scholarly journals Atypical Clinical and Serological Manifestation of Pemphigus Vegetans: A Case Report and Review of the Literature

2017 ◽  
Vol 9 (1) ◽  
pp. 121-130 ◽  
Author(s):  
Rebecca Mergler ◽  
Andreas Kerstan ◽  
Enno Schmidt ◽  
Matthias Goebeler ◽  
Sandrine Benoit

Pemphigus vegetans (PVeg) is a rare variant of pemphigus vulgaris characterized by pustules and/or papillomatous vegetations, preferentially affecting intertriginous and periorificial areas. Exceptional manifestations may be misdiagnosed resulting in delayed diagnosis and treatment. Diagnosis is confirmed by immunofluorescence and detection of anti-desmoglein (Dsg) 3 and/or anti-Dsg1 antibodies. We herein report an unusual manifestation of PVeg. At the time of first presentation, lesions were restricted to the right ring finger’s tip. Although mucous membranes were initially not affected, high levels of anti-Dsg3 antibodies were detected while anti-Dsg1 and anti-desmocollin (Dsc) 1, 2, and 3 antibodies were absent. To compare our immunological findings with previous reports, all accessible Anglophone literature published since December 1988 was evaluated. We identified 52 patients suffering from PVeg, 7 of these showed anti-Dsg3 antibodies without any mucous membrane involvement. Notably, the detection of anti-Dsg1 and anti-Dsg3 antibodies does not necessarily correlate with the involvement of skin and/or mucous membranes. This might be due to more specific and complex antibody constellations in nonclassical or atypical pemphigus.

2021 ◽  
Vol 12 (4) ◽  
Author(s):  
N. B. Kolych ◽  
◽  
N. V. Hudz ◽  

A pathological autopsy was performed on 6 corpses of piglets in the first week of life who died from mycoplasmosis. Examination of the visible mucous membranes revealed hyperemia of the mucous membrane of the nasal cavity and thymus. Simultaneous lesions of the pharyngeal, parotid, cervical, mandibular lymph nodes were noted. They were slightly enlarged, from dark pink to dark red. The heart is irregularly shaped due to the expansion of the right ventricle or the diffuse expansion of all departments. Lungs have doughy consistency, uneven color. In some cases, there are diffuse red areas covering the entire lobe of the lungs, in other cases, there is a defeat of small areas. The liver has a smooth surface, soft or pasty consistency, the parenchyma pattern is slightly smoothed in section. The color of the liver is different: dark red areas, without clear boundaries turn into creamy-clay. Flatulence was a characteristic feature of the stomach and intestines. Catarrhal enteritis was registered in animals, which manifested itself in the form of moderate hyperemia of the intestinal mucosa and serous membranes. Microscopically, there is a significant blood supply to the vessels in the lungs. Alveoli are half fall down, in the form of slit-like lumens. In areas of tissue infiltration by inflammatory infiltrate, the alveolar wall is thickened, alveocytes are in a state of turbid swelling and vacuolar dystrophy, they are impregnated with erythrocytes. Peribronchial pneumonia of lymphocytic character is observed. The liver is in a state of acute venous hyperemia. The central and intraparticle capillaries are sharply dilated and filled with blood in some lobes, and the hepatic beams are compressed accordingly. In the center of other lobes, diffuse infiltration of liver tissue by erythrocytes as a consequence of diapedesis is noted. Hepatocytes are in a state of granular dystrophy. Destructive changes are strongly expressed in the mucous membrane of the small intestine: desquamation of the epithelium, necrosis of epitheliocytes and villi, destruction of crypts. In the brain tissue, there is dilation of the lumens of large and small blood vessels, extracellular and perivascular edema, areas of reactive necrosis.


2014 ◽  
Vol 8 (4) ◽  
pp. 1625-1627
Author(s):  
XUE-LI YANG ◽  
SAN-JUN LU ◽  
JIE XUE ◽  
YAN-FEN WU ◽  
JUN-LING SHI

2021 ◽  
Vol 5 (Supplement_1) ◽  
Author(s):  
Umair Arain ◽  
Abimbola Phillips ◽  
Ben Burton ◽  
Damodar Makkuni

