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

Robert P Feinstein, MD, Associate Clinical Professor, Department of Dermatology, Columbia University College of Physicians and Surgeons

Updated: Feb 19, 2009

Introduction

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 Favre1 and reviewed in detail by Favre and Racouchot in 1951.2

Pathophysiology

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

Frequency

United States

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

Mortality/Morbidity

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

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.

Clinical

History

The patient will relate considerable sun exposure over a long period of time. Recent evidence suggests that the association with smoking is even stronger than the association with sun exposure. Rarely, the patient will mention a past experience of radiation therapy.

Physical

Multiple open and closed comedones are present in the periorbital and temporal areas. Rarely, the lateral neck, postauricular areas, and forearms may be involved. Marked actinically 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.4

Causes

Although the pathogenesis of the disorder is unknown, it develops in individuals with a heavy smoking history and chronic exposure to UV light.3 The disorder also may follow exposure to radiation therapy.5,6

Differential Diagnoses

Acne Vulgaris
Colloid Milium
Milia
Sebaceous Hyperplasia
Syringoma
Trichoepithelioma

Other Problems to Be Considered

Actinic granuloma
Chronic exposure to pitch tar7
Chloracne
Cutis rhomboidalis nuchae
Vellus hair cyst8

Workup

Laboratory Studies

The clinical findings of multiple open and closed comedones with yellowish nodules of elastotic material in a middle-aged to elderly individual are sufficient to establish the diagnosis. In the absence of elastotic nodules, the differential diagnosis includes chloracne. History and porphyrin studies could be helpful.

Histologic Findings

The lesions appear identical to the comedones seen in acne vulgaris. However, the surrounding dermis contains significant actinic elastosis, including nodules of elastin. Although the disorder has been described as containing cysts, many of the apparent cysts really are nodules of elastotic material.9 Gram-positive bacteria that stain periodic acid-Schiff positive have been noted.10 The bacteria are probably Propionibacterium acnes. Hair shafts may or may not be found within the comedones.

Treatment

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 topical retinoids including tretinoin,11 adapalene, or tazarotene in various bases will loosen the comedones and assist in the extraction of individual comedones.

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.12
  • Dermabrasion of the affected area has been reported to produce variable cosmetic results.13
  • The comedones can be removed via careful curettage with good cosmetic results.14
  • 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.

Medication

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

Retinoids

Treatment of choice in the elimination of comedones. These medications assist in the removal of the comedones and also 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.


Tretinoin (Avita, Retin-A)

Inhibits microcomedo formation and eliminates lesions present. Makes keratinocytes in sebaceous follicles less adherent and easier to remove. Available as 0.025, 0.05, and 0.1% creams. Available also 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.

Dosing

Adult

Apply topically twice per wk; if no irritation occurs, may apply more frequently (as often as qd); lower frequency of application if irritation develops

Pediatric

<12 years: Not established
>12 years: Administer as in adults

Interactions

Toxicity increases with coadministration of benzoyl peroxide, salicylic acid, and resorcinol; avoid topical sulfur, resorcinol, salicylic acid, other keratolytics, abrasives, astringents, spices and lime

Contraindications

Documented hypersensitivity

Precautions

Pregnancy

C - Fetal risk revealed in studies in animals but not established or not studied in humans; may use if benefits outweigh risk to fetus

Precautions

Photosensitivity may occur with excessive sunlight exposure; caution in eczema; do not apply to mucous membranes, mouth, and angles of nose


Adapalene (Differin)

Modulates cellular differentiation, inflammation, and keratinization. May be tolerated by individuals who cannot tolerate tretinoin creams. A therapeutic response can be expected following 8-12 wk of therapy. Available as 0.1% gel or solution.

Dosing

Adult

Apply topically twice/wk, then more frequently as tolerated

Pediatric

Not established

Interactions

None reported

Contraindications

Documented hypersensitivity

Precautions

Pregnancy

C - Fetal risk revealed in studies in animals but not established or not studied in humans; may use if benefits outweigh risk to fetus

Precautions

Avoid contact with mucous membranes, eyes, mouth, and nostrils; avoid exposure to sunlight and sunlamps; dryness of skin, scaling, erythema, burning, and pruritus may occur


Tazarotene (Tazorac)

Retinoid prodrug whose active metabolite modulates differentiation and proliferation of epithelial tissue; may also have anti-inflammatory and immunomodulatory properties.

