658
Journal of the American Academy of Dermatology
Brief communications
Fig. 1. Elastorrhexis and elastosis in mid dermis.
The spectrum of the skin manifestations in the L-tryptophan--induced eosinophilia-myalgia syndrome (EMS) has not been well establishedl; those reported thus far inelude rash, peripheral swelling, "tight hard shiny skin, ''1 and ecsinophilic fasciitis--like lesions. 2 We have observed extensive changes of cutaneous elastic fibers, a previously unreported skin change, in two women who fulfilled the criteria of L-tryptophan-induced EMS. Appearing as multiple yellow papules on the lateral parts of the neck and medial aspects of the arms, these lesions clinically resembled pseudoxanthoma elasticum (PXE). Notably, these papules occurred at the sites of sclerodermatous skin and had identical distribution on both patients. Histopathologic examination of papules showed numerous fragmented and clumped elastic fibers without calcification in the mid dermis (Fig. 1). Electron microscopic study did not reveal m0rphologic abnormalities that are found in typical PXE. There was no family history or ocular sign of PXE. Elastorrhexis and elastosis are not components of scleroderma, pseudoscleroderma, or syndromes of hypereosinophilia. PXE, however, has been reported after long-term (up to 19 years), high-dose (2.5 gm/day) D-penieillamine therapy for Wilson's disease and cystinosis. 3,4 Because her condition was previously diagnosed as scleroderma, one of out patients had received D-penicillamine, for a short period (4 months) and in low doses (150 mg/day), but the other had not. We propose that elastic fiber damage might be part of the EMS and should be searched for in patients with this syndrome. Because PXE-like syndrome is induced only by a limited number of toxins (saltpeter, mixture of calcium and ammonium nitrate) 5 or drugs (penicillamine), the development of PXE-like elastic fiber changes might help to elucidate the pathogenesis of EMS. Furthermore, we should pay attention to possible systemic elastic fiber damage and its potential long-term complications.
REFERENCES 1. EidsonM, Philen RM, Sewen CM, et al. L-Tryptophanand eosinophilia-myalgia syndrome in New Mexico. Lancet 1990;335:645-8. 2. Silver RM, MelvynPH, Maize JC, et al. Scleroderma, fasciitis, and eosinophilia associatedwith the ingestion of tryptophan. N Engl J Med 1990;322:874-81. 3. MeyrickTRH, Light N, Avery NC, et al. Pseudoxanthoma elasticum-like skin changes induced by penicillamine. J Roy Soc Med 1984; 77:794-8. 4. Meyrick TRH, Kirby JDT. Elastosis perforans serpiginose and pseudoxanthoma elasticum-like skin change due to D-penicillamine. Clin Exp Dermatol 1985;10:386-91. 5. Christensen OB. An exogenousvariety of pseudoxanthoma elasticum in old farmers. Acta Derm Venereol (Stockh) 1978;58:319-21.