Abstract Case report - Introduction Relapsing polychondritis (RP) was first recognized as a clinical entity in 1923 by Jaksch-Wartenhorst (1923) and reported by him under the title "polychondropathia". The term "relapsing polychondritis" was first used by Pearson, Kline, and Newcomer (1960). Because the ocular findings can be the initial findings of RP, ophthalmologists should know the major ocular findings of this disease. Isaak et al reported that the most common ocular finding is episcleritis (39%) and the second is scleritis (14%). Other signs are iritis (9%), retinopathy (9%), muscle paresis (5%), and optic neuritis (5%). Case report - Case description A 45-year-old female with known rheumatoid arthritis referred by rheumatology in eye clinic due to blurred vision and dry eye. The patient was on hydroxychloroquine and sulfasalazine. No retinal toxicity was found on examination, OCT and Visual Fields. The vision was 6/6 both eyes. Follow-up was in 12 months. She presented 6 months later in casualty with severe pain in her right eye. Examination showed diffuse anterior scleritis with secondary conjunctival inflammation. Anterior chamber cells present. Posterior segment showed no inflammation. Left eye was unremarkable. She was started on Froben 100mg tds with omeprazole. She was seen after a week and condition was improving. She was asked to taper off the meds. Inflammation resolved with 6/5 vision in both eyes and the next appointment was made in a year to monitor for hydroxychloroquine toxicity. In November 2020 she was seen by ENT with inflammation of the right ear cartilage. The pictures showed that the pinna was spared and cartilage was only involved. There was nasal crusting and stuffy nose but without any respiratory symptoms. She was prescribed 50mgs of prednisolone and this helped with her inflammation. She was seen by rheumatology later on and hydroxychloroquine and sulfasalazine was stopped, and she was started on methotrexate 10mgs weekly and folic acid 5mg weekly. Pulmonary function test and echocardiogram was ordered. The case was discussed in MDT rheumatology and it was decided that if joint symptoms got worse than biologics could be started. Methotrexate increased to 15mg subcut. Echocardiogram was normal with satisfactory blood tests. Her next appointment is in October 2021. Case report - Discussion Initially the patient was diagnosed with rheumatoid arthritis with ocular inflammation (anterior scleritis) and was given the standard treatment of steroids to which the patient responded as well. Later when she developed the ear inflammation which involved only the cartilage the diagnosis was revised by rheumatology and changed to RP. As this is a rare life-threatening disease management was switched to immunosuppressive therapy to which she is currently responding well. Case report - Key learning points It is important to consider the possibility that a rheumatology patient may have more than one diagnosis or be open to the idea of revising the diagnosis as the clinical picture evolves over the time. Given the nature of the disease all the systemic features should be examined thoroughly as any one missed area can lead to delayed diagnosis.


2021 ◽  
Vol 7 (4) ◽  
Author(s):  
Anil P Gosavi ◽  
Ravindranath Brahmadeo Chavan ◽  
Jheel Shriniwas Ambike ◽  
Nitika S Deshmukh

: Pemphigus is a chronic autoimmune vesiculobullous disease caused by autoantibodies directed against desmogleins (Dsgs). Pemphigus vegetans is a rare variant of pemphigus vulgaris, characterized by vegetative plaques, primarily occurring in flexures. It is a less common disease and involves the skin and mucosa due to disintegration of cellular adherence (acantholysis), resulting in intradermal split. We report this case of pemphigus vegetans of Neumann type for its rare presentation. We report a case of pemphigus vegetans, involving lips in a middle-aged female and sparing flexural areas. This patient was diagnosed on the basis of histopathology and treated successfully with intralesional steroids, resulting in the resolution of her plaques.


2020 ◽  
Vol 3 (11) ◽  
pp. 01-07
Author(s):  
Marc Vanderheyden ◽  
Sofie Dhaeyer ◽  
Chirik Wah Lau ◽  
Vanessa Meert ◽  
Jan Leeman ◽  
...  

Cardiac angiosarcomas are rare malignant tumors, predominantly affecting the right heart with poor survival outcomes. The current mainstay of treatment consists of surgery with or without chemotherapy, but often yields limited results with local relapse or metastatic recurrence. This case report describes 2 patients with primary angiosarcoma located in the right atrium. One patient received neo-adjuvant and adjuvant chemotherapy; both were scheduled for surgical resection. The course of disease is described followed by a comprehensive review of the literature.


1995 ◽  
Vol 48 (2) ◽  
pp. 177-181 ◽  
Author(s):  
Phillip J. Harrity ◽  
Henry D. Tazelaar ◽  
William D. Edwards ◽  
Thomas A. Orszulak ◽  
William K. Freeman

2007 ◽  
Vol 6 (2) ◽  
pp. 75-76
Author(s):  
J Fingleton ◽  

Seizures are a common presenting complaint in acute medicine and post-ictal patients can pose a diagnostic challenge. Approximately 1% of patients presenting after a seizure will have sustained a fracture. Delayed diagnosis is common and can lead to worse functional outcomes. A case of occult bilateral neck of femur fracture secondary to seizure is presented together with a review of the literature.


2013 ◽  
Vol 92 (2) ◽  
pp. 84-87 ◽  
Author(s):  
Gangadhara Somayaji ◽  
Aroor Rajeshwary ◽  
Sullia Ramesh ◽  
Sullia Dinesh

We report a case of recurrent Pindborg tumor (calcifying epithelial odontogenic tumor) of the maxilla. The patient was a 34-year-old woman who had been previously diagnosed with Pindborg tumor and treated with curettage. She was subsequently referred to us for evaluation of nasal obstruction. Examination revealed the presence of a mass lesion in the right nasal cavity and right maxilla, which was identified as a recurrence of her earlier Pindborg tumor. The patient was treated with maxillectomy with orbital preservation. Pindborg tumor is a rare odontogenic tumor; when it does occur, it is more often seen in the mandible than in the maxilla. While this tumor is often treated with curettage alone, the aggressive nature of the recurrence in our patient necessitated radical surgery. We report this case to highlight the need to be suitably aggressive in treating these types of tumors in order to avoid recurrence.


Sign in / Sign up

Export Citation Format

Share Document