Linear Deposits Of Immunoglobulin Research Articles

Anti-GBM antibody in a patient with diabetic nephropathy; all that glitters is not gold.

We present the case of a 58-year-old male diabetic patient admitted to our department for a slight decrease in kidney function, with nephrotic range proteinuria, hematuria (16,000/ml) and positive anti-glomerular basement membrane antibodies. Kidney biopsy revealed diabetic nephropathy with no evidence of crescent formation or linear immunoglobulin deposits along the basement membrane. We discuss the various clinical settings involving positive anti-glomerular basement membrane in the absence of crescentic glomerulonephritis.

A case report of atypical anti-glomerular basement membrane disease

BackgroundAnti-glomerular basement membrane (anti-GBM) disease is characterized by crescentic necrotizing glomerulonephritis, with linear deposits of immunoglobulin G (IgG) in the GBM. Classic anti-GBM disease is clinically associated with rapidly progressive glomerulonephritis with or without pulmonary hemorrhage. Some patients have a better renal prognosis and milder symptoms than those with classic anti-GBM disease, which is termed atypical anti-GBM disease.Case presentationA 43-year-old Japanese woman was admitted to our hospital complaining of hematuria that had persisted for more than one month. Serological examination revealed negativity for anti-nuclear, anti-neutrophilic cytoplasmic, and anti-GBM antibodies. However, renal biopsy showed cellular crescents. Immunofluorescence revealed strong diffuse linear capillary loop staining for IgG. An indirect immunofluorescence antibody method was performed by applying the patient serum to normal kidney tissue to confirm the presence of autoantibodies binding to the GBM. Using this method, anti-GBM antibodies were detected. The patient was treated with high-dose steroids, cyclophosphamide, and plasma exchange. Aggressive treatment resolved proteinuria and hematuria and improved renal function.ConclusionsRenal biopsy is crucial in the diagnosis of anti-GBM disease, especially when serological tests are negative. Accurately identifying the presence of anti-GBM disease is important to initiate optimal treatment.

Leukocytoclastic vasculitis in association with linear epidermal basement membrane zone immunoglobulin deposition: Linear vasculitis.

Cutaneous leukocytoclastic vasculitis (LCV) has a distinctive clinical and light microscopic presentation; however, the etiologic basis of LCV is varied. Most cases are attributable to immune complex deposition within a vessel wall and represent an Arthus type III immune complex reaction. The prototypic immunoreactant profile is characterized by granular deposits of components of complement activation in concert with immunoglobulin within the cutaneous vasculature. We encountered nine patients with vasculitic and/or vesiculobullous clinical presentations exhibiting an LCV in association with an immunoreactant profile characterized by homogeneous linear deposits of immunoglobulin along the dermal epidermal junction in a fashion resembling an autoimmune vesiculobullous disease. Among the clinical presentations were palpable purpura, urticarial vasculitis, and vesiculobullous eruptions with supervening purpura. Two patients with Crohn disease presented with classic palpable purpura with biopsy-proven LCV, and direct immunofluorescence (DIF) studies demonstrated linear immunoglobulin G (IgG) with floor localization on the salt-split skin assay. Four patients with systemic lupus erythematosus (SLE) showed purpuric vesiculobullous lesions, with evidence of a neutrophilic interface dermatitis and LCV in three of the four. The remaining patient had urticarial nonbullous lesions showing small-vessel vasculitiswith a neutrophilic interface dermatitis. In all of the patients with SLE, DIF studies showed linear immunoglobulin deposits within the basement membrane zone (BMZ). These constellation of findings clinically, light microscopically, and by immunofluorescence were those of a vasculitic presentation of bullous systemic lupus erythematosus. Two patients had linear IgA disease, which was drug induced in one and paraneoplastic in the other, and the dominant morphology on biopsy in both cases was an LCV. One patient microscopically demonstrated drug-associated and eosinophilic enriched LCV with DIF studies showing striking linear deposits of IgG suggestive of bullous pemphigoid, which was consistent with a vasculitic presentation of drug-induced bullous pemphigoid. In all cases, typical granular vascular immunoglobulin and complement deposition compatible with immune complex mediated vasculitis was observed. It is likely that local immune complexes derived from BMZ antigen bound to antibody are pathogenically relevant. We propose the designation of linear vasculitis for this unique scenario of LCV and linear immunoglobulin epidermal BMZ staining, which in some cases represents a vasculitic presentation of conventional autoimmune vesiculobullous disease.

