ELASTOSIS PERFORANS SERPIGINOSA IN A PATIENT WITH CYSTIC FIBROSIS

Summary: A 24-year-old female with a history of cystic fibrosis and associated co-morbidities presented to the dermatology clinic for a pruritic and painful cutaneous eruption on the upper and lower extremities, trunk and scalp. Examination revealed pink to red papules and plaques with central crusting, many arranged in an arcuate pattern. An initial biopsy showed acanthosis and chronic inflammation. A bacterial culture grew Chryseobacterium. The patient was treated with several courses of Trimethoprim-Sulfamethoxazole with minimal improvement. A punch biopsy performed five months later demonstrated a hyperplastic epidermis with a focal invagination showing transepithelial elimination of abnormal fibers mixed with inflammatory cells and keratinaceous debris. A Modified Masson’s trichrome stain highlighted the perforating fibers as elastic fibers. Additional bacterial and fungal stains and cultures were negative. A diagnosis of elastosis perforans serpiginosa (EPS) was made. EPS is a rare skin condition involving transepithelial elimination of distorted elastic fibers. Clinically it can be misconstrued for an infectious etiology. It is most often associated with inherited connective tissue disorders including Marfan and Ehlers-Danlos syndromes, Down Syndrome and D-penicillamine use; our patient’s history did not reveal any known associations. To our knowledge, EPS has not been widely reported to be associated with cystic fibrosis.