Oral Mucous Membrane Pemphigoid Without Skin Involvement Treated
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Central Journal of Dermatology and Clinical Research Case Report *Corresponding author Manas Bajpai, Associate Professor, Departmentt of Oral and Maxillofacial Pathology, NIMS Dental Oral Mucous Membrane College, Jaipur, India, Email: Pemphigoid without Skin Submitted: 08 June 2017 Accepted: 22 June 2017 Published: 23 June 2017 Involvement Treated by Intra- Copyright © 2017 Bajpai et al. Venous Immunoglobulins: A Case OPEN ACCESS Keywords Report with the Correlation of • Mucous membrane pemphigoid • Oral mucosa • Immunoflurescence Clinical, Histopathological and • Intra – venous immunoglobulins Direct Immunoflurescence Findings and a Literature Review of the Treatment Modalities Manas Bajpai*and Nilesh Pardhe Department of Oral and Maxillofacial Pathology, NIMS Dental College, India Abstract Mucous membrane pemphigoid (MMP) is an autoimmune blistering disorder of mucosa, characterized by subepithellial bullae. The involvement of skin is only seen in one quarter of the patients with MMP. We report a case of 37 years old male presented with painful erosions involving gingiva, tongue and palatal mucosa without any skin involvement. Hisopathological features and direct immunofluroscene l to the final diagnosis of MMP. INTRODUCTION since 6 months for which no response was found. The lesions of palate, tongue and lip were extremely painful due to which he group of autoimmune heterogeneous disorder characterized Mucous membrane pemphigoid (MMP) can be defined as a contributory to the presenting symptom. Intra oral examination by subepithelial blistering disease primarily affecting mucous revealedexperienced erosions difficulty of labial in eating. mucosa His (Figure family 1) history and palate was (Figure non – membrane with or without involvement of skin [1,2]. Various 2). Localized desquamative marginal gingivitis was noted with relation to teeth #12, #21, #22 and #33 (Figure 3). Marginal targets of autoantibodies in MMP [3]. Intra–orally MMP shows gingiva showed a tendency to bleed and peel off on pressure. variedcomponents manifestation of basement i.e. painful membrane erosion, have desqumative been identified gingivitis, as erythematous patches etc [2,4]. We present a case of oral MMP Dorsal surface of the tongue showed bullae covered by a without skin and other mucous membrane involvement; we also pseudomembrane with reddish and yellowish in color (Figure 4). No ocular and genital lesions were found. Tzank test was the case. performed which was found to be negative. On clinical grounds a describe the histopathological and immunoflurosence findings of provisional diagnosis of mucous membrane pemphigoid with the CASE PRESENTATION differential diagnosis of pemphigus vulgaris was made. An otherwise healthy 37 year old Indian male presented to a An incisional biopsy was taken from the surrounding mucos private dental clinic with the chief complaint of painful erosions of the bulla of the dorsum of the tongue under local anesthesia of lip, gingiva and palate from 8 months. He had been treated and the tissue was submitted to the Department of Oral and by oral prednisolone (0.5mg/kg/day) from a general physician Maxillofacial Pathology NIMS Dental College Jaipur (India). The Cite this article: Bajpai M, Pardhe N (2017) Oral Mucous Membrane Pemphigoid without Skin Involvement Treated by Intra-Venous Immunoglobulins: A Case Report with the Correlation of Clinical, Histopathological and Direct Immunoflurescence Findings and a Literature Review of the Treatment Modalities. J Dermatolog Clin Res 5(4): 1106. Bajpai et al. (2017) Email: Central was found after the completion of third cycle. The therapy was further continued till six months. The follow up period of 1 year was found to be uneventful. DISCUSSION MMPs are autoimmunaneoe, vesicobullous disease that affects mucosa or mucosa and skin both [1]. There are several variants of MMP, each with distinctive clinical features, pattern of immunopathology and They are oral pemphigoid, anti epiligrin pemphigoid, anti antigenic specificity of autoantibodies. Figure 1 Erosive lesion of labial mucosa. Figure 4 Bullae present on the dorsum of the tongue. Figure 2 Erythematous patch on palate. Figure 5 Histopathological examination shows sub basilar split between epithelium and connective tissue stroma (Hematoxylin and Eosin stain X20). Figure 3 Localized desquamative marginal gingivitis. histopathological examination of hematoxylin and eosin stained subepithelial separation with the underlying connective tissue stromasoft tissue (Figure section 5). revealed The connective a stratified tissue squamous stroma epithelium was densely with infiltrated with chronic inflammatory cells chiefly lymphocytes ofand epithelium plasma