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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 3. Figurate erythema and darkening of the skin in EKVP due to GJA1 mutation.

Figure 3

All subjects report figurate erythema and have experienced progressive skin darkening. (a) In subject 101-1, figurate erythema is present on the back, flank, arm, and upper thigh. (b) Subject 102-1 shows thick, corrugated keratoderma with darkening prominent on the neck, forearm, axilla, and abdomen. There is a patch of erythema on the wrist. (c) In subject 103-1, figurate erythema is present on the upper and lower back. Notably, subject 103-1 reports that figurate erythema became more frequent and prominent at age 10 years but was present throughout childhood. Treatment with acitretin has reduced scaling, but hyperpigmentation is prominent on the flanks, shoulders, and neck. In all cases, erythema is induced by stress and warm conditions and can be accompanied by a stinging or burning sensation.