Background: Pemphigus is a worldwide disease and varies in its clinical profile and epidemiology in different regions of the world. The disease is rare and few epidemiological data are available. Objective: The purpose of this study was to evaluate the epidemiologic, clinical, and therapeutic features of pemphigus in Lumbini Medical College. Methods: We retrospectively investigated a total of 70 patients with pemphigus. The parameters including age at diagnosis, sex, diagnostic methods and treatment outcome of the disease. Results: The mean age at the onset was 41.3 years. The male-to-female ratio was 1.18:1. Most of the patients were cured at the end of the treatment. Rest were clinically improved. Conclusion: Pemphigus vulgaris (PV) is the most common clinical subtype in our centre. The disease is more frequent in the fifth decade of life and has a male predominance in our region

Mikrani, Jameel Akhthar

Sharma, U

Thapa, Sudeep

Journal of Lumbini Medical College

English

Journal of Lumbini Medical College (JLMC)

83Clinical Study of Pemphigus in Lumbini Medical CollegeMikrani JA, Sharma U and Thapa S Department of Dermatology and Venereology, Lumbini Medical College, Palpa, NepalCorresponding author: Dr. Mikrani JA, Lecturer, Dept. of Dermatology and Venereology, Lumbini Medical College, Palpa, Nepal; e-mail: drjameelmikrani@gmail.comABSTRACTBackground: Pemphigus is a worldwide disease and varies in its clinical profi le and epidemiology in diff erent regions of the world. The disease is rare and few epidemiological data are available. ObjecƟ ve: The purpose of this study was to evaluate the epidemiologic, clinical, and therapeuƟ c features of pemphigus in Lumbini Medical College.  Methods: We retrospecƟ vely invesƟ gated a total of 70 paƟ ents with pemphigus. The parameters including age at diagnosis, sex, diagnosƟ c methods and treatment outcome of the disease. Results: The mean age at the onset was 41.3 years. The male-to-female raƟ o was 1.18:1. Most of the paƟ ents were cured at the end of the treatment. Rest were clinically improved. Conclusion: Pemphigus vulgaris (PV) is the most common clinical subtype in our centre. The disease  is more frequent in the fi Ō h decade of life and has a male predominance in our region. Keywords: Pemphigus, dermatological disease, epidemiology, clinical , therapeuƟ cOriginal Article L M Coll J 2013; 1(2): 83-85INTRODUCTIONThe term pemphigus refers to a group of autoimmune blistering diseases of the skin and mucous membranes characterized histologically by intraepidermal blister and acantholyƟ c cells and immunopathologically by the fi nding of IgG, IgA and C3 deposited on the surface of the keraƟ nocytes.1,2,3,4 In the paƟ ent's serum, indirect immunofl uorescence demonstrates the presence of circulating pemphigus autoantibodies that bind to epidermis. The Ɵ ter of circulaƟ ng anƟ body correlates with disease course.5,6,7Pathogenesis of pemphigus is the binding of autoanƟ bodies to keraƟ nocyte cell surface molecules. These intercellular or pemphigus anƟ bodies bind to keratinocyte desmosomes and to desmosome-free areas of the keraƟ nocyte cell membrane. The binding of autoanƟ bodies results in blisters and a loss of cell-cell adhesion, a process termed acantholysis.8,9Pemphigus has the tendency to recurrent and its clinical symptoms is varied. Prognosis is worse in patients with extensive lesion, especially when associated with infecƟ on.10 Therefore, the diagnosis and treatment has been one of the issues to focus on to the dermatologists. That is why we the choose pemphigus to study its features and treatment eff ect.We analyzed and summarized the clinical manifestaƟ ons and the treatment of pemphigus to supply the reference of clinical diagnosis and treatment.MATERIAL AND METHODS All paƟ ents diagnosed as pemphigus from the out and in-paƟ ent Department of Dermatology and Venereology of Lumbini Medical College were selected for the study. Their records were reviewed retrospecƟ vely. PaƟ ents visiƟ ng from 1st of June 2009 to 31st of May 2013 were included in the study. Socio-Demographic data and subtype of disease were studies. All the data was entered into MicrosoŌ  Excel and staƟ sƟ cal analyses were performed by the SPSS17.0 soŌ ware package and expressed with mean and standard deviaƟ on.RESULTSThere were a total of 70 paƟ ents enrolled in the study. Out of those, 38(54%) were male and 32(46%) were female. The extent of paƟ ent’s age was from 16 to 82 years old (mean 41.3 ± 28.5).Disease was classifi ed into following types:1. Pemphigus vulgaris2. Pemphigus vegetans3. Pemphigus foliaceus4. Pemphigus erythematosus5. IgA pemphigus6. HerpeƟ form  pemphigus7. ParaneoplasƟ c pemphigusThere were 42  cases of Pemphigus vulgaris accounƟ ng for 60% of all those cases. Sixteen cases (22.8%) were diagnosed as pemphigus foliaceus. Eight cases (11.4%) were of Pemphigus erythematosus variety. There were 4 cases (5.8%) diagnoses as Pemphigus vegetans. There 84Journal of Lumbini Medical Collegewere no cases of IgA, ParaneoplasƟ c and HerpeƟ form pemphigus.Fig 1: Sex distribuƟ onTHERAPEUTIC