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Direct immunofluorescence microscopy (DIF) of the cutaneous lesions revealed weak in vivo IgG deposition on the keratinocyte cell surface from the mid to upper epidermal layers (weakly positive lace like pattern in the epidermis, original magnification x100).
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Introduction: Pemphigus vulgaris (PV) is a chronic and infrequent autoimmune mucocutaneous disease that is characterized by the loose blisters and erosions on the skin and mucous membrane. Middle-aged adults are affect most frequently and the elderly and juvenile cases are infrequent. Herein, we reported a case of pemphigus vulgaris in an elderly p...
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... physical examination, interestingly, the past lesions were completely healed ( Figure 3) and it clearly demonstrated complete remission achieved four years from diagnosis. Accordingly, the skin biopsy was taken from the erythematous back, and the test showed the weak in vivo IgG deposition at the intercellular level in the stratum basale, stratum spinosum, and the basement membrane in the healed area of the skin (Figure 4). The patient remained under the treatment of 10 mg/day and 100 mg/day of prednisolone and azathioprine, respec- tively. ...
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Background: Pemphigus is a group of autoimmune blistering disease of the skin and mucous membranes with an incidence of
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... The peak onset of this disease occurs in the middle-age group of around 40-60 years 4,7 . Pemphigus vulgaris is uncommon among elders, 8,9 but has been documented in some previous studies [10][11][12] . Despite the unknown disease pathogenesis in this age group, it is thought to be related to immune system dysfunction caused by ageing 13,14 . ...
... The disease was also identified in male patients aged 73 to 79 in Syria 10 and Iran. 9 Studies conducted by Suryawati et al 11 Several studies have confirmed the relationship between the ageing process and autoimmune diseases 11 . The innate and adaptive immune systems decline and become dysfunctional with age. ...
... Masjedi et al 9 showed contradictory results that the immune system is suppressed in elders, implying that immunological diseases will improve with ageing. ...
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Pemphigus vulgaris is an autoimmune disease characterized by mucocutaneous involvement in the form of loose bullae on the skin and mucous membranes. This disorder is typically associated with the poor general condition and can be life-threatening. Furthermore, it has a rare incidence among elders. Pemphigus vulgaris was reported in a 73-year-old female patient. The diagnosis was performed clinically according to the history of the disease course and its typical lesions with a predilection for location. The patient's clinical condition improved after receiving systemic corticosteroids and additional supportivetherapy.
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... There are no mucosal lesions, even with widespread disease. Intradermal acantholysis occurs in the granular layer [6]. ...
... Direct immunofluorescence (DIF) demonstrates an intercellular deposition of IgG and C3 in a "chicken-wire" lattice pattern. For diagnosis of any pemphigus disease, sensitivity of DIF has been found to range from 80-95% [6]. The substrate for indirect immune-florescence (IIF) usually Monkey esophagus (for PV) and Normal Human Skin (for PF); if autoantibodies from the patient's serum are present and bound, the fluorescein conjugated IgG anti-serum will fluorescence under microscopy [4], seen as an intracellular staining. ...
Pemphigus is the name of a group of autoimmune pathological entities characterized by the formation of intraepithelial blisters in the skin and/or mucosa. Pemphigus vulgaris (PV) and Pemphigus foliaceus (PF) are considered as classical forms. Rare, non-classical forms of Pemphigus include: Pemphigus herpetiformis, IgA pemphigus, Paraneoplastic pemphigus, IgG/IgA pemphigus. Aim: Aim of our study is to explore the prognostic influence of clinical, immunological and therapeutic drugs on disease course and remission in different pemphigus variants in Kuwait and to establish a safe and effective multidrug protocol. Plan of the study is to make a correlation between epidemiological factors, therapy used and clinical and immunological remission, as well as, follow up of comorbidities in our patients. 84 patients were evaluated, from 1st January 1990 to 31st December 2013. IVIG is used as a third-line adjuvant treatment initially flanked by high-dose systemic corticosteroids and steroid-sparing immune-suppressants. Thus, this multidrug IVIG regimen made it possible to achieve a rapid control of the pemphigus symptoms, with progression to a stable disease remission, while maintenance an overall safety of treatment.
... Pemphigus vulgaris (PV) is a potentially fatal autoimmune vesiculobullous disease [1]. Autoimmunity, breaks in the mucocutaneous barrier, hospitalization, and immunosuppressive therapy increase the surface colonization by microorganisms and infections [2]. ...
Background Pemphigus vulgaris (PV) is a potentially fatal autoimmune vesiculobullous disease. Staphylococcus aureus is the most common cause of cutaneous bacterial infection in pemphigus. Methicillin-resistant S. aureus (MRSA) is a common multidrug-resistant bacterium. MRSA infection is high among dermatology inpatients and vesiculobullous diseases patients are the commonest infected.
