Favre racouchot syndrome

Favre-Racouchot syndrome also called Favre-Racouchot disease or nodular cutaneous elastosis with cysts and comedones, is a skin disease characterized by coexistence of cysts, comedones and elastotic nodules in actinically damaged skin, typically on the face ¹. Favre Racouchot syndrome lesions are typically restricted to the periorbital area, however reports of similar findings in periocular region ² and atypical locations such as forearms and chest have been made ³. Ultraviolet radiation plays a significant role in the development of the Favre-Racouchot disease. Unilateral manifestation of Favre-Racouchot disease has been described previously ⁴ and was attributed to prolonged occupational unilateral sun exposure or following radiation therapy ⁵.

Favre-Racouchot syndrome is a slowly progressive disease estimated to occur in 1,4% of the general population ⁶ and in 6% of fair-skinned men older than 50 years of age ⁷. Favre Racouchot syndrome is commonly associated with other dermatoses including actinic keratosis and cutis rhomboidalis nuchae ¹.

Favre-Racouchot most commonly presents in elderly white men with a history of long-term sun exposure, heavy smoking and, although rare, a history of radiation exposure ⁸. These risk factors are being shared by squamous cell carcinoma (SCC), the second most common skin cancer accounting for 10-20% of all cutaneous carcinomas ¹.

Histologically, Favre Racouchot syndrome lesions demonstrate significant solar elastosis with epidermal atrophy and basophilic degeneration of the upper dermis. Sebaceous glands typically show atrophy or are absent.

In treating Favre-Racouchot syndrome patient, different approaches, e.g., medication and surgery, can be employed ⁹. Topical retinoids, such as tretinoin, adapalene, or tazarotene, are the most effective pharmacologic treatments ¹⁰. Surgical techniques include excision, dermabrasion, curettage ¹¹, comedone extraction, and laser resurfacing. Although these techniques have yielded poor results when used independently, they provide patients with Favre-Racouchot syndrome a very favorable outcome when used in conjunction with medication ¹⁰.

Figure 1. Favre Racouchot disease

Footnote: Several open comedones are located in the infraorbital regions. The open comedones tip of each horny plug is blackish-grey.

Favre Racouchot syndrome causes

The exact pathogenesis of Favre‐Racouchot syndrome remains obscure but extensive chronic sun exposure is thought to be largely responsible. Heavy smoking ¹² and radiation therapy are other reported predisposing factors. One theory is that sun exposure induces collagen degeneration and sebum retention ¹³. Ultraviolet light exposure may also produce increased free fatty acids and squalene, as well as squalene peroxidases, which may have comedogenic effects. In particular, ultraviolet-B light may promote sebaceous hyperplasia and increased sebum formation.

There may also be a genetic predisposition to this condition.

Medications have not been reported to exacerbate or cause Favre Racouchot syndrome.

Favre Racouchot syndrome symptoms

Clinical presentation consists of a slow and gradual development of 1-2mm non-inflammatory open and closed comedones, nodules, and cysts on a background of photodamaged and poikilodermatous skin (i.e. atrophic or thickened with a diffusely pale to yellowish hue, fine and/or deep rhytides and telangiectasia) on the face and neck (Figure 1).

The periocular regions, malar eminence, temples, retroauricular and posterior and lateral neck (often in conjunction with cutis rhomboidalis nuchae) are most often affected. The lesions are bilateral unless there are specific circumstances where only one side of the face was sun-exposed (e.g. truck driver).

An uncommon related clinical and histologic variant of Favre Racouchot syndrome is actinic comedonal plaque, which consists of a pink to bluish plaque with a cribriform appearance and clusters of deep open comedones, typically found on the flexural forearm. It is histologically indistinguishable from Favre Racouchot syndrome. Other signs of actinic damage such as cutis rhomboidalis nuchae, actinic keratoses, and basal cell or squamous cell carcinoma can accompany Favre Racouchot syndrome.

Favre Racouchot syndrome diagnosis

Histopathologically, comedones of Favre Racouchot syndrome appear as dilated pilosebaceous infundibulum filled with lamellar keratin and lined with flattened squamous epithelium with an extension to the surface. Often evident is a background of solar elastosis with epidermal atrophy and basophilic degeneration of the upper dermis, blue-gray discoloration of elastic fibers, as well as smaller, less numerous or absent sebaceous glands. Early Favre Racouchot syndrome may show amorphous basophilic staining and elastic fiber hyperplasia, while later stages can be accompanied by dense collections of hypertrophic and entangled elastic fibers.

Favre Racouchot syndrome treatment

The treatment of choice is manual extraction, followed by topical retinoids or their derivatives. Large comedones require manual extraction and are unlikely to respond to topical treatment alone.

Retinoids work by normalizing keratinization, promoting exfoliation and comedone extraction, and improving dermal elastic fiber and collagen synthesis. Examples of such products include topical tretinoin, retinoic acid, and retinaldehyde (precursor to retinoic acid, considered less irritating).

Topical therapies require several months of continuous use before improvement is noted. A suggested regimen would be tretinoin 0.04% gel daily, and if tolerated, the strength may be increased to 0.1%. Retinoids in a cream-based vehicle or retinaldehyde are recommended for more sensitive skin.

Other alternative therapies include manual extraction or surgical excision alone (for a small number of lesions), chemical peels, dermabrasion, and resurfacing lasers, with or without topical retinoids. A carbon dioxide laser has been used safely and effectively using two initial passes, followed by manual extraction of comedones, and then a third pass in fifty patients with Fitzpatrick skin type III, with no evidence of relapse 15 to 21 months later ¹⁴. Oral isotretinoin given as 0.05 to 1mg/kg/day over 4 to 5 months, with or without topical retinoids may also be used.

References

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