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Favre-Racouchot Disease Provoked by UV-A1 and UV-B Exposure

Favre-Racouchot Disease Provoked by UV-A1 and UV-B Exposure In 1951, Favre and Racouchot1 described a disease characterized by nodular elastosis with cysts and comedones. It occurs primarily in the periorbital and malar regions, but can occasionally develop in the neck and retroauricular areas. We describe such a case, which could be provoked by UV-A1 and UV-B exposure. To our knowledge, this is the first case with a proven provocation by UV irradiation. Report of a Case A 47-year-old white woman with a previous history of "burned out" discoid lupus erythematosus was treated with systemic corticosteroids until 4 years ago. During the spring, the patient developed comedones and cysts in the face and neck region. Topical treatment with retinoic acid was unsuccessful, and she was referred to our department. On examination, several cysts and comedones were seen on the cheeks, jaw, and neck (Figure 1, A). The skin of the cheeks and in the temples displayed signs of severe elastosis. Histologic examination showed elastosis solaris and no sign of discoid lupus erythematosus. Blood parameters were normal. Tests for antinuclear antibodies were weakly positive and for SS-A and SS-B antibodies were negative. The patient was followed up for 1 year without any treatment and the comedones almost disappeared during winter (Figure 1B). View LargeDownload A, Comedones and cysts in the face and neck region developed during the spring. B, Comedones and cysts disappeared during the winter. C, Comedones and cysts on the right cheek provoked by UV-B irradiation. We undertook a minimal erythema dose (MED) test with both UV-A1 and UV-B. UV-A1 irradiation was provided by Philips TL 10R tubes (350-400 nm) and UV-B irradiation by Philips TL 12 tubes (280-330 nm) (Philips Lighting). The dose that produced a barely perceptible erythema was evaluated 24 hours after exposure. The MED on the back was 2.3 standard erythema dose (SED) (SED = 10 mJ/cm2 at 298 nm using the International Commission on Illumination erythema action spectrum). This dose was used to perform provocation tests for 5 consecutive days on the back without abnormal skin reactions. The patient primarily developed comedones on the face, and we therefore performed provocation tests on facial skin. The right cheek was exposed to UV-B irradiation and the left cheek to UV-A1 irradiation on days 1, 2, 3, 4, 8, 11, 15, 18, and 22. On days 1 through 4 the cheeks were exposed to 2.3 SED and on the remaining days, 3.1 SED of either UV-A1 or UV-B. On day 22, comedones were observed on the right cheek (UV-B provoked) (Figure 1 C) and some small comedones on the left cheek (UV-A1 provoked). During the next weeks the number of comedones increased, particularly on the UV-B–exposed right cheek. Comment Favre-Racouchot disease occurs in approximately 6% of the population aged 40 to 60 years. The pathogenesis of the disease is unclear. However, theories have focused on degeneration of elastic fibers in dermis, allowing the comedones to enlarge. It has been suggested that the UV-induced damage is combined with a particular host susceptibility.2 Our patient had a history of discoid lupus erythematosus and had received systemic corticosteroids for almost 20 years until 4 years ago. Both systemic and topical corticosteroids are known to produce cutaneous atrophy, and it has also been suggested that corticosteroids make the epithelium more vulnerable to comedogenic substances.3 It is thus likely that the long-term treatment with corticosteroids could predispose to Favre-Racouchot disease. Several factors may influence the ductal hypercornification. Sebaceous lipids, in particular free fatty acids and squalene, have been shown to be comedogenic,4 and studies have demonstrated that squalene peroxides formed by UV irradiation are even more comedogenic than squalene itself.5 UV-B irradiation has been found to cause marked hyperplasia of the sebaceous glands with a possible net increase in the sebum production in hairless mice, and possibly also in humans.6 We found that the comedones especially developed on the right cheek exposed to UV-B, rather than on the left cheek exposed UV-A1. In accordance, in winter (low UV-B), the comedones of this patient improved and almost disappeared without treatment. Of importance may also be cycling of normal follicles and of comedones, which may explain the natural resolution of comedones.7 The mechanism behind UV irradiation and comedogenesis is still not clear. However, our observation of a positive provocation test using both UV-A1 and UV-B irradiation, resulting in comedones, confirms in an in vivo human model the relationship between the two. The authors have no relevant financial interest in this article. References 1. Favre MRacouchot J L'élastéidose cutanée nodulaire à kystes et à comédons Ann Dermatol Syphiligr (Paris). 1951;78681- 702Google Scholar 2. Sams Jr WM Sun-induced aging: clinical and laboratory observations in humans Clin Geriatr Med. 1989;5223- 233http://www.ncbi.nlm.nih.gov/htbin-post/Entrez/query?db=m&form=6&Dopt=r&uid=entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_uids=2645999&dopt=AbstractGoogle Scholar 3. Kaidbey KHKligman AM The pathogenesis of topical steroid acne J Invest Dermatol. 1974;6231- 36http://www.ncbi.nlm.nih.gov/htbin-post/Entrez/query?db=m&form=6&Dopt=r&uid=entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_uids=4271838&dopt=AbstractGoogle ScholarCrossref 4. Kligman AMWheatley VRMills OH Comedogenicity of human sebum Arch Dermatol. 1970;102267- 275http://www.ncbi.nlm.nih.gov/htbin-post/Entrez/query?db=m&form=6&Dopt=r&uid=entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_uids=4247928&dopt=AbstractGoogle ScholarCrossref 5. Motoyoshi K Enhanced comedo formation in rabbit ear skin by squalene and oleic acid peroxides Br J Dermatol. 1983;109191- 198http://www.ncbi.nlm.nih.gov/htbin-post/Entrez/query?db=m&form=6&Dopt=r&uid=entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_uids=6223652&dopt=AbstractGoogle ScholarCrossref 6. Lesnik RHKligman LHKligman AM Agents that cause enlargement of sebaceous glands in hairless mice, II: ultraviolet radiation Arch Dermatol Res. 1992;284106- 108http://www.ncbi.nlm.nih.gov/htbin-post/Entrez/query?db=m&form=6&Dopt=r&uid=entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_uids=1610209&dopt=AbstractGoogle ScholarCrossref 7. Cunliffe WJHolland DBClark SMStables GI Comedogenesis: some new aetiological, clinical and therapeutic strategies Br J Dermatol. 2000;1421084- 1091http://www.ncbi.nlm.nih.gov/htbin-post/Entrez/query?db=m&form=6&Dopt=r&uid=entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_uids=10848729&dopt=AbstractGoogle ScholarCrossref http://www.deepdyve.com/assets/images/DeepDyve-Logo-lg.png Archives of Dermatology American Medical Association

