We report the case of a 25-year-old woman who presented to the outpatient dermatology clinic with a history of systemic lupus erythematosus, systemic sclerosis, and primary hypothyroidism. She complained of a one-year history of cutaneous lesions that were pruriginous and evolved into crusts and weeks later resolved with varioliform scarring. Clinicopathological correlation established a diagnosis of acne necrotica varioliformis. This report highlights the clues and pitfalls in its diagnosis and reviews associated systemic diseases.