Positive Direct Immunofluorescence Research Articles

Atypical Presentation of Adult-Onset Hydroa Vacciniforme with Direct Immunofluorescence Positivity—A Case Report

Atypical Presentation of Adult-Onset Hydroa Vacciniforme with Direct Immunofluorescence Positivity—A Case Report

Clinical Characteristics of Ocular Mucous Membrane Pemphigoid: A Systematic Review and Meta-Analysis

ABSTRACT Purpose To synthesize the evidence and generate a combined weighted measure on the frequency of ocular manifestations of mucous membrane pemphigoid (OMMP). Methods Systematic literature review and meta-analysis, searching PubMed, Embase, VHL, and Google Scholar. Articles reporting patients with mucous membrane pemphigoid and ocular involvement were included. At least, two reviewers independently and in parallel participated in all the following phases; preliminary screening, full-text review, risk of bias assessment by validated tools, and data extraction. Qualitative analysis and meta-analysis were conducted. This study was previously registered in PROSPERO (CRD42023451844). Results Thirty-five studies met the inclusion criteria, comprising 1,439 patients and 1,040 eyes summarized in qualitative analysis. Twenty-eight studies were included in the meta-analysis. Ages included ranged from 60.4 to 75 years. Women were reported with more frequency. The mean time for diagnosis was 55.1 months, usually with bilateral ocular disease in 90% (95% CI 78%; 96%). Trichiasis and entropion were the most frequent manifestations in up to 92%, followed by symblepharon and punctate keratitis. Ankyloblepharon, persistent epithelial defects, and visual impairment were less frequent complications. Direct immunofluorescence positivity in conjunctival biopsies was 54% (95% CI 43%; 64%). Extraocular involvement was highly frequent, being oral and skin involvement the most frequently reported. Conclusions Our systematic review and meta-analysis evidenced that patients around 60 years of age are the most affected population with a female preponderance, usually with bilateral ocular involvement. Trichiasis and entropion were the most frequent findings; although visual impairment and persistent epithelial defects were less reported, they should not be overlooked in suspected OMMP.

Utility of C4d Immunohistochemistry in the Diagnosis of Esophageal Pemphigus Vulgaris.

Aims. To assess the utility of C4d immunohistochemistry for esophageal pemphigus vulgaris. Methods and results. We searched for patients with a history of esophageal pemphigus vulgaris who had esophageal biopsies for routine hematoxylin and eosin (H&E) staining. A total of 8 biopsies from 7 patients were available. We also identified 18 non-pemphigus esophageal biopsies for controls. C4d immunohistochemistry was performed on each biopsy. Five of 6 (83%) biopsies with classic pemphigus vulgaris histologic findings were positive for intercellular staining at the basal layer. The negative biopsy was in a patient that had recently received high-dose corticosteroid treatment for a flare. Two biopsies with atypical histologic features for pemphigus vulgaris had negative C4d staining but positive direct immunofluorescence (DIF) studies. Various nonspecific C4d staining patterns were observed in the controls, but none showed the intercellular staining pattern that was observed in pemphigus vulgaris. Conclusions. Suprabasal clefting with acantholysis and "tombstone effect" are described histologic features of pemphigus vulgaris on H&E. However, procedural artifact may mimic these findings. Currently, the gold standard for pemphigus vulgaris is DIF, which is not always available because it cannot routinely be performed on formalin-fixed paraffin embedded tissue. Our study shows that C4d immunohistochemistry may be a useful adjunct in evaluating esophageal pemphigus vulgaris.

High serum total IgE levels correlate with urticarial lesions and IgE deposition in perilesional skin of bullous pemphigoid patients: An observational study.

