Intercellular IgG Deposits Research Articles

P19 Pemphigus foliaceus in a 13-year-old child

Abstract Pemphigus foliaceus (PF) is an autoimmune blistering disease caused by autoantibodies against desmoglein 1, which cause acantholysis and intraepithelial blisters. PF classically presents in adults and, excluding the endemic form of PF (fogo salvagem) found in North Africa, Turkey and South America, it rarely occurs in children. We present a previously fit and well 13-year-old girl who was referred with a 2-year history of multiple small blisters on the legs which gradually spread to the trunk and arms. Concomitantly she had severe abdominal pain and alternated constipation with diarrhoea. A skin biopsy suggested a diagnosis of PF. She was commenced on azathioprine which had to be subsequently discontinued due to severe muscle pain and fatigue. She has had a 15-month-course of oral prednisolone which was difficult to wean due to acute flares. A repeat biopsy showed features of pemphigus, direct immunofluorescence showed intercellular deposition of IgG and C3 in the epidermis and ELISA revealed positive autoantibodies against desmoglein 1 (110 U mL–1), in keeping with PF. She was commenced on erythromycin with small improvement and was therefore switched to azithromycin which was discontinued after 5 days due to severe abdominal pain. No abnormalities were seen on a colonoscopy and upper gastrointestinal endoscopy. Despite treatment with erythromycin and topical tacrolimus she continued experiencing acute flares, particularly over her chest and back. Dapsone 50 mg once daily was prescribed and was increased to 75 mg to fully resolve the skin lesions. She is under follow-up and her disease has now remained quiescent for a year.

Pruritic crusted lesions in an 89‐year‐old woman

Pruritic crusted lesions in an 89‐year‐old woman

Anti-desmoglein 1 antibody-positive mother and antibody-negative child with Darier's disease.

We report a mother and an adult son with Darier's disease. The mother, 76 years old and Japanese, had positivity for anti-desmoglein (Dsg)1 antibodies. She had erythema with hyperkeratosis and seborrheic and interstitial blistering. A high level of anti-Dsg1 antibodies was detected in the serum. Histopathological examination showed acantholysis and direct immunofluorescence testing revealed intercellular IgG and C3 deposition of the epidermis. Although she was diagnosed as having pemphigus foliaceus, the skin lesions slightly improved with immunosuppressive therapy. Her son, 47 years old, had similar skin lesions on the seborrheic and interstitial parts, but the anti-Dsg1 antibodies were negative in his serum. Histopathological examination showed acantholysis and dyskeratotic cells. Although Hailey-Hailey disease was first suspected, no mutation in the ATP2C1 was detected in either patient. Trio-exome analysis including the father showed a heterozygous c.2027C>A transition on exon 14 of ATP2A2, causing a replacement at amino acid 676 (p.Ala676Asp) in the mother and son only. The two patients were then diagnosed as having Darier's disease. Exome analysis further showed that a novel heterozygous missense mutation of DSG1 was identified only in the affected mother. Anti-Dsg1 antibody-positive Darier's disease is reported here for the first time. Very rare coexistence of Darier's disease and anti-Dsg1 antibody-positivity might be associated with this novel heterozygous DSG1 mutation. Experimental evidence is required to validate this hypothesis.

