelic gene carriers [63].…”

Autosomal dominant genodermatoses in adults being heralded by superimposed skin lesions in children

“…(Löhrer et al, 2010). Additional examples of such heralding phenomenon are given in Table 1 (Commens & Shumack, 1987; Dover et al, 1986; Feldman et al, 1991; Kaur et al, 2002; Pearson & Cliff, 2003; Suarez‐Amor et al, 2006). Other reports may also belong to this category, but they are less to the point and thus less instructive (Freyschmidt‐Paul et al, 1999; Garg et al, 2011; Lucker & Steijlen, 1994; Machino et al, 1984; Moreland & Wyre, 1981; Palleschi & Torchia, 2008).…”