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Favre-Racouchot syndrome

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A nodular cutaneous elastosis characterized by cysts and comedones on actinically damaged skin, typically on the face and less commonly on the neck.

Estimated to occur in 1.4% of the adult population, and up to 6% of white men older than 50 years.

May occur in women and dark skinned individuals, as well.

Middle-aged to elderly individuals are most commonly affected.

Cases have occurred in the second decade have been reported.

Chronic exposure to UV light and heavy cigarette smoking are important triggers.

Chronic exposure of UV light to the mid and upper dermis reduces tensile strength and may cause retention of the sebum, leading to the formation of comedones, cysts and nodules.

Chronic smoking may potentiate the effect of solar damage to the skin.

A nodular cutaneous elastosis that is characterized by cysts and comedones on actinically damaged skin, typically on the face and less commonly on the neck.

The skin is solar-damaged and often appears dull, yellowish, thickened, wrinkled, and furrowed.

In severely affected patients confluent, multiple papules, cysts, and nodules may be present.

The prevalence is high in white men.

The process has been observed in women and dark-skinned individuals.

Middle-aged to elderly individuals are most commonly affected.

Cases occurring as early as the second decade of life have also rarely been reported.

Chronic exposure to UV light and heavy cigarette smoking are important triggers.

Chronic exposure to UV light damages the elastic network of the mid and upper dermis reducing its tensile strength, and may cause retention of the sebum, leading to the formation of comedones, cysts, and nodules.

Chronic smoking may increase the effect of solar damage to the affected skin.

Rarely, may follow exposure to radiation therapy.

Histologic findings include: solar elastosis, epidermal atrophy, basophilic degeneration of the upper dermis, dilated pilosebaceous infundibulum filled with lamellar keratin, and atrophic sebaceous glands.

Patients with Favre-Racouchot syndrome present with multiple large comedones on actinically damaged skin.

The comedones may be whiteheads or, more commonly, blackheads.

The comedones are absent inflammation.

The lesions usually symmetrically affect the cheeks, forehead, periorbital area, and/or temples, and sometimes the neck.

Unilateral and circumscribed forms have been reported, presumably as a result of asymmetric solar exposure or radiation.

Other conditions associated: actinically damaged skin include actinic keratosis, cutis rhomboidalis nuchae, trichostasis spinulosa, keratoacanthoma, basal cell carcinoma, and squamous cell carcinoma.

Diagnosis is usually clinical, based on the typical appearance of the lesions and typical locations.

A skin biopsy is seldom necessary for diagnosis.

The differential diagnosis includes acne vulgaris, sebaceous hyperplasia, milia, epidermoid cysts, chloracne, syringomas, and trichoepitheliomas.

Avoidance of sun exposure, regular use of broad-spectrum sunscreens, and wearing of protective clothes when outdoors and smoking cessation should be emphasized.

Topical retinoids such as tretinoin, adapalene, and tazarotene are the mainstay of medical treatment.

Topical retinoids work by repairing collagen and remodeling elastin in the dermis.

Topical retinoids may decrease the activity of matrix metalloproteinases within the skin that may further decrease collagen breakdown.

Other treatments include curettage, excision, comedo extraction, dermabrasion, deep chemical peels, and laser ablation.

Combining medical and surgical treatments yields optimal outcomes.

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