Bullous pemphigoid in a lichen sclerosus lesion

A 72-year-old woman obtained consultation for a 2-week history of anogenital erosions and bullae resulting in pain and a burning sensation. She had a history of lichen sclerosus. Physical examination revealed a white patch surrounding the anogenital area with erosions and ruptured bullae (Figure 1). Histologic examination depicted a subepidermal mononuclear inflammatory infiltrate and subepidermal blistering (Figure 2). Direct immunofluorescence microscopy of a skin biopsy showed linear deposits of immunoglobulin G and C3 along the dermoepidermal junction (Figure 3).

Bullous systemic lupus erythematosus with nephrotic syndrome in an adolescent girl: a case report

Bullous systemic lupus erythematosus is an uncommon autoimmune subepidermal vesiculobullous disease occurring in less than 5% of patients with systemic lupus erythematosus. The diagnosis of bullous systemic lupus erythematosus is based on clinical picture, histopathological findings, direct immunofluorescence and other immunologic tests. Importantly, establishing the diagnosis of bullous systemic lupus erythematosus requires the occurrence of systemic lupus erythematosus according to the American College of Rheumatology criteria. We report a case of bullous systemic lupus erythematosus in a 16-year-old Vietnamese girl, who demonstrated an acquired vesiculobullous eruption associated with nephrotic syndrome and fulfilled the European League Against Rheumatism/ American College of Rheumatology 2019 classification criteria of systemic lupus erythematosus. Clinical examination showed numerous tense, clear fluid-filled vesicles and bullae affecting the whole body with the predilection for the face, neck, chest, abdomen and bilateral inner thigh areas. Erosions were observed on the tongue and the buccal mucosa. Histopathologic examination showed subepidermal vesicle containing abundant neutrophils and perivascular mixed inflammatory cell infiltration. Microabscesses at tips of dermal papillae and the features of a leukocytoclastic vasculitis were not seen. Direct immunofluorescence of perilesional skin biopsies demonstrated linear deposition of IgG, IgA, IgM and C3 in the basement membrane zone. Linear immunoglobulin deposition displayed a u-serrated pattern with the predominant staining intensity of IgG. Serologic tests showed positive antinuclear antibody. Complement components assay revealed low levels of C3 (66.5 mg/dL) and C4 (3,9mg/dL). Proteinuria level was increased to 3.5g/24 hours. The final diagnosis of bullous systemic lupus erythematosus with nephrotic syndrome was made. The patient was treated with a high-dose oral glucocorticoid scheme and showed significant clinical improvement.

Mixed immunobullous disease in infants: Falls in bullous pemphigoid-linear IgA spectrum?

Bullous pemphigoid (BP) and linear immunoglobulin A bullous dermatosis (LABD) are common immunobullous diseases affecting both adults and children, with minor differences in clinical presentation. The only test for a definitive diagnosis is direct immunofluorescence (DIF) study, with linear deposits of immunoglobulin G (IgG) (mainly) at the dermoepidermal junction (DEJ) being diagnosed as BP and linear deposits of IgA at DEJ, diagnosed as LABD. Mixed immunobullous disease is the term given for immunobullous diseases with equal IgA/IgG deposits at the DEJ. Through this case report, a mixed immunobullous disease with clinical diagnosis of LABD and DIF findings of BP is being discussed. Furthermore, the presence of a BP-LABD spectrum of disease that can progress in either direction and present with features of both diseases is discussed.