Dosing

Adult

Apply topically twice/wk; not to exceed >20% of BSA; then more frequently as tolerated

Pediatric

<12 years: Not established
>12 years: Administer as in adults

Interactions

None reported

Contraindications

Documented hypersensitivity

Precautions

Pregnancy

X - Contraindicated; benefit does not outweigh risk

Precautions

May cause burning or stinging sensations; discontinue if excessive irritation; rinse thoroughly if contact with eyes, eyelids, or mouth; may cause severe irritation in eczematous skin; photosensitivity may occur


Isotretinoin (Accutane)

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.
Decreases sebaceous gland size and sebum production. May inhibit sebaceous gland differentiation and abnormal keratinization.
Should only be prescribed by individuals who are completely familiar with 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.

Dosing

Adult

0.05-2 mg/kg/d PO

Pediatric

Not established

Interactions

Toxicity may occur with vitamin A coadministration; pseudotumor cerebri or papilledema may occur when coadministered with tetracyclines; may reduce plasma levels of carbamazepine; in diabetes, isotretinoin may cause alterations in blood glucose; corticosteroids, if used with isotretinoin, may produce hyperlipidemia and benign intracranial hypertension; with methotrexate, may increase risk of liver toxicity; with alcohol, may produce a disulfiramlike reaction or cause elevations in serum triglycerides; with trimethoprim and sulfamethoxazole, may increase risk of intracranial hypertension

Contraindications

Documented hypersensitivity; pregnancy

Precautions

Pregnancy

X - Contraindicated; benefit does not outweigh risk

Precautions

Advise against pregnancy while taking isotretinoin and for at least 1 mo after discontinuing medication; women should use at least 2 effective forms of birth control (an oral contraceptive plus another effective contraceptive); perform pregnancy tests prior to institution of therapy and then monthly; tests must be sensitive and performed within 1 wk of starting drug; start drug on 2nd or 3rd day of menstrual period; repeat pregnancy test monthly until 1 mo after drug is stopped; obtain baseline CBC count, LFTs, fasting triglycerides, and cholesterol prior to institution of therapy with isotretinoin and q2-4wk until response is established

Follow-up

Further Outpatient Care

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 precancers in view of the association with actinic damage.

Prognosis

Prognosis is excellent, if properly treated.

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.

Miscellaneous

Medicolegal Pitfalls

It is essential for the physician to appreciate the significance of chronic actinic damage and the potential for skin cancers and precancers.

References

  1. Favre M. Sur une kystique des appareils pilo-sebaces localis ertaines r'ons de la face. Bull Soc Fr Dermatol Syph. 1932;39:93-6.

  2. Favre M, Racouchot J. [Nodular cutaneous elasteidosis with cysts and comedones.]. Ann Dermatol Syphiligr (Paris). Nov-Dec 1951;78(6):681-702. [Medline].

  3. Keough GC, Laws RA, Elston DM. Favre-Racouchot syndrome: a case for smokers' comedones. Arch Dermatol. Jun 1997;133(6):796-7. [Medline].

  4. Patterson WM, Fox MD, Schwartz RA. Favre-Racouchot disease. Int J Dermatol. Mar 2004;43(3):167-9. [Medline].

  5. Breit S, Flaig MJ, Wolff H, Plewig G. Favre-Racouchot-like disease after radiation therapy. J Am Acad Dermatol. Jul 2003;49(1):117-9. [Medline].

  6. Friedman SJ, Su WP. Favre-Racouchot syndrome associated with radiation therapy. Cutis. Mar 1983;31(3):306-10. [Medline].

  7. Adams BB, Chetty VB, Mutasim DF. Periorbital comedones and their relationship to pitch tar: a cross-sectional analysis and a review of the literature. J Am Acad Dermatol. Apr 2000;42(4):624-7. [Medline].

  8. Morgan MB, Stevens GL, Somach S. Multiple follicular cysts, infundibular type with vellus hairs and solar elastosis of the ears: a new dermatoheliosis?. J Cutan Pathol. Jan 2003;30(1):29-31. [Medline].