A 72-year-old man with nonhealing facial erosions and bullae

A 72-year-old man with nonhealing facial erosions and bullae

A Case of Thymoma-Associated Paraneoplastic Pemphigus Presenting as Psoriasis

Thymoma was reported to be frequently associated with autoimmune diseases, including not only Myasthenia Gravis (MG), but also a variety of dermatoses, such as lichen planus, pemphigus vulgaris, pemphigus foliaceous, graft-vs-host disease, and Paraneoplastic Pemphigus (PNP) [1-3]. Here, we report a rare case of thymoma-associated pemphigus presenting as psoriasis. A 48-year-old man presented with pruritic skin rash all over the body for 2 months. He underwent thymectomy (type B2 thymoma) 9 years ago, followed by postoperative chemotherapy and radiotherapy, but 2 years ago, pleural metastasis was found and he developed MG, which was controlled by pyridostigmine, prednisone and plasmapheresis. Two months before, he developed pruritic skin eruptions. Physical examination revealed annular erythemas with white scales on the trunk and extremities, no oral lesion was found (Figure 1a and 1b). Laboratory examination revealed an elevated SCC antigen level of >100ng/ml (normal 0-2.5 ng/ml). ANA, anti- SSA, anti-SSB, anti-dsDNA, and anti-Sm antibodies were all negative. Histopathology showed parakeratosis, psoriasis-like epidermal hyperplasia, necrotic keratinocytes, basal cell edema and lichenoid lymphocytes infiltration (Figure 1c). DIF showed positive intercellular IgG and C3 deposition. IIF identified positive intercellular IgG on rat bladder epithelium (titer 1: 80). Anti-Dsg1 and anti-Dsg3 antibodies were normal. CT scan revealed pleural metastasis of thymoma (Figure 1d). The patient was diagnosed as PNP based on the skin eruption, histopathology, IIF of rat bladder epithelium and pleural metastasis of the thymoma [4]. The lesions relieved partly with oral prednisone 40mg/d. The patient finally died from respiratory failure within half a year.

Punctate Pattern and Pemphigus: Is There Any Evidence of Punctate Pattern Among Iranian Patients?

To examine the prevalence of this novel pattern among Iranian patients with pemphigus and peruse the relationship between the presence of a punctate pattern with clinical severity of disease and histopathological findings. One hundred recently diagnosed patients with pemphigus were enrolled. DIF evaluation and routine light microscopy were performed on their biopsy specimens. Disease severity was determined using the Pemphigus Disease Area Index. Serum samples were collected to measure autoantibody titers using enzyme-linked immunosorbent assay. All the samples evaluated by DIF showed a continuous linear pattern of intercellular IgG deposition, whereas none of them had a punctate pattern. Despite a significant correlation between the Pemphigus Disease Area Index score and autoantibody values, no association between histopathological findings and disease severity has been found. We could not detect any punctate pattern among Iranian patients with pemphigus. The importance of this pattern in the diagnosis of pemphigus might be different among patients with different ethnic and genetic factors.

Comparative analysis of BIOCHIP mosaic-based indirect immunofluorescence with direct immunofluorescence in diagnosis of autoimmune bullous diseases: A cross-sectional study

Background:Autoimmune bullous diseases (AIBD) are a heterogeneous group of diseases characterized by autoantibodies against desmosomal proteins in the pemphigus group of disorders and adhesion molecules of the dermal-epidermal junction in pemphigoid group of diseases. Direct immunofluorescence (DIF) establishes the diagnosis of AIBD by demonstrating intercellular deposits of IgG and C3 in case of pemphigus and linear deposits of IgG and C3 along the basement membrane zone (BMZ) in bullous pemphigoid (BP). BIOCHIP mosaic-based indirect immunofluorescence (IIF), a novel diagnostic approach employs detection of characteristic staining pattern and target antigens in a single miniature incubation field.Aim:To compare the BIOCHIP mosaic-based IIF with DIF in the diagnosis of AIBD.Materials and Methods:A total of 40 patients of AIBD in the active phase of the disease were included in the study. Skin biopsy was done in these patients for DIF study and serum was subjected to BIOCHIP mosaic-based IIF assay. The results were then compared.Results:DIF revealed a diagnosis of Pemphigus in 18 patients and BP in 22 patients. BIOCHIP showed a diagnosis of pemphigus in 18 patients, BP in 18 patients and floor pattern staining in four patients, which could be attributed to any of the floor pattern staining subepidermal blistering disease.Limitations:Small sample size, lack of control group and no comparison made with ELISA.Conclusion:This study concludes that the result of BIOCHIP shows correlation with the DIF and can be used as a first line-screening tool in the diagnosis of AIBD.

Direct immunofluorescence of the hair follicle in pemphigus: a less invasive method for diagnosis