Clinicopathological characteristics of typical and atypical anti-glomerular basement membrane nephritis.

Anti-glomerular basement membrane (GBM) antibody disease is a rare pathological condition that mainly involves renal and/or pulmonary parenchyma. It is characterized by the presence of circulating anti-GBM antibodies accompanied by a linear deposition of immunoglobulins (Ig) detected through immunofluorescence (IF) technique and typical signs and symptoms of organ dysfunction, such as rapidly progressive glomerulonephritis (RPGN) and pulmonary hemorrhage (PH). However, recently atypical forms of anti-GBM disease have been described and the presence of overlapping diseases contributed to make its diagnosis challenging. In this review will be discussed the entire spectrum of renal anti-GBM related conditions, focusing the attention on the differences in terms of pathogenesis, diagnosis and therapy of these disparate entities.

A clinical and serological study of linear IgA bullous dermatosis without linear immunoglobulin deposition other than IgA at the basement membrane zone using direct immunofluorescence.

Linear IgA bullous dermatosis (LABD) is a heterogeneous disease. Different diagnostic criteria have been used in different reports. To reappraise the characteristic features of LABD with only IgA deposition at the basement membrane zone (BMZ) with direct immunofluorescence (DIF). We retrospectively collected data from 101 patients from our archival records from 1 January 1996 to 31 December 2014 who had: (i) blisters on the skin and/or mucous membranes; (ii) subepidermal blisters in a biopsy specimen; and (iii) linear IgA deposition along the BMZ with/without linear C3 deposition at the BMZ with DIF. Most patients were referred for serological evaluation. Patients who showed concurrent linear IgG and/or IgM deposition at the BMZ under DIF were excluded. Clinical manifestations and serological findings were analysed. Heterogeneity of autoantigens in LABD was confirmed. Fifty-four of 101 patients (53%) had IgG antibodies detected either by indirect immunofluorescence, immunoblotting or enzyme-linked immunosorbent assays (ELISA). No statistical difference in clinical manifestations was found between patients in the IgG antibody-possessing group and patients in the other group. An association of IgG anti-BMZ antibodies with LABD may increase if new IgG immunoblotting or ELISA techniques become available. Consensus for diagnostic criteria for LABD is desired for prospective data storage, although it may be arbitrary.

Glomerular disease with idiopathic linear immunoglobulin deposition: a rose by any other name would be atypical

Nasr etal. (2016) report 20 cases of "atypical anti-GBM disease," characterized by bright, linear glomerular basement membrane (GBM) immunoglobulin deposition in patients who lacked anti-GBM antibodies by conventional testing and who had a relatively benign course. Half had light chain restriction. The term "idiopathic linear immunoglobulin deposition" may be preferable until anti-GBM activity is demonstrable, since there are alternative mechanisms of linear deposition in the GBM, including physicochemical affinity for GBM components and alterations of the GBM itself.

Stevens-Johnson Syndrome-Like Skin Lesions in a Patient with Juvenile Systemic Lupus Erythematosus.

