A brief review of pemphigus vulgaris
Tóm tắt
Pemphigus vulgaris is an autoimmune disorder which presents with painful mucocutaneous blisters and erosions. On the skin, they are flaccid bullae or erosions, and on the mucosa, they present as erosions. This disease is rare but is devastating to those who have it; it also is related—perhaps genetically—to other autoimmune conditions. This is to say that a patient can develop pemphigus vulgaris if they have thyroiditis or diabetes mellitus. Biopsy is needed to obtain histopathological evidence of the breakdown of intercellular connections due to the autoimmune attack on components of desmosomes, which are responsible for intercellular integrity above the basement membrane. When these desmosomes are attacked, loss of connection ensues, and the cells break apart at these connections; this leads to fluid buildup, seen grossly as bullae. Treatment of the disease is difficult and sometimes unsafe. For decades, the mainstay of treatment has been glucocorticoids followed by other drugs. Unfortunately, these drugs are systemically absorbed, and the side effect profile can be unfavorable. In the past several years however, more innovative treatments have emerged that may help ease the cost and safety burden to patients. This review highlights the major points about pemphigus vulgaris, its pathophysiology, and its treatment.
Tài liệu tham khảo
Abasq C, Mouquet H, Gilbert D, Tron F, Grassi V, Musette P, et al. ELISA Testing of Anti–Desmoglein 1 and 3 Antibodies in the Management of Pemphigus. Arch Dermatol. 2009;145(5):529–35.
Amagai M, Komai A, Hashimoto T, Shirakata Y, Hashimoto K, Yamada T, et al. Usefulness of enzyme-linked immunosorbent assay using recombinant desmogleins 1 and 3 for serodiagnosis of pemphigus. Br J Dermatol. 1999;140(2):351–7.
Arin M, Engert A, Krieg T, Hunzelmann N. Anti-CD20 monoclonal antibody (rituximab) in the treatment of pemphigus. Br J Dermatol. 2005;153(3):620–5.
Baum S, Sakka N, Artsi O, Trau H, Barzilai A. Diagnosis and classification of autoimmune blistering diseases. Autoimmun Rev. 2014;13(4–5):482–9.
Beutner E, Jordon R. Demonstration of Skin Antibodies in Sera of Pemphigus Vulgaris Patients by Indirect Immunofluorescent Staining. Exp Biol Med. 1964;117(2):505–10.
Bhol K, Natarajan K, Nagarwalla N, Mohimen A, Aoki V, Ahmed A. Correlation of peptide specificity and IgG subclass with pathogenic and nonpathogenic autoantibodies in pemphigus vulgaris: a model for autoimmunity. Proc Natl Acad Sci. 1995;92(11):5239–43.
Bomm L, Fracaroli T, Sodré J, Bressan A, Gripp A. Off-label use of rituximab in dermatology: pemphigus treatment. An Bras Dermatol. 2013;88(4):676–8.
Bystryn J. The adjuvant therapy of pemphigus. Arch Derm. 1996;132(2):203–12.
Bystryn J, Rudolph J. Pemphigus. Lancet. 2005;366(9479):61–73.
Chams-Davatchi C, Esmaili N, Daneshpazhooh M, Valikhani M, Balighi K, Hallaji Z, et al. Randomized controlled open-label trial of four treatment regimens for pemphigus vulgaris. J Am Acad Dermatol. 2007;57(4):622–8.
Ding X, Diaz L, Fairley J, Giudice G, Liu Z. The Anti-Desmoglein 1 Autoantibodies in Pemphigus Vulgaris Sera are Pathogenic. J Investig Dermatol. 1999;112(5):739–43.
Ellebrecht C, Choi E, Allman D, Tsai D, Wegener W, Goldenberg D, et al. Subcutaneous Veltuzumab, a Humanized Anti-CD20 Antibody, in the Treatment of Refractory Pemphigus Vulgaris. JAMA Dermatology. 2014;150(12):1331.
Garrod D, Chidgey M. Desmosome structure, composition and function. Biochim Biophys Acta Biomembr. 2008;1778(3):572–87.
Herbst A, Bystryn J. Patterns of remission in pemphigus vulgaris. J Am Acad Dermatol. 2000;42(3):422–7.
