scholarly journals MANIFESTASI DAN PENATALAKSANAAN PEMFIGUS VULGARIS DI RONGGA MULUT

2018 ◽  
Vol 2 (2) ◽  
pp. 117-124
Author(s):  
Indra Gunawan ◽  
Riani Setiadhi

Background : Pemphigus Vulgaris (PV) is an autoimmune intraepithelial blistering disease involving the skin and mucous membranes. Oral lesions could be the first sign of the disease followed by the involvement of skin and other mucosa sites.Objectives : This is oral manifestations of pemphigus vulgaris case report, intended to help clinicians to recognize and treat the oral lesions.Case Reported : Male 27 yo admitted to RSUP Dr.Hasan Sadikin hospital with multiple blisters on the skin and oral mucosa, was diagnosed with Pemphigus Vulgaris.Case Management : Patient was treated with steroid mouth wash and oral paste. After two months of treatment, the patient was fully recovered from oral lesion.Discussion : In PV, autoantibodies are produced against desmosomes spesifically desmoglein 3 which responsible for holding the cells of the epithelium together. The loss of adhesive function due to anti Dsg 3 antibodies result in bulla formation on the oral mucosa.The aetiology for PV is still uncertain. Conclusion :Early recognition and treatment of oral lesions is important as it may prevent skin involvement. Early treatment, patient’s compliance and multi disciplinal teamwork ensure the treatment succes for this disease

2011 ◽  
Vol 2011 ◽  
pp. 1-4 ◽  
Author(s):  
Mitsuhiro Ohta ◽  
Seiko Osawa ◽  
Hiroyasu Endo ◽  
Kayo Kuyama ◽  
Hirotsugu Yamamoto ◽  
...  

Pemphigus Vulgaris (PV) is an autoimmune intraepithelial blistering disease involving the skin and mucous membranes. Oral mucosa is frequently affected in patients with PV, and oral lesions may be the first sign of the disease in majority of patients. In some patients, oral lesions may also be followed by skin involvement. Therefore, timely recognition and therapy of oral lesions is critical as it may prevent skin involvement. Early oral lesions of PV are, however, often regarded as difficult to diagnose, since the initial oral lesions may be relatively nonspecific, manifesting as superficial erosions or ulcerations, and rarely presenting with the formation of intact bullae. Lesions may occur anywhere on the oral mucosa including gingiva; however; desquamtive gingivitis is less common with PV than other mucocutaneous conditions such as pemphigoid or lichen planus. This paper describes the case of a patient presenting with a one-year history of painful gingival, who is finally diagnosed as having PV.


2021 ◽  
Vol 2021 ◽  
pp. 1-3
Author(s):  
Bassel Tarakji

Pemphigus vulgaris (PV) is a chronic, autoimmune, intraepidermal blistering disease of the skin and mucous membranes. The first clinical manifestation is often the development of intraoral lesions, and later, the lesions involve the other mucous membranes and skin. The etiological factors of this disease still remain unknown, although the presence of autoantibodies is consistent with an autoimmune disease. A 73-year-old man had bullous lesions on gingiva, oral mucosa first, then scalp, trunk, and face. An oral medicine specialist suspects the lesion in differential diagnosis in the first presentation of oral lesions and follows up the patient, and then these bullous lesions presented on the skin. In this article, a patient had received oral prednisolone (80 mg/kg/day) and azathioprine, then tapered oral prednisolone to 40 mg/day, with a reduction of 5 mg/day every three weeks. The patient shows remission of these lesions, and complication of this treatment includes osteoporosis, hyperglycemia, and hypertension.


2012 ◽  
Vol 16 (2) ◽  
pp. 128-130 ◽  
Author(s):  
Ashley O'toole ◽  
Maureen O'malley

Background: Pemphigus vulgaris is an autoimmune blistering disease of the skin and mucous membranes. Sweet syndrome is an uncommon reactive neutrophilic dermatosis. Objective: This case report describes the rare presentation of a 58-year-old female patient with newly diagnosed pemphigus vulgaris who later presented with erythematous plaques and leukocytosis highly suggestive of Sweet syndrome. Methods: Review of the literature using the PubMed and Medline databases. Results: The clinical presentation of new-onset Sweet syndrome in a patient with pemphigus has been reported in the literature only on one other occasion. Conclusion: The observation of an association between Sweet syndrome and autoimmune diseases, such as pemphigus vulgaris, may ultimately lead to better understanding of the pathophysiology of this disease.


Author(s):  
Tayna Soares De Oliveira ◽  
Lucas Lavareze Dos Reis ◽  
Thuany Servare De Lima ◽  
Danielle Resende Camisasca ◽  
Tânia Regina Grão Velloso ◽  
...  