IgE-bearing Langerhans cells are not specific to atopic eczema but are found in inflammatory skin diseases Thomas Bieber, MD, and Otto Braun-Falco, MD
Munich, Germany IgE-bearing epidermal Langerhans cells (LCs) were initially identified in patients with atopic eezema.l This suggests that LCs may be primarily involved in the pathophysiology of this disease by presenting IgE-bound aeroallergens to specific T cells and thereby provoking an IgE-mediated type IV reaction. 2 Since then it has been believed that this finding is specific to this disease. Recently, we have shown that in atopic eczema the presence of IgE-bearing LCs is somehow related, first, to the presence of an inflammatory infiltrate and, second, to the serum IgE level, because only patients with increased IgE levels (> 100 kU/L) display such features) To verify the specificity of this finding in atopic eczema, we investigated the LCs in various skin diseases (Table I) as described in detail elsewhere. 3 No IgE-positive LCs were observed in uninvolved skin in these diseases. In contrast, in lesional skin IgE-bearing LCs were found in numerous sections of various diseases in patients with elevated IgE serum levels. In our study on IgE-bearing LCs in atopic eczema, 3we pointed out that cytokines locally released by infiltrating ceils like T cells may be involved in the induction and/or regulation of the expression of a specific receptor for IgE molecules on LCs. 4 Furthermore, we have recently shown From the Department of Dermatology,Ludwig-MaximiliansUniversity. Reprint requests: Thomas Bieber, MD, DermatologiseheKlinik und Poliklinik der Ludwig-MaximiliansUniversit/it, Frauenlobstrasse, 9-11, 8000 Munich 2, Germany. 16/4/26884
Volume 24 Number 4 April 1991
Brief communications
T a b l e I. IgE-bearing Langerhans cells (LCs) in lesional skin of atopic eczema and common inflammatory skin diseases Stainingof LC Serum IgE 100 kU/L Diagnosis Atopic eczema (n = 40) Psoriasis vulgaris (n = 25) Lichen tuber planus (n = 5) Discoid LE (n = 9) Subaeute cutaneous LE (n = 2) Mycosis fungoides (n = 10) Parapsoriasis en plaques (n = 3) Lymphocytic infiltration (n = 2) Histiocytosis X (n = 3)
(+/-) . I (+/-) 0/8 30/2 0/15 9/1 0/3 1/I 0/7 2/0 0/1 1/0 0/7 3/0 0/2 1/0 0/1 1/0 0/1 1/l
LE, Lupus erythematosus. that T celi--der tved cytokines like interleukin 4 and/or interferon-'r are able to induce the low-aff-mity receptor for the Fc fragment of IgE (FeeR2/CD23) on normal epidermal LCs. From these observations, we postulate that the expression of an FceR is not intrinsic to LCs from patients with atopic eczema, but rather is related to distinct signals or mediators present in inflamed skin. Thereafter, an increased IgE level, which is not exclusively related to atopy, S may contribute to the binding of IgE molecules on the receptor within inflamed skin areas. However, we believe that IgE-bearing LCs are not an epiphenomenon in atopic eczema because in vitro studies on LCs isolated from atopic eczema show that these cells are able to stimulate autologous antigen-specific T cells.6 Therefore the antigen specificity of IgE molecules bound on the receptor may be the most important feature to consider. REFERENCES 1. BruynzeeI-Koomen C, van Wichen DF, Toonstra J, et al. The presence of lgE molecules on epidermal Langerhans cells in patients with atopic dermatitis. Arch Dermatol Res 1986;278:199-205. 2. Bruynzeel-KoomenC. IgE on Langerhans ceils:new insights into pathogenesis of atopic dermatitis. Dermato~ogica 1986;172:181-3. 3. Bieber T, Dannenberg B, Prinz JC, et al. Occurrence of IgE-bearing epidermal Langerhans cells in atopic eczema: a study of the time course of the lesionsand with regard to the IgE serum level. J Invest Dermatol 1989;93:215-9. 4. Bieber T, Rieger A, Neuchrist C, et al. Induction of Fc~R2/ CD23 on human epidermal Langerhans cells by human recombinant interleukin 4 and y-interferon. J Exp Med 1989;170:309-14. 5. Gurevitch AW, Heiner DC, Reisner RM. IgE in atopic dermatitis and other common dermatosis. Arch Dermatol 1973;107:712-5.
