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Xeroderma pigmentosum lesions related to ultraviolet transmittance by clothes
Volume 24 Number 2, Part 2 February 1991
infections associated with a toxic shock-like syndrome and scarlet fever toxin A. N Engl J Med I989;32l:1-7. 7. Baker CJ. Group B streptococcal cellulitis-adenitis in infants. Am J Dis Child 1982;136:631-3. 8. Rouland V, Bouchez MC, Morisot C, et al. Ad6nite et cellulite inguinale ~ streptocoque B chez un nouveau-n6. Arch Fr Pediatr 1987;44:889-92. 9. Crandal JP, Shah BR. Group B streptococcal lymphaden-
itis in a child with AIDS. Clin Pediatr 1988;27:81. 10. Ho DD, Murata GH. Streptococcal lymphadenitis in homosexual men with chronic lymphadenopathy.Am J Med 1984;77:151-3.
Streptococcal adenitis in AIDS I 1. Schaehter J. Lymphogranuloma venereum and other nonocular Chlarnydia trachomatis infections. In: Hobson D, Holmes KK, eds. Nongonococcal urethritis and related infections. Washington, DC: Society for Microbiology,
1977:91-7. 12. WittDJ, CravenDE, McCabeWR. Bacterialinfectionsin adult patients with the acquired immune deficiencysyndrome (AIDS) and AIDS-related complex. Am J Med 1987;82:900-6.
Xeroderma pigmentosum lesions related to ultraviolet transmittance by clothes Niels Bech-Thomsen, MD, Hans Christian Wulf, MD, PhD, and Susanne Ullman, MD, PhD
Copenhagen, Denmark Xeroderma pigmentosum is associated with severe actinic degeneration of the skin. Our patient with xeroderma pigrnentosum showed increasing actinic damage in some areas covered by clothes. We therefore performed a complete evaluation of the patient's exposure to ultraviolet radiation. This included transmissionmeasurements of the patient's clothes, glasses, and ear windows. The transmission of UVB (280 to 320 nm) by the clothes varied from none to 17.8%. The transmitted UVB radiation was proportional to the clinicalmanifestations ofxerederma pigmentosum. The patient's wardrobe was changed, and UVA-blocking film was applied to the windows of the car and house. A decline in manifestations was seen after 18 months of improved ultraviolet protection. (J AM ACAD DERMATOL1991;24:365-8.)
Xeroderma pigrnentosum (XP) is a group of autosomal recessive diseases characterized by defects in the excision repair of ultraviolet (UV)-induced damage to DNA.1, 2 The manifestations of XP usually begin in childhood as abnormal sun sensitivity, freckling, and skin malignancies) To delay the progress of XP, patients are instructed to avoid sun exposure, use sunscreens, and wear covering clothes. However, various clothing materials transmit UV radiation differently, so the
From the Laboratory of Photobiology,Department of Dermatology, University Hospital, Rigshospital. Reprint requests: Niels Bech-Thomsen,MD, Laboratory of Photobiology, Department of Dermatology,Rigshospital,Blegdamsvej9, DK2100 KCbenhavn 0, Denmark. 16/4/18904
protective effect might be insufficient.4's In this study we examined the relationship between skin lesions and the transmittance of UVB radiation by the clothes of a patient with XP. CASE REPORT A 32-year-old woman had had XP diagnosed at 7 years of age. Since then she has worn trousers and shirts with long sleeves during outdoor activities. The patient's face showed severe actinic damage, but the forehead and ears normally covered by hair and the circumocular areas shielded by glasses were less damaged. The skin of the trunk and arms were variably sun damaged (Fig. 1). The least damaged area was found where the skin was covered both by her undershirt and shirt. A sharp increase in actinic damage was seen below the wrists. The neck and back were actinically damaged down to the waist (Fig. 2). The buttocks had no sun dam-
365
366
Journal of the American Academy of Dermatology
Bech- Thomsen et al.
a III
Fig. 1. Actinic damage on trunk was correlated to transmittance by clothes. Outline of sleeveless undershirt can be seen. Fig. 3. Skin surface subdivided in seven regions after UV exposure. Number of removed skin lesions is listed in Table I.
