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Query: UMLS:C0011168 (dysphagia)
 15,644 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Scleromyxedema is an infiltrative skin disease produced by hyaluronic acid deposition in the dermis. A benign monoclonal gammopathy is usually present. We report 2 patients with scleromyxedema and systemic illnesses. Both patients had muscle weakness, dysphagia, and weight loss in addition to the skin changes. One also had sclerodactyly, telangiectasias, and Raynaud's phenomenon. Scleromyxedema with systemic involvement may mimic rheumatic diseases.
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PMID:Scleromyxedema with systemic involvement mimics rheumatic diseases. 294 92

Scleromyxedema is a rare fibromucinous connective tissue disorder characterized by papular skin lesions associated with sclerosis and a serum monoclonal gammopathy. Little is known about either the natural history or the systemic manifestations of this disease. We reviewed the medical records of 19 patients with biopsy-proven scleromyxedema seen from 1950 to 1985 for evidence of systemic disease. There were 10 males and 9 females with a median age at diagnosis of 53 years. Monoclonal gammopathy was present in 13 patients. Eight patients complained of dysphagia; 3 had proximal esophageal dysfunction and 1 had total esophageal aperistalsis on barium swallow. Proximal muscle weakness was noted in 5, with an inflammatory myopathy in 3. Six patients complained of dyspnea on exertion. Of these, 5 had reduced diffusing capacity, 3 had reduced volumes, and 2 developed cor pulmonale. Pathologic changes characteristic of "scleroderma kidney" were demonstrated in 1 patient at postmortem. One patient had Raynaud's phenomenon and 2 had arthralgias/arthritis with noninflammatory synovial fluids. Although 8 of 12 patients treated with melphalan noted regression of their skin changes, no consistent improvement in the extracutaneous manifestations was demonstrated. Furthermore, 2 patients died of sepsis related to melphalan-induced myelosuppression, and 4 developed hematological malignancies following melphalan therapy. In conclusion, systemic manifestations in scleromyxedema are more prevalent than previously recognized, and can resemble those of scleroderma. Significant toxicity occurred with the use of alkylating agents in these patients, with treatment-related complications developing in 45% of patients treated with melphalan. The lack of definitive data regarding the natural history of this disease complicates the question of optimal therapy, but the use of alkylating agents should be reserved for those patients with severe debilitating skin disease.
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PMID:Scleromyxedema: a scleroderma-like disorder with systemic manifestations. 333 81

Oropharyngeal disease is common in dogs and cats. Signs are seldom apparent to the owner until the disease is well advanced, and often are not specific for a particular disease. Signs include inappetence, pawing at the mouth, drooling of saliva, dysphagia, and halitosis. These signs can result from primary oral disease, or from effects of systemic or skin disease. Oral examination requires a cooperative patient; sedation may be necessary so that the entire oral cavity can be examined without causing the animal pain or risking injury to the examiner.
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PMID:Oral diseases and veterinary dentistry. 357 97

Epidermolysis bullosa is a rare hereditary mechanobullous skin disorder. Four patients with esophageal involvement are reported. These cases and review of reported cases of epidermolysis bullosa dystrophica-recessive (EBD-R) showed distinct clinical and roentgenographie features of esophageal involvement: diffuse inflammatory changes, motility disorders, small blisters or bullae seen as constant nodular-filling defects, esophageal ulcers, scars, pseudodiverticula, transverse and circumferential webs, various size strictures, shortening of the esophagus with development of traction hiatal hernia and gastroesophageal reflux, perforation, and complete obstruction of the esophageal lumen. Dysphagia is reversible when caused by bullae or webs and permanent when due to cicatrizing strictures. The strictures may remain unchanged in size for many years despite variations in dysphagic symptoms.
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PMID:Esophageal involvement in epidermolysis bullosa dystrophica: clinical and roentgenographic manifestations. 685 25

Scleroedema adultorum Buschke is characterized by progressive hardening of the skin. In contrast to scleroderma the hardening occurs in the skin of the trunk while extremities remain largely free. Internal organs are said not to be involved in scleroedema adultorum Buschke. The full picture of the persistent form of scleroedema adultorum Buschke was observed in two patients. One patient complained of increasing dysphagia with regurgitation and aspiration. Manometry and X-ray cinematographic investigation showed inappropriate relaxation of the upper oesophageal sphincter. In the other patient who had not previously had swallowed difficulties manometry showed achalasia of the upper oesophageal sphincter. The functional disturbances of the upper oesophagus indicate the possibility of an involvement of internal organs in scleroedema adultorum Buschke. However, proof of an aetiological connection between disturbances of oesophageal motility and skin disease requires systematic investigations in a larger group of patients.
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PMID:[Cervical dysphagia in scleroedema adultorum Buschke (author's transl)]. 723 18

