Background
Favre-Racouchot syndrome was originally described by Favre in 1932 and was reviewed in detail by Favre and Racouchot in 1951.[1] It is a disorder consisting of multiple open and closed comedones in the presence of actinically damaged skin. The syndrome is limited to the skin; no internal manifestations occur.
Treatment may include the use of sunscreen, salicylate or retinoids, comedo extraction, and laser therapy. (See Treatment.)
Etiology
Although the precise pathogenesis of Favre-Racouchot syndrome has not been defined, the disorder is known to develop in individuals with a heavy smoking history and chronic exposure to ultraviolet (UV) light.[2] Vogel et al described a case of unilateral Favre-Racouchot syndrome that supported an etiologic role for chronic solar damage in this disease.[3] Owing to the strong association with smoking, the name "smokers’ comedones" has been proposed.[2] The syndrome may represent a manifestation of chloracne, though both UV and infrared[4] (IR) light exposure may contribute.
The disorder also may follow exposure to radiation therapy (RT).[5, 6] This was illustrated in a paper by Hoff et al, in which a 71-year-old man underwent RT for a malignant melanoma of the right paranasal sinus.[7] He subsequently developed elastosis, open and closed comedones, and cysts in the radiation field.
Lim et al, in a discussion of the adverse effects of UV radiation exposure from the use of indoor tanning equipment, cited Favre-Racouchot syndrome as one of those adverse effects.[8]
Epidemiology
In the United States and worldwide, Favre-Racouchot syndrome has been reported to occur in 6% of adults older than 50 years. Although it is mostly middle-aged to elderly individuals who are affected, reports of the syndrome developing in young adults exist. Males are affected much more commonly, but cases have been reported in women. Favre-Racouchot syndrome is found most commonly in lighter-skinned people; however, isolated cases have occurred in darker-skinned people.
Prognosis
The prognosis is excellent, if the condition is properly treated. Favre-Racouchot syndrome is of cosmetic concern. It is an indication that the individual has had chronic excessive exposure to UV light. It is also strongly associated with heavy cigarette smoking.[2]
Patient Education
Patients must be strongly advised to avoid sun exposure and to use a sunblock daily. Continued UV damage will aggravate the disorder and potentiate the development of precancers and skin cancers.
History
Typically, a patient with Favre-Racouchot syndrome will relate a history of considerable sun exposure over a long period. Heavy smoking is also associated with this syndrome. Rarely, the patient will mention a past experience of radiation therapy (RT).
Physical Examination
Multiple open and closed comedones are present in the periorbital and temporal areas. Rarely, the lateral neck, postauricular areas, and forearms may be involved. Severely actinic damaged skin is seen, with yellowish discoloration, yellowish nodules, atrophy, wrinkles, and furrows. The eruption is usually bilaterally symmetrical, though one side may predominate, particularly if that side experienced greater sun exposure. The comedones of Favre-Racouchot syndrome show no inflammation, unlike the comedones seen in acne vulgaris.[9]
Two cases of an actinic comedonal plaque variant of Favre-Racouchot syndrome were reported by Cardoso et al.[10] This variant presents with papules, cysts, and comedones producing a yellowish plaque in areas of chronic sun-damaged skin. The involvement is usually on the forearms and chest, with ultraviolet (UV) light exposure being the main factor in its pathogenesis. Although it is more common in skin types 1-4, it has been reported in skin of color.[11] Dermatoscopic features of this variant were described by Melo et al.[12]
Laboratory Studies
The clinical findings of multiple open and closed comedones with yellowish nodules of elastotic material in a middle-aged to elderly individual with actinic damage are sufficient to establish the diagnosis, without a requirement for specific laboratory studies.
Histologic Findings
Histologic evaluation typically reveals significant actinic elastosis and epidermal atrophy. Comedones similar to those of acne vulgaris are seen. Although the disorder has been described as containing cysts, many of the apparent cysts are actually nodules of elastotic material.[15] (See the image below.) Gram-positive bacteria that are positive on periodic acid–Schiff staining have been noted.[16] The comedones are likely colonized with Cutibacterium acnes (formerly Propionibacterium acnes), Staphylococcus albus, and Malassezia yeasts. Hair shafts may or may not be found within the comedones.
View Image | Multiple comedones, dilated and plugged follicular infundibula, and epidermal cyst formation. Image from DermNet New Zealand and Pathlab Bay of Plenty.... |
Medical Care
In general, the most effective medical therapy for Favre-Racouchot syndrome has been the use of topical retinoids (eg, tretinoin, adapalene, or tazarotene). Application of salicylic acid or topical retinoids in various bases loosens the comedones and assists in the extraction of individual comedones.[17, 18, 19] More severe cases have been treated with oral isotretinoin, but such therapy should be reserved for severe cases refractory to topical therapy.
Sun protection may be of benefit in preventing progression. The patient should be advised to avoid sun exposure, particularly between 10:00 AM and 2:00 PM. The sunscreen selected should provide good protection throughout the ultraviolet (UV) range (ie, UV-A and UV-B). Sunscreens containing micropulverized titanium dioxide or Parsol 1789 provide the best protection throughout the UV-A range.
Smoking cessation is also critical.
Surgical Care
Comedo extraction is effective. However, the comedones seen in Favre-Racouchot syndrome are more difficult to extract than those seen in acne vulgaris. Concomitant use of topical retinoids makes the extraction process easier.[20]
Dermabrasion of the affected area has been reported to produce variable cosmetic results.[21]
The comedones can be removed via careful curettage with good cosmetic results.[22]
Chemical peels, laser peels, and staged excision of the involved area have been reported, but the cosmetic result has been highly variable.
Treatment with carbon dioxide laser has been reported.[23] In a study that included 50 patients with Favre-Racouchot syndrome, a superpulsed carbon dioxide laser was used to vaporize epidermis, and both cystic and comedonal material was then extracted via soft pressure with a pair of forceps.[24] The laser treatment was performed without any preceding topical or intralesional anesthesia; no pain was reported with the laser parameters described in the study. The technique proved to be safe and effective in all of the patients treated, with an excellent cosmetic result. Rai et al, using a similar approach, reported similar findings.[25]
Long-Term Monitoring
All cases should be followed for evaluation of the effect of topical retinoids and for subsequent removal of remaining comedones. Patients should be carefully observed for the potential development of skin cancers and precancers, in view of the known association with actinic damage.
Author
Robert P Feinstein, MD, FAAD, Associate Clinical Professor, Department of Dermatology, Columbia University Vagelos College of Physicians and Surgeons
Disclosure: Nothing to disclose.
Specialty Editors
Francisco Talavera, PharmD, PhD, Adjunct Assistant Professor, University of Nebraska Medical Center College of Pharmacy; Editor-in-Chief, Medscape Drug Reference
Disclosure: Received salary from Medscape for employment. for: Medscape.
Edward F Chan, MD, Clinical Assistant Professor, Department of Dermatology, University of Pennsylvania School of Medicine
Disclosure: Nothing to disclose.
Chief Editor
Dirk M Elston, MD, Professor and Chairman, Department of Dermatology and Dermatologic Surgery, Medical University of South Carolina College of Medicine
Disclosure: Nothing to disclose.
){kind=link}