Fig. 2, Sharp increase in actinic damage is observed above patient's waistline.
age. The parts of the legs that were sun exposed until the age of 7 years were moderately freckled. Of 967 skin lesions removed from 1964 to 1987, 563 were located as shown in Table I and Fig. 3. The tumors were classified as b a ~ l cell carcinomas, squamous cell carcinomas, and malignant melanomas. Protection was improved after the patient was instructed to wear either denim or leather when outdoors. The patient reported that the usual increase in chronic erythema and freckles during the summer did not appear. A decrease in hyperpigmentation and a disappearance of the erythema on the arms and the upper part of the trunk were seen. M A T E R I A L AND M E T H O D S The transmission of UVB radiation through the patient's clothes and through silk and leather fabrics was
determined. As a UV source an Oriel solar simulator (model 6730/6740; Oriel Corp. of America, Stratford, Conn.) with filters and a selective mirror was used. The filtered light source emitted from 298 nm to 329 nm with a maximum of 313 nm. Radiation intensities were measured with a research radiometer (IL 1700), connected to a SED 400 detector with a diffuser and a WBS 320 filter (International Light Inc., Newburyport, Mass.). The sensitivity of the detector starts at about 250 nm and ends at about 425 nm with a maximum at 350 nm. Measurements were performed with the detector covered with cloth and uncovered. The transmission fraction was expressed as a percentage. Spectroradiometrie measurements were made on sampies 6, 7, and 22 and on the patient's car windows and glasses. A xenon lamp irradiated a monochromator (2 nm bandpass; Jobin Yvon H20, Jobin Yvon, Longjumeau, France) and the irradiance was measured with the use of an E. G. & G. 550-1 radiometer and a E. G. & G. 550-2 multiprobe detector (E. G, & G. Inc., Wellesley, Mass.). The number and location of suspected skin lesions removed during a 13-year period were recorded. The skin of the patient was examined for tumors and actinic damage during 18 months of improved protective clothing. RESULTS
UV transmission T h e U V B transmission of the measured samples is listed in T a b l e II. M a n y clothes worn on the patient's trunk and upper extremities (samples 1 to 14) transmitted more than 7% of the irradiation in the
Volume 24 Number 2, Part 2 February 1991
Xeroderma pigmentosum and UV transmittance by clothes 367
Table I. Skin tumors removed from 1964 to 1987, located in seven regions shown in Fig. 3
Region
!
,.]
I
AverageUV transmittanceby clothes(%)
II VI III I V IV VII
)
100" 1130" 6 0.6-100" 6 3 0.2
Relativedensity of tumors
I
l
14.6 0.7 0.6 0.5 0.2 0.1 0.0
No. of tumors removed 493 4 38 10 14 4 0
*Uncovered.
Table II. UVB transmittance by 34 samples of clothes Article of clothing Shirts, sweatshirts, blouses Denim jackets Parka Trousers Silk samples Leather samples
I
SampleNo.
Fabric
1 and 2 3-6 7-14 15 and 16 17 18-21 22-28 29-34
100% acrylic Mixed cotton and nylon/polyester 100% cotton 100% cotton Mixed 100% cotton/mixed 100% silk Lamb, pig, calf, and goat skins
UVB region. In contrast, the patient's trousers (samples 18 to 21) transmitted less than 0.5% of the UVB radiation. Spectroradiometric measurements showed that the transmission through clothes was almost independent of the wavelength in a range from 250 to 450 nm. A sharp increase was noted in transmittance above 340 nm by the car windows. The transmission reached a plateau at 400 rim, where 75% of all light was transmitted. The patient's reading glasses and polarized sunglasses showed an increasing transmission at wavelengths above 340 and 380 nm, respectively. DISCUSSION The essential feature of XP is a defect in the excision repair of pyrimidine dimers in DNA. 6 All classic XP cell strains are extremely sensitive to UV-induced DNA alterations. The transmission of UVB radiation through different clothing fabrics is difficult to predict. 4 The most important factor is the structure or weave of the cloth. The more tightly woven a material, the greater the protection. Direct transmission and
UVB transmittance range ( %)
I5.8-17.8 3.4-8.9 0.1-9.3 Up to 0.0002 0 0.01-0.3 0.8-5.5 Up to 0.0001
scattering through the interstices are more important than transmission through the fibers of the fabric. Color, thickness, and type of material are of minor importance. 4 Cotton in denim weave transmitted up to 0.5% of the UVB radiation. Other studies have shown a transmission in the UVB area below 0.1%.4, 5Leather transmits almost no UVB radiation but is not usable in warm weather. Spectroradiometric measurements of UV transmission through cotton, polyester, and silk showed that transmission is almost independent of wavelength. This enables total UV transmission to be determined with UVB or U V A light sources. The shirts, sweatshirts, and blouses previously worn by the patient in the summer transmitted from 0.1% to 17.8% of the UV radiation. The degree of freckling on the upper part of the body correlated with the transmittance by the clothes. Until 1987 more than 100 lesions were removed from this area and new lesions constantly appeared. Below the waistline the patient had never had any cutaneous malignancies. The patient's trousers transmitted from 0.01% to 0.3% of the UVB radiation. Thus a
368
Journal of the American Academy of Dermatology
B e c h - T h o m s e n et al.