Dear Editor: Skin manifestations in systemic lupus erythematosus (SLE) are very heterogeneous. Recently, Stevens-Johnson syndrome (SJS)/toxic epidermal necrolysis (TEN)-like lupus erythematosus (LE) has been reported. Clinically, this condition presents with photodistributed exanthema and minimal mucositis. The onset of the rash is insidious with a prolonged clinical course1. A 16-year-old Korean woman presented with a 4-week history of gradually progressive development of painful, annular, erythematous, flattened, and blistering eruptions. Crusting presented on the face, V-neck area, upper back, and dorsum of the hands, and involved less than 10% of body surface area. The lips had erosions and crusts (Fig. 1). Eye and genital examinations were unremarkable. Previous medical history included malar rash and diffuse alopecia. No relevant medications had been newly administered. Fig. 1 Photodistributed erythematous flattened vesiculobullous eruptions with crusts on the face, V-neck area and the dorsum of hands and erosion on the lips. Skin biopsy from the back revealed a subepidermal blister with exocytosis and superficial perivascular infiltration of lymphocytes and neutrophils. Marked necrosis of keratinocytes of the suprabasal layer and interface dermatitis were manifested (Fig. 2). Direct immunofluorescence microscopy was remarkable in that linear deposits of immunoglobulin G, C3, and C1q were shown at the basement membrane zone. Fig. 2 Subepidermal blister with exocytosis and superficial perivascular infiltration of lymphocytes and neutrophils, marked necrosis of keratinocytes of the suprabasal layer and interface change (H&E, ×100). Laboratory investigations demonstrated anemia (hemoglobin 10.9 g/dl), leukopenia (3,600/mm3), and a normal platelet count. The antinuclear antibody titer was positive (1:640 homogeneous pattern), anti-double-stranded DNA was positive, anti-Ro/SS-A was positive and anti-La/SS-B was negative. Anti-Sm was positive, anti-nucleosome, anti-histones, and rheumatoid factor were negative. C3 and C4 levels were markedly low at 47.2 g/dl and 13.6 g/dl, respectively. Plasma creatinine was 0.65 mg/dl and the 24-hour urine proteinuria was 1,589 mg/day. Lupus nephritis class IIA was diagnosed from kidney biopsy. Liver function tests were unremarkable. Eventually, SJS-like cutaneous presentation with juvenile SLE was diagnosed based on the above findings. Treatment regimen included oral hydroxychloroquine 200 mg, deflazacort 48 mg per day with gradual tapering. Lesions improved after dressing on the denuded area and applying topical corticosteroid. Skin is the second most commonly affected organ system in SLE2. A bullous eruption in the setting of SLE can be a diagnostic challenge. Classic SJS/TEN, SJS/TEN-like LE, drug-induced cutaneous LE, bullous LE, Rowell's syndrome, and immunobullous diseases need to be considered in the differential diagnosis3. Indirect immunofluorescence is an effective method to diagnose bullous LE, but the present patient did not undergo this test. However, bullous LE appears with various-sized tense blisters, which is clinically different from the patient's presentation. Usually, SJS/TEN-like LE develops as a result of severe inflammatory epidermal basal layer damage. Vacuolar degeneration leads to subepidermal blistering and almost full-thickness epidermal necrosis1,4. SJS/TEN-like LE is often triggered by excessive ultraviolet exposure with an underlying predisposition to SLE. These patients often have anti-Ro antibody. Anti-Ro may be associated with increased risk of photosensitive rash4. The standard treatment of SJS/TEN-like LE remains controversial. Corticosteroids were prescribed in the present case because of continuing lupus activity. Chloroquine was combined for immunomodulation. In previous reports of TEN-like acute cutaneous LE, intravenous immunoglobulin, and plasmapheresis were successful5. To our knowledge, SJS-like skin lesions with juvenile SLE have rarely been reported in the literature. This was the first case reported in Korea.

Etiology and Outcome of Crescentic Glomerulonephritis From a Single Center in China: A 10-Year Review

