Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0011168 (dysphagia)
 15,644 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

A case of pemphigus vulgaris with esophageal involvement is discussed and 10 other reported cases are reviewed. Esophageal involvement is a rare but serious occurrence. It is most readily diagnosed by endoscopy when dysphagia or odynophagia in a patient with pemphigus vulgaris or with a history of pemphigus vulgaris does not respond to appropriate adjustment of corticosteroid or other therapy. All patients with esophageal pemphigus vulgaris were middle-aged women. None had skin lesions at the time esophageal disease was diagnosed, and five patients appeared to be in complete remission, with neither skin nor oral lesions, when esophageal involvement was discovered.
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PMID:Pemphigus vulgaris of the esophagus in women. 268 Dec 97

The authors report two cases of bullous rashes with an ENT onset: 1. a benign mucosal pemphigoid; 2. a pemphigus vulgaris. On the basis of these cases, they indicate the need to consider the possibility of bullous lesions in the presence of any persistent erosion of the nasal or bucopharyngeal mucosa, the diagnosis of which should be dominated by the identification of a possible pemphigus, the prognostic and therapeutic consequences of which remain serious. The clinical onset may be confined for a long period to the E.N.T. musoca. It may present with dysphagia and pain, or sometimes by simple epistaxis. The commonest diagnostic errors are aphthous ulcers and candidiasis which may, in the case of the latter, be associated with bullous lesions. They also report the possible problems which may result from endoscopic manoeuvres in the case of pemphigus. Any persistent erosion of the mucosae in the E.N.T. area should be evaluated by diagnostic cytology which would offer positive identification of pemphigus.
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PMID:[Problems of bullous lesions in E.N.T. Based upon two cases (author's transl)]. 699 56

A 5-year-old boy developed hemorrhagic mucocutaneous blisters on various parts of the body leading to fetor, dysphagia, dysuria, anal pruritus, pain on defecation, and weight loss. The histopathology showed the classic features of pemphigus vulgaris, and direct immunofluorescence showed intercellular deposition of IgG and C3 in the epidermis. Circulating pemphigus antibodies were also detected. He was treated with a combination of systemic prednisone and dapsone which induced a rapid remission and controlled the disease well. He has been in remission for 1 year and 7 months with no immunosuppressive therapy except for the use of topical agents for the oral lesions. An adjuvant to corticosteroids has been used only once before in children with pemphigus vulgaris under the age of 12 years. This is the third and the youngest child in the literature treated in this fashion.
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PMID:Childhood pemphigus vulgaris treated with dapsone: a case report. 979 90

The extension of bullous lesions in pemphigus to the esophagus is relatively uncommon, especially in patients who appear to be in clinical remission. Very rarely, pemphigus vulgaris may affect the entire esophagus, resulting in complete sloughing of the mucous membrane. A 20-year-old man with pemphigus vulgaris presented to the emergency room with acute onset of dysphagia, odynophagia, and hemoptysis. There were no cutaneous or oral findings of pemphigus on presentation, since he was being maintained on corticosteroids and azathioprine with excellent results. During initial evaluation in the emergency room, the patient was observed to vomit a cast of the mucosal lining of the esophagus. The morphologic description of such an esophageal cast is termed esophagitis dissecans superficialis. This is the third case of esophagitis dissecans superficialis in pemphigus vulgaris recorded in the medical literature.
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PMID:Esophagitis dissecans superficialis associated with pemphigus vulgaris. 1019 66

We present a 69-year-old white woman with pemphigus vulgaris limited to the oral mucosa who presented with oral pain and difficulty swallowing of 2 days duration, followed by multiple irregular ulcers arising from normal mucosa with no grouping of individual lesions--herpes simplex should be considered in the differential diagnosis of lesions that appear suddenly in patients with PV, particularly if the lesions fail to respond to an increased dose of corticosteroids.
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PMID:Atypical herpes simplex can mimic a flare of disease activity in patients with pemphigus vulgaris. 1064 61

Odynophagia and dysphagia are clinical signs associated with a wide spectrum of potential causes. Our patient was an 84-year-old woman, who suffered from odynophagia and dysphagia for over 10 years. The interstinoscopy showed blister-like lesions and signs of inflammation only in the proximal part of the oesophagus. The diagnosis of pemphigus vulgaris was supported by immunohistological results. Typical oropharyngeal and cutaneous lesions of pemphigus were missing in our patient. Involvement of the oesophagus is described in literature, but it seems to be underdiagnosed in clinical everyday's work. Restriction of the lesions to the oesophagus without oropharyngeal and cutaneous manifestation - as we have seen in our patient - is rare.
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PMID:[Long-Lasting Odynophagia and Haematemesis - the Only Clinical Signs of a Pemphigus vulgaris]. 1205 66

