Sporadic Case of Acrokeratosis Verruciformis of Hopf With Unusual Clinical Presentation: A Case Report and Review of the Literature.

objectiveThis study aims to report a sporadic case of acrokeratosis verruciformis of Hopf (or acrokeratosis verruciformis (AKV)) with an unusual clinical presentation, highlighting its diagnostic challenges and key distinguishing features. CASE REPORT: A 54-year-old female patient with no relevant medical history presented with multiple skin-colored, flat-topped, hyperkeratotic papules on the dorsal aspects of the hands since childhood, and onychopathy for 12 years. Nail changes included thickening of the nail plate and subungual hemorrhages. She presented a soft, warty pink-colored plaque in the right inguinal region. Dermoscopy of the dorsum of the hand showed a white network and papules with a central homogeneous area and a peripheral cobblestone appearance. A biopsy was performed and showed hyperorthokeratosis and epidermal hyperplasia with focal papillomatosis. Treatment was started with keratolytics. The patient has been under follow-up by our department for three months with partial improvement of the lesions. DISCUSSION: AKV, a rare genodermatosis, presents a diagnostic challenge due to its subtle onset, relying primarily on clinical presentation and histopathological characteristics. Genetic studies can aid in identifying underlying mutations, with Darier's disease being the main differential diagnosis. Management typically involves keratolytic agents and retinoids, though superficial ablation is considered first-line treatment.

conclusionAKV should be suspected in patients presenting with numerous flat, skin-colored, polygonal papules on acral areas and long-standing onychopathy. This case emphasizes the critical role of comprehensive clinical assessment and histopathological study for accurate diagnosis, especially given its unusual manifestations.