Clinical presentation and diagnostic delay in pemphigus vulgaris: A prospective study from Turkey

Abstract

BackgroundTo evaluate the diagnostic patterns and factors that may influence delays in the diagnosis of pemphigus vulgaris (PV) with oral involvement.Materials and MethodsIn this prospective cohort study, 36 newly diagnosed PV patients with oral involvement were clinically examined and interviewed about the natural history of the oral lesions, number of medical consultations (Med consultation), medical treatment history, and diagnostic delay time (DD time).ResultsThirty (83%) PV patients presented initially with oral mucosal involvement (OMI) and 6 (17%) presented initially with skin involvement (SI). The mean DD time was 6.19 ± 3.82 months, and the mean number of Med consultation was 5.8 (n = 36). The means of all the parameters were significantly higher for the OMI patients than for the SI patients (P < .05). All of the patients with OMI had been misdiagnosed. The DD time was significantly longer in patients who presented initially with desquamative gingivitis (8.25 ± 3.81) than patients who presented with ulcers and erosions (4.78 ± 1.11) (P < .05). There was a statistically significant positive correlation between DD time and Med consultation (r = 0.91).ConclusionEven with the high frequency of oral involvement and easy access to the oral cavity, diagnostic delays are still common for patients with oral PV. This underlines the need for education to improve healthcare providers' awareness and knowledge of the clinical oral presentation of PV.

Prevalence of oral mucosal lesions in patients with pemphigus vulgaris: A systematic review and meta‐analysis

“…The studies were conducted in 20 countries: Argentina, 7 Brazil, 15,16 Croatia, 17 Egypt, 18 Greece, 19 India, 6,20–23 Iran, 24–29 Italy, 2,3,30 Japan, 31 Korea, 32 Kuwait, 33 Macedonia, 34 Mexico, 35 Morocco, 8,36 Romania, 37 Serbia and Montenegro, 38 Slovakia, 39 Spain, 40,41 Thailand, 42 and Turkey 1,5,9,43–45 . Years of publication ranged from 1999 33 to 2020 3,4,6–9,24 . The most common study design was observational study as retrospective reviews ( n = 20, 50%), followed by retrospective studies with follow‐up ( n = 18, 45%), and two prospective studies (5%).…”

“…The overall prevalence of oral mucosal involvement in PV patients varied substantially, ranging from 55.5% 40 to 100% 2–4,6,15,16,18,19,21,23,31,34,35 Table provides details on studies characteristics and the prevalence of oral mucosal lesions of each study.…”

“…Pemphigus Vulgaris (PV) is the most common variant of pemphigus, a systemic bullous autoimmune disease 1–3 . PV is a rare condition, with an incidence of 0.76 to 16 cases per million per year worldwide 4 . It affects both sexes; however, a slightly higher prevalence on women has been reported, 5 and the disease onset commonly occurs at the 4th and 6th decades of life 1,3 .…”

“…Due to constant mechanical trauma in the mouth, PV blisters are rarely found on the oral mucosa; instead, painful erosions and ulcerations are common manifestations, causing local discomfort, in addition to dysphagia and quick weight loss 1,7 . The definitive diagnosis of PV is achieved by analysis of a perilesional tissue biopsy under histopathological examination, direct immunofluorescence (DIF), or indirect immunofluorescence (IIF) 4,8,9 . The criterion for histopathological diagnosis of PV is the presence of acantholysis with an inflammatory infiltration and Tzank cells, while the DIF reveals intercellular accumulation of IgG against Dsg1 and/or Dsg3 1,4,9,10 .…”

“…The definitive diagnosis of PV is achieved by analysis of a perilesional tissue biopsy under histopathological examination, direct immunofluorescence (DIF), or indirect immunofluorescence (IIF) 4,8,9 . The criterion for histopathological diagnosis of PV is the presence of acantholysis with an inflammatory infiltration and Tzank cells, while the DIF reveals intercellular accumulation of IgG against Dsg1 and/or Dsg3 1,4,9,10 . However, this pattern of immunoreactants is not specific for pemphigus vulgaris and might also be observed in other types of pemphigus, so the association of clinical and histopathological data is essential to PV diagnosis 1,2,4 …”