1995 ◽  
Vol 88 (1) ◽  
pp. 49-53
Author(s):  
Haruo HIRAKAWA ◽  
Yukiko IINO

2015 ◽  
Vol 2015 ◽  
pp. 1-3 ◽  
Author(s):  
Diana Kuriachan ◽  
Rakesh Suresh ◽  
Mahija Janardhanan ◽  
Vindhya Savithri

Pemphigus is a group of potentially fatal dermatoses with both cutaneous and oral manifestations. Characterized by the appearance of vesicle or bullae, their manifestations in the oral cavity often precede those on the skin by many months or may remain as the only symptoms of the disease. It is therefore important that the oral manifestations of the disease are recognized on time, to make a proper diagnosis and initiate timely treatment. Here we present a case of Pemphigus Vulgaris (PV) that presented with oral lesions at multiple sites including tongue, to highlight the importance of timely recognition of the oral lesions during routine dental practice for the diagnosis and management of this disease.


Author(s):  
Deyvid Silva Rebouças ◽  
Lucas Souza Cerqueira ◽  
Tila Fortuna Costa ◽  
Thaise Gomes Ferreira ◽  
Roberta Catapano Naves ◽  
...  

Pemphigus is the general name for a group of rare autoimmune diseases that affect the skin and mucous membranes and is presented with the formation of intraepidermal blisters. Pemphigus vulgaris (PV) is a chronic rare vesicular-bullous autoimmune disease that when not diagnosed and treated in its early stages has severe prognosis. This study aims to report a clinical case of a female patient, 32 years of age who had manifestation of pemphigus vulgaris. The oral manifestations are, in most cases, the first signs of the disease and the dentist has a primary role in the early diagnosis.


2021 ◽  
pp. 222-229
Author(s):  
Maria L. Mihailescu ◽  
Cuoghi Edens ◽  
Mark D. Hoffman

Dermatomyositis (DM) and its variant, clinically amyopathic DM, are widely recognized entities. DM sine dermatitis, a variant without skin involvement, is less widely reported. DM with neither muscle nor skin manifestations has not been reported. We herein describe the first account of a patient with a myositis-specific antibody presenting with an array of clinical findings in the absence of both muscle and pathognomonic skin disease. This case report details the multidisciplinary assessment of an anti-melanoma differentiation-associated gene 5 (MDA5) antibody-positive individual with inflammatory polyarthropathy, mucocutaneous capillary changes, and evidence of interstitial lung disease but lacking overt skin and muscle disease. This presentation is paradoxically but appositely deemed to represent a unique form of DM, which may be best described as “amyopathic hypodermatitic dermatomyositis.” Early recognition and documentation of these cases will help to characterize this variant in the future, determine its frequency, and guide management.


1997 ◽  
Vol 137 (5) ◽  
pp. 1091-1102 ◽  
Author(s):  
Peter J. Koch ◽  
M G. Mahoney ◽  
Hiroyasu Ishikawa ◽  
Leena Pulkkinen ◽  
Jouni Uitto ◽  
...  

In patients with pemphigus vulgaris (PV), autoantibodies against desmoglein 3 (Dsg3) cause loss of cell–cell adhesion of keratinocytes in the basal and immediate suprabasal layers of stratified squamous epithelia. The pathology, at least partially, may depend on protease release from keratinocytes, but might also result from antibodies interfering with an adhesion function of Dsg3. However, a direct role of desmogleins in cell adhesion has not been shown. To test whether Dsg3 mediates adhesion, we genetically engineered mice with a targeted disruption of the DSG3 gene. DSG3 −/− mice had no DSG3 mRNA by RNase protection assay and no Dsg3 protein by immunofluorescence (IF) and immunoblots. These mice were normal at birth, but by 8–10 d weighed less than DSG3 +/− or +/+ littermates, and at around day 18 were grossly runted. We speculated that oral lesions (typical in PV patients) might be inhibiting food intake, causing this runting. Indeed, oropharyngeal biopsies showed erosions with histology typical of PV, including suprabasilar acantholysis and “tombstoning” of basal cells. EM showed separation of desmosomes. Traumatized skin also had crusting and suprabasilar acantholysis. Runted mice showed hair loss at weaning. The runting and hair loss phenotype of DSG3 −/− mice is identical to that of a previously reported mouse mutant, balding (bal). Breeding indicated that bal is coallelic with the targeted mutation. We also showed that bal mice lack Dsg3 by IF, have typical PV oral lesions, and have a DSG3 gene mutation. These results demonstrate the critical importance of Dsg3 for adhesion in deep stratified squamous epithelia and suggest that pemphigus autoantibodies might interfere directly with such a function.


Author(s):  
Mila Nu Nu Htay ◽  
Wai Wai Myint ◽  
Htay Lwin ◽  
Win Htay

Erythroderma is a scaly, erythematous dermatitis of the skin, which occurs in drug allergy, malignancy and underlying skin disorders. The diagnosis is challenging because the extent of skin involvement does not always correlate with the extent of internal organ involvement. Therefore, early recognition of symptoms is vital to minimize morbidity and mortality. Case report: A 52 years old man had asymptomatic hyperuricemia and prescribed allopurinol 300mg, daily. One month later, the rashes started to appear on his trunk and then progressed to the face and upper limbs. Then it continued to spread to the lower extremities. Management involves prompt cessation of the culprit drug, administration of corticosteroids and supportive treatment. It is Concluded that Allopurinol is commonly used in clinical practice for the treatment of symptomatic hyperuricemia and gout. It has been associated with erythroderma especially when used indiscriminately.


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