Bullous Systemic Lupus Erythematosus Research Articles

Current Knowledge on Nail Involvement in Autoimmune Bullous Disorders

<b><i>Background:</i></b> There are few studies, mainly case reports, on the involvement of the nail unit in autoimmune bullous disorders. <b><i>Summary:</i></b> Nail involvement in autoimmune bullous disorders is a significant clinical phenomenon, marked by a range of manifestations, most often not presenting with blisters like on the skin but rather with alterations of the nail unit such as paronychia, onychomadesis, or onycholysis. This involvement is particularly notable due to the unique immunological features of the nail unit, including the expression of various antigens and the presence of Langerhans cells. Conditions like pemphigus vulgaris, bullous pemphigoid, epidermolysis bullosa acquisita, linear IgA disease, and bullous systemic lupus erythematosus can lead to nail abnormalities. The prevalence of nail manifestations varies according to the disorder, and diagnosis often relies on histopathological and immunofluorescence testing. Nail involvement correlates with disease severity and duration, sometimes serving as a herald sign. Further research is needed to guide therapeutic approaches for nail involvement in autoimmune bullous diseases. <b><i>Key Messages:</i></b> Nail involvement in autoimmune bullous nail disorders may be confusing as there are almost never bullae. One should keep the diagnosis in mind when facing an atypical paronychia.

A Case of Bullous Systemic Lupus Erythematosus with Erosions on the Oral and Nasal Mucosa as Initial Symptoms

A Case of Bullous Systemic Lupus Erythematosus with Erosions on the Oral and Nasal Mucosa as Initial Symptoms

Patterns of Periodic Acid–Schiff Staining in Subepidermal Bullous Disorders with Emphasis on Bullous Pemphigoid

Abstract Background: Direct immunofluorescence (DIF) including salt split skin technique is quintessential in the diagnosis of subepidermal bullous disorders. These techniques are expensive and require technical expertise, limiting their diagnostic utility. The property of periodic-acid–Schiff to stain the basement membrane and the patterns produced by it was observed in a cohort of subepidermal bullous disorders, to see if it offers an alternative cost-effective testing method. Materials and Methods: This was a retrospective, observational study conducted in the department of general pathology, in a tertiary care medical center, over a period of 4 years. Only those cases for which paraffin sections, DIF and direct salt split skin immunofluorescence technique were available were included. The levels of anti-bullous pemphigoid (BP) antibodies were collected where available. Results: Of the 21 cases analyzed, 15 were BP, two each of epidermolysis bullosa acquisita (EBA) and lichen planus pemphigoides (LPP), one each of porphyria cutanea tarda (PCT), and bullous systemic lupus erythematosus (SLE). All the cases of BP, LPP and EBA showed a floor pattern of staining with periodic acid–Schiff (PAS) stain. Roof pattern of staining was observed in bullous SLE and PCT. PAS was found to be useful in diagnosing BP, especially in conjunction with an eosinophil rich bulla and anti-BP antibodies. Conclusion: We found that PAS stain could be an adjunct to hematoxylin and eosin stain when the diagnosis of BP is in doubt. However, larger sample size is needed to study its utility in other subepidermal bullous disorders.

44274 Direct immunofluorescence IgG autoantibody subclass analysis as a tool to differentiate between bullous systemic lupus erythematosus and epidermolysis bullosa acquisita

44274 Direct immunofluorescence IgG autoantibody subclass analysis as a tool to differentiate between bullous systemic lupus erythematosus and epidermolysis bullosa acquisita

Clinical presentation of bullous systemic lupus erythematosus in a pediatric patient: a case report

Bullous systemic lupus erythematosus (BSLE) is a rare blistering eruption that can occur in patients with systemic lupus erythematosus. Although 59 to 85% of patients will have skin manifestations, less than 5% will develop the bullous disease, which is even rarer (1%) in the pediatric population. We present the case of a 16-year-old female patient in whom a diagnosis of BSLE was made through clinical presentation, histological study by biopsy, and direct immunofluorescence. Due to the low incidence of cases reported in pediatric patients, it is possible that the diagnosis may not be made immediately, which is why we emphasize the importance of obtaining an accurate diagnosis and providing timely 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.