Background In the pathophysiology of bullous pemphigoid, besides IgG, there has been evidence that supports the role of IgE antibodies. However, there have been no studies to evaluate total serum IgE levels or detect IgE deposits in the skin of Vietnamese patients. Aim To analyse the association between IgE levels in the serum and disease severity as well as eosinophils and IgE basement membrane zone (BMZ) deposition in Vietnamese bullous bullous pemphigoid patients. Methods A single-centre observational research on 35 newly diagnosed and untreated bullous bullous pemphigoid patients. Total serum IgE levels were analysed using enzyme-linked immunosorbent assay (ELISA). For controls, we collected sera of 30 pemphigus patients and 30 elderly patients with pruritus. Perilesional skin biopsies underwent direct immunofluorescence (DIF) staining, with biopsies of pemphigus patients as controls. Results Elevated total serum IgE was observed in 60% of bullous pemphigoid patients, the percentage in the pemphigus group and pruritus group was 20% and 40%, respectively. The mean total serum IgE level among the bullous pemphigoid group was higher than that of the pemphigus group (123.3 ± 102.4 IU/mL vs. 64.3 ± 45.1 IU/mL, p = 0.010). Total serum IgE levels of bullous pemphigoid patients correlated with higher eosinophil counts (r = 0.61; p = 0.018) and urticaria/erythema (U/E) Bullous Pemphigoid Disease Area Index (BPDAI) score (r = 0.50; p = 0.035). Among 35 bullous pemphigoid patients, 5 patients showed positive IgE DIF staining, accounting for 14.3%. Higher serum IgE levels correlated with the deposition of IgE in patients' perilesional skin (p = 0.037). Limitations Due to the rarity of bullous pemphigoid, the effect of the COVID-19 pandemic, and self-treatment issues in Vietnam, we could not recruit a larger number of participants. Conclusions Total serum IgE values correlated with urticarial lesions and IgE deposition in perilesional skin of Vietnamese bullous pemphigoid patients. IgE autoantibodies present in the skin of bullous pemphigoid patients support the role of IgE in bullous pemphigoid pathogenesis.

Ocular Cicatricial Pemphigoid in Young Patients.

The purpose of this study is to report ocular cicatricial pemphigoid (OCP) occurring in young patients. Relevant literature is also reviewed. Medical records of patients aged 30 years or younger who were treated for OCP between August 2021 and May 2023 at a tertiary care eye institute were reviewed. The most common differential diagnoses of cicatrizing conjunctivitis, such as Stevens-Johnson syndrome sequelae, chemical injury, chronic topical/systemic drug use, autoimmune connective tissue disorders, or allergic eye diseases, were ruled out based on clinical history, examination, and the Cicatrizing Conjunctivitis Score described by Shanbhag et al. The diagnosis of OCP was confirmed by positive direct immunofluorescence of oral mucosal and/or conjunctival biopsy in a majority of the patients. Seven patients fulfilled the criteria for a diagnosis of OCP. The mean age at presentation was 21.86 ± 5.25 years (13-28 years). Some of the patients presented with relatively atypical features for OCP such as corneal immune ring infiltrate and bilateral corneal perforation. Six patients exhibited systemic mucosal lesions, and the direct immunofluorescence yield was 85.71%. All patients required aggressive immunosuppressive treatment. OCP is classically described as a rare disease that occurs in old age. This case series highlights the importance of a higher index of suspicion for diagnosing OCP at a younger age. Early administration of immunosuppressive agents can potentially control severe ocular surface inflammation and its sequelae.

Diagnostic and prognostic values of conjunctival and oral biopsies analyzed by direct immunofluorescence in patients with mucous membrane pemphigoid.

Mucous membrane pemphigoid (MMP) is diagnosed on the basis of a characteristic clinical picture (a predilection for mucosal involvement and scarring in the affected tissues) and a positive direct immunofluorescence (DIF) result. In this study, we compare the diagnostic and prognostic values of conjunctival and oral biopsies analyzed by DIF in patients with MMP. Sixteen patients with MMP and mucosal involvement as a predominant symptom were classified into three groups based on the clinical picture. Oral and conjunctival DIF were performed on all patients. Our study showed that patients with simultaneous oral and conjunctival involvement had a positive oral DIF in 83% and a positive ocular DIF in 100% of the examined cases, respectively. Patients with isolated ocular MMP had a positive oral DIF in 50% and a positive ocular DIF in 66% of the examined cases, respectively. Patients with only oral involvement with MMP had a positive oral DIF in 100% and a positive ocular DIF in 50% of the examined cases, respectively. Oral biopsy should be performed first and is usually sufficient for the diagnosis, even in patients with exclusively ocular MMP, whereas in patients without clinical ocular involvement, ocular DIF is positive in half of the cases and may be a predictive factor for ocular lesions in the future.