Favre-Racouchot Disease Provoked by UV-A1 and UV-B Exposure

Archives of Dermatology , Volume 140 (1) – Jan 1, 2004

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Publisher
American Medical Association
Copyright
Copyright © 2004 American Medical Association. All Rights Reserved.
ISSN
0003-987X
eISSN
1538-3652
DOI
10.1001/archderm.140.1.129
Publisher site
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Abstract

In 1951, Favre and Racouchot1 described a disease characterized by nodular elastosis with cysts and comedones. It occurs primarily in the periorbital and malar regions, but can occasionally develop in the neck and retroauricular areas. We describe such a case, which could be provoked by UV-A1 and UV-B exposure. To our knowledge, this is the first case with a proven provocation by UV irradiation. Report of a Case A 47-year-old white woman with a previous history of "burned out" discoid lupus erythematosus was treated with systemic corticosteroids until 4 years ago. During the spring, the patient developed comedones and cysts in the face and neck region. Topical treatment with retinoic acid was unsuccessful, and she was referred to our department. On examination, several cysts and comedones were seen on the cheeks, jaw, and neck (Figure 1, A). The skin of the cheeks and in the temples displayed signs of severe elastosis. Histologic examination showed elastosis solaris and no sign of discoid lupus erythematosus. Blood parameters were normal. Tests for antinuclear antibodies were weakly positive and for SS-A and SS-B antibodies were negative. The patient was followed up for 1 year without any treatment and the comedones almost disappeared during winter (Figure 1B). View LargeDownload A, Comedones and cysts in the face and neck region developed during the spring. B, Comedones and cysts disappeared during the winter. C, Comedones and cysts on the right cheek provoked by UV-B irradiation. We undertook a minimal erythema dose (MED) test with both UV-A1 and UV-B. UV-A1 irradiation was provided by Philips TL 10R tubes (350-400 nm) and UV-B irradiation by Philips TL 12 tubes (280-330 nm) (Philips Lighting). The dose that produced a barely perceptible erythema was evaluated 24 hours after exposure. The MED on the back was 2.3 standard erythema dose (SED) (SED = 10 mJ/cm2 at 298 nm using the International Commission on Illumination erythema action spectrum). This dose was used to perform provocation tests for 5 consecutive days on the back without abnormal skin reactions. The patient primarily developed comedones on the face, and we therefore performed provocation tests on facial skin. The right cheek was exposed to UV-B irradiation and the left cheek to UV-A1 irradiation on days 1, 2, 3, 4, 8, 11, 15, 18, and 22. On days 1 through 4 the cheeks were exposed to 2.3 SED and on the remaining days, 3.1 SED of either UV-A1 or UV-B. On day 22, comedones were observed on the right cheek (UV-B provoked) (Figure 1 C) and some small comedones on the left cheek (UV-A1 provoked). During the next weeks the number of comedones increased, particularly on the UV-B–exposed right cheek. Comment Favre-Racouchot disease occurs in approximately 6% of the population aged 40 to 60 years. The pathogenesis of the disease is unclear. However, theories have focused on degeneration of elastic fibers in dermis, allowing the comedones to enlarge. It has been suggested that the UV-induced damage is combined with a particular host susceptibility.2 Our patient had a history of discoid lupus erythematosus and had received systemic corticosteroids for almost 20 years until 4 