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September 1985

Ichthyosis Follicularis With Alopecia and Photophobia

Author Affiliations

From the Divisions of Dermatology (Drs Eramo and Esterly), Ambulatory Pediatrics (Dr Zieserl), and Ophthalmology (Dr Stock), Department of Pediatrics and Pediatric Surgery, Northwestern University Medical School, Chicago, and the Division of Genetics, Milwaukee Children's Hospital, Milwaukee (Dr Herrmann).

Arch Dermatol. 1985;121(9):1167-1174. doi:10.1001/archderm.1985.01660090081019

• We treated two unrelated boys with ichthyosis follicularis, a rare skin disorder characterized by extensive noninflammatory spiny follicular hyperkeratoses, severe photophobia, and generalized noncicatricial alopecia. This disorder must be differentiated from keratosis follicularis spinulosa decalvans; ulerythema ophryogenes; keratosis pilaris rubra atrophicans faciei; atrichia with papular lesions; atrophodermia vermiculata; and keratitis, ichthyosis, and deafness syndrome, all of which share some clinical features. Ichthyosis follicularis with alopecia and photophobia appears to be a familial disorder, but too few cases have been reported to establish the exact mode of inheritance.

(Arch Dermatol 1985;121:1167-1174)