scholarly journals Mucous membrane pemphigoid - a report of four cases

2021 ◽  
pp. 98-98
Author(s):  
Ivana Jocic ◽  
Dragana Dakovic ◽  
Lidija Kandolf-Sekulovic ◽  
Ljubomir Novakovic ◽  
Zeljko Mijuskovic
Keyword(s):  
Mucous Membrane ◽  
Complex Disease ◽  
Basal Membrane ◽  
Chronic Inflammatory Disease ◽  
Elisa Test ◽  
Mucous Membrane Pemphigoid ◽  
Direct Immunofluorescence ◽  
Ocular Involvement ◽  
Clinical Appearance ◽  
Split Skin

Introduction. Mucous membrane pemphigoid (MMP) is a rare autoimmune, chronic inflammatory disease which affects mucous membranes, most commonly the eyes and 3 mouth, with or without skin involvement. It is a complex disease with several complications including scarring, especially on conjunctival mucosa that can lead to visual loss. Case report. We report four patients (two men and two women) with MMP. In all patients the disease started in their seventies. The diagnosis was confirmed on the basis of clinical appearance, histology, direct and indirect immunofluorescence studies, indirect split skin technique and ELISA test. The majority of lesions were on the gums and buccal mucosa, one patient had laryngeal involvement and a lesion on the umbilicus. No ocular involvement and no malignancy were detected. Direct immunofluorescence tests revealed continuous linear IgG deposition in the basal membrane zone in two patients, and they were treated with oral nicotinamide and tetracycline hydrochloride. In two patients we detected IgG along with IgA linear deposition; they received treatement with methylprednisolone. Complete remission was achieved in all patients. Conclusion. Early diagnosis as well as a multidisciplinary therapeutic approach is necessary for the MMP treatment in long term disease control and reduction of the disease-related complications.

scholarly journals 126 Clinical implications of negative direct immunofluorescence (DIF) results in Mucous Membrane Pemphigoid (MMP) with ocular disease

2016 ◽  
Vol 136 (5) ◽  
pp. S23
Author(s):  
M. Labowsky ◽  
C. Shieh ◽  
S. Stinnett ◽  
M. Daluvoy ◽  
R. Hall
Keyword(s):  
Mucous Membrane ◽  
Ocular Disease ◽  
Mucous Membrane Pemphigoid ◽  
Direct Immunofluorescence ◽  
Clinical Implications

scholarly journals Autoantibody Detection for Diagnosis in Direct Immunofluorescence-Negative Mucous Membrane Pemphigoid

Ophthalmology ◽  
2020 ◽  
Author(s):  
John Dart ◽  
Jane Setterfield ◽  
Richard W. Groves ◽  
John B. Mee ◽  
Gilles F.H. Diercks ◽  
...  
Keyword(s):  
Mucous Membrane ◽  
Mucous Membrane Pemphigoid ◽  
Direct Immunofluorescence ◽  
Autoantibody Detection

scholarly journals Mucous Membrane Pemphigoid with Ocular Involvement

Ophthalmology ◽  
2018 ◽  
Vol 125 (4) ◽  
pp. 496-504 ◽  
Author(s):  
Hon Shing Ong ◽  
Jane F. Setterfield ◽  
Darwin C. Minassian ◽  
John K. Dart ◽  
Debbie Booth ◽  
...  
Keyword(s):  
Mucous Membrane ◽  
Mucous Membrane Pemphigoid ◽  
Ocular Involvement

Clinical Implications of Direct Immunofluorescence Findings in Patients With Ocular Mucous Membrane Pemphigoid

2017 ◽  
Vol 183 ◽  
pp. 48-55 ◽  
Author(s):  
Mary T. Labowsky ◽  
Sandra S. Stinnett ◽  
Jason Liss ◽  
Melissa Daluvoy ◽  
Russell P. Hall ◽  
...  
Keyword(s):  
Mucous Membrane ◽  
Mucous Membrane Pemphigoid ◽  
Direct Immunofluorescence ◽  
Clinical Implications

The use of rituximab in refractory mucous membrane pemphigoid with severe ocular involvement

2009 ◽  
Vol 93 (4) ◽  
pp. 421-422 ◽  
Author(s):  
A H Ross ◽  
P Jaycock ◽  
S D Cook ◽  
A D Dick ◽  
D M Tole
Keyword(s):  
Mucous Membrane ◽  
Mucous Membrane Pemphigoid ◽  
Ocular Involvement

scholarly journals Unilateral Oral Mucous Membrane Pemphigoid: Refractory Atypical Presentation Successfully Treated with Intravenous Immunoglobulins

