Abstract

Perforating calcific elastosis, also known as periumbilical perforating pseudoxanthoma elasticum, is a rare, acquired condition associated with the alteration of elastic fibers. It is predominantly seen in middle-aged woman presenting as periumbilical papules. It differs from hereditary pseudoxanthoma elasticum (PXE), which is a systemic disease involving the elastic tissues of the skin, eyes and blood vessels. PXE typically manifests as small, yellow papules on the neck in young adults. The presence of underlying PXE in rare PCE cases necessitates a comprehensive evaluation for systemic disease. We report a case of a 78-year-old woman who presented with mildly painful clusters of 2 to 4 mm periumbilical hyperpigmented papules that had persisted for over a year. Her past medical history was significant for diabetes, hypertension, and non-alcoholic cirrhosis. The patient was initially treated with doxycycline and intralesional steroid injection. However, upon worsening symptoms, a punch biopsy was performed. The histology showed epidermal hyperplasia overlying a calcium deposit surrounded by collections of inflammatory cells and calcified, fragmented elastic fibers. Her complete blood count, metabolic panel including blood glucose, and serum calcium level were unremarkable. The diagnosis of perforating calcific elastosis was made based on the classic clinical presentation and lack of systemic involvement. The lesions partially regressed with intralesional corticosteroids, and the patient reported improvement in symptoms. This case highlights the importance of considering perforating calcific elastosis in the differential diagnosis of periumbilical papules.