EFFECT:Pemphigus vulgaris: Out of 42 cases, 32 were clinically cured and 8 cases were improved. Pemphigus foliaceus: All the 16 cases were cured.Pemphigus erythematosus: All 8 cases were cured.Pemphigus vegetans: All 4 cases were cured as shown in table 2.Table-1: Types of PemphigusDisease Number PercentagePemphigus vulgaris 42 60Pemphigus vegetans 4 5.8Pemphigus foliaceus 16 22.8Pemphigus erythematosus 8 11.4IgA pemphigus 0 0HerpeƟ form  pemphigus 0 0ParaneoplasƟ c pemphigus 0 0Table 2: Results aŌ er treatmentDisease Total Cured ImprovedPemphigus vulgaris 42 34 8Pemphigus vegetans 4 4 NAPemphigus foliaceus 16 16 NAPemphigus erythematosus 8 8 NAIgA pemphigus NA NA NAHerpeƟ form  pemphigus NA NA NAParaneoplasƟ c pemphigus NA NA NANA= Not available or Non applicableDISCUSSIONPemphigus is a worldwide disease and varies its presentaƟ on, clinical profi le, and incidence in diff erent regions of the world. In general, the incidence ranges from 0.76 to 5 new cases per million per year.11There was a male dominance in our study with M:F raƟ o of 1.18. Overall, the sex raƟ o for pemphigus appears to be equivalent or close to equivalent. However, a few studies have found large imbalances in the sex distribuƟ on, such as a study that found a 4:1 raƟ o of females to males with pemphigus foliaceus in Tunisia and a study that found a 19:1 raƟ o of males to females in an endemic locaƟ on in Columbia.12,13Pemphigus vulgaris was the most common variety in our study. This result was comparable to those reported in the majority of the studies.12,13Treatment was very effective in early and limited diseases with 100% cure rate in many cases. Some advanced cases were parƟ ally improved. Similar results were shown by studies.14,15,16CONCLUSIONSPemphigus is a common dermatological disease. It aff ects males more than females. Pemphigus vulgaris is the most common clinical type of Pamphigus, accounƟ ng for 60% of the paƟ ents, followed by pemphigus foliaceus, accounƟ ng for 22.8%. IgA pemphigus, ParaneoplasƟ c andherpeƟ form pemphigus are rare.The total cure rate is 88.6%. Treatment is very eff ecƟ ve for vegetans, foliaceus and erythematosus types. Whereas it is eff ecƟ ve for Pemphigus vulgaris with cure rate of 81%. The eff ect of treatment in paƟ ents with mild to moderate condiƟ on of pemphigus vulgaris, pemphigus foliaceus, pemphigus erythematosus and pemphigus vegetans was best, while in serious condiƟ on of pemphigus vulgaris it was parƟ al. REFERENCES8. Scully C, Challacombe SJ. Pemphigus vulgaris: update on eƟ opathogesis, oral manifestaƟ ons, and management. J Crit Rev Oral Biol Med 2002; 13(5): 397-408.9. FermandesNC, PerezM. Treatment of pemphigus vulgaris and pemphigus foliaceus: experience with 71 paƟ ents over a 20 year period. Rev Inst Med Trop 2001; 43(1): 33-6.10. Hertl M, Eming R, Veldman C. T cell control in autoimmune bullous skin disorders. J Clin Invest 2006; 116: 1159.11. Tsuruta D, Ishii N, Hamada T et al. IgA pemphigus. Clin Dermatol 2011; 29: 437.12. Amagai M. Pemphigus. In: Dermatology, 3rd ed, Bolognia JL, Jorizzo JL, Schaff er JV, et al.. (Eds), Elsevier, 2012. Vol 1, p.461.13. Ishii K, Amagai M, Hall RP, et al. Characterization of autoantibodies in pemphigus using antigen-specific enzyme-linked immunosorbent assays with baculovirus-expressed recombinant desmogleins. J Immunol 1997; 159: 2010.14. Abasq C, Mouquet H, Gilbert D, et al. ELISA tesƟ ng of anƟ -desmoglein 1 and 3 anƟ bodies in the management of pemphigus. Arch Dermatol 2009; 145: 529. 15. Kljuic A, Bazzi H, Sundberg JP, et al. Desmoglein 4 in hair follicle diff erenƟ aƟ on and epidermal adhesion: evidence from inherited hypotrichosis and acquired pemphigus vulgaris. Cell 2003; 113:249. 16. Funakoshi T, Lunardon L, Ellebrecht CT, et al. Enrichment 85JA Mikrani et alof total serum IgG4 in paƟ ents with pemphigus. Br J Dermatol 2012; 167:1245. 17. Herbst A, Bystryn JC. PaƩ erns of remission in pemphigus vulgaris. J Am Acad Dermatol 2000; 42: 422.18. Kneisel A, Hertl M. Autoimmune bullous skin diseases. Part 1: Clinical manifestaƟ ons. J Dtsch Dermatol Ges 2011; 9: 844. 19. Bastuji-Garin S, Souissi R, Blum L, et al. ComparaƟ ve epidemiology of pemphigus in Tunisia and France: unusual incidence of pemphigus foliaceus in young Tunisian women. J Invest Dermatol 1995; 104:302. 20. Abrèu-Velez AM, Hashimoto T, Bollag WB, et al. A unique form of endemic pemphigus in northern Colombia. J Am Acad Dermatol 2003; 49: 599. 21. Chams-Davatchi C, Esmaili N, Daneshpazhooh M, et al. Randomized controlled open-label trial of four treatment regimens for pemphigus vulgaris. J Am Acad Dermatol 2007; 57: 622. 22. Ioannides D, Apalla Z, Lazaridou E, Rigopoulos D. EvaluaƟ on of mycophenolate mofeƟ l as a steroid-sparing agent in pemphigus: a randomized, prospecƟ ve study. J Eur Acad Dermatol Venereol 2012; 26: 855. 23. Chams-Davatchi C, Mortazavizadeh A, Daneshpazhooh M, et al. Randomized double blind trial of prednisolone and azathioprine, vs. prednisolone and placebo, in the treatment of pemphigus vulgaris. J Eur Acad Dermatol Venereol 2012. 

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