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. Author manuscript; available in PMC: 2015 Dec 1.
Published in final edited form as: J Invest Dermatol. 2014 Nov 14;135(6):1540–1547. doi: 10.1038/jid.2014.485

Figure 1. Clinical and histologic features of EKVP due to GJA1 mutation.

Figure 1

(a) The knee of 101-1 was normal at birth, but became thickened and scaly when he began to crawl; shown at age 30 months. (b) The knee of 6-year-old 102-1 shows progression to corrugated, thickened, scaly skin which has extended beyond sites of friction. (c) The knee of 30-year-old 103-1 shows marked hyperkeratosis with peeling scale; a patch of figurate erythema is present inferiorly. (d) Histology of affected leg skin of 102-1 shows a compact, thickened stratum corneum (SC) with retained nuclei (arrow), papillomatosis, a thickened granular layer (G), and a perivascular lymphocytic infiltrate (asterisk).