Infantile systemic hyalinosis.

Shin, Helen T; Paller, Amy; Hoganson, George; Willner, Judith P; Chang, Mary Wu; Orlow, Seth J · J Am Acad Dermatol · 2004

case_report · Level V

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Abstract

Infantile systemic hyaloinosis is a rare, progressive, and fatal disease that is inherited in an autosomal recessive fashion. We describe 2 patients in whom thickened skin; small nodules of the perianal region, face, and neck; joint contractures; growth failure; diarrhea; and frequent infections developed within the first few weeks of life. Both patients died before 2 years of age.

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