  9. Lewis KG, Bercovitch L, Dill SW, Robinson-Bostom L. Acquired disorders of elastic tissue: part I. Increased elastic tissue and solar elastotic syndromes. J Am Acad Dermatol. Jul 2004;51(1):1-21; quiz 22-4. [Medline].

  10. Sanchez-Yus E, del Rio E, Simon P, Requena L, Vazquez H. The histopathology of closed and open comedones of Favre-Racouchot disease. Arch Dermatol. Jun 1997;133(6):743-5. [Medline].

  11. Kligman AM, Plewig G, Mills OH Jr. Topically applied tretinoin for senile (solar) comedones. Arch Dermatol. Oct 1971;104(4):420-1. [Medline].

  12. Sharkey MJ, Keller RA, Grabski WJ, McCollough ML. Favre-Racouchot syndrome. A combined therapeutic approach. Arch Dermatol. May 1992;128(5):615-6. [Medline].

  13. English DT, Martin GC, Reisner JE. Dermabrasion for nodular cutaneous elastosis with cysts and comedones. Favre-Racouchot syndrome. Arch Dermatol. Jul 1971;104(1):92-3. [Medline].

  14. Mohs FE, McCall MW, Greenway HT. Curettage for removal of the comedones and cysts of the Favre-Racouchot syndrome. Arch Dermatol. May 1982;118(5):365-6. [Medline].

  15. Jansen T, Plewig G. Favre-Racouchot Syndrome. Clin Dermatol. 1975;Unit 4-44:1-4.

  16. Plewig G, Kligman AM. Acne and Rosacea. Munich, Germany: Universitat Munchen; 1993:79-81, 83, 93, 97, 113, 119, 121, 129, 396.

Keywords

l'élasteïdose cutanée nodulaire à kystes et à comédons, nodular cutaneous elastoidosis with cysts and comedones, senile comedones, solar comedones, smoker's comedones

Contributor Information and Disclosures

Author

Robert P Feinstein, MD, Associate Clinical Professor, Department of Dermatology, Columbia University College of Physicians and Surgeons
Robert P Feinstein, MD is a member of the following medical societies: American Academy of Dermatology, American Medical Association, Noah Worcester Dermatological Society, and Phi Beta Kappa
Disclosure: Nothing to disclose.

Medical Editor

James W Patterson, MD, Director of Dermatopathology, Professor of Pathology and Dermatology, Departments of Pathology and Dermatology, University of Virginia Medical Center
James W Patterson, MD is a member of the following medical societies: American Academy of Dermatology, American College of Physicians, American Medical Association, American Society of Dermatopathology, Medical Society of Virginia, Royal Society of Medicine, Society for Investigative Dermatology, and United States and Canadian Academy of Pathology
Disclosure: Nothing to disclose.

Pharmacy Editor

David F Butler, MD, Professor of Dermatology, Texas A&M University College of Medicine; Chair, Department of Dermatology, Director, Dermatology Residency Training Program, Scott and White Clinic
David F Butler, MD is a member of the following medical societies: Alpha Omega Alpha, American Academy of Dermatology, American Medical Association, American Society for Dermatologic Surgery, American Society for MOHS Surgery, Association of Military Dermatologists, and Phi Beta Kappa
Disclosure: Nothing to disclose.

Managing Editor

Edward F Chan, MD, Clinical Assistant Professor, Department of Dermatology, University of Pennsylvania School of Medicine
Edward F Chan, MD is a member of the following medical societies: American Academy of Dermatology, American Society of Dermatopathology, and Society for Investigative Dermatology
Disclosure: Nothing to disclose.

CME Editor

Catherine Quirk, MD, Clinical Assistant Professor, Department of Dermatology, Brown University
Catherine Quirk, MD is a member of the following medical societies: Alpha Omega Alpha and American Academy of Dermatology
Disclosure: Nothing to disclose.

Chief Editor

William D James, MD, Paul R Gross Professor of Dermatology, University of Pennsylvania School of Medicine; Vice-Chair, Program Director, Department of Dermatology, University of Pennsylvania Health System
William D James, MD is a member of the following medical societies: American Academy of Dermatology and Society for Investigative Dermatology
Disclosure: elsevier Royalty Other; american college of physicians Honoraria Other

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