direct relationship existed between U V transmission of the clothes and the a p p e a r a n c e of skin lesions ( T a b l e I and Fig. 3). O u r patient was instructed to wear leather or denim shirts that t r a n s m i t little U V radiation. U V A - b l o c k i n g film was applied to the windows of the patient's house and car. T h e increased protection decreased the clinical manifestations of XP.
REFERENCES 1. Robbins JH, Kraemer KH, Lutzner MA, et al. Xerederma pigmentosum: an inherited disease with sun sensitivity,
2. 3. 4. 5. 6.
multiple cutaneous neoplasms and abnormal DNA repair. Ann Intern Med 1974;80:221-48. Cleaver JE, Bootsma D. Xeroderma pigmentosum: biochemical and genetic characteristics. Ann Rev Genet 1975;9:18-38. Kraemer KH, Lee MM, Scotto J. Xeroderrna pigmentosum. Arch Dermatol 1987;123:241-50. Welsh C, Diffey BL. The protection against solar actinic radiation afforded by common clothing fabrics. Clin Exp Dermatol 1981;6:577-82. Berne B, Fischer T. Protective effects of various types of clothesagainst UV radiation. Acta Derm Venereol (Stock_h) 1980; 60:459-60. Cleaver JE, Karentz D. DNA repair in man: regulation by a multigene family and association with human diseases. Bioessays 1987;6:122-7.
infections associated with a toxic shock-like syndrome and scarlet fever toxin A. N Engl J Med I989;32l:1-7. 7. Baker CJ. Group B streptococcal cellulitis-adenitis in infants. Am J Dis Child 1982;136:631-3. 8. Rouland V, Bouchez MC, Morisot C, et al. Ad6nite et cellulite inguinale ~ streptocoque B chez un nouveau-n6. Arch Fr Pediatr 1987;44:889-92. 9. Crandal JP, Shah BR. Group B streptococcal lymphaden-
itis in a child with AIDS. Clin Pediatr 1988;27:81. 10. Ho DD, Murata GH. Streptococcal lymphadenitis in homosexual men with chronic lymphadenopathy.Am J Med 1984;77:151-3.
Streptococcal adenitis in AIDS I 1. Schaehter J. Lymphogranuloma venereum and other nonocular Chlarnydia trachomatis infections. In: Hobson D, Holmes KK, eds. Nongonococcal urethritis and related infections. Washington, DC: Society for Microbiology,
1977:91-7. 12. WittDJ, CravenDE, McCabeWR. Bacterialinfectionsin adult patients with the acquired immune deficiencysyndrome (AIDS) and AIDS-related complex. Am J Med 1987;82:900-6.
Xeroderma pigmentosum lesions related to ultraviolet transmittance by clothes Niels Bech-Thomsen, MD, Hans Christian Wulf, MD, PhD, and Susanne Ullman, MD, PhD
Copenhagen, Denmark Xeroderma pigmentosum is associated with severe actinic degeneration of the skin. Our patient with xeroderma pigrnentosum showed increasing actinic damage in some areas covered by clothes. We therefore performed a complete evaluation of the patient's exposure to ultraviolet radiation. This included transmissionmeasurements of the patient's clothes, glasses, and ear windows. The transmission of UVB (280 to 320 nm) by the clothes varied from none to 17.8%. The transmitted UVB radiation was proportional to the clinicalmanifestations ofxerederma pigmentosum. The patient's wardrobe was changed, and UVA-blocking film was applied to the windows of the car and house. A decline in manifestations was seen after 18 months of improved ultraviolet protection. (J AM ACAD DERMATOL1991;24:365-8.)