S2313 Esophageal Stricture Recalcitrant to Repeated Dilation in Complex Rheumatological and Bullous Disease

Introduction: Many rheumatological diseases have been well characterized to affect the esophagus. Esophageal involvement in autoimmune bullous disease is rare and almost always occurs with severe cutaneous symptoms. We report a case of severe esophageal strictures recalcitrant to repeated dilatation in a patient with complex rheumatological disease and isolated esophageal bullous disease. Case Description/Methods: A 50-year-old female presented with complete inability to tolerate oral intake for several weeks. Past history included Sjogren's syndrome and esophageal strictures with many previous dilatations. Physical exam findings were relevant for poor skin turgor, dry mucous membranes, and skin hyperpigmentation on her hands and forearms. Laboratory studies showed hypokalemia, hypoalbuminemia, and anemia. The patient was admitted. Her esophagram and esophagogastroduodenoscopy (EGD) showed a proximal esophageal stricture which was dilated using a push style dilator to 14mm. The stricture recurred, requiring repeated dilatation and placement of a percutaneous gastrojejunostomy. Once oral intake was tolerated, she was discharged with outpatient follow-up. Outpatient rheumatological workup showed antibodies to ANA, RNP, SSA, CCP, and Smith indicating mixed connective tissue disease with features of systemic lupus erythematosus, rheumatoid arthritis, and scleroderma. She required multiple dilatations over several months and was started on mycophenolate mofetil with some clinical improvement. The dysphagia recurred 2 months later and EGD showed and esophageal stricture measuring 7mm requiring a neonatal upper gastroscope to facilitate balloon dilatation. Biopsies examined for immunofluorescence showed continuous linear deposition along the basement membrane zone for IgG and C3, consistent with atypical esophageal bullous systemic lupus erythematosus or atypical esophageal bullous pemphigoid. The patient declined systemic steroids. Her mycophenolate mofetil dose was increased and she received weekly dilatations to prevent Koebnerization and allow time for medical therapy effect. After 3 months of treatment, she had symptom improvement (Figure). Discussion: In patients with systemic immune mediated disorders including rheumatological disease, bullous diseases can be considered as a cause of esophageal stricture. Bullous disease with esophageal involvement can be difficult to treat, especially in this patient who declined corticosteroids. A combination approach using both immune suppression and dilatation was effective.Figure 1.: a. Pre- dilatation during repeated dilation treatments. Diameter 8mm. b. Post- dilatation. Diameter 11mm. c. Immunofluorescence showing IgG deposition along the basement membrane.

Bullous systemic lupus erythematosus in a 16-year-old male patient

Bullous systemic lupus erythematosus (BSLE) is a vesiculobullous disease mediated by autoantibodies in patients with systemic lupus erythematosus (SLE). Tense vesicles and bullae are seen on an edematous, erythematous and sometimes normal skin in any body region, including mucous membranes and non-photoexposed areas. This is the case of a 16-year-old male patient with lupus nephritis who, on the seventh day of hospitalization, presented multiple serous and hemorrhagic blisters on the face, trunk, abdomen and upper extremities. The histological study showed a subepidermal bullousdermatosis with numerous neutrophils.

A Rare And Severe Complication Of SLE: Bullous Systemic Lupus Erythematous

Bullous systemic lupus erythematosus (BSLE) is a rare antibody-mediated blistering eruption of subepidermal tissues in patients with underlying SLE. We report a case of a 19 years old Indian unconscious male who presented with acute generalized blistering lesion and widespread tense vesicles and Bullae at the superior trunk, proximal superior limbs, and face(lips) with the symmetrical distribution. Bullae contained haemorrhagic fluid and bleeding was also seen on the ruptured site. The lesions are also seen on mucosal areas such as perioral, pharyngeal, laryngeal, and genital areas. Multiple erosions and crusting lesions are also seen over the body. Multiple scalp plaque erythematous peeling lesions with foul smells. Blood investigation showed leucocytosis and metabolic acidosis with a diagnosis of septic shock secondary to infected BSLE. He was treated with IV hydrocortisone 200mg, analgesic, and IV Ceftriaxone. Unfortunately, he collapsed 24 hours later and passed away. This case report highlights the importance of early detection of BSLE as an acute skin complication in patients with underlying SLE and its management steps.

