September 1983

Familial Occurrence of Stevens Johnson Syndrome

Author Affiliations

University of Utah Medical Center Salt Lake City

Am J Dis Child. 1983;137(9):914-916. doi:10.1001/archpedi.1983.02140350086023

Stevens-Johnson syndrome is an illness characterized by systemic toxicity, erythema multiforme skin lesions, and vesicular or bullous eruptions of mucous membranes. Its occurrence is most common in children and young adults,1 and the outcome, especially if the eyes are involved or if infection is superimposed, may include permanent disability or death. The precise causal and pathogenetic mechanisms involved in this syndrome are not well understood. Familial occurrence of Stevens-Johnson syndrome is rare. The twins described here had a contributory family history for an allergic drug reaction and for Stevens-Johnson syndrome (Figure). These cases represent the unusual familial incidence of Stevens-Johnson syndrome and suggest that genetic predisposition might be an important causal factor.

Report of Cases.—Case 1.—A 3-year-old female firstborn twin was seen at the University of Utah Medical Center, Salt Lake City, with fever, severe skin rash, and poor oral intake. Twelve days before admission, she was