Direct Immunofluorescence Findings and Factors Affecting Conjunctival Biopsy Positivity in Ocular Mucous Membrane Pemphigoid.

The aim of this study was to describe the direct immunofluorescence (DIF) findings and factors affecting conjunctival biopsy positivity in patients clinically diagnosed with ocular mucous membrane pemphigoid (OMMP). This retrospective observational case series included patients with clinical OMMP who underwent conjunctival biopsy for DIF in at least 1 eye between 2018 and 2021 in an institutional setting. The primary outcome measures were association of age and chronic ocular complications with biopsy positivity. Of 61 patients, DIF positivity was seen in 33 (54.1%) clinically suspected cases of OMMP. Of 39 patients who underwent bilateral biopsy, 23 (59%) were positive, of which 12 (52%) were positive in both eyes while 11 (48%) were positive in 1 eye. Of 22 patients who underwent unilateral biopsy, 10 (45%) were positive. Of the 100 biopsied eyes, 45 (45%) were DIF positive. Among the immunoreactants studied, linear deposition of C3 was seen in all 45 positive eyes (100%). Increasing age was significantly associated with higher likelihood of biopsy negativity ( P = 0.032), whereas a greater Sotozono chronic ocular complication score, indicative of disease severity, was associated with low likelihood of biopsy positivity ( P = 0.0042) and lower overall expression of immunoreactants on DIF ( P = 0.0007). Older patients and patients with more severe ocular surface disease sequelae are likely to have negative DIF results. To optimize the chances of confirming the diagnosis of OMMP by DIF, both eyes should be biopsied early in the disease course. If 1 eye is being biopsied, the less affected eye must be chosen.

Evaluation of the Diagnostic Value of Oesophageal Biopsies for Direct Immunofluorescence Microscopy in Mucous Membrane Pemphigoid.

Mucous membrane pemphigoid is an autoimmune blistering disorder characterized by predominant involvement of surface-close epithelia and linear depositions of immunoreactants at the dermal-epithelial junction on direct immunofluorescence microscopy. A major diagnostic difficulty is the frequent need for multiple biopsies to facilitate the diagnosis. Although oesophageal involvement is a rare, but life-threatening manifestation, the relevance of oesophageal direct immunofluorescence sampling is unclear. This retrospective monocentric study evaluated 67 non-lesional biopsies from 11 patients with mucous membrane pemphigoid and clinical symptoms suggestive of oesophageal involvement, comprising 31 samples from the oesophagus and 36 samples from other anatomical sites. Five patients (45.5%) exhibited endoscopic findings compatible with oesophageal involvement of mucous membrane pemphigoid. No correlation was identified between the presence of oesophageal lesions and direct immunofluorescence positivity in lesions from the oesophagus (p = 1.0). Oral and cutaneous samples were significantly more frequently positive by direct immunofluorescence than were oesophageal biopsies (p < 0.0001 and p = 0.0195, respectively). Oesophageal samples yielded significantly less IgG reactivity than oral and cutaneous lesions (p < 0.0001 and p = 0.0126, respectively), and less IgA antibody response than oral lesions (p = 0.0036). In conclusion, oesophageal direct immunofluorescence samples were inferior to oral and cutaneous biopsies for the diagnosis of mucous membrane pemphigoid even when oesophageal lesions compatible with mucous membrane pemphigoid were present at the time of biopsy.

A retrospective study on the direct immunofluorescence findings in pigmented purpuric dermatosis.

