You are in: eMedicine Specialties > Dermatology > PHOTO-RELATED DISEASES Favre-Racouchot Syndrome (Nodular Elastosis With Cysts and Comedones)Article Last Updated: Dec 5, 2006AUTHOR AND EDITOR INFORMATIONAuthor: Robert P Feinstein, MD, Associate Clinical Professor, Department of Dermatology, Columbia University College of Physicians and Surgeons Robert P Feinstein is a member of the following medical societies: American Academy of Dermatology, American Medical Association, Noah Worcester Dermatological Society, and Phi Beta Kappa Editors: James W Patterson, MD, Director of Dermatopathology, Professor of Pathology and Dermatology, Departments of Pathology and Dermatology, University of Virginia Medical Center; David F Butler, MD, Professor of Dermatology, Texas A&M University College of Medicine; Director, Division of Dermatology, Scott and White Clinic; Director Dermatology Residency Training Program, Scott and White Clinic; Edward F Chan, MD, Clinical Assistant Professor, Department of Dermatology, University of Pennsylvania School of Medicine; Catherine Quirk, MD, Clinical Assistant Professor, Department of Dermatology, Brown University; 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 Author and Editor Disclosure Synonyms and related keywords: l'élasteïdose cutanée nodulaire à kystes et à comédons, nodular cutaneous elastoidosis with cysts and comedones, senile comedones, solar comedones, smoker's comedones INTRODUCTIONBackgroundFavre-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 and reviewed in detail by Favre and Racouchot in 1951. PathophysiologyThis syndrome is limited to the skin. No internal manifestations occur. FrequencyUnited StatesIn the United States and worldwide, this disorder has been reported to occur in 6% of adults older than 50 years. Mortality/MorbidityFavre-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. RaceThis disorder is found most commonly in whites, but isolated cases have been reported in dark-skinned people. SexMales are affected much more commonly, but cases have been reported in women. AgeMiddle-aged to elderly individuals mostly are affected, although reports of young adults developing the problem exist. CLINICALHistoryThe 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. PhysicalMultiple 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. CausesAlthough the pathogenesis of the disorder is unknown, it develops in individuals with a heavy smoking history and chronic exposure to UV light. The disorder also may follow exposure to radiation therapy. DIFFERENTIALSAcne Vulgaris Colloid Milium Milia Sebaceous Hyperplasia Syringoma Trichoepithelioma
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| Drug Name | Tretinoin (Avita, Retin-A) |
|---|---|
| Description | 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. |
| Adult Dose | 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 Dose | <12 years: Not established >12 years: Administer as in adults |
| Contraindications | Documented hypersensitivity |
| 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 |
| 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 |
| Drug Name | Adapalene (Differin) |
|---|---|
| Description | 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. |
| Adult Dose | Apply topically twice/wk, then more frequently as tolerated |
| Pediatric Dose | Not established |
| Contraindications | Documented hypersensitivity |
| Interactions | None reported |
| 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 |
| Drug Name | Tazarotene (Tazorac) |
|---|---|
| Description | Retinoid prodrug whose active metabolite modulates differentiation and proliferation of epithelial tissue; may also have anti-inflammatory and immunomodulatory properties. |
| Adult Dose | Apply topically twice/wk; not to exceed >20% of BSA; then more frequently as tolerated |
| Pediatric Dose | <12 years: Not established >12 years: Administer as in adults |
| Contraindications | Documented hypersensitivity |
| Interactions | None reported |
| 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 |
| Drug Name | Isotretinoin (Accutane) |
|---|---|
| Description | 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. |
| Adult Dose | 0.05-2 mg/kg/d PO |
| Pediatric Dose | Not established |
| Contraindications | Documented hypersensitivity; pregnancy |
| 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 |
| 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 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 is excellent, if properly treated.
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.
It is essential for the physician to appreciate the significance of chronic actinic damage and the potential for skin cancers and precancers.
Favre-Racouchot Syndrome (Nodular Elastosis With Cysts and Comedones) excerpt
Article Last Updated: Dec 5, 2006