Clinical and histological characterization of oral pemphigus lesions in patients with skin diseases: a cross sectional study from Sudan

Abstract

BackgroundPemphigus is a rare group of life-threatening mucocutaneous autoimmune blistering diseases. Frequently, oral lesions precede the cutaneous ones. This study aimed to describe clinical and histological features of oral pemphigus lesions in patients with skin disease has been canceled aged 18 years and above, attending outpatient’s facility of Khartoum Teaching Hospital - Dermatology Clinic, Sudan. In addition, the study aimed to assess the diagnostic significance of routine histolopathology along with immunohistochemical (IHC) examination of formalin-fixed, paraffin-embedded biopsy specimens in patients with oral pemphigus.MethodsA cross-sectional hospital-based study was conducted from October 2008 to January 2009. A total of 588 patients with confirmed skin has been canceled disease diagnosis completed an oral examination and a personal interview. Clinical evaluations supported with histopathology were the methods of diagnosis. IHC was used to confirm the diagnosis. Location, size, and pain of oral lesions were used to measure the oral disease activity.ResultsTwenty-one patients were diagnosed with pemphigus vulgaris (PV), 19 of them (mean age: 43.0; range: 20–72 yrs) presented with oral manifestations. Pemphigus foliaceus was diagnosed in one patient. In PV, female: male ratio was 1.1:1.0. Buccal mucosa was the most commonly affected site. Exclusive oral lesions were detected in 14.2% (3/21). In patients who experienced both skin and oral lesion during their life time, 50.0% (9/18) had oral mucosa as the initial site of involvement, 33.3% (6/18) had skin as the primary site, and simultaneous involvement of both skin and oral mucosa was reported by 5.5% (1/18). Two patients did not provide information regarding the initial site of involvement. Oral lesion activity score was higher in those who reported to live outside Khartoum state, were outdoor workers, had lower education and belonged to Central and Western tribes compared with their counterparts. Histologically, all tissues except one had suprabasal cleft and acantholytic cells. IHC revealed IgG and C3 intercellularly in the epithelium.ConclusionsPV was the predominating subtype of pemphigus in this study. The majority of patients with PV presented with oral lesions. Clinical and histological pictures of oral PV are in good agreement with the literature. IHC confirmed all diagnoses of PV.

Oral mucosal manifestations of autoimmune skin diseases

“…Oral lesions are hallmark of PV and occur in almost all the 391 cases usually at the onset of the disease[10,19,20,[60][61][62][63]. The preva-392 lence of oral involvement as the initial site varies in different population 393 studies, ranging between 37% and 77.5%[60,61,[64][65][66].…”

“…Oral lesions are hallmark of PV and occur in almost all the 391 cases usually at the onset of the disease[10,19,20,[60][61][62][63]. The preva-392 lence of oral involvement as the initial site varies in different population 393 studies, ranging between 37% and 77.5%[60,61,[64][65][66]. Importantly, the 394 high frequency of initial isolated oral mucosal involvement may result 395 in delayed diagnosis despite the fact that patients seek medical help at 396 an early stage due to pain and discomfort associated with mastication, 397 swallowing and speech[67,36].398 Oral lesions of pemphigus vulgaris may present as multiple chronic 399 ulcers involving any part of the oral mucosa, with sites subjected to fric-400 tion trauma.…”

Successful treatment of oral pemphigus vulgaris using intralesional corticosteroids and levamisole immunomodulation: A case report

“…Individual lesions of pemphigus do no heal and Nikolsky's sign will generally be positive. [2,3,[5][6][7][8][9] Some studies have found palate to be the most common followed by buccal mucosa, gingiva, and labial mucosa. Another study found that gingiva to be the most common location.…”

“…Another study found that gingiva to be the most common location. [9] On an average, oral lesions are present for about 5 months before the skin lesions appear [10] and oral lesions will be present for about 5-10 months before diagnosed and treated. [3] Tzank test is considered as a rapid test to diagnose pemphigus as it shows acantholytic cells.…”

“…[3] Tzank test is considered as a rapid test to diagnose pemphigus as it shows acantholytic cells. [9] Furthermore, a biopsy of the lesion with H and E stains will show the presence of a supraepithelial split with inflammatory infiltrate, and some studies state that H and E stain is sufficient to diagnose cases of pemphigus in about 90% of cases. [9] Direct or indirect immunofluorescence has been utilized to diagnose autoimmune mucocuteneous disorders.…”