Pigmented purpuric dermatosis (PPD) is characterized by grouped petechiae, purpuric macules, and pigmentation in the bilateral lower extremities. It runs a chronic and relapsing course. Pathophysiology is poorly understood, but it has been proposed to be an immune-complex disease or capillaritis. This study aimed to determine the incidence and patterns of positive direct immunofluorescence (DIF) findings in patients with clinically and histopathologically confirmed PPD. The association between DIF deposition type and clinical profile was also analyzed. Patients with a clinical and histopathologic PPD diagnosis who had undergone DIF studies at a tertiary medical center with attached dermatopathology and immunofluorescence diagnostic centers between January 2002 and December 2021 were included in this study. Data on age, sex, disease duration, comorbidities, and drug intake were collected from medical records. There were 65 patients who satisfied the inclusion criteria. Among them, 58 (89%) had at least one positive finding and 53 (82%) were vascular deposition of immunoglobulin (Ig), complement, or fibrinogen. The most common vascular deposition was fibrinogen (71%) followed by C3 (62%), IgM (18%), IgA (6%), and IgG (3%). Fibrinogen deposition was associated with hypertension (p < 0.03). There was no association between vascular DIF deposition of IgG, IgA, and C3, with age, sex, comorbidities, disease duration, and drug history. The most common DIF findings in PPD were vascular deposition of fibrinogen and C3, with or without Ig presence. DIF findings supported a vascular origin in PPD but not an immune complex-mediated disease. Hypertension was associated with fibrinogen deposition and may play a role in its pathophysiology.

PA04 (P05) A rare presentation of blistering in an adolescent

Abstract A previously well 16-year-old male presented with florid and widespread tense bullae and erosions. He gave a 2-week history of an erythematous rash consisting of crusted erosions that started on his chest and face. He denied any new medications or over-the-counter preparations. There was no preceding systemic illness, and no history of cold sores or sexual contacts. In the 24 h prior to his admission, his rash had become widespread, involving his trunk, limbs, hands, ears, face, lips, oral and genital mucosa. He had started to feel systemically unwell and was in significant pain. On examination, he had a combination of heavily crusted erosions and extensive tense bullae containing straw-coloured fluid on a background of inflamed skin. The morphology was herpetiform in some areas. There was notable extension of bullae onto the palms and penis, as well as intraoral and labial erosions. Viral skin swabs were negative, and his biopsy subsequently demonstrated subepidermal blistering with neutrophil-rich inflammation and positive direct immunofluorescence (IMF) for C3 and IgG only. Serum autoantibodies were negative. Childhood bullous pemphigoid (BP) was initially the working diagnosis, but indirect IMF showed localization to the base of the blister and anticollagen VII antibodies later proved positive, consistent with a diagnosis of epidermolysis bullosa acquisita (EBA). Given the clinical features, it was classified as BP-like EBA. He was initially treated with high-dose oral prednisolone, superpotent topical steroids and analgesia as an inpatient. Mycophenolate mofetil (MMF) was initiated, and his corticosteroid dose was slowly tapered. His condition was completely controlled with MMF 500 mg twice daily at 4 months. The patient then elected to stop taking MMF and remains in complete remission at 7 months. The EBA is rare in adults and even more so in children. The mean age of onset is 44–54 years, but up to 5% of reported cases are in children/adolescents. Four subtypes are described: classic mechanobullous, BP-like, mucous membrane pemphigoid-like and linear IgA bullous dermatosis-like. The BP-like EBA is characterized by widespread vesicles and tense bullae, commonly on an inflammatory background and lacking skin fragility, scarring or milia. Oral and genital mucosae are frequently affected in paediatric cases of EBA. Various steroid-sparing treatment modalities are described, including dapsone, colchicine and MMF. The prognosis in the paediatric population is favourable compared with that of adults: the majority achieve complete remission or complete control on treatment.

