A Case of Recurrent Caffey's Disease Treated With Naproxen

Abstract

Caffey's disease (infantile cortical hyperostosis) is considered to be a benign self-limiting disease of uncertain etiology that typically appears in early infancy. The following case of Caffey's disease in a young girl is significant from 2 standpoints. First, the course of the disease is atypical because of its severe recurrent nature. The patient experienced 6 highly symptomatic episodes of cortical hyperostosis involving multiple new sites and previously healed lesions. These recurrent episodes persisted into her fourth year of life. In general, the reported cases of late recurrence of Caffey's disease describe patients with minimal symptoms and mild bony involvement. The second area of significance involves the successful use of naproxen to control the symptoms and arrest the progression of the patient's disease. Reports of hyperostosis in patients receiving prostaglandin E to maintain a patent ductus arteriosus suggest that prostaglandins may play a role in the etiology of Caffey's disease. Given this evidence, it seems plausible that there may be a therapeutic role for prostaglandin inhibitors in the successful treatment of Caffey's disease. The patient in this case had immediate and complete resolution of her symptoms while receiving naproxen with no recurrent bone formation while being treated with antiinflammatory drugs.