A Rare And Severe Complication Of SLE: Bullous Systemic Lupus Erythematous

Bullous systemic lupus erythematosus (BSLE) is a rare antibody-mediated blistering eruption of subepidermal tissues in patients with underlying SLE. We report a case of a 19 years old Indian unconscious male who presented with acute generalized blistering lesion and widespread tense vesicles and Bullae at the superior trunk, proximal superior limbs, and face(lips) with the symmetrical distribution. Bullae contained haemorrhagic fluid and bleeding was also seen on the ruptured site. The lesions are also seen on mucosal areas such as perioral, pharyngeal, laryngeal, and genital areas. Multiple erosions and crusting lesions are also seen over the body. Multiple scalp plaque erythematous peeling lesions with foul smells. Blood investigation showed leucocytosis and metabolic acidosis with a diagnosis of septic shock secondary to infected BSLE. He was treated with IV hydrocortisone 200mg, analgesic, and IV Ceftriaxone. Unfortunately, he collapsed 24 hours later and passed away. This case report highlights the importance of early detection of BSLE as an acute skin complication in patients with underlying SLE and its management steps.

Bullous systemic lupus erythematosus in females.

Bullous systemic lupus erythematosus (BSLE) is a rare blistering presentation of systemic lupus erythematosus, typically affecting women with the highest incidence in those of African descent. The key pathogenic insult includes the formation of autoantibodies against type VII collagen, which weaken the basement membrane zone and lead to the formation of subepidermal blisters. The acute vesiculobullous eruptions in BSLE generally tend to affect photo-distributed areas, although they can arise unrelated to sun exposure (eg, mucous membranes, axillae). The bullae can arise from erythematous macules, inflammatory plaques, or previously normal skin. Their appearance can range from small, grouped vesicles reminiscent of lesions in dermatitis herpetiformis to large, tense blisters, similar to bullous pemphigoid. Internal organ involvement occurs in up to 90% of those affected. This mostly includes lupus nephritis (classes III–V, lifetime prevalence of up to 90%), arthralgias/arthritis, and cytopenias, while serositis and neuropsychiatric involvement are rare. First-line management with dapsone should be considered in mild disease with stable underlying systemic lupus erythematosus. As discussed in this review, the off-label use of rituximab (an anti-CD20 B-cell depleting agent) has been shown to be safe and effective in several refractory cases of BSLE unresponsive to dapsone, glucocorticoids, or steroid-sparing immunosuppressants.

Clusters of clinical and immunologic features in patients with bullous systemic lupus erythematosus: experience from a single-center cohort study in China

BackgroundBullous systemic lupus erythematosus (BSLE) is a rare subtype of systemic lupus erythematosus (SLE) that is clinically characterized by subepidermal tense vesicles or bullae. We aimed to investigate the clinical and laboratory features of patients with BSLE.MethodsWe retrospectively reviewed all patients who fulfilled the diagnostic criteria for BSLE in our institution from 2015 to 2021. Cutaneous lesions, systemic manifestations, treatment options, and outcomes were evaluated. For each case of BSLE, four controls were randomly selected from patients with single SLE. Major clinical and laboratory characteristics were compared between the two groups.ResultsAmong 4221 patients with SLE, 12 developed BSLE. Vesiculobullous lesions were the first sign in five of the BSLE patients (5/12, 41.7%) and appeared after SLE diagnosis in the remaining seven patients (7/12, 58.3%), with a median duration from SLE onset of 36 months (4–115 months). The most common BSLE-affected sites were the head and neck (10/12, 83.3%), extremities (9/12, 75.0%), trunk (7/12, 58.3%), and mucosae (6/12, 50.0%). All patients with BSLE had extra-cutaneous involvement. The SLE disease activity index score exceeded 5 in 10/12 (83.3%) patients, which indicated high disease activity. Patients in the BSLE group had significantly higher incidences of proteinuria (83.3% vs. 47.9%, P = 0.027), hematuria (75% vs. 31.3%, P = 0.006), hemolytic anemia (33.3% vs. 0%, P = 0.000), and leukopenia (66.7% vs. 25.0%, P = 0.006) than those in the control group. The use of systemic corticosteroids, immunosuppressants, dapsone, and skin care was effective in controlling disease.ConclusionsVesiculobullous lesions may be the first manifestation and indicate a high disease activity in patients with BSLE. Early diagnosis using clinical, histopathological, and immunological evaluations can lead to appropriate treatment of this progressive disease and improve prognosis.

A Rare Manifestation of Bullous Systemic Lupus Erythematosus in Children: A 10-year Retrospective Study in a Tertiary Care Hospital.