659
6. Mudde GC, Vanreijsen FC, Boland GJ, et al. Allergen presentation by epidermal Langerhans cells from patients with atopic dermatitis is mediated by IgE. Immunology 1990;69:335-41. The qualitative determination of radiation output from a grenz ray apparatus E. K. Edwards, Jr., MD, FACP, and E. K. Edwards, St., MD Pompano Beach, Florida With the current revival of iterest in the use of grenz ray treatment of a host of benign diseases, many dermotologists are purchasing a variety of older grenz-ray machines. 1-3 Most older units can be rebuilt and recalibrated for less than $1000 and will continue to give good service for many years. Many of these second-hand units are sold "as is," with no guarantee, either warranted or implied. A unit may have been stored or handled improperly so it is important to know before purchase whether the machine is still capable of generating radiation. We devised a simple qualitative test that can b e performed before purchase to ascertain whether the unit is producing radiation. In this test the machine need not even be set up; only a 110 V power source is needed. Place the dial of a wristwatch with a plastic watch crystal and luminous numbers directly under the port or cone of the unit; set the control panel for 15 kVp at 5 mA; completely darken the room and turn power on. If radiation is being produced, the numbers of the wristwatch will glow. With our units, the glow starts at 15 kVp and stops at 12 kVp, so it is important to generate radiation with quality hard enough to penetrate the watch crystal. With our grenz apparatus, radiation produced at 15 kVp has a haLf-value layer of 0.030 mm aluminum. This quick, effective qualitative test can be performed by a prospective buyer before the purchase of a unit without the need for expensive calibration equipment. We emphasize, however, that this is strictly a qualitative test; if the machine is found to be functional, it will need to be calibrated by a radiation physicist familiar with dermatologic radiotherapy equipment. REFERENCES 1. Edwards EK Jr, Edwards EK Sr. Grenz ray therapy: a commentary and re-assessment. Int J DermatoI 1990;29:17-8. 2. Edwards EK Jr, Edwards EK Sr. Dermatologic radiation therapy: pyrex versus beryllium window units. Int J Dermatol 1990;29:156. 3. Edwards EK Jr, Edwards EK Sr. The effect of superficial radiation therapy on Langerhans cells in human skin. Int J Dermatol (In press.) Reprintrequests:E. K. Edwards,Jr., MD, or E. K. Edwards,St., MD, Ridge-EdwardsDermatologyCemer, 180t3North FederalHighway, Pompano Beach,FL 33062.
16/4/25737
Journal of the American Academy of Dermatology
Brief communications
Fig. 1. Elastorrhexis and elastosis in mid dermis.
The spectrum of the skin manifestations in the L-tryptophan--induced eosinophilia-myalgia syndrome (EMS) has not been well establishedl; those reported thus far inelude rash, peripheral swelling, "tight hard shiny skin, ''1 and ecsinophilic fasciitis--like lesions. 2 We have observed extensive changes of cutaneous elastic fibers, a previously unreported skin change, in two women who fulfilled the criteria of L-tryptophan-induced EMS. Appearing as multiple yellow papules on the lateral parts of the neck and medial aspects of the arms, these lesions clinically resembled pseudoxanthoma elasticum (PXE). Notably, these papules occurred at the sites of sclerodermatous skin and had identical distribution on both patients. Histopathologic examination of papules showed numerous fragmented and clumped elastic fibers without calcification in the mid dermis (Fig. 1). Electron microscopic study did not reveal m0rphologic abnormalities that are found in typical PXE. There was no family history or ocular sign of PXE. Elastorrhexis and elastosis are not components of scleroderma, pseudoscleroderma, or syndromes of hypereosinophilia. PXE, however, has been reported after long-term (up to 19 years), high-dose (2.5 gm/day) D-penieillamine therapy for Wilson's disease and cystinosis. 3,4 Because her condition was previously diagnosed as scleroderma, one of out patients had received D-penicillamine, for a short period (4 months) and in low doses (150 mg/day), but the other had not. We propose that elastic fiber damage might be part of the EMS and should be searched for in patients with this syndrome. Because PXE-like syndrome is induced only by a limited number of toxins (saltpeter, mixture of calcium and ammonium nitrate) 5 or drugs (penicillamine), the development of PXE-like elastic fiber changes might help to elucidate the pathogenesis of EMS. Furthermore, we should pay attention to possible systemic elastic fiber damage and its potential long-term complications.