The disease spectrum of crescentic glomerulonephritis (GN) has been described in only a few previous studies, and detailed epidemiologic data from China are unavailable to date. Case series. 528 patients with biopsy-proven crescentic GN in 2003 to 2013 from a single center. Crescentic GN was classified into 3 types according to immunofluorescence findings: type I was defined as linear deposition of immunoglobulins along the glomerular basement membrane; type II, as glomerular deposition of immune complex; and type III, as pauci-immune deposition. Demographic, clinical, and serologic characteristics. Of 528 cases identified, 208 (39.4%) were men, with a mean age of 37.6±16.4 (SD) years at kidney biopsy. 61 (11.6%) patients had type I crescentic GN, 331 (62.7%) had type II (lupus nephritis, 34.3%; immunoglobulin A [IgA] nephropathy, 17.4%), and 136 (25.8%) had type III. Proportions of patients with acute kidney injury (AKI), acute kidney diseases and disorders without AKI, and chronic kidney disease were 86.9%, 0%, and 13.1% for type I; 42.0%, 19.6%, and 38.4% for type II; and 84.6%, 2.9%, and 12.5% for type III crescentic GN, respectively. Serum antineutrophil cytoplasmic antibodies were detected in 11 (18.0%) patients with type I, 15 (4.5%) with type II, and 117 (86.0%) with type III. Anti-glomerular basement membrane antibodies were found in 60 (98.4%) patients with type I, 3 (0.9%) with type II, and 5 (3.7%) with type III. 5-year cumulative renal survival rates for patients with types I, II, and III were 17.6%, 70.1%, and 44.3%, respectively. Retrospective study, single-center experience. Lupus nephritis may be the most common type of crescentic GN in China, followed by pauci-immune crescentic GN and IgA nephropathy. Almost half the patients presented with AKI, whereas 28.8% of cases showed chronic kidney disease. Clinical manifestations and outcomes varied according to crescentic GN type. The distinction between subtypes based on immunofluorescence and serologic findings has important implications for therapy and outcome.

Oral lichen planus pemphigoides: a series of four cases

Lichen planus pemphigoides (LPP), which is a rare autoimmune blistering mucocutaneous disease of the pemphigoid family of diseases, is characterized by the development of vesiculobullous lesions on or adjacent to the areas of lichen planus (LP). LPP primarily affects the skin, and oral involvement alone is rare. The objective of this case series was to report four new cases of oral LPP. We present four cases with clinical, histologic, and direct immunofluorescence (DIF) features characteristic of LPP, with three cases having oral involvement only. The four patients (including two males) were aged 49, 50, 51, and 61 years; only one patient had skin lesions. All patients had typical reticular, erythematous, or ulcerative oral LP involving the gingiva and the buccal mucosa. Mucosal biopsies showed features consistent with LP, mucous membrane pemphigoid (MMP), or a combination of both, and DIF studies in all 4 cases showed linear deposition of immunoglobulin G (IgG) and C3 at the interface. Correlation of clinical findings, routine histopathology, and DIF studies is essential for the diagnosis.

In vivo analysis of IgE autoantibodies in bullous pemphigoid: A study of 100 cases

BackgroundBullous pemphigoid (BP) is an acquired autoimmune blistering disease characterized by subepidermal blister formation, in vivo linear deposition of immunoglobulin G (IgG) and complements at the dermal–epidermal junction (DEJ). The circulating IgG autoantibodies are directed against two epidermal hemidesmosomal glycoproteins: BP180, also known as type XVII collagen (COL17), and BP230. In addition, recent studies have shown that IgE autoantibodies may be involved in the pathogenesis of BP, although in vivo IgE deposition in lesional skin has not been fully characterized in large numbers of BP patients. ObjectiveThis study investigated the incidence of in vivo deposition of IgE autoantibodies at the DEJ in lesional skin from a large number of BP patients. MethodsPeri-lesional skin samples from 100 patients who met the clinical and histopathological criteria for BP were investigated by direct immunofluorescence for the deposition of autoantibodies and complement. Patients’ sera were also investigated by enzyme-linked immunosorbent assay and indirect immunofluorescence. Results18% of BP patients were found to show IgE deposition at the DEJ. Disease severity, clinical course and outcome did not differ between IgE-positive and IgE-negative patients. In 3 IgE-positive cases, IgG was undetectable in vivo, and these cases showed atypical manifestations. ConclusionThe results of in vivo IgE deposition may not be useful in predicting the disease course of BP, although predominant IgE deposition could alter the pattern of clinical manifestations.