P05 A rare presentation of blistering in an adolescent

Abstract A previously well 16-year-old male presented with florid and widespread tense bullae and erosions. He gave a 2-week history of an erythematous rash consisting of crusted erosions that started on his chest and face. He denied any new medications or over-the-counter preparations. There was no preceding systemic illness, and no history of cold sores or sexual contacts. In the 24 h prior to his admission, his rash had become widespread, involving his trunk, limbs, hands, ears, face, lips, oral and genital mucosa. He had started to feel systemically unwell and was in significant pain. On examination, he had a combination of heavily crusted erosions and extensive tense bullae containing straw-coloured fluid on a background of inflamed skin. The morphology was herpetiform in some areas. There was notable extension of bullae onto the palms and penis, as well as intraoral and labial erosions. Viral skin swabs were negative, and his biopsy subsequently demonstrated subepidermal blistering with neutrophil-rich inflammation and positive direct immunofluorescence (IMF) for C3 and IgG only. Serum autoantibodies were negative. Childhood bullous pemphigoid (BP) was initially the working diagnosis, but indirect IMF showed localization to the base of the blister and anticollagen VII antibodies later proved positive, consistent with a diagnosis of epidermolysis bullosa acquisita (EBA). Given the clinical features, it was classified as BP-like EBA. He was initially treated with high-dose oral prednisolone, superpotent topical steroids and analgesia as an inpatient. Mycophenolate mofetil (MMF) was initiated and his corticosteroid dose was slowly tapered. His condition was completely controlled with MMF 500 mg twice daily at 4 months. The patient then elected to stop taking MMF and remains in complete remission at 7 months. EBA is rare in adults and even more so in children. The mean age of onset is 44–54 years, but up to 5% of reported cases are in children/adolescents. Four subtypes are described: classic mechanobullous, BP-like, mucous membrane pemphigoid-like and linear IgA bullous dermatosis-like. BP-like EBA is characterized by widespread vesicles and tense bullae, commonly on an inflammatory background and lacking skin fragility, scarring or milia. Oral and genital mucosae are frequently affected in paediatric cases of EBA. Various steroid-sparing treatment modalities are described, including dapsone, colchicine and MMF. The prognosis in the paediatric population is favourable compared with that of adults: the majority achieve complete remission or complete control on treatment.

P19 Diagnostic conundrums in paraneoplastic pemphigus

Abstract Paraneoplastic pemphigus (PNP) is a subset of pemphigus, a group of autoimmune blistering skin disorders. A 51-year-old man presented with paraneoplastic pemphigus associated with a hepatic neoplasm. He described an epigastric mass and associated mucositis affecting the orogenital mucosae, as well as the sclerae. Both computed tomography and magnetic resonance imaging demonstrated a 14-cm mass arising from the left lobe of the liver. A liver biopsy showed features of an atypical spindle-cell neoplasm, but a definite diagnosis was not possible. A diagnostic skin biopsy was taken from his outer lip mucosae. Histology showed surface ulceration with an acute inflammatory infiltrate, lichenoid lymphoplasmacytic infiltrate with focal basal vacuolar change, degenerate keratinocytes and adjacent suprabasal acantholysis and spongiosis. Perilesional direct immunofluorescence (DIF) was negative; however, indirect immunofluorescence (IIF) showed intercellular IgG antibodies on transitional epithelium with a high titre of 1 : 800 to rat bladder. These finding were consistent with the suspected clinical diagnosis of paraneoplastic pemphigus. The patient was commenced on oral prednisolone 1 mg kg−1 daily. Topical therapy included budesonide mixed with sweetener applied to the oral mucosae. Following this, a trial of 3 days of intravenous methylprednisolone along with 2% cocaine mouthwash, triple mouthwash (betamethasone, doxycycline and nystatin) and Dermovate® ointment was commenced, with improvement noted in pain, haemorrhage and ulceration. Intravenous immunoglobulin was administered preoperatively. The patient was subsequently transferred to a specialist centre for hepatobiliary surgery and listed for debulking of the liver mass. Postoperatively, he had plasmapheresis, with a noted reduction in mucosal ulceration and significant improvement in pain. Chemotherapy with doxorubicin and ifosfamide was initiated thereafter. PNP is a severe autoimmune blistering disorder characterized clinically by flaccid blisters and erosions. Although much is yet to be understood regarding the pathophysiology of PNP, studies show that the development of autoantibodies to the plakin family and desmogleins 1 and 3 play a particular role in its pathogenesis. In addition to autoantibodies, CD8+ T cells have also been shown to contribute to the development of PNP. In adults, the majority of cases are associated with an underlying haematological malignancy and, rarely, a solid-organ tumour. DIF findings include IgG and C3 deposition along the intercellular and basement membrane zones, while IIF detects autoantibodies to proteins found in transitional or stratified squamous epithelium. As in our case, reports have shown that the DIF can be negative, with one study showing a positive DIF in only 41% of cases. This case highlights the complex diagnostic and treatment challenges practitioners face when dealing with PNP and the patients it affects.

