BACKGROUND: Paraneoplastic pemphigus (PNP) in the oral cavity is a rare variant of blistering pemphigus disease closely associated with mostly malignant tumors. The diagnosis may even precede an underlying malignancy enabling early detection. Here, we describe a previously unreported case of PNP associated with HPV-related tonsillar squamous cell carcinoma. METHODS AND RESULTS: A 50-year-old woman was referred to a dentist because of painful oral lesions resembling aphthae major and minor. Later, blisters appeared and an incisional biopsy was performed. Histological examination revealed an unusual coexistence of subepithelial and intraepithelial blisters raising suspicion of paraneoplastic pemphigus. The patient underwent 18F-FDG PET/MRI, showing a metabolically active process in the left palatal tonsil. Diagnostic biopsy revealed HPV type 16 associated tonsillar squamous cell carcinoma. A left tonsillectomy with elective left-sided neck dissection was performed. The postoperative period was complicated by bilateral fluidothorax. Two weeks after radical tumor removal, the mucosal and skin lesions of PNP disappeared. The patient currently shows no evidence of recurrence either of malignancy or PNP eight months after the surgery. CONCLUSION: PNP is a rare autoimmune blistering disease characterized by polymorphous cutaneous and mucosal lesions associated with internal neoplasms including HPV associated squamous cell carcinoma of a tonsil. In order to identify an occult malignancy, a whole-body PET/CT or PET/MRI scan is recommended. Rarely, accurate patient management may depend on the dentist being familiar with this entity and on interdisciplinary cooperation involving dermatologist, radiologist, pathologist, and pneumologist. A strict patient follow-up is indicated.
- Klíčová slova
- 18F-FDG PET/MRI, oral mucosa lesions; paraneoplastic autoimmune multiorgan syndrome, paraneoplastic pemphigus, pulmonary complications, tonsillar carcinoma HPV associated,
- MeSH
- autoimunitní nemoci * komplikace MeSH
- infekce papilomavirem * komplikace diagnóza MeSH
- krční mandle patologie MeSH
- lidé středního věku MeSH
- lidé MeSH
- paraneoplastické syndromy * etiologie diagnóza patologie MeSH
- pemfigus * diagnóza etiologie MeSH
- PET/CT škodlivé účinky MeSH
- puchýř komplikace MeSH
- spinocelulární karcinom * komplikace diagnóza MeSH
- Check Tag
- lidé středního věku MeSH
- lidé MeSH
- ženské pohlaví MeSH
- Publikační typ
- kazuistiky MeSH
Background: Isolated laryngeal pemphigus vulgaris (LPV) is rare; however, early diagnosis is crucial in determining its course and prognosis. This paper aims to describe mucosal vascular changes typical for LPV using advanced endoscopic methods, which include Narrow Band Imaging (NBI), IMAGE1-S video-endoscopy and enhanced contact endoscopy (ECE). Materials and Methods: Retrospective analysis of all laryngeal mucosal lesion examined using advanced endoscopic methods during 2018-2020 at tertiary hospital was performed. Results: Videolaryngoscopy examination records of 278 patients with laryngeal mucosal lesions were analyzed; three of them were diagnosed with LPV. Epithelial vascularization of LPV included specific pattern. Intraepithelial papillary capillary loops were symmetrically stratified and were organized into "contour-like lines". This specific vascularization associated with LPV were different from other laryngeal mucosal pathologies. Conclusions: Using advanced endoscopic methods supports early diagnosis of LPV and accelerate the diagnosis and treatment.