Hidden Tiger: An Atypical Presentation of Anti-Glomerular Basement Membrane Disease

Anti-glomerular basement membrane (anti-GBM) disease is characterized by linear deposition of immunoglobulin G (IgG) along the GBM. The concurrence of anti-GBM disease with another type of glomerulonephritis has been well described. We report a case of necrotizing glomerulonephritis in association with prodromal systemic symptoms, deterioration in kidney function less rapid than expected, and the presence of circulating anti-GBM antibody. This was initially diagnosed histologically as immunoglobulin A (IgA) nephropathy based on immunohistochemistry (IHC) staining, but immunofluorescence (IF) staining on a subsequent biopsy was more consistent with a diagnosis of anti-GBM disease with co-existing mild IgA nephropathy. Prompt initiation of standard treatment of anti-GBM disease normalized kidney function. This case highlights that anti-GBM disease can present atypically and may also co-exist with another form of glomerulonephritis. World J Nephrol Urol. 2014;3(3):124-128 doi: http://dx.doi.org/10.14740/wjnu173w

Retrospective Study of the Clinical, Histologic, and Immunologic Features of Epidermolysis Bullosa Acquisita in 9 Patients

BackgroundEpidermolysis bullosa acquisita (EBA) is an autoimmune subepidermal blistering disease caused by autoantibodies to type VII collagen. The clinical presentation is variable, with skin and mucosal lesions that can cause significant dysfunction. Different treatment options exist, but the results are often unsatisfactory. ObjectiveTo review all the cases of epidermolysis bullosa acquisita (EBA) diagnosed at our hospital over a 26-year period. Materials and methodsWe performed a retrospective review of the clinical, histologic, and immunologic features of EBA in 9 patients. ResultsMean age at presentation was 37 years and 66.67% of the patients were women. EBA occurred in association with malignant tumors, inflammatory bowel disease, and autoimmune disorders. The most common variant was inflammatory EBA (6 of the 9 cases). In all 9 patients, histology revealed a subepidermal blister and direct immunofluorescence showed linear deposits of immunoglobulin G and C3 in the basement membrane zone. Indirect immunofluorescence performed on salt-split skin substrate was positive in 6 patients and showed a dermal pattern in all cases. Five patients were tested for autoantibodies to type VII collagen using enzyme-linked immunosorbent assay, with positive results in 2 cases. Immunoblotting using recombinant noncollagenous domains (NC1) of type VII collagen was positive in all 6 cases in which it was performed. Response to treatment was variable. ConclusionsEBA is a rare disease with a variable clinical presentation that can be confused with that of other subepidermal blistering diseases. Correct diagnosis requires a high level of clinical suspicion and the use of all available diagnostic tests. Thorough evaluation of cutaneous and mucosal involvement and prompt initiation of appropriate treatment will ensure the detection and prevention of dysfunction and treatment-related complications.

Estudio retrospectivo de las características clínicas, histológicas e inmunológicas en una serie de 9 pacientes con epidermólisis ampollosa adquirida

IntroductionEpidermolysis bullosa acquisita (EBA) is an autoimmune subepidermal blistering disease caused by autoantibodies to type VII collagen. The clinical presentation is variable, with skin and mucosal lesions that can cause significant dysfunction. Different treatment options exist, but the results are often unsatisfactory. ObjectiveTo review all the cases of epidermolysis bullosa acquisita (EBA) diagnosed at our hospital over a 26-year period. Materials and methodsWe performed a retrospective review of the clinical, histologic, and immunologic features of EBA in 9 patients. ResultsMean age at presentation was 37 years and 66.67% of the patients were women. EBA occurred in association with malignant tumors, inflammatory bowel disease, and autoimmune disorders. The most common variant was inflammatory EBA (6 of the 9 cases). In all 9 patients, histology revealed a subepidermal blister and direct immunofluorescence showed linear deposits of immunoglobulin G and C3 in the basement membrane zone. Indirect immunofluorescence performed on salt-split skin substrate was positive in 6 patients and showed a dermal pattern in all cases. Five patients were tested for autoantibodies to type VII collagen using enzyme-linked immunosorbent assay, with positive results in 2 cases. Immunoblotting using recombinant noncollagenous domains (NC1) of type VII collagen was positive in all 6 cases in which it was performed. Response to treatment was variable. ConclusionsEBA is a rare disease with a variable clinical presentation that can be confused with that of other subepidermal blistering diseases. Correct diagnosis requires a high level of clinical suspicion and the use of all available diagnostic tests. Thorough evaluation of cutaneous and mucosal involvement and prompt initiation of appropriate treatment will ensure the detection and prevention of dysfunction and treatment-related complications.