years ago. Both systemic and topical corticosteroids are known to produce cutaneous atrophy, and it has also been suggested that corticosteroids make the epithelium more vulnerable to comedogenic substances.3 It is thus likely that the long-term treatment with corticosteroids could predispose to Favre-Racouchot disease. Several factors may influence the ductal hypercornification. Sebaceous lipids, in particular free fatty acids and squalene, have been shown to be comedogenic,4 and studies have demonstrated that squalene peroxides formed by UV irradiation are even more comedogenic than squalene itself.5 UV-B irradiation has been found to cause marked hyperplasia of the sebaceous glands with a possible net increase in the sebum production in hairless mice, and possibly also in humans.6 We found that the comedones especially developed on the right cheek exposed to UV-B, rather than on the left cheek exposed UV-A1. In accordance, in winter (low UV-B), the comedones of this patient improved and almost disappeared without treatment. Of importance may also be cycling of normal follicles and of comedones, which may explain the natural resolution of comedones.7 The mechanism behind UV irradiation and comedogenesis is still not clear. However, our observation of a positive provocation test using both UV-A1 and UV-B irradiation, resulting in comedones, confirms in an in vivo human model the relationship between the two. The authors have no relevant financial interest in this article. References 1. Favre MRacouchot J L'élastéidose cutanée nodulaire à kystes et à comédons Ann Dermatol Syphiligr (Paris). 1951;78681- 702Google Scholar 2. Sams Jr WM Sun-induced aging: clinical and laboratory observations in humans Clin Geriatr Med. 1989;5223- 233http://www.ncbi.nlm.nih.gov/htbin-post/Entrez/query?db=m&form=6&Dopt=r&uid=entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_uids=2645999&dopt=AbstractGoogle Scholar 3. Kaidbey KHKligman AM The pathogenesis of topical steroid acne J Invest Dermatol. 1974;6231- 36http://www.ncbi.nlm.nih.gov/htbin-post/Entrez/query?db=m&form=6&Dopt=r&uid=entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_uids=4271838&dopt=AbstractGoogle ScholarCrossref 4. Kligman AMWheatley VRMills OH Comedogenicity of human sebum Arch Dermatol. 1970;102267- 275http://www.ncbi.nlm.nih.gov/htbin-post/Entrez/query?db=m&form=6&Dopt=r&uid=entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_uids=4247928&dopt=AbstractGoogle ScholarCrossref 5. Motoyoshi K Enhanced comedo formation in rabbit ear skin by squalene and oleic acid peroxides Br J Dermatol. 1983;109191- 198http://www.ncbi.nlm.nih.gov/htbin-post/Entrez/query?db=m&form=6&Dopt=r&uid=entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_uids=6223652&dopt=AbstractGoogle ScholarCrossref 6. Lesnik RHKligman LHKligman AM Agents that cause enlargement of sebaceous glands in hairless mice, II: ultraviolet radiation Arch Dermatol Res. 1992;284106- 108http://www.ncbi.nlm.nih.gov/htbin-post/Entrez/query?db=m&form=6&Dopt=r&uid=entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_uids=1610209&dopt=AbstractGoogle ScholarCrossref 7. Cunliffe WJHolland DBClark SMStables GI Comedogenesis: some new aetiological, clinical and therapeutic strategies Br J Dermatol. 2000;1421084- 1091http://www.ncbi.nlm.nih.gov/htbin-post/Entrez/query?db=m&form=6&Dopt=r&uid=entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_uids=10848729&dopt=AbstractGoogle ScholarCrossref

Journal

Archives of DermatologyAmerican Medical Association

Published: Jan 1, 2004

Keywords: ultraviolet rays,ultraviolet b radiation

References