Xeroderma pigrnentosum (XP) is a group of autosomal recessive diseases characterized by defects in the excision repair of ultraviolet (UV)-induced damage to DNA.1, 2 The manifestations of XP usually begin in childhood as abnormal sun sensitivity, freckling, and skin malignancies) To delay the progress of XP, patients are instructed to avoid sun exposure, use sunscreens, and wear covering clothes. However, various clothing materials transmit UV radiation differently, so the
From the Laboratory of Photobiology,Department of Dermatology, University Hospital, Rigshospital. Reprint requests: Niels Bech-Thomsen,MD, Laboratory of Photobiology, Department of Dermatology,Rigshospital,Blegdamsvej9, DK2100 KCbenhavn 0, Denmark. 16/4/18904
protective effect might be insufficient.4's In this study we examined the relationship between skin lesions and the transmittance of UVB radiation by the clothes of a patient with XP. CASE REPORT A 32-year-old woman had had XP diagnosed at 7 years of age. Since then she has worn trousers and shirts with long sleeves during outdoor activities. The patient's face showed severe actinic damage, but the forehead and ears normally covered by hair and the circumocular areas shielded by glasses were less damaged. The skin of the trunk and arms were variably sun damaged (Fig. 1). The least damaged area was found where the skin was covered both by her undershirt and shirt. A sharp increase in actinic damage was seen below the wrists. The neck and back were actinically damaged down to the waist (Fig. 2). The buttocks had no sun dam-
365
366
Journal of the American Academy of Dermatology
Bech- Thomsen et al.
a III
Fig. 1. Actinic damage on trunk was correlated to transmittance by clothes. Outline of sleeveless undershirt can be seen. Fig. 3. Skin surface subdivided in seven regions after UV exposure. Number of removed skin lesions is listed in Table I.
Fig. 2, Sharp increase in actinic damage is observed above patient's waistline.
age. The parts of the legs that were sun exposed until the age of 7 years were moderately freckled. Of 967 skin lesions removed from 1964 to 1987, 563 were located as shown in Table I and Fig. 3. The tumors were classified as b a ~ l cell carcinomas, squamous cell carcinomas, and malignant melanomas. Protection was improved after the patient was instructed to wear either denim or leather when outdoors. The patient reported that the usual increase in chronic erythema and freckles during the summer did not appear. A decrease in hyperpigmentation and a disappearance of the erythema on the arms and the upper part of the trunk were seen. M A T E R I A L AND M E T H O D S The transmission of UVB radiation through the patient's clothes and through silk and leather fabrics was
determined. As a UV source an Oriel solar simulator (model 6730/6740; Oriel Corp. of America, Stratford, Conn.) with filters and a selective mirror was used. The filtered light source emitted from 298 nm to 329 nm with a maximum of 313 nm. Radiation intensities were measured with a research radiometer (IL 1700), connected to a SED 400 detector with a diffuser and a WBS 320 filter (International Light Inc., Newburyport, Mass.). The sensitivity of the detector starts at about 250 nm and ends at about 425 nm with a maximum at 350 nm. Measurements were performed with the detector covered with cloth and uncovered. The transmission fraction was expressed as a percentage. Spectroradiometrie measurements were made on sampies 6, 7, and 22 and on the patient's car windows and glasses. A xenon lamp irradiated a monochromator (2 nm bandpass; Jobin Yvon H20, Jobin Yvon, Longjumeau, France) and the irradiance was measured with the use of an E. G. & G. 550-1 radiometer and a E. G. & G. 550-2 multiprobe detector (E. G, & G. Inc., Wellesley, Mass.). The number and location of suspected skin lesions removed during a 13-year period were recorded. The skin of the patient was examined for tumors and actinic damage during 18 months of improved protective clothing. RESULTS
UV transmission T h e U V B transmission of the measured samples is listed in T a b l e II. M a n y clothes worn on the patient's trunk and upper extremities (samples 1 to 14) transmitted more than 7% of the irradiation in the
Volume 24 Number 2, Part 2 February 1991
Xeroderma pigmentosum and UV transmittance by clothes 367
Table I. Skin tumors removed from 1964 to 1987, located in seven regions shown in Fig. 3
Region
!
