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Læknablaðið - 15.10.1999, Qupperneq 45

Læknablaðið - 15.10.1999, Qupperneq 45
LÆKNABLAÐIÐ 1999; 85 807 Table 1. Cases of esophageal pemphigus vulgaris. Case No (Reference) Age (years) Involvement Duration of disease Gastrointestinal symptoms Endoscopic appearance DIF* esophagus IIF* Site of initial presentation 1 (5) 54 mouth 9 months dysphagia stricture, erosions ND 1:320 mouth, esophagus 2(6) 47 skin, mouth 18 months odynophagia # POS 1:40 skin 3(7) 56 skin, mouth 16 years odynophagia erosions ND NEG skin, mouth 4(8) 40 mouth 6 months epigastric pain ulcers, erosions ND ND mouth 5(9) 47 mouth 3 years hematemesis # POS 1:10 mouth 6(10) 34 mouth 2 years odynophagia bullae POS ND mouth 7(11) 65 skin 10 years epigastric pain ulcer, erythema ND ND skin 8(12) 45 mouth 3 years none erythema ND ND ? 9(12) 55 mouth 9 odynophagia, dysphagia bullae, erosions ND ND ? 10(12) 48 mouth, genitalia 3 years odynophagia, dysphagia bullae, erosions ND ND ? 11 (12) 50 skin, mouth 3 years none normal ND ND ? 12(13) 66 mouth 5 months odynophagia, dysphagia ND POS 1:20 mouth, esophagus 13(13) 64 mouth 3 months odynophagia, dysphagia linear erythema POS 1:60 mouth, esophagus 14(14) 76 mouth 2 months none normal POS 1:40 ? 15(14) 54 skin, mouth 3 months none erosions POS 1:40 ? 16(15) 40 mouth,skin,genit 7 months odynophagia, dysphagia erosions ND 1:160 mouth 17 (present) 68 mouth, genitalia ? odynophagia, dysphagia stricture, erosions POS NEG ? * DIF = direct immunofluoroscence; IIF = indirect immunofluoroscence # esophagitis dissecans superficialis ND = not done speglun og sýnatöku úr vélinda. Hjá þessum sjúklingum reyndist vélindabólga lítil eða eng- in. Við speglun er oftast lýst fleiðrum (erosions) en í nokkrum tilvikum sáust sár eða blöðrur. I einu tilviki leit slímhúðin eðlilega út en breyt- ingar á vefjasýni voru dæmigerðar. Skimað var fyrir mótefnum í vefjasýnum frá átta sjúklingum (DIF) og fékkst jákvæð svörun í öllum tilvikum. Leitað var að mótefnum í sermi (IIF) 10 sjúklinga og var svörun jákvæð í átta tilvikum með títer 1:10-1:320. Mögulegt er að algengi pemphigus vulgaris í vélinda sé vanmetið. Vélindasjúkdómur getur verið einkennalaus og hans því ekki leitað (13) og einkenni frá öðrum líffærum geta yfirgnæft sjúkdómsmyndina. Við ályktum að þótt vélindabólga af völdum pemphigus vulgaris sé sjaldgæf, ætti að hafa hana í huga hjá sjúklingum með sjúkdóminn, einkum konum með einkenni frá efri hluta meltingarfæra. HEIMILDIR 1. Becker BA, Gaspari AA. Pemphigus vulgaris and vegetans. Dermatol Clin 1993; 11: 429-52. 2. Wick MR, Ritter JH, Humphrey PA, Swanson PE. Immuno- patholopgy of Nonneoplastic Skin Disease. A brief Review. Am J Clin Pathol 1995; 105:417-29. 3. Korman NJ. Pemphigus. Dermatologic Clinics 1990; 8: 689-700. 4. Boh EE, Millikan LE. Vesiculobullous disease with promi- nent immunologic features. JAMA 1992; 268: 2893-8. 5. Raque CJ, Stein KM, Samitz MH. Pemphigus vulgaris involving the esophagus. Arch Dermtol 1970; 102: 371-3. 6. Kaplan RP, Touloukian J, Ahmed AR, Newcomer VD. Eso- phagitis dissecans superficialis associated with pemphigus vulgaris. J Am Acad Dermatol 1981; 4: 682-7. 7. Wood DR, Patterson JB, Orlando RC. Pemphigus vulgaris of the esophagus. Ann Int Med 1982; 96: 189-91. 8. Yamamoto H, Kozawa Y, Otake S, Shimokawa R. Pemphi- gus vulgaris involving the mouth and esophagus. Int J Oral Surg 1983; 12: 194-200. 9. Kaneko F, Mori M, Tsukinaga I. Pemphigus vulgaris of eso- phageal mucosa. Arch Dermatol 1985; 121: 272-3. 10. Bames LM, Clark ML, Estes SA, Bongiovanni GL. Pem- phigus vulgaris involving the esophagus. A case report and review of the literature. Dig Dis Sci 1987; 32: 655-9. 11. Kamm MA, Brenan JA, Davies DJ, Desmond PV. Pemphi- gus vulgaris of the esophagus. J Clin Gastroenterol 1988; 10: 324-6. 12. Eliakim R, Goldin E, Livshin R, Okon E. Esophageal in- volvement in pemphigus vulgaris. Am J Gastroenterol 1988;83:155-7. 13. Lurie R, Trattner A, Sandbank M. Esophageal involvement in pemphigus vulgaris: report of two cases and review of the literature. Dermatologica 1990; 181: 233-6. 14. TrattnerA, Lurie R, LeiserA, David M, Hazaz B, Kadish U, Sandbank M. Esophageal involvement in pemphigus vulgaris: a clinical, histologic and immunopathologic study. J Am Acad Dermatol 1991; 24: 223-6. 15. Goldberg NS, Weiss SS. Pemphigus vulgaris of the eso- phagus in women. J Am Acad Dermatol 1989; 21:1115-8.
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