- Klíčová slova
- IMAGE1-S, Narrow Band Imaging (NBI), enhanced contact endoscopy (ECE), larynx, pemphigus vulgaris,
- MeSH
- endoskopie MeSH
- lidé MeSH
- nádory hrtanu * MeSH
- pemfigus * diagnóza MeSH
- retrospektivní studie MeSH
- úzkopásmové zobrazení MeSH
- Check Tag
- lidé MeSH
- Publikační typ
- časopisecké články MeSH
BACKGROUND: The current standard in the serologic diagnosis of autoimmune bullous diseases (AIBD) is a multistep procedure sequentially applying different assays. In contrast, the BIOCHIP Mosaic technology combines multiple substrates for parallel analysis by indirect immunofluorescence. METHODS: Sera from 749 consecutive, prospectively recruited patients with direct immunofluorescence-positive AIBD from 13 international study centers were analyzed independently and blinded by using (1) a BIOCHIP Mosaic including primate esophagus, salt-split skin, rat bladder, monkey liver, monkey liver with serosa, recombinant BP180 NC16A, and gliadin GAF3X, as well as HEK293 cells expressing recombinant desmoglein 1, desmoglein 3, type VII collagen, and BP230 C-terminus and (2) the conventional multistep approach of the Department of Dermatology, University of Lübeck. RESULTS: In 731 of 749 sera (97.6%), specific autoantibodies could be detected with the BIOCHIP Mosaic, similar to the conventional procedure (725 cases, 96.8%). The Cohen κ for both serologic approaches ranged from 0.84 to 1.00. In 6.5% of sera, differences between the 2 approaches occurred and were mainly attributed to autoantigen fragments not present on the BIOCHIP Mosaic. LIMITATIONS: Laminin 332 and laminin γ1 are not represented on the BIOCHIP Mosaic. CONCLUSIONS: The BIOCHIP Mosaic is a standardized time- and serum-saving approach that further facilitates the serologic diagnosis of AIBD.
- Klíčová slova
- autoimmune bullous diseases, biochip, immunofluorescence, pemphigoid, pemphigus,
- MeSH
- autoimunitní nemoci krev diagnóza MeSH
- bulózní pemfigoid krev diagnóza imunologie MeSH
- dítě MeSH
- dospělí MeSH
- fluorescenční protilátková technika nepřímá metody MeSH
- lidé středního věku MeSH
- lidé MeSH
- mladiství MeSH
- mladý dospělý MeSH
- pemfigus krev diagnóza imunologie MeSH
- prospektivní studie MeSH
- senioři nad 80 let MeSH
- senioři MeSH
- Check Tag
- dítě MeSH
- dospělí MeSH
- lidé středního věku MeSH
- lidé MeSH
- mladiství MeSH
- mladý dospělý MeSH
- mužské pohlaví MeSH
- senioři nad 80 let MeSH
- senioři MeSH
- ženské pohlaví MeSH
- Publikační typ
- časopisecké články MeSH
- multicentrická studie MeSH
BACKGROUND: Several European countries recently developed international diagnostic and management guidelines for pemphigus, which have been instrumental in the standardization of pemphigus management. OBJECTIVE: We now present results from a subsequent Delphi consensus to broaden the generalizability of the recommendations. METHODS: A preliminary survey, based on the European Dermatology Forum and the European Academy of Dermatology and Venereology guidelines, was sent to a panel of international experts to determine the level of consensus. The results were discussed at the International Bullous Diseases Consensus Group in March 2016 during the annual American Academy of Dermatology conference. Following the meeting, a second survey was sent to more experts to achieve greater international consensus. RESULTS: The 39 experts participated in the first round of the Delphi survey, and 54 experts from 21 countries completed the second round. The number of statements in the survey was reduced from 175 topics in Delphi I to 24 topics in Delphi II on the basis of Delphi results and meeting discussion. LIMITATIONS: Each recommendation represents the majority opinion and therefore may not reflect all possible treatment options available. CONCLUSIONS: We present here the recommendations resulting from this Delphi process. This international consensus includes intravenous CD20 inhibitors as a first-line therapy option for moderate-to-severe pemphigus.
- Klíčová slova
- CD20 inhibitor, consensus, guidelines, pemphigus foliaceus, pemphigus vulgaris, treatment,
- MeSH
- akademie a ústavy normy MeSH
- antigeny CD20 imunologie MeSH
- delfská metoda MeSH
- dermatologie metody normy MeSH
- glukokortikoidy aplikace a dávkování MeSH
- imunologické faktory aplikace a dávkování MeSH
- intravenózní podání MeSH
- kombinovaná farmakoterapie metody normy MeSH
- kombinovaná terapie metody normy MeSH
- konsensus MeSH
- lidé MeSH
- pemfigus diagnóza imunologie terapie MeSH
- plazmaferéza * MeSH
- rituximab aplikace a dávkování MeSH
- směrnice pro lékařskou praxi jako téma * MeSH
- stupeň závažnosti nemoci MeSH
- Check Tag
- lidé MeSH
- Publikační typ
- časopisecké články MeSH
- Geografické názvy
- Evropa MeSH
- Názvy látek
- antigeny CD20 MeSH
- glukokortikoidy MeSH
- imunologické faktory MeSH
- rituximab MeSH
Severe forms of pemphigus vulgaris (PV) that are resistant to standard treatment present a life-threatening disease with a mortality of 5-10%. The treatment is usually individualized. The most popular procedures used today include intravenous applications of immunoglobulins and rituximab. Currently the common use of pulse corticosteroids, often in first-line treatment, is being neglected. This particular case documented the severity of the disease and also the need for combined and comprehensive care, in which corticosteroid pulse therapy still plays an important role.
