Ekaterinburg, Russian Federation
employee
Ekaterinburg, Russian Federation
Ekaterinburg, Russian Federation
Ekaterinburg, Russian Federation
Autoimmune bullous dermatoses are a group of acquired and inherited diseases caused by the production of autoantibodies directed against protein structures of the epidermis and dermo-epidermal junction. The most severe and potentially dangerous bullous dermatoses are acantholytic pemphigus and bullous pemphigoid. Mortality from acantholytic pemphigus is 10.0 - 30.0 %. Aim. To demonstrate the diversity of clinical manifestations and the need for multidisciplinary interaction in the management of patients with autoimmune bullous dermatoses. Materials and methods. The literature review of materials of domestic and foreign researchers describe the clinical course of autoimmune bullous dermatoses using the search engines Pubmed, Medline, Cochrane library, Elibrary (total 73). The clinical course of bladder dermatosis varies from localized forms with a relatively mild degree of severity to generalized lethal forms that are characterized by the formation of bullas that open with the formation of long-term non-healing erosions that can occur both on the skin and on the mucous membranes of the eyes, nose, mouth, esophagus, genitalia. The article describes the most significant complaints from patients with lesions on mucous membranes, the description of the endoscopic picture of lesions in the gastrointestinal tract in patients with autoimmune bullous dermatoses, the description of the classical clinical picture of pemphigus acantholyticus, represented by blisters with serous contents, with listless, flabby cover and erosions prone to peripheral growth, a description of the clinical symptoms of Nikolsky, Asbo-Khansen and Sheklov, most significant for the differential diagnosis of bullous dermatoses. Furthermore authors describe cases with non typical clinical findings autoimmune bullous dermatoses and unusual site of the pathologic process. That can cause diagnostic errors leading the process to spread, postponement of the start of treatment, which in turn requires the appointment of high doses of systemic glucocorticosteroids. Improving the prognosis and quality of life of patients is possible only with the interdisciplinary interaction of a dermatovenereologist with adjacent specialists
autoimmune bullous dermatoses, pemphigus, bullous pemphigoid, bulla, erosion.
1. Ghiasi M., Daneshpazhooh M., Ismonov M., Chams-Davatchi C. Evaluation of Autoimmune Bullous Diseases in Elderly Patients in Iran: A 10-Year Retrospective Study. Skinmed, 2017, vol. 15, no. 3, pp. 175–180.
2. Sovershenstvovanie diagnostiki vul'garnoy puzyrchatki / E. A. Batkaev, Yu. A. Gallyamova, N. I. Syuch, L. T. Togoeva [i dr.] // Rossiyskiy zhurnal kozhnyh i venericheskih bolezney. – 2006. – № 5. – S. 49–51.
3. Zavadskiy, V. N. K voprosu diagnostiki i lecheniya seboreynoy puzyrchatki / V. N. Zavadskiy // Rossiyskiy zhurnal kozhnyh i venericheskih bolezney. – 2013. – № 1. – S. 18–21.
4. Baican A., Chiorean R., Leucuta D.C., Baican A., Baican C. et al. Prediction of survival for patients with pemphigus vulgaris and pemphigus foliaceus: a retrospective cohort study. Orphanet J Rare Dis, 2015, no. 10, pp. 48. doi: 10.1186/s13023-015-0263-4
5. Kridin K., Zelber-Sagi S., Khamaisi M., Cohen A.D. et al. Remarkable differences in the epidemiology of pemphigus among two ethnic populations in the same geographic region. J Am Acad Dermatol, 2016, vol. 75, no. 5, pp. 925–993. doi: 10.1016/j.jaad.2016.06.055
6. Rahbar Z., Daneshpazhooh M., Mirshams-Shahshahani M., Esmaili N. et al. Pemphigus disease activity measurements: pemphigus disease area index, autoimmune bullousskin disorder intensity score, and pemphigus vulgaris activity scor0065. JAMA Dermatol, 2014, vol. 150, no. 3, pp. 266–272. doi: 10.1001/jamadermatol.2013.8175
7. Bulgakova, A. I. Rasprostranennost', etiologiya i klinicheskie proyavleniya puzyrchatki / A. I. Bulgakova, Z. R. Hismatulina, G. F. Gabidullina // Medicinskiy vestnik Bashkortastana. – 2016. – T. 11, № 6(66). – S. 86–90.