Background Demonstration of intercellular deposition of immunoglobulin (Ig)G on the cell surface of keratinocytes by direct immunofluorescence (DIF) of the skin is the gold standard in diagnosis of pemphigus. In the past few years, the intercellular deposition of IgG in the outer root sheath has shown to be useful. Objective The aim was to compare the DIF of hair (plucked anagen and telogen and telogen obtained by combing) with that of skin for diagnosis of patients with pemphigus vulgaris (PV) and those with pemphigus foliaceus (PF). Materials and methods A total of 30 patients [24 with PV and six with PF] with active disease were included. Clinical evaluation (ABASIS score), histopathological examination, and DIF of skin and hair were done. Presence of intercellular deposits of IgG and or C3 in skin and outer root sheath of the hair was considered positive. Results DIF of skin was positive in all patients for IgG and in 25 patients for C3 and also DIF in anagen hair was positive in all patients, whereas in telogen hair, it was positive in 23 patients (17 with PV for both IgG and C3, and six with PF for IgG, and four of them for C3). All patients showed a positive relation between clinical, histopathological, DIF skin, and DIF of anagen hair in both PV and PF. Regarding telogen hair, the same findings was found in all six (100%) patients with PF, but in PV, only 17 (70.83%) patients showed these positive relations, whereas the other seven (29.2%) had positive DIF of both skin and anagen hair only. The sensitivity of hair DIF was 100% for anagen hair and 76.67% for telogen hair in patients with pemphigus. Conclusion Anagen and telogen hair DIF is a simple, noninvasive, and cost-effective procedure and can be used as an additional procedure for diagnosis in patients with PV and PF.

Comparative Analysis of BIOCHIP Mosaic-Based Indirect Immunofluorescence with Direct Immunofluorescence in Diagnosis of Autoimmune Bullous Diseases: A Cross-Sectional Study.

Background:Autoimmune bullous diseases (AIBD) are a heterogeneous group of diseases characterized by autoantibodies against desmosomal proteins in the pemphigus group of disorders and adhesion molecules of the dermal-epidermal junction in pemphigoid group of diseases. Direct immunofluorescence (DIF) establishes the diagnosis of AIBD by demonstrating intercellular deposits of IgG and C3 in case of pemphigus and linear deposits of IgG and C3 along the basement membrane zone (BMZ) in bullous pemphigoid (BP). BIOCHIP mosaic-based indirect immunofluorescence (IIF), a novel diagnostic approach employs detection of characteristic staining pattern and target antigens in a single miniature incubation field.Aim:To compare the BIOCHIP mosaic-based IIF with DIF in the diagnosis of AIBD.Materials and Methods:A total of 40 patients of AIBD in the active phase of the disease were included in the study. Skin biopsy was done in these patients for DIF study and serum was subjected to BIOCHIP mosaic-based IIF assay. The results were then compared.Results:DIF revealed a diagnosis of Pemphigus in 18 patients and BP in 22 patients. BIOCHIP showed a diagnosis of pemphigus in 18 patients, BP in 18 patients and floor pattern staining in four patients, which could be attributed to any of the floor pattern staining subepidermal blistering disease.Limitations:Small sample size, lack of control group and no comparison made with ELISA.Conclusion:This study concludes that the result of BIOCHIP showed a significant correlation with the DIF and can be used as a first line-screening tool in the diagnosis of AIBD.

Plucked Hair as a Substrate for Indirect Immunofluorescence in Cases of Pemphigus Vulgaris.

Introduction:Indirect immunofluorescence (IIF) is used to determine the circulating autoantibodies in cases of Pemphigus. For IIF in Pemphigus several substrates had been used. This study was done to determine whether plucked hair can be utilized as a substrate for IIF in cases of Pemphigus. Aim & Objectives: To determine the efficacy of utilizing the plucked hair as a substrate for indirect immunofluorescence among patients with Pemphigus vulgaris.Methodology:Thirty two consecutive patients with active and fresh diagnosed cases of pemphigus vulgaris (PV) who did not undergo any treatment and those patients who had positive DIF findings of characteristic fish net or chicken wire pattern of intercellular IgG deposits in the epidermis of perilesional skin were included in the study. A total of 32 control subjects without any auto immune disorders were selected and the blood samples were taken from these patients for IIF analysis. Anagen hair were collected from the healthy volunteers without pemphigus in the same way as for trichogram. Telogen hair were obtained by combing the hair and collecting the loose strands of hair on the comb and further confirmed under microscope. Three hairs each of anagen and telogen stage were collected from each subject to be utilized as a substrate for IIF.Results:Out of 32 samples 20 samples showed positive results for Ig G alone and 5 samples showed positivity for Ig G and C3. One sample showed positivity for C3alone. All these 26 samples were considered to have positive IIF test based on the intercellular pattern of deposit. Positive IIF results were observed in anagen hair samples and were negative in all the telogen hair samples. Six anagen hair samples did not show any positive findings in IIF for the study group. All the 32 control samples showed negative reports in IIF.Conclusion:In conclusion, IIF microscopy on plucked hair as a substrate is a more sensitive immunoassay for the detection of circulating intercellular autoantibodies in PV and the lower negative predictive value of this substrate is a limitation. Further large scale studies might provide better information regarding the practical utility.

