Pénfigo vulgar / Pemphigus vulgaris

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Thoracoabdominal computed tomography neoplasia detection in patients with paraneoplastic pemphigus: the importance of collaboration between specialists.

INTRODUCTION: Paraneoplastic pemphigus (PNP) is an autoimmune disorder that is almost always linked to an underlying neoplasia. General radiologists are usually not aware of what kind of neoplasia can be associated with PNP. Therefore, this study evaluates the effect of a dermatology lecture on radiologists' neoplasia diagnosis performance. METHODS: Two radiologists evaluated thoracoabdominal computed tomography (CT) examination images of 43 patients with PNP in separate reading sessions blinded to each other's assessments. Six months after the first CT image evaluation session, the two radiologists attended a lecture by two dermatologists about PNP, and 6 months later the two radiologists assessed the same CT examinations again. RESULTS: Statistical analysis showed statistically significant differences in CT sensitivity between the first and the second round of image evaluation for both radiologists (reader 1: p = 0.0313; reader 2: p = 0.0156). CONCLUSIONS: This is the first study to evaluate the effectiveness of a dermatology lecture on diagnostic performance. It is very important for radiologists to be familiar with the particular neoplasms that can be associated with PNP because this can have a direct clinical impact on diagnostic performance.

Ultra-high frequency ultrasound in the differential diagnosis of oral pemphigus and pemphigoid: An explorative study.

OBJECTIVES: Intraoral ultra-high frequency ultrasound (UHFUS) is an emerging technique in oral medicine, due to its possibility to provide submillimeter resolution imaging of superficial mucosal structures. In this study, the potential role of UHFUS in the diagnosis of oral pemphigus vulgaris (PV) and mucous membrane pemphigoid (MMP) is assessed. MATERIALS AND METHODS: Consecutive patients with suspected oral PV or MMP were enrolled. All patients underwent clinical examination, laboratory tests, intraoral UHFUS scan, and biopsy. Histology and direct immunofluorescence were set as benchmark for diagnosis confirmation. The sensitivity and specificity of UHFUS compared to histology were assessed. Mann-Whitney test was performed to evaluate the presence of differences in the echogenicity of PV and MMP. P-value was set at P < 0.05. RESULTS: Twenty-five patients were included. Thirteen patients were diagnosed with PV, and twelve with MMP. The UHFUS features of PV and MMP lesions were described. Image analysis showed statistically significant differences between the echogenicity of PV and MMP lesions (P < 0.05). Good concordance between UHFUS and histology was found. UHFUS showed 75% sensitivity in the diagnosis of PV and 66.7% in the diagnosis of MMP. CONCLUSIONS: UHFUS appears a valuable tool in the diagnosis of PV and MMP. Although histology and immunofluorescence remain the gold standard, UHFUS role in the diagnostic algorithm of PV and MMP seems promising as a chair-side tool consistently enhancing clinical evaluation of oral bullous lesions.

Pemphigus scalp lesions: is trichoscopy helpful in clinical diagnosis?

BACKGROUND: Pemphigus is an autoimmune intraepidermal bullous disease which can affect skin and∕or mucous membranes. Trichoscopy is a non-invasive tool that can help in the clinical diagnosis especially if the patient is presented with only scalp lesions. We conducted this study to investigate the trichoscopic features of pemphigus scalp lesions. METHODS: A total of 100 patients with confirmed diagnosis of pemphigus entered the study; Seventy-nine patients were diagnosed as pemphigus vulgaris (PV) and 21 as pemphigus foliaceus (PF). Trichoscopy of the scalp lesions was performed, and three dermatologists examined separately the trichoscopic images of each patient. The results were re-checked simultaneously by the examiners. Association between variables was analyzed by using different tests including: chi-square test or Fischer's exact test, independent T-Test or Mann-Whitney nonparametric test and logistic regression model when applicable. RESULTS: Mean age of the patients was 45.41 ± 11.13 years, and 58% of cases were male. The most common trichoscopic features observed among all cases were diffuse scaling (71/100), presence of any types of vessels (67/100), and extravasation (63/100). Positive anagen pull test, extravasation, yellow hemorrhagic crust, and polymorphic vessels were more commonly seen in PV than PF cases (P value < 0.05), and by having data of these variables, we can differentiate between PV and PF scalp lesions with 74% sensitivity and 91% specificity. CONCLUSION: Trichoscopy can help in clinical diagnosis of pemphigus scalp lesions but should be followed by other modalities such as biopsy and direct immunofluorescence for definite diagnosis.