Bullous systemic lupus erythematosus (BSLE) is an uncommon cutaneous presentation that occurs even less frequent in the pediatric population. A retrospective review was performed from January 2012 to December 2021 in all pediatric patients (aged <18 years) who fulfilled the diagnostic criteria for BSLE to evaluate the clinical characteristics, extracutaneous involvement, histopathologic features, immunofluorescence patterns, serological abnormalities, internal organ involvement, treatments, and outcomes. Among 1,415 patients with SLE, five patients were validated for the diagnosis of BSLE, accounting for 0.35%. The mean age at diagnosis was 12.2 years (standard deviation, 1.92). The clinical features of BSLE in the study population were generalized tense bullae and large extensive vesicles on the lips and perioral and mucosal areas. Pediatric BSLE in the study population revealed high SLE disease activity with multiple organ involvement. Hematologic abnormalities, serositis, and renal involvement were found in all patients, while polyarthritis (40%) and neurological abnormalities (40%) were less frequently observed. Systemic corticosteroids, intravenous immunoglobulin, immunosuppressants, antimalarials, and dapsone were prescribed in the study population. The cutaneous lesions subsided in all patients with a median clearance duration of 14 days (range, 5–56 days). BSLE in the pediatric population has auxiliary manifestations with high disease activity. Multiple organ involvement, especially hematologic abnormalities, serositis, and renal involvement, was frequently found in the study population. Although cutaneous lesions in BSLE subsided in all patients, involvement of other organs, especially renal impairment, required aggressive treatment, and long-term follow-up.

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.

Successful remission with tofacitinib in a patient with refractory bullous systemic lupus erythematosus.

Successful remission with tofacitinib in a patient with refractory bullous systemic lupus erythematosus.

Successful treatment of bullous lupus with corticosteroids and belimumab: A case report.

Cutaneous lupus erythematosus (CLE) is one of the most common manifestations of systemic lupus erythematosus (SLE), although it can manifest as an independent entity as well. Bullous systemic lupus erythematosus (BSLE) is a rare cutaneous manifestation of SLE presenting as tense vesiculobullous eruptions in a photosensitive distribution. Pathophysiology is secondary to autoantibodies against noncollagenous domain 1 and 2 (NC1 and NC2) type VII collagen, and histopathology reveals dense neutrophilic infiltration of the dermis with direct immunofluorescence showing IgG deposition at dermoepidermal junction. There is lack of data on available therapeutic options to treat BSLE, and varying responses to dapsone, methotrexate, azathioprine and corticosteroids have been reported. Belimumab, a fully humanised Change to Immunoglobulin G1λ (IgG1λ) monoclonal antibody targeting soluble B lymphocyte stimulator protein, was the first Food and Drug Administration-approved drug for SLE and has been reported to be effective for CLE. We present the case of a 41-year-old black female with SLE presenting with BSLE, who was successfully treated with corticosteroids and belimumab and did not experience disease relapse even after discontinuation of corticosteroids. To our knowledge, this is the first reported case of successful treatment of BSLE with belimumab, and further research can help determine the role of belimumab in the treatment of BSLE.

Diffuse blistering rash with ocular involvement

Diffuse blistering rash with ocular involvement

English

BACKGROUND Autoimmune blistering diseases contribute to a significant number of patients attending the dermatology department. As they have a varied presentation, definite clinical diagnosis is not possible always. Histopathology is also inconclusive sometimes. Immunofluorescence studies are now considered to be most valid in diagnosing these problems. Direct immunofluorescence study of perilesional skin will help us to identify the type of immunoreactants and their exact site of location. This may help dermatologist to make accurate and prompt diagnosis. Hence we decided to conduct this study. METHODS We conducted a diagnostic tool evaluation cross sectional study in 34 patients, who attended dermatology outpatient department (OPD) of a major tertiary care centre in South Kerala, with a clinical diagnosis of immunobullous disease. We performed histopathology and direct immunofluorescence (DIF) in all our patients. RESULTS Age group of study population ranged from 31 – 90 years with a definite female preponderance. 32.4 % of our patients had intraepidermal immunobullous diseases while rest had subepidermal disorders. Pemphigus vulgaris was the commonest intraepidermal disease reported, with most patients presenting in fifth and sixth decade. Oral erosions were the presenting symptom in 97.5 % patients. Classical histopathology was seen in all except one, clinicopathological correlation being 87.5 %. Pemphigus erythematosus could be diagnosed only by DIF. Direct immunofluorescence had 100 % negative predictive value (NPV). Among subepidermal immunobullous diseases, bullous pemphigoid was seen in 19 patients with age ranging from 41 - 90 years. Clinical, histopathological and DIF correlation in bullous pemphigoid was 100 %. Clinical diagnosis of lichen planus pemphigoids and bullous systemic lupus erythematosus could be confirmed by histopathology and DIF while linear IgA disease showed features of bullous pemphigoid in DIF. Positive and negative predictive value reached 100 % in the present study. CONCLUSIONS Direct immunofluorescence study is a useful investigation for the correct diagnosis of various immunobullous diseases although histopathology is more relevant in diagnosis of certain subtypes of pemphigus. KEY WORDS Immunobullous Diseases, Direct Immunofluorescence, Histopathology.