2015 ◽  
Vol 2015 ◽  
pp. 1-3 ◽  
Author(s):  
André Laureano ◽  
Jorge Cardoso
Keyword(s):  
Mucous Membrane ◽  
Intravenous Immunoglobulins ◽  
Mucous Membrane Pemphigoid ◽  
Direct Immunofluorescence ◽  
Oral Mucous Membrane ◽  
Mucosal Involvement ◽  
Oral Corticosteroids ◽  
Local Trauma ◽  
Palate Mucosa ◽  
The Right

A 57-year-old male presented with a 6-month history of blisters and painful erosions on the right buccal mucosa. No skin or other mucosal involvement was seen. The findings of histopathological and direct immunofluorescence examinations were sufficient for the diagnosis of oral mucous membrane pemphigoid in the context of adequate clinical correlation. No response was seen after topical therapies and oral corticosteroids or dapsone. Intravenous immunoglobulin was started and repeated every three weeks. Complete remission was achieved after three cycles and no recurrence was seen after two years of follow-up. The authors report a rare unilateral presentation of oral mucous membrane pemphigoid on the right buccal and hard palate mucosa, without additional involvement during a period of five years. Local trauma or autoimmune factors are possible etiologic factors for this rare disorder, here with unique presentation.

Incidence and Clinical Characteristics of Ocular Involvement in Mucous Membrane Pemphigoid

2018 ◽  
Vol 27 (5) ◽  
pp. 821-825 ◽  
Author(s):  
Gloria H. Hong ◽  
Irfan R. Khan ◽  
Amde Selassie Shifera ◽  
Chinwenwa Okeagu ◽  
Jennifer E. Thorne
Keyword(s):  
Mucous Membrane ◽  
Clinical Characteristics ◽  
Mucous Membrane Pemphigoid ◽  
Ocular Involvement

scholarly journals The Second Study of Clinical and Immunological Findings in Anti-laminin 332-Type Mucous Membrane Pemphigoid Examined at Kurume University—Diagnosis Criteria Suggested by Summary of 133 Cases

2021 ◽  
Vol 12 ◽  
Author(s):  
Hua Qian ◽  
Yohei Natsuaki ◽  
Hiroshi Koga ◽  
Tamihiro Kawakami ◽  
Chiharu Tateishi ◽  
...  
Keyword(s):  
Human Skin ◽  
Mucous Membrane ◽  
Indirect Immunofluorescence ◽  
Mucous Membrane Pemphigoid ◽  
Direct Immunofluorescence ◽  
Inclusion Criteria ◽  
Normal Human Skin ◽  
Mucosal Lesions ◽  
Positive Direct ◽  
Laminin 332

BackgroundRecently, we published an article retrospectively summarizing the results in 55 anti-laminin 332 (LM332)-type mucous membrane pemphigoid (MMP) cases examined at Kurume University, which were diagnosed by strict inclusion criteria, including positive reactivity in direct immunofluorescence and absence of antibodies to non-LM332 autoantigens. However, indirect immunofluorescence using 1M-NaCl-split normal human skin (ssIIF) is also valuable for diagnosis of anti-LM332-type MMP.MethodsIn this second study, we selected 133 anti-LM332-type MMP cases, which were diagnosed by our different inclusion criteria: (i) immunoglobulin G (IgG) deposition to basement membrane zone (BMZ) by direct immunofluorescence or IgG reactivity with dermal side of split skin by ssIIF, (ii) positivity for at least one of the three subunits of LM332 by immunoblotting of purified human LM332, and (iii) the presence of mucosal lesions. Clinical, histopathological, and immunological findings were summarized and analyzed statistically. Although these cases included the 55 previous cases, the more detailed study for larger scale of patients was conducted for further characterization.ResultsClinically, among the 133 patients, 89% and 43% patients had oral and ocular mucosal lesions, respectively, 71% had cutaneous lesions, and 17% had associated malignancies. Histopathologically, 93% patients showed subepidermal blisters. The sensitivities of ssIIF and direct immunofluorescence are similar but are significantly higher than indirect immunofluorescence using non-split human skin (both p < 0.001). In immunoblotting of purified LM332, patient IgG antibodies most frequently reacted with LMγ2 subunit (58%), followed by LMα3 (49%) and LMβ3 (36%). Thirty-four percent patients recognized additional non-LM332 autoantigens. Statistical analysis revealed that autoantibodies against non-LM332 autoantigens might stimulate the production of anti-LMγ2 antibodies.ConclusionsThis retrospective study further characterized in more detail the clinical and immunological features of 133 cases of anti-LM332-type MMP, in which the new diagnostic criteria without positive direct immunofluorescence reactivity were useful for the diagnosis. Higher frequency with anti-LMγ2 antibodies suggested more significant pathogenic role of this subunit. Additional autoantibodies to non-LM332 autoantigens detected in one-third of the patients may contribute to complexity in anti-LM332-type MMP, including the induction of anti-LMγ2 antibodies.

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