8. Sanchez-Perez J., Garcia-Diez A. Pemphigus. Actas Dermosifiliogr, 2005, vol. 96, no. 6, pp. 329–356.
9. Baum S., Astman N., Berco E., Solomon M. et al. Epidemiological data of 290 pemphigus vulgaris patients: a 29-year retrospective study. Eur J Dermatol, 2016, vol. 26, no. 4, pp. 382–387. doi: 10.1684/ejd.2016.2792
10. Abbas Z., Safaie Naraghi Z., Behrangi E. Pemphigus vulgaris presented with cheilitis. Case Rep Dermatol Med, 2014. doi: 10.1155/2014/147197
11. Mandel V.D., Farnetani F., Vaschieri C., Manfredini M. et al. Pemphigus with features of both vulgaris and foliaceus variants localized to the nose. J Dermatol, 2016, vol. 43, no. 8, pp. 940–943. doi: 10.1111/1346-8138.13314
12. Rebello M.S., Ramesh B.M., Sukumar D., Alapatt G.F. Cerebriform Cutaneous Lesions in Pemphigus Vegetans. Indian J Dermatol, 2016, vol. 61, no. 2, pp. 206–208. doi: 10.4103/0019-5154.177760
13. Obyknovennaya puzyrchatka: osobennosti terapii v polosti rta / V. V. Chebotarev, A. G. Sirak, F. M. S. Al'-Asfari, S. V. Sirak // Medicinskiy vestnik Severnogo Kavkaza. – 2014. – T. 9, № 3. – S. 215–217.
14. Razrabotka i primenenie polikomponentnoy adgezivnoy mazi dlya lecheniya erozivnyh porazheniy slizistoy obolochki polosti rta u pacientov s obyknovennoy puzyrchatkoy / S. V. Sirak, V. V. Chebotarev, A. G. Sirak, A. A. Grigor'yan // Sovremennye problemy nauki i obrazovaniya. ‒ 2013. ‒ № 2. ‒ S. 15‒22.
15. Zabolevaniya slizistoy obolochki polosti rta i gub / pod red. prof. E. V. Borovskogo, prof. N. F. Mashkilleysona. – Moskva: MEDpress, 2001. – S. 177–194.
16. Arvind Babu R.S., Chandrashekar P., Kiran Kumar K., Sridhar Reddy G. et al. A Study on Oral Mucosal Lesions in 3500 Patients with Dermatological Diseases in South India. Ann Med Health Sci Res, 2014, vol. 4, no. 2, pp. 84–93. doi: 10.4103/2141-9248.138019
17. Kumar S.J., Anand S.P.N., Gunasekaran N., Krishnan R. Oral pemphigus vulgaris: A case report with direct immunofluorescence study. J Oral Maxillofac Pathol, 2016, vol. 20, no. 3, pp. 549–555. doi: 10.4103/0973-029X.190979
18. Gunther C. Involvement of mucous membranes in autoimmune bullous diseases. Hautarzt, 2016, vol. 67, no. 10, pp. 774–779. doi: 10.1007/s00105-016-3871-6
19. Uludag H.A., Uysal Y., Kucukevcilioglu M., Ceylan O.M. et al. An uncommon ocular manifestation of pemphigus vulgaris: conjunctival mass. Ocul Immunol Inflamm, 2013, vol. 21, no. 5, pp. 400–402. doi: 10.3109/09273948.2013.791924
20. Akhyani M., Keshtkar-Jafari A., Chams-Davatchi C., Lajevardi V. et al. Ocular involvement in pemphigus vulgaris. J Dermatol, 2014, vol. 41, no. 7, pp. 618–621. doi: 10.1111/1346-8138.12447
21. Tan J.C., Tat L.T., Francis K.B., Mendoza C.G. et al. Prospective study of ocular manifestations of pemphigus and bullous pemphigoid identifies a high prevalence of dry eye syndrome. Cornea, 2015, vol. 34, no. 4, pp. 443–448. doi: 10.1097/ICO.0000000000000335
