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Category: Volume 07 Issue 10 October 2019
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Title: Clinicoepidemiological Profile of Pemphigus

Authors: Dr Sheena Ann Simon, Dr Suja V

 DOI: https://dx.doi.org/10.18535/jmscr/v7i10.126

Abstract

   

Introduction: Pemphigus refers to a group of acquired, chronic autoimmune blistering disorders presenting clinically by flaccid intraepidermal blisters, erosions and ulcerations on skin and mucosa. The study was aimed at evaluating the epidemiological and clinical features of pemphigus.

Methods: After taking history of clinically diagnosed pemphigus cases, characteristics of the lesions were noted. Extent of involvement of both skin and mucosa was graded using appropriate methods. Data was analysed using statistical package for social science version 10.

Results: Among the 30 patients, onset of the disease was noted in the younger age group in 16.7 %.  In majority of the patients, duration of the disease was less than 6 months. Onset of the disease on mucosa alone was seen in 50 % cases and only 3.3% had mucocutaneous onset. Pain or burning sensation was present in 60% cases and pruritus in 40%. Based on body surface area, 53.3% had moderate degree and 26.7% had severe degree of skin involvement. Nikolsky’s sign was positive in only 75 % patients. Two pemphigus foliaceus cases had oral lesions and 1 had nasal lesions. Grade 1 oral mucosal involvement was found in 40.2% cases and 16.7% had grade 3 involvement. Two pemphigus vulgaris patients had paronychia and oral candidiasis was present in 12 patients.

Conclusion: Severe mucocutaneous pemphigus and onset in the younger age group may have social and medical implications. Nikolsky’s sign was found as less sensitive. Mucosal involvement was seen in pemphigus foliaceus patients.

Keywords: pemphigus vulgaris, foliaceus.

References

  1. Kitajima Y. Current and prospective understanding of clinical classification, pathomechanisms and therapy in pemphigus. Arch Dermatol Res .2003; 295 : 17-23.
  2. Chmurova N, Svecova P. Pemphigus vulgaris: a 11 year review. Bratisl Lek Listy.2009;110(8):500-3.
  3. Herbst A, Bystryn JC. Patterns of remission in pemphigus vulgaris. J Am Acad of Dermatol. 2000;42( 3 ):422-7.
  4. Camacho-Alonso F, López-Jornet P, Bermejo-Fenoll A. Pemphigus vulgaris. A presentation of 14 cases and review of the literature. Med Oral Patol Oral Cir Bucal. 2005 ;10(4):282-8.
  5. Ramassamy S, Agrawal P, Sathishkumar D, Mathew L, Peter JV, Mani T, George R. Clinical, immunological profile and follow up of patients with pemphigus: A study from India. Indian Journal of Dermatology, Venereology, and Leprology. 2018 Jul 1;84(4):408.
  6. Asia AJ, Laul RA. Clinico-Epidemiological Profile of Pemphigus: An Observational Study. JMSCR. 2015;3(8): 7090-94.
  7. ZHU X, PAN J,YU Z, WANG Y, CAI L, ZHENG S. Epidemiology of pemphigus vulgaris in the Northeast China:A 10-year retrospective study. Journal of Dermatology 2014; 41: 70–5.
  8. Mahajan A, Kaur J, Manjari M, Sharma S, Bains P, Awal G et al. Oral pemphigus vulgaris: clinicopathological study of 35 cases at a tertiary care centre in North India.Int J Res Dermatol.2017 ;3(2):192-5.
  9. Chowdhury J, Datta PK, Chowdhury SN, Das NK. A clinicopathological study of pemphigus in Eastern India with special reference to direct immunofluorescence. Indian journal of dermatology. 2016 ;61(3):288.-94.
  10. Suliman NM, Åstrøm AN, Ali RW, Salman H, Johannessen AC. Clinical and histological characterization of oralpemphigus lesions in patients with skin diseases: a cross sectional study from Sudan. BMC Oral Health.2013; 13:66.
  11. Shamim T, Varghese V, Shameena PM, Sudha S. Pemphigus vulgaris inoral cavity: Clinical analysis of 71 cases. Med Oral Patol Oral Cir Bucal.2008;13(10): 622-6.
  12. Kavusi S. Outcome of pemphigus vulgaris. J Eur Acad Dermatol Venereol. 2008; 22(5): 580-4.
  13. Huda MM, Afsar MJ. A clinicopathological study of pemphigus. Indian J Dermatol. 2001; 46(2):75-9.
  14. Arya SR, Valand AG, Krishna K. A clinico-pathological study of 70 cases of pemphigus. Indian J Dermatol Venereol Leprol. 1999;65:168-71.
  15. Uzun S, Durdu M. The specificity and sensiivity of Nikolsky sign in the diagnosis of pemphigus. J Am Acadn Dermatol 2006;54:411-5.
  16. Javidi Z, Meibodi NT, Nahidi Y. Epidemiology of pemphigus in northeast Iran: A 10-year retrospective study. Indian J Dermatol. 2007;52(4):188-91.
  17. Pietkiewicz P, Bowszyc- Dmochowska M, Gornowicz-Porowska J, Dmochowski M. Involvement of Nail Apparatus in Pemphigus Vulgaris in Ethnic Poles Is Infrequent. Front Med (Lausanne). 2018; 5: 227.
  18. N,  Mortazavi H, Noormohammadpour P, Boreiri M, Soori T, Farahani IV et al. Pemphigus Vulgaris and Infections: A Retrospective Study on 155 Patients. Autoimmune Dis. 2013;  2013 :834295.

Corresponding Author

Dr Sheena Ann Simon

Department of Dermatology & Venereology, Government Medical College, Thiruvananthapuram