Hertl M, Zillikens D, Borradori L, Bruckner-Tuderman L, Burckhard H, Eming R, et al. Recommendations for the use of rituximab (anti-CD20 antibody) in the treatment of autoimmune bullous skin diseases. J Dtsch Dermatol Ges. 2008;6(5):366–73.
Jackson A, Hall A, McLelland J. Thiopurine methyltransferase levels should be measured before commening patients on azathioprine. Br J Dermatol. 1997;136(1):133–4.
Janeway C. Immunobiology: The Immune System in Heath and Disease. 5th ed. London: Harcourt Brace and Company; 1999.
Joly P, Litrowski N. Pemphigus group (vulgaris, vegetans, foliaceus, herpetiformis, brasiliensis). Clin Dermatol. 2011;29(4):432–6.
Kavala M, Altıntaş S, Kocatürk E, Zindancı İ, Can B, Ruhi Ç, et al. Ear, nose and throat involvement in patients with pemphigus vulgaris: correlation with severity, phenotype and disease activity. J Eur Acad Dermatol Venereol. 2011;25(11):1324–7.
Kavala M, Topaloğlu Demir F, Zindanci I, Can B, Turkoğlu Z, Zemheri E, et al. Genital involvement in pemphigus vulgaris (PV): Correlation with clinical and cervicovaginal Pap smear findings. J Am Acad Dermatol. 2015;73(4):655–9.
Kumaran M, Kanwar A, Seshadri D. Acantholysis revisited: Back to basics. Indian J Dermatol Venereol Leprol. 2013;79(1):120.
Kwon O, Brautbar C, Weintrob N, Sprecher E, Saphirman C, Bloch K, et al. Immunogenetics of HLA class II in Israeli Ashkenazi Jewish, Israeli non-Ashkenazi Jewish, and in Israeli Arab IDDM patients. Hum Immunol. 2001;62(1):85–91.
Martin L, Werth V, Villaneuva E, Murrell D. A systematic review of randomized controlled trials for pemphigus vulgaris and pemphigus foliaceus. J Am Acad Dermatol. 2011;64(5):903–8.
Meggitt S, Anstey A, Mohd Mustapa M, Reynolds N, Wakelin S. British Association of Dermatologists’ guidelines for the safe and effective prescribing of azathioprine 2011. Br J Dermatol. 2011;165(4):711–34.
Morrison L. Direct immunofluorescence microscopy in the diagnosis of autoimmune bullous dermatoses. Clin Dermatol. 2001;19(5):607–13.
Mustafa M, Porter S, Smoller B, Sitaru C. Oral mucosal manifestations of autoimmune skin diseases. Autoimmun Rev. 2015;14(10):930–51.
Parameswaran A, Attwood K, Sato R, Seiffert-Sinha K, Sinha A. Identification of a new disease cluster of pemphigus vulgaris with autoimmune thyroid disease, rheumatoid arthritis and type I diabetes. Br J Dermatol. 2015;172(3):729–38.
Pemphigus. Pemphigus Pemphigoid Foundation (IPPF) 2014. http://www.pemphigus.org/research/clinically-speaking/pemphigus/. Accessed 11 Feb 2017.
Pisanti S, Sharav Y, Kaufman E, Posner L. Pemphigus vulgaris: Incidence in Jews of different ethnic groups, according to age, sex, and initial lesion. Oral Surg Oral Med Oral Pathol. 1974;38(3):382–7.
Razzaque Ahmed A, Moy R. Death in pemphigus. J Am Acad Dermatol. 1982;7(2):221–8.
Saha M, Powell A, Bhogal B, Black M, Groves R. Pulsed intravenous cyclophosphamide and methylprednisolone therapy in refractory pemphigus. Br J Dermatol. 2009;162(4):790–7.
Schacke H. Mechanisms involved in the side effects of glucocorticoids. Pharmacol Ther. 2002;96(1):23–43.
Stanley J, Amagai M. Pemphigus, Bullous Impetigo, and the Staphylococcal Scalded-Skin Syndrome. N Engl J Med. 2006;355(17):1800–10.
Venugopal S, Murrell D. Diagnosis and Clinical Features of Pemphigus Vulgaris. Dermatol Clin. 2011;29(3):373–80.