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Related to keratosis follicularis: Darier's disease, keratosis pilaris, ichthyosis vulgaris
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Less frequent causes were SLE in 5% cases followed by scleroderma, dermatomyositis, keratosis follicularis spinulosa decalvans, aplasia cutis, kerion, follicular mucinosis, pemphigus, dissecting cellulitis of scalp/ pyogenic folliculitis and acne keloidalis nuchae in 2.
16 Linear or segmental keratosis follicularis (Darier disease) has been shown in some cases to result from genetic mosaicism of ATP2A2.