cells and basement(Figure 6). membrane Direct immunuflurosence (Figure 7). Based on(DIF) these of peribullous mucosa revealed a linear band of IgA, IgG at the zone Figure 6 features a final diagnosis of MMP was rendered. Intravenous repeated every three months. Complete remission of the lesions Connective tissue stroma shows dense infiltration of chronic immunoglobulin (IVIG) (2gm/Kg/cycle) started which was inflammatory cells (Hematoxylin and Eosin stain X40). J Dermatolog Clin Res 5(4): 1106 (2017) 2/5 Bajpai et al. (2017) Email: Central as a clinical differential diagnosis of blistering diseases (Table 1). Histopathologically it shows subepithelial blisters without acantholysis and connective tissues shows a dense infiltration of junctioninflammatory between cells the [3-5]. connective Direct immunofluorescent tissue and epithelium techniques [1]. The show homogeneous IgG and C3 complement deposits along the bypresent subepithelial case revealed blisters a withlinear the band production of IgG and of autoantibodiesIgG on DIF at targetedepithelium to – certainbasement components membrane of zone. the MMPbasal is lamina characterized of the [11].epithelium The diagnosis immunoglobulin of MMP Gis based(IgG) (97%),upon the C3 correlation complement of factor (78%) and, to a lesser degree, IgA (27%) and IgM (12%) Figure 7 clinical, histopathological and DIF findings. DIF is a useful adjunct Direct immunoflurescence shows a linear band of IgG, IgA and C3 at the zone of epithelium and basement membrane. (Original helpsfor the to definitive rule out thediagnosis differential of bullous diagnoses diseases, which especially differ in when their magnification X40). the clinical and histopathological findings are not conclusive.DIF BP antigen mucosal pemphigoid, ocular pemphigoid & multiple the response of the treatment [3]. treatment protocol. DIF may also be used in order to determine case reports of MMP in English language revealed that MMP is The treatment of MMP depends upon the severity of antigens. An exhaustive literature review of the published disease ranging from topical steroids to systemic steroids hand, it is up to 3 times more common than pemphigus, which itselfapproximately has an annual 7 times incidence less common of 0.5 tothan 3.2 BP per [4]. 100000 On the people other modulators comprised of polyclonal antibodies derived and ant metabolites [12]. (Table 2) IVIgs are biologic immune [1,2]. There are no known racial or geographic predilections. from a large pool of healthy plasma donors [20]. They are Most patients ranged from 23 to 75 years age; maximum being 50 approved treatment modality for several diseases including immune thrombocytopenic purpura, primary and secondary to 60 years old. The female/male ratio was found to be 1.8:1[3]. by soft and hard palate, presenting as thick walled bullae immunodeficiency, pediatric HIV, Systemic lupus eryhematosus Intra – orally, it most commonly involves gingiva followed persisting for 1 to 2 days before rupturing, leaving raw, eroded clearly(SLE), understood,Kawasaki althoughdisease, severalmultiple theories sclerosis, have been pemphigus, proposed erythematous or bleeding surface [2,3,5]. The present case pemphigoid etc [20-23]. The mechanism of action of IVIg is not showed the involvement of gingiva, labial mucosa and hard palate. Skin lesions are seen in only 25% of the patients. unresponsive(Figure 8). For to autoimmune the conventional diseases therapy IVIg is notand considered patients withas a Erythema multiforme, pemphigus vulgaris, bullous pemphigoid. first line treatment and it is reserved for the patients who are bullous lichen planus and lichenoid reaction can be considered are the well accepted mode of treatment for the patients with rapidly progressive disease [22]. Immuno suppressive drugs Figure 8 Mode of action of IVIg. J Dermatolog Clin Res 5(4): 1106 (2017) 3/5 Bajpai et al. (2017) Email: Central Table 1: S. No Differential diagnosesDifferential of MMP diagnosis with differentiating features [6-10]. Differentiating features EM is generally associated with reaction infections of herpes simplex and mycoplasma 1 Erythema multiforme (EM) easily differentiated from EM. and/or drug reactions while MMP is autoimmune disease. Histopathologically MMP can be 2 Histopathologically PV shows suprabasilar splitting and MMP shows sub basilar splitting between basement membrane zone and underlying connective tissue stroma. In Pemphigus vulgaris (PV) epitheliumpemphigus, and direct basement immunofluorescence membrane zone. shows deposition of IgG and C3 in lower layer of BPepithelium, seldom affects in MMP mucosa direct unlikeimmunofluorescence MMP. Histopathologically exhibits a linear BP exhibits band of relatively IgG, IgA andnormal C3 at 3 Bullous pemphigoid (BP) epithelium without acantholysis and the basement