Subramania Adityan, M (2007) A Clinicopathological study of Autoimmune Vesiculobullous Diseases. Masters thesis, Madurai Medical College, Madurai.
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Abstract
INTRODUCTION : Vesicles and bullae are reaction patterns of the skin to injury, and thus can be caused by a wide variety of conditions. Many skin diseases present with blisters, but only in some of them does blistering occur as a primary event. This group of disorders has been traditionally termed ‘the vesiculobullous disorders’. Most primary vesiculobullous diseases are either genetic or immunologic. The latter group, also called the ‘autoimmune vesiculobullous diseases’ is the focus of this study. The autoimmune vesiculobullous diseases are a heterogeneous group of diseases. They are classified on the basis of their clinical, histopathological, and immunopathological features. Thus, they can be broadly classified histopathologically into epidermal and subepidermal blistering dermatoses. These can be further categorized, based on their immunopathological features, into several individual diseases. Though these disorders are rare in the general population, for a given patient, the impact of the diseases on the quality of life can be devastating. The severity is often variable and the course is unpredictable, and may even be fatal. Moreover, some auto immune vesiculobullous diseases may be markers for internal malignancy. Since the days of Walter lever who differentiated pemphigus from pemphigiod, dramatic progress has been made in the understanding of these diseases, with the advent of investigative techniques such as electron microscopy, immunoprecipitation, immunoblotting and molecular genetic analysis. In the therapeutic front, dexamethasone cyclophosphamide pulse therapy has improved the prognosis, and emerging therapies like biologicals hold much promise for the future. But despite the explosion of knowledge regarding the etiopathogenesis, the exact mechanism that triggers autoimmunity in these patients remains largely unclear. AIMS OF THE STUDY : This study of autoimmune vesiculobullous diseases was undertaken. 1. To find out the presence of different autoimmune vesiculobullous diseases and their incidence encountered among patients attending skin OPD. 2. To find out the various morphological patterns, symptoms, and systemic association if any. 3. To correlate the clinical, cytological, histopathological and immunofluorescence features of various autoimmune vesiculobullous diseases. MATERIALS AND METHODS The material for this study was from the patients attending the skin OPD, Government Rajaji Hospital, Madurai Medical College, Madurai, with autoimmune vesiculobullous diseases of the skin during the period of May 2004 – May 2006. In total 40 patients with vesiculobullous lesions were subjected to a detailed clinical evaluation, followed by cytological, hispathological and direct immunofluorescence studies. Out of this 40 cases, 32 were confirmed as cases of autoimmune vesiculobullous diseases, and were included in the study after getting informed consent. The remaining 8 cases whose cytology, histopathology and immunofluorescence were found to be otherwise, were excluded from the study. SUMMARY : 32 patients, diagnosed with autoimmune vesiculobullous diseases, based on history, clinical features, cytology, histopathology, and immunofluorescence were included in the study. Incidence : The incidence was 1.7:10,000, among 1,92,750 patients who attended the skin OPD during the period of study. Sex : 13 patients were male (40.6%) and 19 patients were female (59.4%). The male:female ratio was approximately 2:3. Age : 78.1% of the patients were between 21-60 years of age. Morphology and distribution : The morphology of lesions and their distribution were concordant with those described in the literature. Oral mucosal involvement and flaccid bullae was noted in 100% of the cases of pemphigus vulgaris. Tense bullae (100%) and targetoid lesions (62.5%) were the commonest lesions seen in bullous pemphigoid. Nikolsky sign and Asboe-Hansen sign : These signs were positive in 100% of the pemphigus patients, and one patient with paraneoplstic bullous pemphigoid. Tzanck smear : Acantholytic cells were demonstrated in 100% cases of pemphigus. Eosinophils could be seen in 75% of bullous pemphigoid cases multinucleated cells were seen in 23% of pemphigus vulgaris patients. Histopathology : Histopathological features consistent with pemphigus vulgaris were seen in 13 patients, pemphigus foliaceus in 2 patients, pemphigus erythematosus in 3 patients, IgA pemphigus in one patient, bullous pemphigoid in 8 patients, linear IgA dermatosis in one patient, and bullous SLE, acute LE and pemphigoid vegetans in one patient each. One patient who was diagnosed as cicatricial pemphigoid based on histopathology, was later diagnosed as linear IgA dermatosis based on DIF. Immunofluorescence : Based on direct immunofluorescence findings, a diagnosis of pemphigus vulgaris was made in 13 patients, pemphigus foliaceus in 2 patients, pemphigus erythematosus in 1 patient, bullous pemphigoid in 8 patients, linear IgA dermatosis in 2 patients acute cutaneous LE in one, bullous SLE in one patient and pemphigoid vegetans in one patient. One patient who had DIF findings of pemphigus vulgaris initially and bullous pemphigoid 2 months later, was subjected to indirect immunofluorescence and was finally diagnosed as pemphigus vulgaris. Association : Non insulin dependent diabetes mellitus was the commonest associated disease, seen in 18.75% cases, followed by hypertension (6.25%), ischaemic heart disease (3%), internal malignancy (3%) and diclofenac ingestion (3%). CONCLUSION : 1. Autoimmune vesiculobullous diseases were rare skin diseases comprising only a very small percentage of patients attending skin OPD. 2. Pemphigus vulgaris was the most common autoimmune vesiculobullous disease, followed by bullous pemphigoid. 3. Pemphigus vulgaris occurred more commonly in females. 4. Majority of the patients were between 21-60 yrs of age. 5. Tzanck smear is a useful bedside tool which points towards the final diagnosis. 6. Histopathological examination is of paramount importance in arriving at the diagnosis. 7. Direct immunofluorescence technique is a useful supplement to routine histopathology, and may prove to be of diagnostic value and therapeutic significance in selected cases. 8. Indirect immunofluorescence is helpful in arriving at the correct diagnosis in selected cases.
| Item Type: | Thesis (Masters) |
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| Uncontrolled Keywords: | Autoimmune Vesiculobullous Diseases ; Clinicopathological study. |
| Subjects: | MEDICAL > Dermatology Venereology and Leprosy |
| Depositing User: | Subramani R |
| Date Deposited: | 02 Mar 2018 03:00 |
| Last Modified: | 02 Mar 2018 03:00 |
| URI: | http://repository-tnmgrmu.ac.in/id/eprint/5966 |
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