Anti-p200 pemphigoid responding to dapsone

Abstract Anti-p200 pemphigoid is a rare autoimmune subepidermal blistering disease. Clinical presentation is similar to standard bullous pemphigoid (BP) but mucous membranes and cephalic lesions are more frequent. Histology and direct immunofluorescence (IF) are identical to BP but indirect IF discloses linear deposits of immunoglobulin G (IgG) on the dermal side of artificial salt-split skin. Specific diagnosis is based on western immunoblotting that shows circulating IgG recognizing a 200-kDa protein localized on the dermal extract. The 200-kDa antigen was recently identified as laminin γ1. Anti-p200 pemphigoid should be considered before all atypical or topical steroid-resistant bullous disease, as well as mucous membranes pemphigoid or epidermolysis bullosa acquisita. Dapsone appears to be the most effective treatment and should be used as the first option in combination with topical steroids. In this report, we describe the case of a patient with a typical clinical and immunopathological anti-p200 pemphigoid, responding to a combination of topical steroids and dapsone.

A necessidade da imunofluorescência direta no diagnóstico da dermatose bolhosa por IgA

A dermatose bolhosa por imunoglobulina da classe A linear (DbIgA) do adulto é uma doença autoimune rara caracterizada por formação de bolhas subepidérmicas e depósito linear de imunoglobulina da classe A (IgA) na zona da membrana basal (ZMB). Por possuir aspectos clínicos e histológicos semelhantes a outras dermatoses bolhosas, principalmente a dermatite herpetiforme e o penfigoide bolhoso, faz-se necessária a realização de imunofluorescência direta para confirmação diagnóstica. Apresenta-se então, neste artigo, relato de caso ilustrando essa necessidade.

Ocular and oral mucous membrane pemphigoid (cicatricial pemphigoid)

Mucous membrane pemphigoid, a heterogeneous group of autoimmune blistering diseases, affects primarily the mucous membranes. Although oral and ocular mucosae can both be affected in a given patient, patients with involvement restricted to oral mucosae tend to have a benign outcome, whereas those with ocular disease commonly face treatment resistance, resulting in scarring and blindness. Diagnosis requires direct immunofluorescence microscopy to demonstrate a linear deposition of immunoglobulin (Ig) G or IgA, or complement component 3 (C3), at the epithelial basement membrane. Although the target antigens vary, subsets of patients affected exclusively by oral and ocular mucosal diseases have autoantibodies targeting α-6 and β-4 integrins, respectively.

Heterogeneous disease: A child case of lichen planus pemphigoides triggered by varicella

Lichen planus pemphigoides (LPP) is a rare and controversial disease. It is characterized clinically by tense bullae arising both on lichen planus papules and on uninvolved skin, histologically by the demonstration of subepidermal bullae and by linear deposits of immunoglobulin G and C3 along the basement membrane zone on immunofluorescence of peribullous skin. Some authors consider LPP as the combination of lichen planus and bullous pemphigoid. Others think that it most likely encompasses a heterogeneous group of subepidermal autoimmune blistering disorders occurring in association with lichen planus. We present a child case that supports the heterogeneous condition of this disease triggered by varicella.