Favre-Racouchot syndrome.

A 75-year-old man presented with a peculiar facial appearance, having a diffuse yellowish hue, atrophic skin, and deep wrinkles and furrows involving the entire face, most prominently the forehead, zygomatic, and malar areas [Figure 1]. Multiple large comedones and numerous 2-6 mm sized yellow-to-brown colored papules and nodules were present, clustered in the periocular region and over the dorsum of a patulous nose. The lesions had been present for at least 15 years with gradual coarsening of facial features. Lateral madarosis of both eyebrows was evident, but leprosy was clinically ruled out based on absence of history suggestive of any sensory or motor impairment and lack of cutaneous lesions suggestive of leprosy, Hypoesthesia, thickening of peripheral nerves, any motor deficit or deformity on examination. He also had depigmentation of lower lips but was not bothered by it and did not seek any treatment for the same. The patient was a farmer by occupation with history of significant daily sun exposure and had never followed any specific sun-protection measures or used sunscreens. He had been a chronic heavy smoker with 50 smoking pack years (25 cigarettes per day for 40 years). The patient was totally unconcerned about the facial disfigurement and wanted to consult regarding itching in his groin region, which on examination revealed tinea cruris. Histopathology from the cheek revealed grossly solar damaged epidermis with abundant grey-blue ribbon-like elastotic fibers in upper- and mid-dermis, agglomerating to form elastic globules at places. The upper dermis shows an increased number of thin-walled capillaries and sparse superficial perivascular lymphohistiocytic infiltrate with thinning of reticular dermis [Figure 2]. We diagnosed him as a case of Favre-Racouchot syndrome (FRS) with tinea cruris. Special stains were not done as the histopathology on hematoxylin and eosin staining was characteristic of this syndrome. He was prescribed a 2-week course of twice daily terbinafine cream that completely cleared the fungal infection when examined on follow-up. He refused any treatment for the facial skin.

Figure 1

Deeply wrinkled, furrowed, and atrophic facial skin with multiple comedones, papules, and nodules over the nose, cheeks, and forehead

Figure 2

Histopathology from the cheek revealing sparse superficial perivascular lymphohistiocytic infiltrate with abundant grey blue ribbon like elastotic fibers in upper- and middermis, agglomerating to from elastic globules at places. Overlying epidermis is unaffected. The upper dermis shows an increased number of thin-walled capillaries and reticular dermis appears thinned

FRS, also referred to as senile comedones, solar comedones, and nodular elastosis with cysts and comedones, was originally described in 1932 by Favre and later reviewed in detail by Favre and Racouchot in 1951.[1] Though mostly reported in Caucasian men (with an estimated prevalence of 6% in adults older than 50 years), cases have also been reported in dark-skinned people including Indians, albeit rarely.[2] Cases occurring as early as the second decade of life have also been documented.[3] Males clearly outnumber females, presumably due to more sun-exposure and the effect of smoking.

Clinically, this peculiar syndrome is characterized by a diffuse yellowish hue with the presence of large, open, black comedones symmetrically distributed on the temporal and periorbital areas, and deep wrinkles and furrows over a background of actinically damaged and atrophic skin.[3] In severely affected patients, multiple small papules, nodules and cystic lesions develop, especially involving the periocular region and the nose. Uncommonly, the lateral neck, retroauricular areas, earlobes, and forearms may also be involved. The eruption is usually bilaterally symmetrical. The differential diagnosis includes acne comedones, milia, colloid milium, syringomas, trichoepitheliomas, and sebaceous hyperplasia.[3] Conditions closely linked to FRS include cutis rhomboidalis nuchae which it often accompanies and the actinic comedonal plaque, which is considered to be a variant of FRS.[3] Other conditions reported to be associated with FRS include cutaneous myxoma, actinic keratosis, basal and squamous cell carcinoma, trichostasis spinulosa, keratoacanthoma, and eyelid papilloma.[4]

Diagnosis is primarily clinical and histopathology is rarely required. However, the histologic features of FRS are very characteristic with presence of dilated pilosebaceous openings, atrophic sebaceous glands and large, round cyst-like spaces lined by a flattened epithelium and filled with layered horny material.[5] Evidence of significant solar elastosis is often present with presence of epidermal atrophy and massive basophilic degeneration of the upper dermis.[3] Vellus hair shafts, copious amounts of bacteria, sebum, and eosinophilic laminated horny material have been demonstrated within the cyst-like spaces, suggesting that they actually represent oblique histologic cuts of closed comedones rather than true infundibular cysts.[35] The comedones are colonized with Propionibacterium acnes, Corynebacterium acnes, Staphylococcus albus, and Malassezia yeasts. Inflammation is conspicuously absent.

Though the precise pathogenesis of FRS remains unclear, implicated factors include chonic exposure to ultraviolet radiation and heavy smoking, which may act synergistically. Additionally, radiation therapy has been linked to development of FRS in at least three reported cases till now.[35]

Measures to halt the progression of the disease include meticulous sun-protective measures like avoiding outdoors activities during peak hours of sun-exposure and the use of broad spectrum sunscreens.[4] Cessation of smoking is strongly advised. Isolated use of pharmacologic treatments or surgical techniques has so far resulted in unsatisfactory results. For best therapeutic outcome, one needs to target the solar elastosis, nodulocystic lesions, and the small as well as large comedones that require mechanical removal. Thus, combining medical and surgical treatments seems to be the best approach at present. Owing to their exfoliative and collagen remodeling properties, topical retinoids constitute the mainstay of medical treatment. Their regular use results in expulsion of small comedones and improvement in photodamaged skin. Daily oral Isotretinoin (0.05-0.1 mg/kg/day) used in conjunction with topical tretinoin has also been found effective.[3] Surgical techniques include comedone extraction, curettage, simple or multiple-stage excision, dermabrasion, and laser resurfacing.[4] The most recent addition to the therapeutic armamentarium of FRS is the combination of superpulsed carbon dioxide laser to vaporize the epidermis followed by extraction of cystic and comedonic material with a pair of forceps, with the entire procedure performed without anesthesia. After an initial encouraging outcome observed in two cases of unilateral FRS, Mavilia et al.,[6] reported excellent cosmetic results, rapid recovery, and good safety profile of this combination therapy in 50 patients of FRS with Fitzpatrick skin type III, and have suggested that it may become the treatment of choice for FRS in future. The prognosis of this condition is excellent if the patient is properly counseled and treated.