Objective This study aimed at determining the causative organisms of the secondary bacterial skin infections in PV patients, identifying MRSA, doing antibiotic sensitivity pattern for MRSA, and determining the risk factors for acquiring MRSA infection among PV patients in the locality.
Patients and methods This cross-sectional study included 54 PV patients. All patients were subjected to history taking, general, and dermatological examinations. Pemphigus disease area index score was calculated. Three swabs for bacterial culture and antibiotic sensitivity testing were obtained (one from vesicle or bulla, one from pustule, and the third from the nose).
Results In all, 51 (94.4%) patients showed positive cultures for S. aureus either as nasal carrier, skin colonization, or skin infection. There was overlap as some patients showed more than one type of positive culture at the same time. Of the patients, 19 out of 51 patients (37.25%) had positive cultures for MRSA. All MRSA strains were sensitive to vancomycin. Also, they had good sensitivity to trimethoprim–sulfamethoxazole, amikacin, ciprofloxacin, and erythromycin, while all MRSA strains were resistant to cefoxitin and ampicillin. Hospitalization, previous antibiotics use in the last 4 weeks prior to this study, length of hospital stay, history of hospital-acquired infections, and community-acquired infections were significantly higher among MRSA-positive patients.
Conclusion There is high MRSA infection/colonization among PV patients. MRSA responds to cheap antibiotics as trimethoprim–sulfamethoxazole, amikacin, and ciprofloxacin.
... Plasmapheresis and the monoclonal anti-CD20 antibody may be used in addition to the systemic drugs to decrease the antibodies titre in the bloodstream. Unfortunately, the use of high-dose systemic corticosteroids is associated with complications such as osteoporosis and corticosteroid-induced hyperglycaemia [5]. ...
Background: Pemphigus is an autoimmune disorder of skin and mucous membranes that appears in the form of blistering on skin and mucous membrane and may lead to loss of life in the absence of proper treatment. Treatment is effected by using immunosuppressant drugs such as Azathioprine cyclophosphamide and some others. Objective: Our purpose of this study was to survey about blood, hepatic and renal side effects of dapsone with the patients affected by Pemphigus. Methods: The test was carried out on 37 patients who were suffering from acute Pemphigus and had undergone some therapy with dapsone amongst which 5 patients were excluded from the research because of hepatic and hematic symptoms. The study was carried on with 31 patients with an average age of 40 ± 17.5. They had no symptoms necessitating the stop of administration of the medicine. Results: Statistical analysis indicated no significant difference in the test results before and after the therapy and only platelet counts showed significant difference before and after the therapy (p<0.05). Conclusion: This study indicated that among all disagreements about dapsone treatment, dapsone can be applied as adjuvant therapy.
... 7 The average age at the onset is between the fourth and sixth decades of life, 7 but can also arise in children and older people. 8,9 PV is a rare condition, with a reported incidence ranging from 0.75-5 cases per million per year to 0.5-3.2 cases per 100,000 per year, depending on the population, with an increased incidence among people of Jewish and Mediterranean descent. ...
OBJECTIVE: This paper reports a case of pemphigus vulgaris (PV) in a young adult male affecting the oral mucosa to highlight the importance of early diagnosis and treatment of this lesion
CASE REPORT: A 33 years old student presented with a six week history of multiple ulcers in the oral cavity. Intra-oral examination revealed multiple irregular shaped ulcers with erythematous floor on the soft palate, buccal mucosa, anterior pillar of fauces, ventral surface of the tongue and floor of the mouth. An initial diagnosis of erythema multiforme was made. Investigations with full blood count, retroviral screening and fasting blood sugar showed normal findings. A definitive diagnosis of pemphigus vulgaris was made following histopathological examination.
The initial treatment administered were Prednisolone tablets 60mg/day for one week, then the dose was stepped down to 40mg daily for a week. Triamcinolone dental paste was applied topically twice daily for 2 weeks. The oral lesion improved drastically and the dose of prednisolone was further stepped down to 20mg daily in 2 weeks. There was an exacerbation of the oral lesions at a dose of 20mg daily of prednisolone, hence the daily dose was adjusted to 60mg daily for 4 weeks at which the oral lesions started improving. The patient has been on maintenance dose of prednisolone of 30 mg/day and Dexamethasone 1 mg dissolved in 10 ml of water as mouth rinse daily for 5 months.
CONCLUSION: This study reported a case of PV involving the oral mucosa found in a young adult male. Early diagnosis and treatment of the oral lesions with local and systemic steroid resulted in remission within 6 months.