Clinicopathological Features of Cutaneous Vasculitis: A Cross-sectional Study from a Tertiary Care Centre in Karnataka, India

Introduction: Cutaneous vasculitis is an inflammatory disease of the dermal blood vessels with varying clinical presentations. It is not a single disease but a spectrum of entities that present as cutaneous vasculitis. Hence, histopathological evaluation is essential to confirm the diagnosis and determine the type of vasculitis. Direct Immunofluorescence (DIF) studies add credibility to the diagnosis. Aim: To investigate the spectrum of cutaneous vasculitis, its aetiological factors, and the clinicopathological features. Materials and Methods: This cross-sectional study was conducted over a three-year period (February 2015-January 2018). All cases of biopsy-proven cutaneous vasculitis diagnosed in the Department of Pathology, Kasturba Medical College, Manipal, Karnataka, India were included in the study. The clinical data, along with laboratory investigations including skin biopsy and DIF, were analysed. Results: A total of 137 cases of cutaneous vasculitis were diagnosed during the study period. The age of the patients ranged from 1-73 years. The peak incidence of cutaneous vasculitis was observed in the fourth decade 31 (22.6%), with no significant gender preponderance. Palpable purpura over the lower extremities was the most common skin lesion at the time of presentation seen in 47 (34.3%). Most cases of vasculitis were primary cutaneous vasculitis, while 11 cases showed evidence of systemic vasculitis such as Wegener’s granulomatosis, Polyarteritis Nodosa (PAN), and Churg-Strauss syndrome. No underlying aetiology was identified in the majority of cases 82 (59.9%), while a possible underlying aetiology like connective tissue disorder, drug intake, infections, etc., could be identified in 55 (40.1%) cases. Small vessel vasculitis was the most frequent, with leukocytoclastic vasculitis being the predominant type seen in 89 (65%) cases. DIF positivity was sensitive, with positivity around the blood vessel wall observed in89 (87.3%) of cases (N=102). Conclusion: Vasculitis is a broad, poorly defined category of diseases and can manifest with a variety of clinical presentations. Therefore, compiling clinical, laboratory, and pathological findings is essential for formulating the diagnosis.

Can Immunofluorescence on Skin/Mucosal Scraping Smear for Pemphigus Diagnosis Substitute Direct Immunofluorescence on Skin Biopsy?

IntroductionFew studies have been conducted on the use of Direct Immunofluorescence (DIF) on skin/mucosal scraping smear for diagnosis of pemphigus disease; however, the diagnostic value of DIF on the smear has not been fully evaluated.ObjectivesThe present study was carried out to assess the sensitivity and specificity of DIF on skin/mucosal smear for diagnose of pemphigus in the patients presenting with mucocutaneous erosive lesions.MethodologyA total of 89 patients including 40 males and 49 females aged between 23 and 80 years old with various bullous disorders were enrolled in the study. For definite diagnosis, all the patients were subjected to lesional biopsy for pathological studies and perilesional biopsy for DIF studies. In all the cases, skin/mucosal scraping smears were prepared from the perilesional healthy skin/mucosa and were stained with immunofluorescence conjugated anti-IgG.ResultsOf 89 patients, 56 (63%) patients were diagnosed with pemphigus. Immunodeposits favoring the pemphigus were demonstrated in the 46 smears of 56 cases of pemphigus (sensitivity of 82%). No case with other types of bullous disease had positive DIF on the smear (specificity of 100%).ConclusionThe findings of the study showed that the sensitivity of DIF on the smear is not high enough to allow us replacing the conventional DIF with smear-DIF for diagnosis of pemphigus, while the specificity of 100% would allow the unequivocal identification of a subset of patients with pemphigus.

A Cross-Sectional Study to Correlate Serum Complement C3 and C4 Levels With Clinical and Pathological Severity in Cutaneous Small-Vessel Vasculitis.