IgG/IgA pemphigus presenting with mixed clinical features of pemphigus erythematosus and IgA pemphigus

IgG/IgA pemphigus is a phenotype of atypical pemphigus with variable clinical, histological, and immunological manifestations. We describe a unique case of IgG/IgA pemphigus in a 58-year-old male presenting with mixed features of pemphigus erythematosus and IgA pemphigus. The diagnosis is made based on the histopathological examination and intercellular deposition of IgG and IgA throughout the epidermis on direct immunofluorescence test. The patient responded favorably to the combination therapy of dapsone and low-dose systemic steroids.

Continuing Medical Education: Paraneoplastic pemphigus: a clinical, laboratorial, and therapeutic overview

AbstractParaneoplastic pemphigus is a rare and severe autoimmune blistering disease characterized by mucocutaneous lesions associated with benign and malignant neoplasms. Diagnostic criteria include the presence of chronic mucositis and polymorphic cutaneous lesions with occult or confirmed neoplasia; histopathological analysis exhibiting intraepidermal acantholysis, necrotic keratinocytes, and vacuolar interface dermatitis; direct immunofluorescence with intercellular deposits (IgG and C3) and at the basement membrane zone (IgG); indirect immunofluorescence with intercellular deposition of IgG (substrates: monkey esophagus and simple, columnar, and transitional epithelium); and, autoreactivity to desmogleins 1 and 3, desmocollins 1, 2, and 3, desmoplakins I and II, envoplakin, periplakin, epiplakin, plectin, BP230, and a-2-macroglobulin-like protein 1. Neoplasias frequently related to paraneoplastic pemphigus include chronic lymphocytic leukemia, non-Hodgkin lymphoma, carcinomas, Castleman disease, thymoma, and others. Currently, there is no standardized treatment for paraneoplastic pemphigus. Systemic corticosteroids, azathioprine, mycophenolate mofetil, cyclosporine, rituximab, cyclophosphamide, plasmapheresis, and intravenous immunoglobulin have been used, with variable outcomes. Reported survival rates in 1, 2, and 5 years are 49%, 41%, and 38%, respectively.

Paraneoplastic pemphigus: Retrospective study of a case series

Paraneoplastic pemphigus (PNP) is a rare condition associated with poor prognosis. It associates polymorphic mucocutaneous manifestations with neoplasia. Diagnosis is difficult because of the various clinical and histological features involved and the lack of specificity of immunological examinations. We retrospectively analyzed the records of patients presenting with PNP in the Poitou-Charentes region between 2000 and 2015. Seven patients were included. They presented 9 neoplasias (1 lymphoma, 1 melanoma, and 7 carcinomas) diagnosed from 4 months before to 25 months after the occurrence of cutaneous (6/7) and/or mucosal (6/7) polymorphic lesions. Histological examination revealed epidermal acantholysis (7/7), keratinocytic necrosis (4/7), and interface lichenoid dermatitis (5/7). Intercellular deposits of IgG and C3 or along the dermo-epidermal junction were detected with direct immunofluorescence (IF) (7/7). Four of 6 patients tested had positive indirect IF on rat bladder epithelium. Follow-up ranged from 1-132 months with a one-year survival of 85.7%. The clinical and histopathological presentations observed in our patients were polymorphic, with overlap between the clinical and histological features of PNP and classical pemphigus. Prognosis and survival appear better in our series than in the literature. It is possible that in some cases, the association of pemphigus with neoplasia was fortuitous, which might account for the better prognosis. A new consensus on the diagnostic criteria for PNP is needed to help practitioners to consensually diagnose it for prognostic or therapeutic trials.

How can immunohistochemistry improve the diagnosis of pemphigus foliaceus?