Key words: Pemphigus vulgaris, oral lesions, Steroid therapy
Background: This paper reports a case of pemphigus vulgaris in a middle-aged male with oral lesions and skin eruptions Objective: To highlight the complications of prolonged steroid therapy. Case Report A 48-year old male presented with an eight-month history of recurrent mouth ulcers. There was positive history of cutaneous ulcers on the upper extremities, back and genital areas. Patient had been placed on long term steroid therapy prescribed by general practitioners. General examination showed bilateral pitting pedal oedema, moon face and low blood pressure. Intra-oral examination revealed multiple irregular shaped ulcers and erosions with erythematous floor on the buccal mucosa, labial mucosa, floor of the mouth, soft palate and anterior pillar fauces. The dorsum of the tongue had areas of ulcerations with necrotic slough. An impression of pemphigus vulgaris was made based on high index of clinical suspicion and patient was commenced on azathioprine 50mg and prednisolone 40mg daily for a week. Topical use of clobetasol ointment (0.05%) was also commenced to be applied twice daily. There was improvement with the oral lesions on a one-week review. The dose of prednisolone was then stepped down to 20mg daily for one week and 0.2% chlorhexidine mouth rinse twice daily was introduced. Punch biopsy of an intact labial mucosa was done on a subsequent visit and histopathology examination confirmed the diagnosis of pemphigus vulgaris. Patient was referred to the cardiologist on account of the hypotension and bilateral pedal oedema which was suggestive of complications of long-standing steroid use. Patient was placed on maintenance dose of prednisolone 10mg and azathioprine 50mg daily, and topical steroid oral rinse. Conclusion: This study reports a case of pemphigus vulgaris with oral and skin lesions in a middle-aged male. Patient developed some complications due to the prolonged duration of steroid therapy. Regular patient monitoring, adjustment of steroid therapy combined with adjuncts like steroid-sparing drugs are essential to minimizing the steroid-induced adverse effect
Background : Pemphigus Vulgaris (PV) is an autoimmune intraepithelial blistering disease involving the skin and mucous membranes. Oral lesions could be the first sign of the disease followed by the involvement of skin and other mucosa sites.Objectives : This is oral manifestations of pemphigus vulgaris case report, intended to help clinicians to recognize and treat the oral lesions.Case Reported : Male 27 yo admitted to RSUP Dr.Hasan Sadikin hospital with multiple blisters on the skin and oral mucosa, was diagnosed with Pemphigus Vulgaris.Case Management : Patient was treated with steroid mouth wash and oral paste. After two months of treatment, the patient was fully recovered from oral lesion.Discussion : In PV, autoantibodies are produced against desmosomes spesifically desmoglein 3 which responsible for holding the cells of the epithelium together. The loss of adhesive function due to anti Dsg 3 antibodies result in bulla formation on the oral mucosa.The aetiology for PV is still uncertain. Conclusion :Early recognition and treatment of oral lesions is important as it may prevent skin involvement. Early treatment, patient’s compliance and multi disciplinal teamwork ensure the treatment succes for this disease
Pemphigus vulgaris (PV) is a chronic autoimmune blistering disease of the skin, in which loss of adhesion between keratinocytes is caused by autoantibodies. It has been hypothesized that cytokines play an essential role in the pathogenesis of PV. This study aimed to investigate the other immunopathological aspects of PV by determining the serum levels of cytokines in PV patients to find another treatment strategy except corticosteroid therapy.
Twenty-three patients with PV and a control group consisting of 24 healthy subjects were studied. Interleukin (IL)-2, IL-4, IL-6, IL10, IL-12, IL-17 and interferon-gamma (IFN-γ) were measured in the sera of patients by the enzyme-linked immunosorbent assay (ELISA) method.
The serum levels of IL-2, IL-4, IL-17 and IFN-γ in most patients and controls were undetectable. The serum concentrations of IL-10 in the patients and controls were undetectable, nevertheless, the mean serum levels of this cytokine was 64.375 pg/mL in four patients. The mean serum levels of pro-inflammatory cytokine IL-6 increased significantly in the patients, compared to the controls (169.50 vs. 75.62 pg/mL) (P < 0.05). The same was observed for another pro-inflammatory cytokine, IL-12 (135.33 vs. 86.28 pg/mL) (P < 0.05).
Based on the results of this study it can be concluded that the Type 2 T helper cytokine (IL-6) and macrophage-derived cytokine (IL-12) have essential roles in PV pathophysiology. In addition, the potential clinical application of Th1/Th2 type cytokine-based therapy in PV should be considered in next studies.