- Klíčová slova
- own experience, resistant form of pemphigus vulgaris, therapeutic possibilities,
- MeSH
- antibakteriální látky terapeutické užití MeSH
- antifungální látky terapeutické užití MeSH
- azathioprin škodlivé účinky MeSH
- biopsie MeSH
- časové faktory MeSH
- hormony kůry nadledvin aplikace a dávkování MeSH
- imunosupresiva škodlivé účinky MeSH
- intravenózní imunoglobuliny terapeutické užití MeSH
- kombinovaná farmakoterapie MeSH
- kůže účinky léků patologie MeSH
- leukopenie chemicky indukované diagnóza terapie MeSH
- lidé středního věku MeSH
- lidé MeSH
- pemfigus diagnóza farmakoterapie MeSH
- pulzní dávkování léků MeSH
- recidiva MeSH
- sepse chemicky indukované diagnóza terapie MeSH
- výsledek terapie MeSH
- Check Tag
- lidé středního věku MeSH
- lidé MeSH
- ženské pohlaví MeSH
- Publikační typ
- časopisecké články MeSH
- kazuistiky MeSH
- Názvy látek
- antibakteriální látky MeSH
- antifungální látky MeSH
- azathioprin MeSH
- hormony kůry nadledvin MeSH
- imunosupresiva MeSH
- intravenózní imunoglobuliny MeSH
The case report is describing patient with an autoimmune disease, Pemphigus Vulgaris. This patient arrived to the general practitioner with Pemphigus Vulgaris after 20 days from the first manifestation of the disease. Despite intensive care, patient dies forty-seventh day after first clinical manifestation of the illness of candidemia. Candidiasis was diagnosed only in postmortem examination.
- MeSH
- fatální výsledek MeSH
- fungemie etiologie patologie MeSH
- kandidóza etiologie patologie MeSH
- lidé MeSH
- pemfigus diagnóza farmakoterapie mikrobiologie patologie MeSH
- Check Tag
- lidé MeSH
- ženské pohlaví MeSH
- Publikační typ
- časopisecké články MeSH
- kazuistiky MeSH
- MeSH
- chronická lymfatická leukemie komplikace patologie MeSH
- kůže patologie MeSH
- lidé středního věku MeSH
- lidé MeSH
- paraneoplastické syndromy diagnóza patologie MeSH
- pemfigus komplikace diagnóza patologie MeSH
- Check Tag
- lidé středního věku MeSH
- lidé MeSH
- ženské pohlaví MeSH
- Publikační typ
- dopisy MeSH
- kazuistiky MeSH
- MeSH
- bulózní pemfigoid diagnóza terapie MeSH
- dermatitis herpetiformis diagnóza terapie MeSH
- dospělí MeSH
- lidé středního věku MeSH
- lidé MeSH
- pemfigus diagnóza terapie MeSH
- vezikulobulózní nemoci kůže * diagnóza terapie MeSH
- Check Tag
- dospělí MeSH
- lidé středního věku MeSH
- lidé MeSH
- mužské pohlaví MeSH
- ženské pohlaví MeSH
- Publikační typ
- anglický abstrakt MeSH
- časopisecké články MeSH
- MeSH
- alkalická fosfatasa krev MeSH
- dermatitis herpetiformis diagnóza MeSH
- dospělí MeSH
- klinické enzymatické testy MeSH
- leukocyty enzymologie MeSH
- lidé středního věku MeSH
- lidé MeSH
- pemfigus diagnóza MeSH
- Check Tag
- dospělí MeSH
- lidé středního věku MeSH
- lidé MeSH
- mužské pohlaví MeSH
- ženské pohlaví MeSH
- Publikační typ
- anglický abstrakt MeSH
- časopisecké články MeSH
- Názvy látek
- alkalická fosfatasa MeSH