PurposePemphigus foliaceus (PF) is a rare, autoimmune blistering disorder characterized by the production of autoantibodies against desmoglein 1. The mainstay for diagnosis is the demonstration of immune complex deposition by direct immunofluorescence (DIF) in fresh tissue samples. Immunohistochemistry (IHC) recognizes autoantibodies in formalin-fixed paraffin-embedded specimens, but studies regarding its use in PF are scarce. This study aims to evaluate immunoglobulin and C3 deposition using IHC in patients with confirmed PF by DIF and indirect immunofluorescence (IIF). Material and methodsSix biopsies obtained from five patients with PF and six healthy individuals were included in this study. Anti-C3c, -IgG, -IgM, and -IgA antibodies were used for DIF and automated IHC. After digitalizing the slides, staining was classified as negative (0) or positive (1=mild/2=intense). ResultsDIF revealed intraepidermal intercellular deposition of IgG and C3c (n=6), without deposits in dermal structures. IHC was positive in the intercellular spaces between keratinocytes with anti-IgG (n=6) and anti-C3c antibodies (n=6); no intercellular immune complexes deposition was observed in healthy individuals. In patients with PF, inflammatory cells were tagged by anti-IgG and anti-C3c (n=6), anti-IgM (n=1), and anti-IgA (n=1); and immune complexes at vessel walls were detected with anti-C3c, anti-IgG, anti-IgA (n=6), and anti-IgM (n=5) antibodies. Adnexal positivity occurred with anti-C3c and anti-IgG (n=6), anti-IgM (n=1), and anti-IgA (n=3). Healthy individuals also presented positivity in inflammatory cells with anti-IgG and anti-C3c (n=4), anti-IgM (n=1), and anti-IgA (n=3); vessels were stained with anti-IgG and anti-C3c (n=5), anti-IgM and anti-IgA (n=4); adnexa were not represented in all samples obtained from healthy individuals. ConclusionIHC may serve as a reliable method to assess PF diagnosis. Immune deposits in dermal structures suggest their participation in autoimmune/inflammatory processes in PF. IHC may contribute to evaluate disease mechanisms, prognostic factors, and target-oriented treatment in PF.

Comparison of Direct Immunofluorescence of Plucked Hair and Skin for Evaluation of Immunological Remission in Pemphigus.

Background:Pemphigus is a chronic autoimmune bullous disorder characterized by autoantibodies directed against desmoglein 3 and/or 1. Demonstration of intercellular deposition of IgG on the cell surface of keratinocytes by direct immunofluorescence (DIF) of the skin is the gold standard in the diagnosis of pemphigus. Recently, DIF of plucked hair demonstrating intercellular deposition of IgG in the outer root sheath (ORS) has shown to be useful.Objective:To compare the DIF of plucked hair and skin for the evaluation of immunological remission in pemphigus vulgaris patients in clinical remission.Materials and Methods:A total of 30 patients with pemphigus vulgaris with positive DIF of the skin and hair at baseline were included, and DIF of skin and hair was repeated after 6 months or more of clinical remission (with no new/non-healing lesions). Presence of intercellular deposits of IgG and or C3 in skin and ORS of the hair was considered as positive.Results:Of the 30 patients, DIF of skin was positive in 10 patients and hair was positive 14 patients. The findings of hair and skin DIF correlated with each other in 22 patients. In 6 (20%) patients DIF of hair was positive even though the DIF of skin was negative. The sensitivity of hair DIF was 80% and specificity was 70%.Limitations:Small sample size.Conclusion:DIF of hair is a simple, non-invasive, and cost effective procedure and can be used as an additional procedure for the assessment of immunological remission in patients with pemphigus vulgaris.

Cyclosporine-Induced Posterior Reversible Encephalopathy Syndrome in a Patient with Pemphigus Vulgaris.