We have treated seven patients with multiple esophageal webs without a predisposing disorder. The patients ranged in age from 34 to 61 years and had suffered from dysphagia for 4 to 26 years. None had skin disease, a history of ingestion of a potentially caustic substance, or evidence of gastroesophageal reflux disease by history or endoscopy. All patients responded to esophageal dilation, but dysphagia recurred in all during follow-up ranging from 6 months to 4 years, often requiring repeated dilation. Study of our patients and similar patients reported previously leads us to believe this unusual disorder is congenital.
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PMID:Multiple esophageal webs: treatment and follow-up of seven patients. 925 40

Cicatricial pemphigoid (CP) is a chronic subepidermal bullous dermatosis which primarily involves the mucous membranes. The oral cavity and the eye are most frequently involved. Since extension of the lesion into the pharynx and esophagus causes sore throat and dysphagia and progressive ocular lesions may cause blindness, early and valid diagnosis is very important. Here we present a case of cicatricial pemphigoid with onset at age 45 in a patient who manifested severe periodontal disease and showed the lesion on the mucous membranes of the mouth (desquamative gingivitis), skin, and eyes. Since definite diagnosis is very important, we describe how we made a differential diagnosis from other diseases which also accompany desquamative gingivitis. We examined the clinical manifestations, blood test results, HLA-genotype, histopathologic findings of the affected tissue, and immunological findings in relation to autoimmunity. Since many of the CP cases are first referred to periodontists or dentists, we believe that the diagnostic strategy described in the present study will be quite informative for making rapid and definite diagnoses of similar cases.
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PMID:Immunopathological diagnosis of cicatricial pemphigoid with desquamative gingivitis. A case report. 1128 99

Toxic epidermal necrolysis (TEN) is a rare and potentially fatal skin disorder, precipitated by severe allergic drug reaction, and is one of a spectrum of conditions, which includes Stevens-Johnson syndrome (SJS). Mucosal involvement is common, resulting in extreme pain on swallowing and poor oral intake. The aim of this study was to describe swallow function in TEN and SJS and define the role of Speech Pathology in management. The Burns Unit database was reviewed for patients that presented over a five-year period with TEN and SJS. Diagnosis of TEN and SJS was confirmed by skin biopsy. Information specific to swallow function, treatment approaches, and adequacy of oral intake was collected. Fourteen patients' medical records were studied: eight TEN, two TEN/SJS spectrum, and four SJS. The majority had mucosal involvement causing odynophagia, poor oral intake, an ability to tolerate fluids more easily than solids, and increased aspiration risk. These symptoms were confirmed by Speech Pathology swallowing assessment. Severe mucosal involvement resulting in odynophagia, dysphagia, and poor oral intake is common in TEN and SJS. The speech pathologist is able to assess swallow function and provide recommendations to promote safe oral intake, minimize odynophagia, and facilitate nutritional input critical to optimizing recovery.
Dysphagia 2007 Jul
PMID:Management of dysphagia in toxic epidermal necrolysis (TEN) and Stevens-Johnson syndrome (SJS). 1728 25

Scleroderma is a generalized autoimmune disease with variable involvement of the skin and major organs (esophagus, lung, heart and kidney). Scleroderma is essentially a skin disease that frequently involves the digestive system. In scleroderma, the esophagus is the most frequently affected organ of the digestive system, and esophageal dysmotility, reflux and stricture may be observed in the advanced stage. Balloon dilatation and bougienage are generally sufficient in patients developing stricture, and the number of cases in whom resection is performed is very low. In a 20-year-old patient with difficulty in taking even liquid foods, tests revealed sclerodermal involvement of the distal end of the esophagus and stricture. Esophageal resection and gastric replacement were performed. Such systemic diseases as scleroderma, although rare, must be considered in the differential diagnosis of nonmalignant dysphagia, and resection must be borne in mind as a surgical option in cases of advanced stricture.
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PMID:Esophagectomy in scleroderma: report of a case. 2189 6

Epidermolysis bullosa is a genetically transmitted skin disorder that typically manifests with trauma-induced skin blistering, scarring and in some cases mucosal involvement. Esophageal webs, strictures or stenosis can be found in about a third of the patients with the recessive dystrophic type of this disease. We report a six-year-old girl with recessive dystrophic epidermolysis bullosa and progressive dysphagia. Almost complete esophageal occlusion was found on barium swallow and esophagoscopy (endoscopic signs of esophagitis were also seen). She was treated successfully with fluoroscopically guided balloon dilatation and esomeprazole. Fluoroscopically guided balloon dilatation and postdilatation use of esomeprazole is a safe and effective therapy for recessive dystrophic epidermolysis bullosa patients with almost complete esophageal occlusion and esophagitis.
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PMID:Esophagitis and almost complete esophageal occlusion in a girl with epidermolysis bullosa. 2309 44


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