22. Broussard K.C., Leung T.G., Moradi A., Thorne J.E. et al. Autoimmune bullous diseases with skin and eye involvement: Cicatricial pemphigoid, pemphigus vulgaris, and pemphigus paraneoplastica. Clin Dermatol, 2016, vol. 34, no. 2, pp. 205–213. doi: 10.1016/j.clindermatol.2015.11.006
23. Espana A., Iranzo P., Herrero-González J., Mascaro J.M. et al. Ocular involvement in pemphigus vulgaris - a retrospective study of a large Spanish cohort. J Dtsch Dermatol Ges, 2017, vol. 15, no. 4, pp. 396–403. doi: 10.1111/ddg.13221
24. Nakamura R., Omori T., Suda K., Wada N. et al. Endoscopic findings of laryngopharyngeal and esophageal involvement in autoimmune bullous disease. Dig Endosc, 2017. doi: 10.1111/den.12893
25. Shah R., Thoguluva V., Bansal N., Manocha D. Esophageal dissecans: a rare life-threatening presentation of recurrent pemphigus vulgaris. Am J Emerg Med, 2015, vol. 33, no. 12, pp. 1845. doi: 10.1016/j.ajem.2015.04.053
26. Al-Janabi A., Greenfield S. Pemphigus vulgaris: a rare cause of dysphagia. BMJ Case Rep, 2015. doi: 10.1136/bcr-2015-212661
27. Cecinato P., Laterza L., De Marco L., Casali A. et al. Esophageal involvement by pemphigus vulgaris resulting in dysphagia. Endoscopy, 2015, vol. 47, no. 1, pp. 271–272. doi: 10.1016/j.ajem.2015.04.053
28. Zehou O., Raynaud J.J., Le Roux-Villet C., Alexandre Casali M. et al. Oesophageal involvement in 26 consecutive patients with mucous membrane pemphigoid. Br J Dermatol, 2017. doi: 10.1111/bjd.15592
29. Niho K., Nakasya A., Ijichi A., Tsujita J. et al. A case of bleeding duodenal ulcer with pemphigus vulgaris during steroid therapy. Clin J Gastroenterol, 2014, vol. 7, no. 3, pp. 223–227. doi: 10.1007/s12328-014-0476-4
30. Fairbanks Barbosa N.D., de Aguiar L.M., Maruta C.W., Aoki V. et al. Vulvo-cervico-vaginal manifestations and evaluation of Papanicolaou smears in pemphigus vulgaris and pemphigus foliaceus. J Am Acad Dermatol, 2012, vol. 67, no. 3, pp. 409–416. doi: 10.1016/j.jaad.2011.10.004
31. Iijima S., Hamada T., Kanzaki M., Ohata C. et al. Sibling cases of Hailey-Hailey disease showing atypical clinical features and unique disease course. JAMA Dermatol, 2014, vol. 150, no. 1, pp. 97–99. doi: 10.1001/jamadermatol.2013.5666
32. Reyes M.V., Halac S., Mainardi C., Kurpis M. et al. Familial benign pemphigus atypical localization. Dermatol Online J, 2016, vol. 22, no. 4.
33. Cozzani E., Gasparini G., Burlando M., Drago F. et al. Atypical presentations of bullous pemphigoid: Clinical and immunopathological aspects. Autoimmun Rev, 2015, vol. 14, no. 5, pp. 438–445. doi: 10.1016/j.autrev.2015.01.006
34. Twine C.P., Malik G., Street S., Williams I.M. Bullous pemphigoid presenting as dry gangrene in a revascularized limb. J Vasc Surg, 2010, vol. 51, no. 3, pp. 732–734. doi: 10.1016/j.jvs.2009.10.110
35. Ikeda T., Okamoto K., Furukawa F. Case of atypical bullous pemphigoid with generalized pruritus and eczema as the prodrome for 10 years. J Dermatol, 2012, vol. 39, no. 8, pp. 720–721. doi: 10.1111/j.1346-8138.2011.01388.x