Dear Editor: Cyclosporine is an immunosuppressive drug originally isolated from Tolypocladium inflatum in 19701. Here, we report a case of cyclosporine-induced posterior reversible encephalopathy syndrome (PRES) in a patient with pemphigus vulgaris. A 41-year-old Korean woman who had no underlying disease except pemphigus vulgaris was admitted to the emergency room owing to a sudden onset of severe headache, nausea, vomiting, and vision loss. A few minutes after admission, she presented with generalized tonic-clonic convulsive movement and became confused. Transient hypertension (160/100 mmHg) accompanied the episode. Laboratory tests showed leukocytosis (17.9×109/L) with neutrophilia (92.2%); cerebrospinal fluid was normal. Multiple ill-defined high-signal enhanced lesions were visible in the cortex and subcortical white matter of the bilateral parieto-occipital lesion on a T2-weighted brain magnetic resonance imaging (MRI) (Fig. 1A). Six months earlier, the patient presented with highly crusted vegetating lesions on her lower lip (Fig. 2A) as well as several flaccid blisters and scaly crusted erosions on her trunk (Fig. 2B). Histopathological examination showed suprabasal acantholysis (Fig. 2C), and a direct immunofluorescence study on the perilesional dorsal skin showed intercellular epidermal IgG deposition (Fig. 2D). Therefore, she was diagnosed with pemphigus vulgaris. Initially, the patient received prednisolone 100 mg/day for 2 weeks and tapered for 4 months; this was followed by cyclosporine 200 mg/day (4 mg/kg) for 2 months. We considered acute hypertension and cyclosporine administration as possible causes of the convulsive encephalopathy. Cyclosporine was discontinued immediately, and conservative therapy was administered. The patient showed a complete clinical recovery in 6 weeks, and signal enhancement also decreased on follow-up brain MRI (Fig. 1B). Cyclosporine is rarely reported to cause PRES; its association with cyclosporine was first described by Adams et al.2 in 1987. PRES is characterized by clinical symptoms including headache, mental confusion, vomiting, visual disturbance, and multiple generalized tonic-clonic seizures; it is associated with characteristic radiological abnormalities in the posterior area of the cerebral white matter3. In addition to immunosuppressive or cytotoxic drugs, reported causes of PRES also include acute hypertension, infection, autoimmune disease, eclampsia, and pre-eclampsia3. Although the pathophysiology of PRES remains unclear, several mechanisms have been proposed. Cyclosporine has a direct toxic effect on vascular endothelial cells, causing them to release endothelin, prostacyclin, and thromboxane; these may cause microthrombi and also damage the blood-brain barrier3. Many PRES cases have been reported in organ and bone marrow transplant recipients receiving high-dose cyclosporine4. However, in the present case, the patient had been taking low-dose cyclosporine 4 mg·kg-1·day-1 and had no associated risk factors for neurotoxicity. We finally diagnosed the patient with PRES because of her favorable response to cyclosporine discontinuation and the ultimately near-full reversal of encephalopathy. Thus, the possibility of PRES in patients taking even low-dose cyclosporine who present with neurologic symptoms should be emphasized. Physicians should rapidly diagnose this disease and withdraw the causative drug(s), because delayed diagnosis could lead to permanent sequelae. Fig. 1 (A) Axial T2-weighted magnetic resonance imaging (MRI) showing an area of high signal intensity in the bilateral white matter of the posterior parieto-occipital area. (B) Six weeks later, axial T2-weighted MRI showing significant improvement after cyclosporine ... Fig. 2 (A) Highly crusted vegetating lesions on the lower lip. (B) Several flaccid blisters and scaly crusted erosions are distributed on her trunk. (C) Separation of the epidermis above the basal layer and scattered acantholytic cells (H&E, ×200). ...

Pemphigus vulgaris presenting as gingival hypertrophy in a child

Pemphigus vulgaris is a mucocutaneous autoimmune disease caused by autoantibodies against the desmosomal proteins desmoglein 3 and desmoglein 1. Oral pemphigus vulgaris has rarely been reported to occur in children and adolescents. A current literature review reported 50 cases, 7 of which presented with oral lesions exclusively. Oral lesions of pemphigus commonly present as vesicles or bullae that ulcerate easily and occur most frequently in the buccal, palatal and labial mucosa. Gingival lesions usually present as desquamative gingivitis. We report a case of a healthy 17-year old Hispanic female, taking no medications, who presented with an 8-year history of gingival hypertrophy and no other oral, skin or other mucosal lesions. Clinically she had severe gingival hypertrophy affecting both the facial and lingual surfaces and covering most of the clinical crowns. The surface epithelium appeared eroded in areas and the tissues bled easily. Surgical excision of all hypertrophied gingival tissue was done in the operating room. The final diagnosis on light microscopy was inflammatory hyperplasia and ancantholysis. Direct immunofluorescence showed intercellular deposition of IgG and C3 and indirect immunofluorescence showed IgG positive intercellular staining a 1:80 dilution. Testing for serum antibodies against desmoglein 1 and 3 however was negative. After further evaluation with dermatologist, a diagnosis of atypical pemphigus vulgaris was made. Only 2 other reported cases of pemphigus vulgaris have presented with oral nodules or masses but none exclusive to the gingiva. This is the first documented case of pemphigus vulgaris presenting as gingival hypertrophy in a child without any other mucosal or skin lesions.

