Case Report: Variety of Target Antigens During 1 Year Follow-Up of a Patient Initially Diagnosed With Bullous Pemphigoid

Autoimmune bullous diseases (AIBDs), presenting cutaneous and/or mucosal bullous lesions, are classified into pemphigus and pemphigoid diseases. A longtime observation for complicated AIBD cases is rarely reported. In this study, serum samples of one AIBD patient were collected at seven different time points during the disease course including a relapse, which were examined by our conventional and newly developed methods for the detection of autoantibodies. Interestingly, we found changes of both the presence and the titers of various autoantibodies in accordance with the changes of clinical features during the whole disease course, which indicated that the patient started as bullous pemphigoid and relapsed as concurrence of bullous pemphigoid and mucosal-dominant-type pemphigus vulgaris.

An Unusual Case of Pemphigus Vulgaris

<b><i>Introduction:</i></b> Pemphigus is a potential life-threatening skin disorder belonging to the group of the autoimmune bullous diseases affecting the skin and mucosa. The most common subtypes are pemphigus foliaceus (PF) and pemphigus vulgaris. <b><i>Case Presentation:</i></b> We present the case of a young woman with scalp manifestations diagnosed as seborrhiasis who came to our office where a more careful history and clinical examination directed us toward another diagnostic suspicion. The histological examination confirmed our suspicion of pemphigus and therefore we believe it is important to report our experience to avoid misdiagnosis. <b><i>Discussion/Conclusion:</i></b> Our case may be useful in the literature to identify cases of PF with atypical manifestations that may mimic other diseases.

A relapse of pemphigus vulgaris in pemphigus herpetiformis or a phenotypic “switch”

Sir, Pemphigus herpetiformis (PH) was originally described by Jablonska et al. in 1975. Clinically, PH presents itself as a herpetiform dermatitis with immunopathological characteristics of pemphigus [1,2]. We report an exceptional case of typical pemphigus vulgaris (PV) relapsing after 36 years in PH. A 65-year-old patient, followed for PV for 36 years and treated with corticosteroid therapy with a remission for more than thirty years, consulted for pruriginous lesions evolving for the previous eight months. A dermatological examination revealed urticariform pruriginous ring lesions surmounted by small peripheral vesicles spread throughout the body (Fig. 1), sparing the mucous membranes, and without Nikolsky’s sign. After two non-specific skin biopsies, the histological examination revealed an intraepidermal bubble with acantholytic cells and eosinophilic spongiosis (Figs. 2a and 2b). Direct immunofluorescence confirmed the diagnosis of pemphigus and indirect immunofluorescence was at the upper limit. The diagnosis of a PV relapse in PH was retained and a dapsone-based treatment was initiated at a dose of 150 mg/day and stopped seven days later when met with hemolytic anemia. Oral corticosteroid therapy involving prednisone at a dose of 1 mg/kg/day was initiated but, given the persistence of the pruritus, the decision was to combine methotrexate at a dose of 12.5 mg/week. A good evolution and a decline within eight months were observed. An improved pruritus and the disappearance of the skin lesions were achieved after one month of treatment. PV and PH are two different anatomical and clinical entities of the autoimmune disease pemphigus, with distinct clinical, histopathological, and immunopathological characteristics [1,2]. Our observation documents a complete phenotypic “switch” of pemphigus with a transition from PV to PH both clinically, histologically, and immunologically. Several rare cases of PV switching to superficial pemphigus (SP) (“phenotypic switch”) have, since 1991, been reported, with a higher frequency this direction than otherwise; the transition period varies from six months to twenty years [3]. To the best of our knowledge, no case has been described of a progression from PV to PH. Having observed one firsthand, we are first to describe the case of a complete phenotypic switch from PV to PH. The mechanism of such a transition remains poorly understood and is often observed during a relapse. Some authors suggest that the effect of immunosuppressants on the desmoglein DSG3 more marked than on DSG1 could explain the relapse of PS in PH [3,4]. Future studies on the immunological factors and predictors of PV relapses after the discontinuation of treatment would be useful to better understand the mechanisms of a relapse in pemphigus, with or without a phenotypic transition.

