Favre-Racouchot Syndrome (Nodular Elastosis With Cysts and Comedones)

Background

Favre-Racouchot syndrome is a disorder consisting of multiple open and closed comedones in the presence of actinically damaged skin. The disease was originally described in 1932 by Favre^[1] and reviewed in detail by Favre and Racouchot in 1951.^[2]

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Pathophysiology

This syndrome is limited to the skin. No internal manifestations occur.

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Etiology

Although the precise pathogenesis of the disorder is unknown, it develops in individuals with a heavy smoking history and chronic exposure to UV light.^[3]  Vogel et al described a case of unilateral Favre-Racouchot syndrome indicating the etiologic role of chronic solar damage in this disease.^[4]  Owing to the strong association with smoking, and the name "smokers’ comedones" has been proposed.^[5] It may represent a manifestation of chloracne, although both ultraviolet and infrared light exposure may contribute.

The disorder also may follow exposure to radiation therapy.^{[6, 7]} This was illustrated in a paper by Hoff et al, in which a 71-year-old man treated with radiation therapy for a malignant melanoma of the right paranasal sinus. He subsequently developed elastosis, open and closed comedones, and cysts in the radiation field.^[8]

Lim et al, in a discussion of the adverse effects of UV radiation from the use of indoor tanning equipment, has cited Favre-Racouchot syndrome as one of these adverse effects.^[9]

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Epidemiology

US frequency

In the United States and worldwide, this disorder has been reported to occur in 6% of adults older than 50 years.

Race

This disorder is found most commonly in whites, but isolated cases have been reported in dark-skinned people.

Sex

Males are affected much more commonly, but cases have been reported in women.

Age

Middle-aged to elderly individuals mostly are affected, although reports of young adults developing the problem exist.

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Prognosis

Prognosis is excellent, if 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.^[3]

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Patient Education

Strongly advise the patient to avoid sun exposure and to use a sunblock daily, as continued UV damage will aggravate the disorder and potentiate the development of precancers and skin cancers.

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History

The patient will relate considerable sun exposure over a long period of time. Heavy smoking is also associated with Favre-Racouchot syndrome. Rarely, the patient will mention a past experience of radiation therapy.

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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 with yellowish discoloration, yellowish nodules, atrophy, wrinkles, and furrows are present. The eruption is usually bilaterally symmetrical, although one side may predominate, particularly if that side experienced greater sun exposure. No inflammation is present, unlike the comedones seen in acne vulgaris.^[10]

An actinic comedonal plaque-variant of Favre-Racouchot syndrome has been described. This variant presents with papules, cysts, and comedones producing a yellowish plaque in areas of chronic sun-damaged skin. Two cases from Brazil are presented. The involvement is usually on the forearms and chest, with UV light exposure being the main factor in its pathogenesis.^[11]

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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.

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Histologic Findings

Significant actinic elastosis and epidermal atrophy are usually seen. Comedones similar to those of acne vulgaris are seen. Although the disorder has been described as containing cysts, many of the apparent cysts really are nodules of elastotic material.^[14] Gram-positive bacteria that stain periodic acid-Schiff positive have been noted.^[15] The comedones are likely colonized with Propionibacterium acnes, Corynebacterium acnes, Staphylococcus albus, and Malassezia yeasts. Hair shafts may or may not be found within the comedones.

See the image below.

View Image

Multiple comedones, dilated and plugged follicular infundibula, and epidermal cyst formation. Courtesy of DermNet New Zealand and Pathlab Bay of Plent....

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Medical Care

Sun protection may be of benefit in preventing progression. Advise the patient to avoid sun exposure, particularly between 10:00 am and 2:00 pm. Select sunscreen to provide good protection throughout the UV-A and UV-B range. Sunscreens containing micropulverized titanium dioxide or Parsol 1789 provide the best protection throughout the UV-A range. Smoking cessation also is critical. Application of salicylic acid or topical retinoids including tretinoin, adapalene, or tazarotene in various bases, loosens the comedones and assists in the extraction of individual comedones.^{[16, 17, 18]}

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Surgical Care

Comedo extraction is effective. However, these comedones are more difficult to extract than are those seen in acne vulgaris. The concomitant use of topical retinoids assists in the extraction process.^[19]

Dermabrasion of the affected area has been reported to produce variable cosmetic results.^[20]

The comedones can be removed via careful curettage with good cosmetic results.^[21]

Chemical peels, laser peels, and staged excision of the involved area have been reported, but the cosmetic result is highly variable.

Treatment with carbon dioxide laser has been reported. Mavilia et al evaluated 50 patients with a superpulsed carbon dioxide laser in an attempt to vaporize epidermis.^[22] They then extracted both cystic and comedonal material via soft pressure with a pair of forceps. The laser treatment was performed without topical or intralesional anesthetics, as no pain was reported using the laser parameters they described. The technique proved to be safe and effective in all of the patients treated, assuring an excellent cosmetic result. Rai et al, using a similar approach, confirmed these findings.^[23]

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Long-Term Monitoring

Follow up all cases to note the effect of topical retinoids and for subsequent removal of remaining comedones. Carefully observe the patient to note the development of skin cancers and pre-cancers in view of the association with actinic damage.

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Medication Summary

In general, the most effective medication has been the use of topical retinoids including tretinoin, adapalene, or tazarotene.

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Tretinoin topical (Avita, Retin-A, Retissa, Renova)

• Dosing, Interactions, etc.

Clinical Context:  Tretinoin inhibits microcomedo formation and eliminates lesions. It makes keratinocytes in sebaceous follicles less adherent and easier to remove. Tretinoin is available as 0.025, 0.05, and 0.1% creams and as 0.01 and 0.025% gels. Cream formulations usually are well tolerated. Individuals may begin with the 0.025% cream. Those who do not respond to this concentration may require the 0.05% or 0.1% concentration.

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Adapalene (Differin)

• Dosing, Interactions, etc.

Clinical Context:  Adapalene modulates cellular differentiation, inflammation, and keratinization. It may be tolerated by individuals who cannot tolerate tretinoin creams. A therapeutic response can be expected following 8-12 weeks of therapy. Adapalene is available as 0.1% gel or solution.

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Tazarotene (Tazorac, Avage)

• Dosing, Interactions, etc.

Clinical Context:  Tazarotene is a retinoid prodrug whose active metabolite modulates differentiation and proliferation of epithelial tissue; it may also have anti-inflammatory and immunomodulatory properties.

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Isotretinoin (Amnesteem, Claravis, Sotret)

• Dosing, Interactions, etc.

Clinical Context:  Isotretinoin is an oral agent that treats serious dermatologic conditions. Isotretinoin is the synthetic 13-cis isomer of the naturally occurring tretinoin (trans-retinoic acid). Both agents are structurally related to vitamin A.

Isotretinoin decreases sebaceous gland size and sebum production. It may inhibit sebaceous gland differentiation and abnormal keratinization.

Isotretinoin should only be prescribed by individuals who are completely familiar with the drug and appropriate prescribing practices and precautions. A US Food and Drug Administration–mandated registry is now in place for all individuals prescribing, dispensing, or taking isotretinoin. For more information on this registry, see iPLEDGE. This registry aims to further decrease the risk of pregnancy and other unwanted and potentially dangerous adverse effects during a course of isotretinoin therapy.

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Class Summary

Retinoid-like agents are the treatment of choice in the elimination of comedones. These medications assist in the removal of the comedones and may reduce the appearance of actinic damage in the area. More severe cases have been treated with oral isotretinoin, but oral therapy should be reserved for severe cases refractory to topical therapy.

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