Analysis of oral mucosa erosive-ulcerative lesions by reflectance confocal microscopy.

In vivo Reflectance Confocal Microscopy (RCM) allows to optically biopsy vital tissues, non-invasively and in real time. It results in horizontal virtual slices at a microscopic resolution and correlating with conventional histopathology. The aim of the present work is to describe RCM cellular and architectural findings in oral mucosae affected by erosive-ulcerative diseases, thus highlighting in vivo the wellknown histological peculiarities. A series of conventionally diagnosed Recurrent Aphthous stomatitis (RAS) and Pemphigus Vulgaris (PV) erosive and/or ulcerative oral lesions underwent RCM imaging to establish the application of RCM imaging to this kind of inflammatory non-tumoral lesions. A total of 12 RAS-related lesions and 8 PV-related lesions were considered. RCM imaging was capable to visualize their microscopic peculiarities, mainly inflammatory infiltrate, vessel dilation (RAS) and acantholytic cells, intraepithelial clefts and inflammatory cell carpets (PV). Despite RCM may result unnecessary to diagnose oral lesions referred to RAS and PV, its capability to highlight their main microscopic features could be advantageously used to monitor the healing or worsening of the clinical situation as well as the responsiveness/refractoriness to therapy.

Paraneoplastic pemphigus and myasthenia gravis as the first manifestations of a rare case of pancreatic follicular dendritic cell sarcoma: CT findings and review of literature.

BACKGROUND: Follicular dendritic cell sarcoma (FDCS) is a rare neoplasm that originates from follicular dendritic cells in lymphoid tissue while paraneoplastic pemphigus (PNP) is an autoimmune blistering disease associated with neoplasms. Pancreatic FDCS associated with PNP and myasthenia gravis (MG) is even rarer and highly malignant. We present the clinical data, pathological materials and computed tomography (CT) features of a rare case of this disease. CASE PRESENTATION: A 49-year-old woman presented with repeated ptosis of both eyelids, oral ulcers and erosions. Her laboratory results showed a slight elevation of CA125 and positivity of some autoimmune antibodies. CT revealed a round solid mass with central necrosis in the pancreatic tail. The solid component of the mass showed slight enhancement and serpentine feeding arteries in the arterial phase, moderate enhancement with a draining vein around the tumor in the portal venous phase and persistent enhancement in the delayed phase. Surgical resection was performed, and the pathological diagnosis was FDCS. However, the patient died of inability to excrete sputum and occlusion of the respiratory tract. CONCLUSIONS: Pancreatic FDCS manifested as PNP and MG is very rare. Its CT features are not specific, and the disease should be differentiated from neuroendocrine tumors, solid pseudopapillary neoplasms and acinar cell carcinoma.

Reflectance confocal microscopy and optical coherence tomography for the diagnosis of bullous pemphigoid and pemphigus and surrounding subclinical lesions.

BACKGROUND: Diagnosis of bullous pemphigoid (BP) and pemphigus is based on clinical features, histology, immunofluorescence and laboratory data. OBJECTIVES: To evaluate features of BP and pemphigus at reflectance confocal microscopy (RCM) and optical coherence tomography (OCT) in order to provide a rapid non-invasive bed-side diagnosis. Secondary objective was to evaluate the detectability of clinically non-visible lesions. METHODS: This was an observational, retrospective, multicentre study in which patients with suspicious lesions for BP or pemphigus underwent clinical assessment, RCM, OCT, blood tests and skin biopsy for histological and direct immunofluorescence examinations from January 2014 to December 2015. A total of 72 lesions in 24 selected patients were evaluated. Additionally, apparently unaffected skin at two different distances [near (1-2 cm) and far (2-3 cm)] from each lesion was examined to test subclinical lesion detectability. RESULTS: RCM was able to detect subepidermal and intra-epidermal blisters, respectively, in 75% and 50% of the patients affected by BP and pemphigus. At OCT, the exact blister level was identified in all patients. Acantholytic cells were observed only at RCM in pemphigus (62.5%). Fibrin deposition inside the blisters was only found in BP, evidenced both at RCM and OCT. Among patients with BP, subclinical blisters were detected in nine (9.4%) clinically healthy skin, while among patients with pemphigus were observed in 10 (20.8%) apparently unaffected skin. CONCLUSION: RCM and/or OCT provide useful information for a rapid diagnosis of BP and pemphigus and for the identification of biopsy site. Combined use of RCM and OCT is optimal because associates the higher resolution of RCM with the greater penetration depth of OCT. OCT could be an optimal tool for treatment monitoring, especially in the cases of subclinical lesions. However, histopathologic and immunologic examinations remain the gold standard for establishing the final diagnosis.

