Darier’s disease masquerading as eczema: A case report and diagnostic challenges

Darier’s disease (DD) is a rare autosomal dominant genodermatosis that is commonly presented by hyperkeratotic papules and some may have nail dystrophy changes. However, due to its clinical featured resemble common dermatological conditions such as eczema or seborrheic dermatitis, leading to misdiagnosis and prolonged ineffective treatment. We presented a case of 47-year-old man with a history of long-standing pruritic papules affecting the face, neck, upper chest, anterior torso, and lower abdomen include distal nail splitting changes. His first symptoms appear during adolescence, progressively worsened over time and were aggravated by sun exposure, heat, as well as seafood consumption. Despite numerous medical consultations and treatment with topical corticosteroids as well emollients, his condition remained unchanged. A positive family history of similar skin manifestations prompted further evaluation. A skin biopsy was done and confirmed DD based on hallmark histopathological findings, including suprabasal acantholysis, corps ronds, and dyskeratosis. He then started on acitretin 35 mg daily and topical emollients. With a proper targeted treatment his symptoms response well based on eight weeks follow up. This case highlights the diagnostic challenges of DD in primary care and underscores the importance of recognizing its distinctive clinical and histopathological features. Early diagnosis and appropriate management with systemic retinoids and supportive therapy can significantly improve patient outcomes and quality of life.