Comparison of desmoglein 1 and 3 enzyme-linked immunosorbent assay and direct immunofluorescence for evaluation of immunological remission in pemphigus vulgaris

Drug withdrawal is the ultimate goal in the management of patients with pemphigus. Direct immunofluorescence (DIF) has long been considered the gold-standard test to predict immunological remission in pemphigus vulgaris (PV); however, there have been no comparisons between DIF and antidesmoglein (anti-Dsg) ELISA. To compare anti-Dsg ELISA with DIF in patients with PV for evaluation of immunological remission. The study enrolled 46 patients with PV who had absence of any lesion, and had a daily prednisolone dosage of ≤ 10 mg without adjuvant drug treatment in the preceding 6 months. Biopsy specimens were taken from patients and processed for DIF. Intercellular deposition of IgG and/or C3 was considered positive. Serum samples were also collected for anti-Dsg1 and anti-Dsg3 ELISA, and an ELISA index value of > 20.0 was considered positive. DIF and anti-Dsg ELISA were positive for 11 (23.9%) and 18 patients (39.1%), respectively. Anti-Dsg ELISA had a sensitivity of 100%, a specificity of 80%, a positive predictive value of 61.1% and a negative predictive value of 100%. The high sensitivity of anti-Dsg ELISA proves that this simple serological test is a good substitute for DIF for evaluation of immunological remission in PV. As none of the DIF-positive patients was anti-Dsg-negative, it is possible that during the course of immunological remission, results for DIF may become negative before the results for Dsg ELISA do so.

Induction of Skin Lesions by Ultraviolet B Irradiation in a Case of Pemphigus Erythematosus

© 2014 The Authors. doi: 10.2340/00015555-1781 Journal Compilation © 2014 Acta Dermato-Venereologica. ISSN 0001-5555 Pemphigus erythematosus (PE) is a variant of pemphigus foliaceus. PE patients exhibit the immunological features of both pemphigus and lupus erythematosus (LE), i.e. granular immunoglobulin (Ig) and C3 deposition in the basement membrane zone (BMZ), intercellular IgG and C3 deposition in the epidermis and the presence of circulating antinuclear antibodies (ANA) (1, 2). This report describes a case of PE in which skin lesions were induced by UVB irradiation.

Putative paraneoplastic pemphigus and myasthenia gravis in a cat with a lymphocytic thymoma

Paraneoplastic pemphigus (PNP) is a rare and severe autoimmune blistering skin disease of humans. It is associated mainly with haematopoietic neoplasia and a unique autoantibody profile. At this time and in animals, PNP has only been reported in dogs. To report clinical, histological and immunological findings in a cat with suspected thymoma-associated PNP. An 8-year-old female spayed Himalayan cat. The cat presented with a thoracic mass diagnosed as a lymphocytic thymoma. Two weeks postremoval, the cat developed myasthenia gravis, which was controlled with prednisolone and pyridostigmine. The myasthenia gravis resolved, but the cat developed severe cutaneous erosions and ulcers primarily affecting the caudal ventral abdomen and perineum, but also the ventral chest, axillae and medial pinnae. Histopathological analysis revealed changes consistent with pemphigus vulgaris and erythema multiforme, a combination of lesions highly suggestive of PNP. Direct immunofluorescence revealed intercellular epidermal IgG deposition in the lower epidermis, especially in areas of suprabasal clefts. Indirect immunofluorescence revealed antikeratinocyte IgG to basal keratinocytes and bladder epithelial cells. Immunological results supported a diagnosis of PNP. Skin lesions were treated with prednisolone and chlorambucil and, after clinical remission was obtained, the drugs were tapered and eventually discontinued without further recurrence of skin lesions. In this cat, clinical, microscopic and immunological findings were consistent with a diagnosis of PNP, with thymoma being the suspected cause.