Mutation of ATP2C1 on chromosome 3q coding a calcium-dependent ATPase gives rise to calcium dysfunction within keratinocytes, resulting in acantholysis due to a signal transduction disorder. Erythema with erosions appeared 7 months previously when she reported consistent mild diarrhea. No family members had similar lesions. Physical examination revealed clustered white or skin-colored, hard, smooth papules of 0. White lichenoid lesions were found in her gluteal sulcus.
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