IntroductionThe role of serum C3 and C4 levels as a marker of disease activity in cutaneous small-vessel vasculitis (CSVV) has been sparsely studied, especially in India. The primary objective was to determine the correlation between clinico-histopathological severity and serum C3 and C4 levels in CSVV. The secondary objective was to determine the association between direct immunofluorescence (DIF) findings and serum C3 and C4 levels and clinico-histopathological findings.MethodThis prospective cross-sectional study included all the clinically diagnosed cases of CSVV that satisfied the pathological criteria for CSVV. A clinical disease activity grade and a histopathological severity grade were calculated in all patients (N=50).ResultsSerum C3 and C4 levels (n=44) were diminished in 4.5% of cases. There was no significant correlation between the serum C3 and C4 levels and the clinical and histopathological severity. DIF was positive in 60.0% of cases (n=45), and IgA was the predominant immune deposit (46.7%). No significant association was detected between the DIF findings and the serum C3 and C4 levels, histopathological severity, and clinical disease activity grade. Positive DIF findings were significantly associated with palpable purpura and cutaneous necrosis. A significant association was detected between gastrointestinal involvement and IgA positivity.ConclusionIn CSVV, serum C3 and C4 may not be used as markers of disease severity, and a positive DIF finding may indicate an underlying gastrointestinal involvement.

Cutaneous lupus: Immunofluorescence and lupus band test

Cutaneous lupus erythematosus is an autoimmune disease with a broad range of clinical findings. There are several methods to conduct a diagnostic approach, among these stands direct immunofluorescence, in which we can find the Lupus Band Test (LBT) consisting of a band of granular deposits of immunoglobulins and complement along the dermo-epidermal junction. It can have a high sensitivity and specificity for diagnosis in some cases, according to the biopsy site, the constituents of immunorreactants found at the dermoepidermal junction, and the morphology and intensity of the immunofluorescent band. Certain limitations of the test should be considered when interpreting the results. Although useful in diagnosis, the lupus band is not considered a pathognomonic sign of erythematous lupus. About one-third of patients with positive direct immunofluorescence on a skin biopsy do not have systemic lupus erythematosus (SLE). A positive LBT in non-lesional photoprotected skin represents a criterion with high specificity to identify patients with SLE.

Real-life impact of immunologic tests to predict relapse after treatment cessation in patients with bullous pemphigoid: A French multicenter retrospective study

A high level of anti-BP180 antibodies on enzyme-linked immunosorbent assay and a persistent positive direct immunofluorescence at the end of treatment (immunologic tests, [ITs]) are predictors of relapse after treatment cessation (TC) in patients with bullous pemphigoid. To evaluate the real-life impact of the immunologic-based decision of TC on the 3- and 6-month relapse rates after TC in bullous pemphigoid. Retrospective multicentric study included patients followed almost 6months after TC. Patients were classified according to whether the TC decision was in accordance with the results of ITs performed during the 3months before TC, despite the results of ITs or without ITs performed. We included 238 patients. Three months after TC, 36 patients showed relapse: 14 of 95 patients with TC in accordance with IT results (14.7%); 5 of 21 with TC despite ITs (23.8%); and 17 of 122 with TC without ITs (13.9%; P=.5). Six months after TC, the relapse rate was 18.9%, 28.6%, and 18.9% (P=.56), respectively, in the 3 groups. The retrospective design and the limited follow up. In real-life practice, in bullous pemphigoid, the 3- and 6-month relapse rates were not significantly reduced with TC decision based on results of ITs as compared with a classic clinical-based decision.

Brunsting-Perry pemphigoid: a systematic review.