REFERENCES 1. EidsonM, Philen RM, Sewen CM, et al. L-Tryptophanand eosinophilia-myalgia syndrome in New Mexico. Lancet 1990;335:645-8. 2. Silver RM, MelvynPH, Maize JC, et al. Scleroderma, fasciitis, and eosinophilia associatedwith the ingestion of tryptophan. N Engl J Med 1990;322:874-81. 3. MeyrickTRH, Light N, Avery NC, et al. Pseudoxanthoma elasticum-like skin changes induced by penicillamine. J Roy Soc Med 1984; 77:794-8. 4. Meyrick TRH, Kirby JDT. Elastosis perforans serpiginose and pseudoxanthoma elasticum-like skin change due to D-penicillamine. Clin Exp Dermatol 1985;10:386-91. 5. Christensen OB. An exogenousvariety of pseudoxanthoma elasticum in old farmers. Acta Derm Venereol (Stockh) 1978;58:319-21.
IgE-bearing Langerhans cells are not specific to atopic eczema but are found in inflammatory skin diseases Thomas Bieber, MD, and Otto Braun-Falco, MD
Munich, Germany IgE-bearing epidermal Langerhans cells (LCs) were initially identified in patients with atopic eezema.l This suggests that LCs may be primarily involved in the pathophysiology of this disease by presenting IgE-bound aeroallergens to specific T cells and thereby provoking an IgE-mediated type IV reaction. 2 Since then it has been believed that this finding is specific to this disease. Recently, we have shown that in atopic eczema the presence of IgE-bearing LCs is somehow related, first, to the presence of an inflammatory infiltrate and, second, to the serum IgE level, because only patients with increased IgE levels (> 100 kU/L) display such features) To verify the specificity of this finding in atopic eczema, we investigated the LCs in various skin diseases (Table I) as described in detail elsewhere. 3 No IgE-positive LCs were observed in uninvolved skin in these diseases. In contrast, in lesional skin IgE-bearing LCs were found in numerous sections of various diseases in patients with elevated IgE serum levels. In our study on IgE-bearing LCs in atopic eczema, 3we pointed out that cytokines locally released by infiltrating ceils like T cells may be involved in the induction and/or regulation of the expression of a specific receptor for IgE molecules on LCs. 4 Furthermore, we have recently shown From the Department of Dermatology,Ludwig-MaximiliansUniversity. Reprint requests: Thomas Bieber, MD, DermatologiseheKlinik und Poliklinik der Ludwig-MaximiliansUniversit/it, Frauenlobstrasse, 9-11, 8000 Munich 2, Germany. 16/4/26884
Volume 24 Number 4 April 1991
Brief communications
T a b l e I. IgE-bearing Langerhans cells (LCs) in lesional skin of atopic eczema and common inflammatory skin diseases Stainingof LC Serum IgE 100 kU/L Diagnosis Atopic eczema (n = 40) Psoriasis vulgaris (n = 25) Lichen tuber planus (n = 5) Discoid LE (n = 9) Subaeute cutaneous LE (n = 2) Mycosis fungoides (n = 10) Parapsoriasis en plaques (n = 3) Lymphocytic infiltration (n = 2) Histiocytosis X (n = 3)
(+/-) . I (+/-) 0/8 30/2 0/15 9/1 0/3 1/I 0/7 2/0 0/1 1/0 0/7 3/0 0/2 1/0 0/1 1/0 0/1 1/l
LE, Lupus erythematosus. that T celi--der tved cytokines like interleukin 4 and/or interferon-'r are able to induce the low-aff-mity receptor for the Fc fragment of IgE (FeeR2/CD23) on normal epidermal LCs. From these observations, we postulate that the expression of an FceR is not intrinsic to LCs from patients with atopic eczema, but rather is related to distinct signals or mediators present in inflamed skin. Thereafter, an increased IgE level, which is not exclusively related to atopy, S may contribute to the binding of IgE molecules on the receptor within inflamed skin areas. However, we believe that IgE-bearing LCs are not an epiphenomenon in atopic eczema because in vitro studies on LCs isolated from atopic eczema show that these cells are able to stimulate autologous antigen-specific T cells.6 Therefore the antigen specificity of IgE molecules bound on the receptor may be the most important feature to consider. REFERENCES 1. BruynzeeI-Koomen C, van Wichen DF, Toonstra J, et al. The presence of lgE molecules on epidermal Langerhans cells in patients with atopic dermatitis. Arch Dermatol Res 1986;278:199-205. 