Pemphigus vulgaris: a clinical study of 44 cases over a 20‐year period

Svecova, D

doi:10.1111/ijd.12644

Cited by 31 publications

(43 citation statements)

References 19 publications

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“…For pemphigus, relapses were observed in all our patients with a mean delay of 8.75 months and these relapses could be late (up to 34 months). Complete remission was long to obtain, as for adults . In our series, only 7 patients were in complete remission, within a mean delay of 49.1 months (17–96 months).…”

Section: Discussionmentioning

confidence: 48%

Long‐term evolving profile of childhood autoimmune blistering diseases: Retrospective study on 38 children

Welfringer‐Morin

Bekel

Bellon

et al. 2019

Acad Dermatol Venereol

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Background Autoimmune bullous dermatoses (AIBDs) in children are uncommon, and their long‐term evolution remains unknown. Objective The aim of this retrospective study was to characterize the long‐term prognosis of AIBDs that started during childhood. Methods We conducted a monocentric retrospective study, in the French dermatology centre, by including all children affected by AIBDs. The long‐term outcome was obtained through a phone call questionnaire. Results Sixty‐three patients were included from January 1993 to December 2015, 34 female and 29 males: 27 Linear immunoglobulin A disease (LAD), 12 bullous pemphigoid (BP), 12 pemphigus, 8 herpetiform dermatitis (DH) and 4 epidermolysis bullosa aquisita (EBA). The mean age was 4.7 years old. Twenty‐five patients were lost during the follow‐up. For the 38 remaining patients, the mean follow‐up duration for all pathologies was 6.6 years. Twenty‐nine of them had at least one relapse. Late relapses were observed in two cases of DH and six cases of pemphigus (7–34 months). The mean treatment duration was 30.6 months with variability according to the AIBDs. Topical corticosteroids were used alone, effectively, for seven patients and in association with other treatment in 19 patients in complete remission. Complete remission was noted in 34/38 children with a follow‐up of 4.4 years (0.08–19.5). The mean duration to complete remission was 30.5 months (6–114 months). Late nasal synechiae were reported in one EBA only. There was no significant associated comorbidity, but an association with a primary immune deficiency (PID) was observed in two cases. Conclusion Childhood AIBDs appear to be of good overall prognosis but a long‐term follow‐up is mandatory, as relapses can be late, except for BP. The use of topical corticosteroids is frequently effective alone or in association. The association with PID leads to think about the possibility of a possible underlying dysimmunity in the child.

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Section: Discussionmentioning

confidence: 48%

Long‐term evolving profile of childhood autoimmune blistering diseases: Retrospective study on 38 children

Welfringer‐Morin

Bekel

Bellon

et al. 2019

Acad Dermatol Venereol

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“…Additionally, in the same study, anti-Dsg3 IgG4 autoantibody levels were significantly higher in patients who carried one or both of the disease-associated HLA alleles (DRB1*04:02 and DQB1*05:03) than in carriers of other HLA alleles. 16 Other authors have documented poor prognoses in PV patients aged ≤40 years with initial mucosal involvement. Analysis of the DRB1* and DQB1* alleles with additional clinical parameters highlights the association of DRB1*04:02 alleles with severe PV (P = 0.031) and with the mucocutaneous type of PV (P = 0.024); further, the frequency of the alleles is greater than in carriers of other HLA alleles.…”

Section: Discussionmentioning

confidence: 97%

“…6 Our previous study confirmed high allele frequencies of DRB1 and DQB1 in PV patients in Slovakia. 16 Furthermore, the presence of the DQB1*03:02 allele has been shown to be increased significantly in PV patients. 7 Because the course of PV is frequently unpredictable, the identification of parameters that may predict the course and prognosis of the disease are desirable.…”

Section: Discussionmentioning

confidence: 99%

HLA DRB1* and DQB1* alleles are associated with disease severity in patients with pemphigus vulgaris

et al. 2014

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“…Pemphigus vulgaris (PV) is a chronic, autoimmune intraepithelial blistering disease that affects any mucosal or cutaneous surface lined by squamous epithelium . Its incidence is 0.1 to 0.5 per 100,000 patients per year, and it commonly affects 40‐ to 60‐year‐old women of Jewish or Mediterranean descent, despite being described in all ages and every ethnic group …”

Section: Introductionmentioning

confidence: 99%

“…Before the introduction of oral corticosteroids in the 1950s, PV had a 90% mortality rate . Even today, a 6% to 10% mortality rate is typically reported for PV patients . A better prognosis is usually associated with early diagnosis and treatment.…”

Section: Introductionmentioning

confidence: 99%

Metoprolol‐Induced Pemphigus‐Like Reaction

Patel

Kim²,

Allen

2018

Clin Adv Periodontics

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Introduction Pemphigus vulgaris (PV) is a relatively rare, potentially life‐threatening autoimmune disease that, in most cases, has an unknown etiology. Medications for hypertension have been linked to the onset and exacerbation of PV‐like symptoms. The diagnosis of medication‐related PV can be challenging because it has an identical appearance to the clinical and histologic appearance of idiopathic PV and cases may not resolve after discontinuation of the drug. Case Presentation We present a case of an elderly patient with gingival and cutaneous erosions, who underwent several medical and dental consultations without an appropriate diagnosis. After biopsy and a thorough review of her medical history, metoprolol was suspected as the offending agent. After consulting with her cardiologist, metoprolol was discontinued, and a complete resolution of all lesions resulted. Conclusions To our knowledge, the current case is the first reported case of metoprolol‐induced PV in the English‐language literature. As such, it highlights the potential of medication involvement in some immune‐mediated diseases. Because the oral mucosa is often the first site of involvement in PV, knowledge of drug‐related PV is crucial in the diagnosis, treatment, and management of dental patients.

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Pemphigus vulgaris: a clinical study of 44 cases over a 20‐year period

Cited by 31 publications

References 19 publications

Long‐term evolving profile of childhood autoimmune blistering diseases: Retrospective study on 38 children

Long‐term evolving profile of childhood autoimmune blistering diseases: Retrospective study on 38 children

HLA DRB1* and DQB1* alleles are associated with disease severity in patients with pemphigus vulgaris

Metoprolol‐Induced Pemphigus‐Like Reaction

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