Brunsting-Perry pemphigoid (BPP) is a rare, autoimmune bullous skin disorder classified within the spectrum of mucous membrane pemphigoid (MMP). An a priori protocol was designed based on PRISMA guidelines. PubMed and Scopus databases were searched for English-language articles concerning BPP published between 1950 and July 2021. Thirty-six articles including 63 BPP patients were analyzed. The mean age at diagnosis was 62.9 years (range: 27-86). BPP was shown to be characterized by vesiculobullous lesions (46/63, 73.0%) on an erythematous base, erosions or ulcerations (27/63, 42.9%), atrophic scars (49/63, 77.8%), and milia (4/63, 6.3%). Exclusive oral mucosal involvement was documented in 22.2% of cases, usually manifesting after the cutaneous onset of the disease. Subepidermal blistering was a constant finding, often with an eosinophil-rich inflammatory infiltrate (21/58, 36.2%). Positive direct immunofluorescence was found in 92.0% of patients, almost always with linear IgG ± C3 deposits along the basement membrane (43/46, 93.5%). BP180 (12/15, 80.0%), BP230 (5/15, 33.3%), and laminin 332 (3/15, 20.0%) were the most frequently identified target antigens. BPP nosologic position remains uncertain, given the overlap with other autoimmune bullous diseases, such as MMP, bullous pemphigoid, and epidermolysis bullosa acquisita, particularly in its BPP-like variant. Nonpredominant oral mucosal lesions may appear during the course of the disease, generally after cutaneous manifestations. Positivity of DIF and anti-BP180/230 autoantibodies detected on ELISA/immunoblotting in the absence of anticollagen VII antibodies may provide guidance in diagnosing BPP.

Cutaneous balamuthiasis: A clinicopathological study

IntroductionBalamuthia mandrillaris, a free-living amoeba, causes an uncommon infection that is characterized by cutaneous and neurological involvement, which carries a poor prognosis.MethodsThis is a retrospective observational study including patients with clinical suspicion of cutaneous balamuthiasis, their skin biopsies, and/or a positive direct immunofluorescence test. The data were collected from the Dermatology and Pathology service of the Hospital Cayetano Heredia and the Instituto Tropical Alexander von Humboldt, Lima, Peru, from January 1985 to June 2007. We identified 60 biopsies from 35 patients, from which clinical data were available in 30.ResultsTwenty-two (73%) patients had centrofacial lesions, mostly located on the nose. The classical lesion was an asymptomatic, erythematous, or violaceous infiltrated plaque. Twenty-two (73%) patients had neurologic involvement. Fifty (83%) biopsies showed granulomatous dermatitis and 75% showed ill-defined tuberculoid granulomas without caseous necrosis. Multinucleated giant cells were observed in 52 (87%) biopsies. Trophozoite forms were identified in the biopsies of 25 (71%) patients. Direct immunofluorescence was positive in 25 (71%) patients.ConclusionB. mandrillaris is a pathogen that is capable of inducing a characteristic skin lesion with a reaction pattern of ill-defined tuberculoid granulomas and many giant cells.

Value of the BIOCHIP mosaic-based indirect immunofluorescent technique in the diagnosis of dermatitis herpetiformis among patients with chronic pruritus

Background: The BIOCHIP mosaic-based indirect immunofluorescence technique is a practical, standardized test, and it has been used successfully in the diagnosis of autoimmune bullous dermatosis in recent years. Objectives: The study aimed to examine the diagnostic value of the BIOCHIP to identify dermatitis herpetiformis (DH) in patients with chronic pruritus (CP). Materials and Methods: This single-center case–control study included patients who applied to a dermatology clinic between July 2020 and December 2020. The diagnosis of DH was confirmed by direct immunofluorescence (DIF) test. In cases without DIF positivity, the diagnosis was established with a complete response to a long-term gluten-free diet and/or a swift response to dapsone treatment. All analyses were performed using SPSS version 21 (SPSS Inc., Chicago, IL, USA). The diagnostic performance of the variables was evaluated using receiver operating characteristic (ROC) curve analysis. P values < 0.05 were considered statistically significant. Results: GAF 3X (gliadin analog fusion peptide), as measured by the BIOCHIP method, had an area under the ROC curve of 0.854 (95% confidence interval: 0.688–1.000) for DH diagnosis with sensitivity, specificity, positive predictive, and negative predictive values of 72.73%, 100%, 100%, and 93.62%, respectively, demonstrating an overall accuracy of 94.55%. Conclusion: DH could be determined with nearly excellent accuracy by BIOCHIP GAF 3X analysis among patients with CP. BIOCHIP-based determination of GAF 3X was found to be superior to Enzyme-Linked ImmunoSorbent Assay (ELISA)-based determination of GAF 3X.