Peculiarities of Quality of Life in Patients with Pemphigus Vulgaris according to the Results of the Analysis of the SF-36V2 Questionnaires and Dermatological Index of Quality of Life in the Background of Glucocorticosteroid Therapy

The purpose of the study was to carry out a comparative assessment of the parameters of the quality of life in patients with pemphigus vulgaris according to the results of the analysis of the SF-36V2 questionnaires and the dermatological index of the quality of life against the background of glucocorticosteroid therapy. Materials and methods. The results of the study are based on the data of a comprehensive study and further follow-up of 47 patients with pemphigus vulgaris (of which 30 are the main group and 17 are the comparison group) who were hospitalized at the Zaporizhzhya Regional Skin and Venereal Clinical Dispensary, Zaporizhzhia. At the time of observation, the majority of patients were aged 61-70 years old. As a control group, 20 apparently healthy people were examined, randomized by age and sex. Results and discussion. Pemphigus vulgaris is a severe systemic dermatosis, which, according to the literature, accounts for about 1% in the structure of dermatological pathology, the main pathomorphological sign of which is acantholysis. Although the share of pemphigus vulgaris in the structure of dermatological pathology is 0.6 per 100 thousand people per year, the severity of the disease and its outcome determine the relevance of further study of the pathogenesis of dermatosis and the search for new effective therapies. The etiology of true pemphigus still remains unclear. The obtained results of questioning the patients with the SF-36V2 questionnaires and the dermatological index of quality of life indicate a significant negative impact of pemphigus vulgaris on the key parameters of the quality of life of patients in the main group and in the comparison group before treatment – the ability to perform their daily duties, work or study, have personal relationships, and also on the desire to be treated and adhere to all the rules of the therapy. Patients with pemphigus vulgaris in the group of monotherapy with glucocorticosteroids had statistically worse indicators of the quality of life after treatment than patients with additional use of Armadin, which was characterized by a low level of physical activity, an increase in the role of physical and psychoemotional problems in everyday life, general discomfort and subjective health. Conclusion. Evaluation of the quality of life of patients allowed for a more qualified study of the health disorders of patients with pemphigus vulgaris, to see the essence of the clinical problem, to select the most optimal treatment regimen for each patient, and also to assess the expected results in terms of parameters that are on the verge of a scientific approach and evidence-based medicine on the one hand, and also the subjective point of view of patients with pemphigus vulgaris on the other hand

Case Report: Management and Outcomes of COVID-19 in a Patient with Cirrhosis of Liver

Introduction: The outbreak of coronavirus disease 2019 (COVID-19) has recently become a major problem affecting thousands of individuals around the world. It is understood that a significant proportion of patients infected with COVID-19 have disturbed liver function tests. This is a case report of a patient with liver cirrhosis and COVID-19. Presenting Complaints and Investigations: A 39 year old female patient was admitted in AVBRH on 06/02/2021 with chief complaint of distension of abdomen, breathlessness, shortness of breath, reduced urine output over last 3 months. She had skin lesions over the hand, foot, abdomen and back with itching since 4 months. Physical examination, blood investigations and abdominal ultrasound showed cirrhosis of liver with gross ascites seen clinically. She had mild splenomegaly and gall bladder was enlarged. Laboratory tests showed elevated total bilirubin level. In peripheral blood examination, RBC count was low (3.66m/cu mm), Haemoglobin level was 8.2 gm/dl, Platelets count was low (1.19 lakh per cu. mm). WBC count was 3600 cu mm. Doctor diagnosed this as the case of cirrhosis of liver with pemphigus vulgaris with COVID-19. Past History: 6 months ago, patient was admitted in Aarogyadham hospital Yawatmal with chief complaint of abdominal pain, loss of appetites, fever. On ultrasonography, she had splenomegaly for which she took the treatment. The Main Diagnosis, Therapeutic Intervention and Outcomes: This case was diagnosed as a case of COVID-19 with Cirrhosis of liver. Interferon alpha 2b solution was given for 10 days to help improve the immunity. Tab. Ursoldiolis (ursodeoxycholic acid) was used to dissolve gallstones. Conclusion: Good clinical assessment, appropriate care, good nursing care by trained nurses and appropriate treatment can save lives even in complicated COVID-19 infected cases.