DP15 A case series of four patients with elastosis perforans serpiginosa with differing predisposing factors

Abstract

Elastosis perforans serpiginosa (EPS) is a rare skin disorder first described in 1953. It is characterized by transepithelial elimination of elastin fibres and focal dermal elastosis. Noted associations with EPS include connective-tissue diseases such as cutis laxa, pseudoxanthoma elasticum, Ehlers–Danlos syndrome (EDS), Marfan syndrome and systemic sclerosis, trisomy 21, Rothmund–Thompson syndrome and medications such as D-penicillamine. The exact pathomechanism of EPS is unknown. We present a series of four patients in our department with EPS of differing aetiologies. The first patient was a 16-year-old girl with a background medical history of trisomy 21, psoriatic arthritis and scalp psoriasis. Regular medications were notable for tofacitinib to treat her psoriatic arthritis. On examination, she had an annular rash with peripheral studded keratotic papules. Histology revealed prominent degenerative elastic fibres with aggregates of neutrophils in the follicles, consistent with EPS. The second patient was a 34-year-old man who presented with a 4-year history of asymptomatic annular keratotic plaques in his axillae. His medical history was significant for Wilson disease treated with penicillamine. Histology revealed a florid increase of deep dermal coarse elastic fibres with extension into superficial hair follicles. No transepidermal expulsion of elastic fibres was noted. Clinicopathological correlation was consistent with EPS. The third patient was a 17-year-old boy who presented with a 3-year history of a rash on his neck. He had no medical history and took no regular medications. Histology was consistent with EPS. The fourth patient was a 28-year-old woman who was attending dermatology for management of hidradenitis suppurativa. She had a background history of trisomy 21 and had a history of EPS diagnosed in the paediatric setting. This series highlights the variety of presentations of EPS and the importance of considering it as a differential diagnosis, especially in patients with comorbidities that predispose them to EPS.