Pemphigus vulgaris: A rare disease in childhood

Virtuoso, João; Ribeiro, Joana Filipe; Silva, Íris Santos; Santos, Stéfanie Vanessa; Fernandes, Pedro; Cabral, Fatima

doi:10.1111/jpc.15866

Cited by 3 publications

(3 citation statements)

References 3 publications

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“…Prognosis in children seems to be better than in adults, but it may remain reserved due to the potentially harmful side effects of the steroids and immunosuppressive therapy. Deaths from pemphigus vulgaris in pediatric age are usually caused by massive cutaneous involvement (greater than 70%), sepsis, pneumonia and serum electrolyte alterations [2,7].…”

Section: Discussionmentioning

confidence: 99%

“…The majority of patients with CPV have lesions in both mucosa and skin, with the appearance of oral lesions being the first manifestation of the disorder. Mucous membranes of the anus, conjunctiva, and genital areas are frequently involved [2]. We present a case of CPV in a young male that came to our attention for the onset of painful oral erosions just before the COVID-19 outbreak, which hit the city of Bergamo in March 2020.…”

Section: Introductionmentioning

confidence: 99%

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Childhood Pemphigus Vulgaris during COVID-19 Outbreak Successfully Treated with Prednisone and Azathioprine: A Case Report and Literature Review

Vezzoli

Parietti

Carugno

et al. 2022

JCM

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Introduction: pemphigus vulgaris is a rare autoimmune blistering disease that involves the skin and mucous membranes and rarely occurs in pediatric age. Methods: we present a case of childhood pemphigus in a 9-year-old patient from Burkina Faso, which initially manifested with erosive lesions symmetrically distributed in the oral cavity. After a few months, we also observed hyperchromic lesions of the back. Histopathological examination of skin samples showed intraepidermal acantholysis, while direct immunofluorescence showed deposits of complement (C3) and immunoglobulins G (IgG) in the epidermidis; an ELISA test highlighted the presence of circulating autoantibodies against desmoglein 3. Results: the follow-up of this patient was made difficult by the advent of the COVID-19 outbreak. However, after about one year of combined therapy with systemic steroids and azathioprine the patient reached clinical remission.

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Section: Discussionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

Childhood Pemphigus Vulgaris during COVID-19 Outbreak Successfully Treated with Prednisone and Azathioprine: A Case Report and Literature Review

Vezzoli

Parietti

Carugno

et al. 2022

JCM

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“…In European countries, the average age at onset of PV varies from 50 to 60 years ( 9 ). Conversely, PV is extremely rare during childhood ( 10 ).…”

Section: Introductionmentioning

confidence: 99%

Clinical Patterns, Survival, Comorbidities, and Treatment Regimens in 149 Patients With Pemphigus in Tuscany (Italy): A 12-Year Hospital-Based Study

et al. 2022

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IntroductionPemphigus encompasses a group of muco-cutaneous autoimmune bullous diseases characterized by the loss of adhesion between keratinocytes. The disease is associated with increased morbidity and mortality.Materials and MethodsWe characterized clinical patterns, survival, comorbidities, and drug prescriptions in patients with pemphigus referred to the Section of Dermatology of the University of Florence from January 2010 to December 2021.ResultsA total of 149 patients were identified (female/male sex ratio = 2.0). Median age at diagnosis was 57.7 ± 17.2 years; 108 patients were diagnosed with pemphigus vulgaris (PV) (72.5%) and 35 (23.5%) with pemphigus foliaceus (PF). Paraneoplastic pemphigus (PNP) and IgA-pemphigus accounted for three patients each. The overall survival rate was 86.9%. Accordingly, 14 (9%) patients died during the study period. The average age at death was 77.8 ± 9.3. Age at diagnosis was a risk factor for death in patients with pemphigus. Average concentration of Dsg3-IgG and Dsg1-IgG was 85.6 ± 68.8 and 75.9 ± 68.4, respectively. The most serious comorbid diseases included cerebro- and cardiovascular accidents and malignancies. Regarding the treatment regimen, we found a substantially stable use of systemic steroids in the 2010–2018 period; the prevalence of use of mycophenolic acid increased, whereas that of azathioprine decreased. The use of rituximab showed the highest increase in the 2013–2018 period. Proton-pump inhibitors and antibiotics were the most frequently prescribed non-immunomodulating drugs.ConclusionsIn this large series of the patients, patients with pemphigus showed a high incidence of serious comorbid diseases, highlighting the importance of a multidisciplinary approach for a proper management of the patients. Rituximab was the immunomodulating drug showing the highest increase in use over time, reflecting the growing evidence of its efficacy as a first-line treatment in pemphigus.

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Hand, Foot, and Mouth Disease: A Narrative Review

Leung

Lam

Barankin³

et al. 2022

RAIAD

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Background: Hand, foot, and mouth disease is a common viral disease in childhood. Because the disease has the potential to reach epidemic levels and the mortality is high in some countries, early recognition of this disease is of paramount importance. Objective: This purpose of this article is to familiarize pediatricians with the clinical manifestations and management of hand, foot, and mouth disease. Methods: A search was conducted in February 2022 in PubMed Clinical Queries using the key term " hand, foot, and mouth disease". The search strategy included all clinical trials, observational studies, and reviews published within the past 10 years. Only papers published in the English literature were included in this review. Results: Hand, foot, and mouth disease is characterized by a painful oral enanthem and asymptomatic exanthem on the palms and soles. Children younger than 5 years are most commonly affected. Hand, foot, and mouth disease caused by enterovirus A71 is more severe and has a higher rate of complications than that attributed to other viruses such as coxsackievirus A16. Circulatory failure secondary to myocardial impairment and neurogenic pulmonary edema secondary to brainstem damage are the main causes of death. Fortunately, the disease is usually benign and resolves in 7 to10 days without sequelae. Given the self-limited nature of most cases, treatment is mainly symptomatic and supportive. Intravenous immunoglobulin should be considered for the treatment of severe/complicated hand, foot, and mouth disease and has been recommended by several national and international guideline committees. Currently, there are no specific antiviral agents approved for the treatment of the disease. Drugs such as ribavirin, suramin, mulberroside C, aminothiazole analogs, and sertraline have emerged as potential candidates for treatment of hand, foot, and mouth disease. Vaccination of susceptible individuals in high-risk areas and good personal hygiene are important preventative measures to combat the disease. Conclusion: Familiarity of the disease including its atypical manifestations is crucial so that a correct diagnosis can be made, and appropriate treatment initiated. A timely diagnosis can help avoid contact with the affected individual and decrease the risk of an outbreak.

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Pemphigus vulgaris: A rare disease in childhood

Cited by 3 publications

References 3 publications

Childhood Pemphigus Vulgaris during COVID-19 Outbreak Successfully Treated with Prednisone and Azathioprine: A Case Report and Literature Review

Childhood Pemphigus Vulgaris during COVID-19 Outbreak Successfully Treated with Prednisone and Azathioprine: A Case Report and Literature Review

Clinical Patterns, Survival, Comorbidities, and Treatment Regimens in 149 Patients With Pemphigus in Tuscany (Italy): A 12-Year Hospital-Based Study

Hand, Foot, and Mouth Disease: A Narrative Review

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