The epidemiology of autoimmune bullous diseases in Sudan between 2000 and 2016.

Autoimmune bullous diseases vary in their clinico-epidemiological features and burden across populations. Data about these diseases was lacking in Sudan. We aimed to describe the epidemiological profile and to estimate the burden of autoimmune bullous diseases in Sudan. This was a retrospective cross-sectional study conducted at Khartoum Dermatological and Venereal Diseases Teaching Hospital. We used routinely collected health care data, and included all patients with an autoimmune bullous disease who presented to the hospital between 2001 and 2016. Out of the 4736 patients who were admitted to the hospital during the study period, 923 (19.5%) had an autoimmune bullous disease. The average rate of patients at the hospital was 57.7 per year representing 1.3 per 100,000 population per year. After exclusion of patients where the final diagnosis was missing, 585 were included in the further analysis. Pemphigus vulgaris was the most common disease (50.9%), followed by bullous pemphigoid (28.2%), linear IgA disease/chronic bullous disease of childhood (8.4%), and pemphigus foliaceous (8.2%). Pemphigoid gestationis and IgA pemphigus constituted 1.4% and 1.2% of the cohort, respectively. Paraneoplastic pemphigus, mucous membrane pemphigoid, lichen planus pemphigoidis, bullous systemic lupus erythematosus, and dermatitis herpetiformis were rare. None of the patients had epidermolysis bullosa acquisita. The clinico-epidemiological characteristics vary among the types of autoimmune bullous diseases. Females were more predominant in most of them. Sudanese patients tended in general to present at a younger age than other populations. The pool of Sudanese patients with autoimmune bullous diseases is large which requires investigation for the local risk factors and presents a field for future trials.

Bullous Systemic Lupus Erythematosus Mimicking Bullous Pemphigoid: A Case Report

Introduction: Bullous systemic lupus erythematosus (BSLE) is an infrequent but distinct presentation of systemic lupus erythematosus (SLE) in less than 5% of lupus cases. It is characterized by vesicobullous skin eruption in SLE that can develop either before or after SLE diagnosis has been established. Distinguish between BSLE with other autoimmune blistering diseases such as bullous pemphigoid (BP), dermatitis herpetiformis, linear IgA, etc., is very important to prevent misdiagnosis. The physician must be able to combine clinical, histological and immunofluorescence finding for the diagnosis approach. We report a case of blistering skin eruption in SLE patient. Case Report: A 19-year-old female patient complained of tense blistering on her lip, face and wrists since one month ago. She was diagnosed with SLE two weeks ago. The dermatological state showed bullae and vesicle on erythematous/ normal base, erosions, excoriation and blackish red crust on the lip, face, armpit, neck, abdomen and wrists. Histopathological examination of the lesion showed sub-epidermal bullae containing PMN leukocytes consist of abundant neutrophils, only occasional eosinophils and the presence of keratotic plugs. Direct immunofluorescence (DIF) of the skin showed linear deposition of IgG, IgA, IgM and C1q at the dermo-epidermal junction. This patient exhibited similar features to both BSLE and BP with tensed clear blisters and subepidermal cleft. BSLE differ from BP by abundant neutrophils found on histopathological examination, whereas BP has abundant eosinophils. Conclusion: Immunofluorescence examination shows linear IgG in BP, whereas linear or granular IgG in BSLE. Establishing the correct diagnosis is important to prevent misdiagnosis and mistreatment.