A Clinicopathological Study of Immunobullous Diseases
Keywords:
pemphigus, bullous pemphigoid, Immunobullous lesions
Abstract
BACKGROUND Vesiculobullous disorders are a group of disorders involving the formation of a fluid filled cavity within or beneath the epidermis, due to the presence of autoantibodies against adhesion molecules in epidermis and dermis. The clinical presentation and the microscopic features of different diseases belonging to this group vary greatly. This study was undertaken to describe the clinical features and the histopathological patterns of immunobullous diseases. METHOD 30 cases of immunobullous disorders who attended Dermatology Outpatient Department were included in the study. Relevant clinical details of these cases were collected and punch biopsies were taken from the site of lesion which were used for routine histopathology. RESULTS Among the 30 cases included in our study, males were found to be more commonly affected than females. The most common immunobullous lesions encountered were Pemphigus Vulgaris(13/30) and Bullous Pemphigoid (10/30). In pemphigus group of diseases, patients presented with small cutaneous and oral vesicles. Pemphigus foliaceous patients showed cutaneous lesions only. Intraepidermal vesicles containing acantholytic cells and inflammatory cells were characteristic histological features of the Pemphigus group. Bullous pemphigoid lesions were large bullae on the limbs and within the oral cavity. Most of the cases of Bullous Pemphigoid showed the formation of subepidermal bullae predominantly containing eosinophils. CONCLUSION Both the clinical findings and the histological features are invaluable in arriving at a definite diagnosis in immunobullous diseases.References
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15. Kundu B ,Sadhu A, Meyur R, Kundu S, De A, Sarkar S. Original article : Comparative microanatomy of the normal skin with that of immunobullous condition. Indian Journal of Basic and Applied Medical Research 2013;2(8):1146–1152.
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17. Emmerson RW, Wilson-Jones E. Eosinophilic spongiosis in pemphigus. A report of unusual histological change in pemphigus. Arch Dermatol. 1968;97:252-257.
18. Khandpur S, Verma P. Bullous pemphigoid. Indian J Dermatol Venereol Leprol. 2011;77(4):450.
19. Chen M, Kim GH, Prakash L, Woodley DT. Epidermolysis bullosa acquisita: Autoimmunity to anchoring fibril collagen. Autoimmunity. 2012;45(1):91–101.
20. Grover C, Khurana A, Sharma S, Singal A. Bullous Systemic Lupus Erythematosus Indian J Dermatol. 2013 Nov-Dec; 58(6): 492-495
21. Nischal KC, Khopkar U. An approach to the diagnosis of neutrophilic dermatoses: A histopathological perspective. Indian J Dermatol Venerol Leprol 2007;73:22-30.
2. Kambi DP, Deepthi,Santosh CS,Suguna BV,Gopal MG, Abhishek V. Direct immunofluorescence study in autoimmune bullous disorders of skin. International Journal of Biomedical and Advance Research. 2013;4(9).610-618.
3. Langan SM, Smeeth L, Hubbard R, Fleming KM, Smith CJP, West J. Bullous pemphigoid and pemphigus vulgaris--incidence and mortality in the UK: population based cohort study. BMJ. 2008;337:a180.
4. Rizvi SR, Sadiq S. Use of direct immunofluorescent microscopy in the diagnosis of vesiculobullous disorders of skin. Pak J Med Sci. 2010;26(2):411-415.
5. Ajithkumar K. Incidence of Pemphigus in Thrissur District, South India. Indian J Dermatol Venereol Leprol. 2008;74:349-351.
6. James K, Culton D, Diaz L. Diagnosis & Clinical Features of Pemphigus Foliaceus. Dermatol Clin. 2011;29(3):405–12.
7. Leroy D, Lebrun J, Maillard V, Mandard JC, Deschamps P. Pemphigus vegetant , a clinical type of chronic pustular dermatitis of Hallopeau. Ann Dermatol Venereol 1982;109:549-555.
8. Neff AG, Turner M, Mutasim DF. Treatment strategies in mucous membrane pemphigoid. Ther Clin Risk Manag. 2008;4(3):617–626.
9. Arya SR, Valand AG, Krishna K. A clinic-pathological study of 70 cases of Pemphigus. Indian J Dermatol Venerol Leprol 1999;65:168-71.
10. Patel PR, Kapuria DP, Chiplonkar SG. A Clinico-Pathological Study of 22 Cases of Pemphigus National Journal of Medical Research. 2012;2(3):269–271.
11. Mahoney MG, Wang Z, Rothenberger K, Koch PJ, Amagai M, Stanley JR. Explanations for the clinical and microscopic localization of lesions in pemphigus foliaceus and vulgaris. J Clin Invest. 1999;103(4):461–468.
12. Kambil SM, Madavamurthy P. Immunobullous disorders: Clinical histopathological and immunofl uorescence study of thirty-six cases. Muller J Med Sci Res 2014;5:134-138.
13. Aswani V, Vaz B, Shah S, Malkani RH. Bullous Systemic Lupus Erythematosus. Indian J Dermatol Venerol Leprol 1993;59:97-100
14. Liu Z, Li N, Diaz L a. Immunopathological mechanisms of acantholysis in pemphigus vulgaris: An explanation by ultrastructural observations. J Invest Dermatol. 2004;122(5):xiii-xiv
15. Kundu B ,Sadhu A, Meyur R, Kundu S, De A, Sarkar S. Original article : Comparative microanatomy of the normal skin with that of immunobullous condition. Indian Journal of Basic and Applied Medical Research 2013;2(8):1146–1152.
16. Arundhathi S, Raghunatha S, Mahadeva KC. A Cross-sectional Study of Clinical, Histopathological and Direct Immunofluorescence Spectrum of Vesiculobullous Disorders. J Clin Diagnostic Res [Internet]. 2013;7(12):2788–2792.
17. Emmerson RW, Wilson-Jones E. Eosinophilic spongiosis in pemphigus. A report of unusual histological change in pemphigus. Arch Dermatol. 1968;97:252-257.
18. Khandpur S, Verma P. Bullous pemphigoid. Indian J Dermatol Venereol Leprol. 2011;77(4):450.
19. Chen M, Kim GH, Prakash L, Woodley DT. Epidermolysis bullosa acquisita: Autoimmunity to anchoring fibril collagen. Autoimmunity. 2012;45(1):91–101.
20. Grover C, Khurana A, Sharma S, Singal A. Bullous Systemic Lupus Erythematosus Indian J Dermatol. 2013 Nov-Dec; 58(6): 492-495
21. Nischal KC, Khopkar U. An approach to the diagnosis of neutrophilic dermatoses: A histopathological perspective. Indian J Dermatol Venerol Leprol 2007;73:22-30.
Published
2019-02-15
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