Pemphigus is an uncommon chronic disease with dermatologic and mucosal manifestations. Primary laryngeal involvement without skin lesions is extremely rare. The present paper describes a 72-year old man who presented with a 2-month history of hoarseness, haemoptisis and dysphagia. Clinical examination revealed an erythematous oral mucosa without ulcerations. Indirect laryngoscopy revealed supraglottic ulcerations mainly in the laryngeal surface of the epiglottis and in the right arytenoid. The lesions had characteristic gray color membranes. The patient underwent microlaryngoscopy under general anesthesia and biopsies were taken for histology that revealed inflammatory and granular lesions with necrosis. The diagnosis of pemphigus was based on immunohistopathology and the clinical examination. The patient underwent intravenous treatment with high doses of corticosteroids (prezolon 75 mg/24 h) for 10 days and gradually the dose was reduced to 10 mg/24 h. The patient had a very good response to the treatment and after a week approximately 80% of the lesions disappeared. However, the dose of 10 mg prednisolone per day was sustained for 3 months because any attempt of prednisolone discontinuation was related with reappearance of the clinical symptoms. After 3 months, finally the treatment was discontinued without problems. Now, 15 months later, the patient is well and without symptoms. He is under long-term follow-up. ENT surgeons should be aware of pemphigus as primary laryngeal manifestation in order to investigate and manage patients accordingly.
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PMID:Laryngeal pemphigus without skin manifestations and review of the literature. 1711 Nov 2

This article describes a case of pemphigus vulgaris in a relatively young patient with exacerbated characteristics, widespread lesions, and rapid evolution. A 40-year-old woman sought treatment, complaining about severe oral pain and dysphagia. Intraoral inspection revealed ulcerated lesions on the lips, buccal mucosa, and floor of the mouth. The number of lesions quickly increased and resembled erythema multiforme. She also presented blistering lesions on the back, abdomen, neck, and eyes. Incisional oral biopsy revealed pemphigus vulgaris. This report emphasizes the importance of early diagnosis of an oral biopsy in systemic diseases and the management of the lesions.
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PMID:Severe manifestation of oral pemphigus. 2043 9

A 76-year-old woman presented with a pruritic photodistributed rash and dysphagia. Serum anti-nuclear antibody was positive (titre 1/1280) and skin and muscle biopsies confirmed a diagnosis of dermatomyositis. She was treated with oral prednisolone (5-50 mg/day), mometasone furoate 0.1% ointment and lotion, and tacrolimus 0.03% ointment. Four years later she presented with multiple painful scaly erosions on the face, scalp and trunk. Histopathology and direct and indirect immunofluorescence confirmed a diagnosis of pemphigus vulgaris. Repeated malignancy screens were negative. She was treated with methotrexate (10 mg/week) and prednisolone (50 mg/day slowly tapered to 5 mg/day), with good control of both diseases.
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PMID:Dermatomyositis and pemphigus vulgaris: association or coincidence? 2160 86

The stratified squamous epithelial lining of the esophagus is similar to that of the skin, and occasionally conditions that affect the skin tend to have esophageal manifestations. Autoimmune dermatological conditions that present with blisters are referred to as bullous dermatoses and have been found to rarely present with esophageal involvement. Esophageal epithelium is thought to be involved in rare occasions because of its identical epithelial lining to the skin and tends to be more frequent among patients with pemphigus vulgaris than in those with pemphigoid. Common symptoms from esophageal involvement in bullous dermatoses may include heartburn, odynophagia or dysphagia. Rarely, it may present with vomiting of sloughed cast of esophageal mucosa or haematemesis. We present S A, a 47 year old female who was on outpatient treatment in 2010 at King Fahad Medical City for bullous pemphigoid but developed progressive odynophagia with massive haematemesis. She was admitted and resuscitated, and endoscopy revealed sloughing of esophageal mucosa and bleeding in keeping with esophagitis dissecans superficialis. Although association of esophageal dissecans superficialis with pemphigus vulgaris has been sparsely reported; to our knowledge its association with bullous pemphigoid as in our case has been rarely reported in English medical literature.
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PMID:Esophagitis dissecans superficialis presenting with massive haematemesis in a patient with bullous pemphigoid. 2428


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