Favre–Racouchot syndrome

A 78-year-old male truck driver presented with gradually progressive, asymptomatic, skin lesions on the face of 20 years duration. He was a chronic smoker. He gave a history of chronic sun exposure and denied exposure to any chemicals or radiation. His face showed deep wrinkles, atrophic skin with a diffuse yellowish hue, multiple large comedones, yellow-to-brown-colored papules, and cysts. The lesions were clustered on the cheeks, bridge of the nose, and the zygomatic process [Figure 1]. He also manifested cutis rhomboidalis nuchae. We made a clinical diagnosis of Favre–Racouchot syndrome (FRS).

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Figure 1:

Deep wrinkles, a diffuse yellow hue with atrophic skin, multiple large comedones, yellow-to-brown-colored papules, and cysts clustered on the cheeks, bridge of the nose, and the zygomatic process in a patient with Favre–Racouchot syndrome.

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The exact etiology of FRS remains unclear. The condition is thought to be triggered by chronic sun exposure, smoking, and exposure to radiation. The skin lesions are attributed to the sebum retention and the collagen and elastic degeneration in the upper dermis. Asymptomatic elastotic nodules, large open, black comedones, and cysts on actinically damaged, and atrophic skin characterize the syndrome. The common sites affected are the periorbital and malar areas, nose, and temporal region.

The diagnosis is clinical, which may be confirmed by histopathology. Histopathology findings include epidermal atrophy, basophilic degeneration of upper dermis, elastosis, dilated pilosebaceous opening, atrophic sebaceous gland, and cyst-like spaces lined with flattened epithelium, and absence of dermal inflammation.

Photoprotective measures and avoidance of smoking may be beneficial. The treatment options include extraction of comedones, topical retinoids, surgical excision, salicylic acid peel, dermabrasion, laser resurfacing, and plasma exeresis. Our patient was started on sunscreen and topical retinoids.