,.]
I
AverageUV transmittanceby clothes(%)
II VI III I V IV VII
)
100" 1130" 6 0.6-100" 6 3 0.2
Relativedensity of tumors
I
l
14.6 0.7 0.6 0.5 0.2 0.1 0.0
No. of tumors removed 493 4 38 10 14 4 0
*Uncovered.
Table II. UVB transmittance by 34 samples of clothes Article of clothing Shirts, sweatshirts, blouses Denim jackets Parka Trousers Silk samples Leather samples
I
SampleNo.
Fabric
1 and 2 3-6 7-14 15 and 16 17 18-21 22-28 29-34
100% acrylic Mixed cotton and nylon/polyester 100% cotton 100% cotton Mixed 100% cotton/mixed 100% silk Lamb, pig, calf, and goat skins
UVB region. In contrast, the patient's trousers (samples 18 to 21) transmitted less than 0.5% of the UVB radiation. Spectroradiometric measurements showed that the transmission through clothes was almost independent of the wavelength in a range from 250 to 450 nm. A sharp increase was noted in transmittance above 340 nm by the car windows. The transmission reached a plateau at 400 rim, where 75% of all light was transmitted. The patient's reading glasses and polarized sunglasses showed an increasing transmission at wavelengths above 340 and 380 nm, respectively. DISCUSSION The essential feature of XP is a defect in the excision repair of pyrimidine dimers in DNA. 6 All classic XP cell strains are extremely sensitive to UV-induced DNA alterations. The transmission of UVB radiation through different clothing fabrics is difficult to predict. 4 The most important factor is the structure or weave of the cloth. The more tightly woven a material, the greater the protection. Direct transmission and
UVB transmittance range ( %)
I5.8-17.8 3.4-8.9 0.1-9.3 Up to 0.0002 0 0.01-0.3 0.8-5.5 Up to 0.0001
scattering through the interstices are more important than transmission through the fibers of the fabric. Color, thickness, and type of material are of minor importance. 4 Cotton in denim weave transmitted up to 0.5% of the UVB radiation. Other studies have shown a transmission in the UVB area below 0.1%.4, 5Leather transmits almost no UVB radiation but is not usable in warm weather. Spectroradiometric measurements of UV transmission through cotton, polyester, and silk showed that transmission is almost independent of wavelength. This enables total UV transmission to be determined with UVB or U V A light sources. The shirts, sweatshirts, and blouses previously worn by the patient in the summer transmitted from 0.1% to 17.8% of the UV radiation. The degree of freckling on the upper part of the body correlated with the transmittance by the clothes. Until 1987 more than 100 lesions were removed from this area and new lesions constantly appeared. Below the waistline the patient had never had any cutaneous malignancies. The patient's trousers transmitted from 0.01% to 0.3% of the UVB radiation. Thus a
368
Journal of the American Academy of Dermatology
B e c h - T h o m s e n et al.
direct relationship existed between U V transmission of the clothes and the a p p e a r a n c e of skin lesions ( T a b l e I and Fig. 3). O u r patient was instructed to wear leather or denim shirts that t r a n s m i t little U V radiation. U V A - b l o c k i n g film was applied to the windows of the patient's house and car. T h e increased protection decreased the clinical manifestations of XP.
REFERENCES 1. Robbins JH, Kraemer KH, Lutzner MA, et al. Xerederma pigmentosum: an inherited disease with sun sensitivity,
2. 3. 4. 5. 6.
multiple cutaneous neoplasms and abnormal DNA repair. Ann Intern Med 1974;80:221-48. Cleaver JE, Bootsma D. Xeroderma pigmentosum: biochemical and genetic characteristics. Ann Rev Genet 1975;9:18-38. Kraemer KH, Lee MM, Scotto J. Xeroderrna pigmentosum. Arch Dermatol 1987;123:241-50. Welsh C, Diffey BL. The protection against solar actinic radiation afforded by common clothing fabrics. Clin Exp Dermatol 1981;6:577-82. Berne B, Fischer T. Protective effects of various types of clothesagainst UV radiation. Acta Derm Venereol (Stock_h) 1980; 60:459-60. Cleaver JE, Karentz D. DNA repair in man: regulation by a multigene family and association with human diseases. Bioessays 1987;6:122-7.