Paraneoplastic pemphigus as a first sign of metastatic retroperitoneal inflammatory myofibroblastic tumor: (18)F-FDG PET/CT findings.

A 30-year-old female presented with a 3-month history of erosive stomatitis and bullous lesions, along with recurrent episodes of abdominal pain. She was found to have a retroperitoneal lump in left lumbar region. Skin biopsy revealed bullous disorder. CT guided biopsy of the retroperitoneal mass was suggestive of inflammatory myofibroblastic tumor (IMT). She was started on oral steroids and supportive care, and surgery was being planned when she developed respiratory failure. CT chest revealed vertebral metastases. PET/CT for whole body work up revealed a left para-aortic mass along with multiple skeletal metastases. The patient was kept on conservative management. After 3 months, the patient has shown clinical improvement, and an exploratory laparotomy is now being planned for the excision of the tumor, followed by chemotherapy. This case of retroperitoneal IMT is rare in terms of skeletal metastases with paraneoplastic pemphigus.

Mediastinal follicular dendritic cell sarcoma with paraneoplastic pemphigus.

Follicular dendritic cell sarcoma is a rare neoplasm of immune accessory cells. It occurs primarily in lymph nodes. Occurrences in the mediastinum are rarely reported. Diagnosis and management of follicular dendritic cell sarcoma remain unclear, and it is an under-recognized clinical entity. Only a few cases of paraneoplastic pemphigus as the first presentation of follicular dendritic cell sarcoma have been reported. We report an unusual case of follicular dendritic cell sarcoma of the anterior mediastinum, presenting as paraneoplastic pemphigus in a 62-year-old man. Typical histological features confirmed the diagnosis of follicular dendritic cell sarcoma, and surgical resection was successfully performed.

A rare case of pemphigus vegetans mimicking malignancy on F-18 FDG PET/CT.

A 42-year-old man with a history of chronic smoking and alcoholism was suspected to harbor a malignancy involving the perioral region and the scalp. He underwent an F-18 FDG PET/CT scan which revealed abnormal F-18 FDG accumulation in the oral mucosa and lips, extending to the nose, scalp, and bilateral cervical lymph nodes. Further work-up ultimately revealed the lesions to be secondary to pemphigus vegetans. Pemphigus vegetans is a rare variant of pemphigus vulgaris, an autoimmune bullous cutaneous disorder and involves mainly the flexural regions of the body. Unlike pemphigus vulgaris, lesions of pemphigus vegetans present as heaped up, eroded, and ulcerative plaques in the intertriginous regions of the body. Occasional lesions may be present on scalp and elsewhere in the Hallopeau variant of the disease. The use of F-18 FDG PET in the field of oncology is rapidly evolving; however, it is not tumor specific. The integration of CT into PET has increased the specificity of this modality. Nevertheless, there are many physiologic and benign conditions that may result in high accumulation of FDG, and may mimic malignancy. Familiarity with F-18 FDG-avid nonmalignant lesions may extend the use of F-18 FDG PET imaging beyond the field of oncology. To the best of our knowledge, this is the first description of PET/CT findings in pemphigus vegetans.

[Venous thromboembolism in a patient with noninvasive thymoma and a history of pemphigus]. / Zylna choroba zakrzepowo-zatorowa u pacjenta z nieinwazyjnym grasiczakiem i wieloletnia pecherzyca.

A case of a 69-year-old man, admitted due to an acute episode of venous thromboembolism (VTE) is described. He had a 3-year history of 'asymptomatic' (revealed on routine chest X-ray, but undiagnosed) tumor of anterior mediastinum, and a 8-year history of pemphigus vulgaris treated mainly with steroids. Three months following VTE episode he underwent tumor resection and histopathologic evaluation showed noninvasive, type AB thymoma. Gradual pemphigus remission started some weeks later. We analyse the relationship between thymoma, pemphigus and VTE, and emphasize the usefulness of echocardiographic thymoma imaging as a method complementary to computed tomography/magnetic resonance.