Multiple arcuate lesions over neck of an adult: a case of atypical Elastosis Perforans Serpiginosa

Shrayan Pal, Department of Dermatology, Venereology and Leprosy, ICARE Institute of Science and Research, Haldia, West Bengal, India Swarnali Maiti, Department of Dermatology, Venereology & Leprosy, Barasat Government Medical College, Kolkata, West Bengal, India

Elastosis perforans serpiginosa (EPS) is a rare disorder of the skin, usually present in early adulthood over the neck, upper arms, or face, associated with various genetic, cardiac, and renal comorbidities. A 38-year-old male presented with multiple painless, intensely itchy lesions over the neck for 6 months with insidious onset and gradually progressive number. There was no significant drug history or family history, or associated comorbidities or history of any intellectual disability. On examination, multiple non-tender arcuate plaques were present over the nape of the neck, without any scaling or ulceration. Due to a diagnostic dilemma, a biopsy was done, which showed elongated rete ridges forming a channel containing basophilic debris with numerous thick twisted wavy fibers with lymphocytic infiltrates. Verhoeff-Van Gieson staining came positive for elastic fibers. Hence, the case was diagnosed as idiopathic elastosis perforance serpiginosa. Lesions resolved after application of 0.05% tretinoin cream for 3 months. EPS is an uncommon disease of childhood or early adulthood, usually asymptomatic and associated with various genetic, cardiac, or renal comorbidities or drug intake. But here, lesions appeared at late adulthood, were intensely pruritic with no associated factors. Hence, the case was atypical in terms of onset, symptoms, and causal association; hence presented here.

Keywords: Elastosis perforans serpiginosa. Arcuate. Elastin. Verhoeff-Van Gieson. Basophilic debris.