2. Bruynzeel-KoomenC. IgE on Langerhans ceils:new insights into pathogenesis of atopic dermatitis. Dermato~ogica 1986;172:181-3. 3. Bieber T, Dannenberg B, Prinz JC, et al. Occurrence of IgE-bearing epidermal Langerhans cells in atopic eczema: a study of the time course of the lesionsand with regard to the IgE serum level. J Invest Dermatol 1989;93:215-9. 4. Bieber T, Rieger A, Neuchrist C, et al. Induction of Fc~R2/ CD23 on human epidermal Langerhans cells by human recombinant interleukin 4 and y-interferon. J Exp Med 1989;170:309-14. 5. Gurevitch AW, Heiner DC, Reisner RM. IgE in atopic dermatitis and other common dermatosis. Arch Dermatol 1973;107:712-5.
659
6. Mudde GC, Vanreijsen FC, Boland GJ, et al. Allergen presentation by epidermal Langerhans cells from patients with atopic dermatitis is mediated by IgE. Immunology 1990;69:335-41. The qualitative determination of radiation output from a grenz ray apparatus E. K. Edwards, Jr., MD, FACP, and E. K. Edwards, St., MD Pompano Beach, Florida With the current revival of iterest in the use of grenz ray treatment of a host of benign diseases, many dermotologists are purchasing a variety of older grenz-ray machines. 1-3 Most older units can be rebuilt and recalibrated for less than $1000 and will continue to give good service for many years. Many of these second-hand units are sold "as is," with no guarantee, either warranted or implied. A unit may have been stored or handled improperly so it is important to know before purchase whether the machine is still capable of generating radiation. We devised a simple qualitative test that can b e performed before purchase to ascertain whether the unit is producing radiation. In this test the machine need not even be set up; only a 110 V power source is needed. Place the dial of a wristwatch with a plastic watch crystal and luminous numbers directly under the port or cone of the unit; set the control panel for 15 kVp at 5 mA; completely darken the room and turn power on. If radiation is being produced, the numbers of the wristwatch will glow. With our units, the glow starts at 15 kVp and stops at 12 kVp, so it is important to generate radiation with quality hard enough to penetrate the watch crystal. With our grenz apparatus, radiation produced at 15 kVp has a haLf-value layer of 0.030 mm aluminum. This quick, effective qualitative test can be performed by a prospective buyer before the purchase of a unit without the need for expensive calibration equipment. We emphasize, however, that this is strictly a qualitative test; if the machine is found to be functional, it will need to be calibrated by a radiation physicist familiar with dermatologic radiotherapy equipment. REFERENCES 1. Edwards EK Jr, Edwards EK Sr. Grenz ray therapy: a commentary and re-assessment. Int J DermatoI 1990;29:17-8. 2. Edwards EK Jr, Edwards EK Sr. Dermatologic radiation therapy: pyrex versus beryllium window units. Int J Dermatol 1990;29:156. 3. Edwards EK Jr, Edwards EK Sr. The effect of superficial radiation therapy on Langerhans cells in human skin. Int J Dermatol (In press.) Reprintrequests:E. K. Edwards,Jr., MD, or E. K. Edwards,St., MD, Ridge-EdwardsDermatologyCemer, 180t3North FederalHighway, Pompano Beach,FL 33062.
16/4/25737
