1/15. Paraneoplastic pemphigus associated with Castleman tumor, myasthenia gravis and bronchiolitis obliterans.BACKGROUND: Cases of paraneoplastic pemphigus (PNP) have been reported associated with various lymphoproliferative malignancies and benign Castleman tumors, with the most severe course and fatal outcome seen in patients with bronchiolitis obliterans. OBJECTIVE: The aim was to establish immunologic associations by coexistence of Castleman tumor, myasthenia gravis, and bronchiolitis obliterans and to evaluate the treatment modalities. methods: Clinical studies included computed tomography of the mediastinum, computed tomography and magnetic resonance imaging of the abdominal cavity, and quantitative electromyography. Direct and indirect immunofluorescence on various substrates, immunoblot analysis, immunoprecipitation, and specific enzyme-linked immunosorbent assay using recombinant desmogleins (Dsg) were performed as immunologic assays. RESULTS: Direct and indirect immunofluorescence including rat bladder showed intercellular antibodies. immunoblotting disclosed antibodies to envoplakin (210 kd protein) and periplakin (190 kd protein); in addition, immunoprecipitation detected antibodies to desmoplakin I (250 kd protein). antibodies to Dsg3 (pemphigus vulgaris antigen) were detected by specific enzyme-linked immunosorbent assay. myasthenia gravis was controlled by drugs; however, mucocutaneous changes were not fully responsive to corticosteroids and cyclophosphamide pulses, cyclosporine, and intravenous immunoglobulins. The surgical removal of Castleman tumor did not change the course of the disease. The fatal outcome was the result of bronchiolitis obliterans that occurred after the surgery and was only transitionally controlled by plasmapheresis. CONCLUSION: This is the first case of paraneoplastic pemphigus associated with Castleman tumor, myasthenia gravis, and bronchiolitis obliterans. Despite a benign character of the tumor the patient died, as do all patients with bronchiolitis obliterans. Massive plasmapheresis has only a transient effect. We confirmed the presence of antibodies to Dsg 3, in addition to the set of specific paraneoplastic pemphigus antibodies against various proteins of plakin family.- - - - - - - - - - ranking = 1keywords = cavity (Clic here for more details about this article) |
2/15. stevens-johnson syndrome: case presentation.erythema multiforme (EM) is a rare mucocutaneous disease with a variety of clinical manifestations. EM it was recognized in the early 1800's, and still the etiology is unknown. It has been recently suggested erythema multiforme (EM) major and stevens-johnson syndrome (SJS) could be separated as two distinct clinical disorders with similar mucosal erosions, but different patterns of cutaneous lesions. In particular SJS should be used for a syndrome characterized by mucous membrane erosions and widespread small blisters that appear on erythematous or purpuric maculae, which are different from classic targets. In SJS mouth, eyes, skin, genitalia and occasionally the esophagus and respiratory track may be affected. Oral lesions may cause severe pain and usually lips may become encrusted. Concerning ocular involvement, if there is conjunctivitis or uveitis this may lead to scarring and blindness. Also, the course of disease and the prognosis are in most cases severe.- - - - - - - - - - ranking = 22.693595387191keywords = mouth (Clic here for more details about this article) |
3/15. stevens-johnson syndrome in a boy with nephrotic syndrome during prednisolone therapy.stevens-johnson syndrome (SJS) is a mucocutaneous disease that can be lethal. It can occur in association with altered immunological conditions and the administration of various drugs, including corticosteroids. We report a case of SJS in a 14-year-old male with nephrotic syndrome, who was treated with oral prednisolone for 6 weeks. He presented symptoms of fever, skin lesions consisting of multiple vesiculopapular rashes, pruritic maculae and bullae, and mucosal involvement of the eyes, lips, oral cavity, and anorectal junction. His condition improved without complications following the discontinuation of oral prednisolone and replacement with intravenous methylprednisolone. Following the improvement of the symptoms of SJS, he received alternate-day oral prednisolone without any cutaneous eruption.- - - - - - - - - - ranking = 35.615155310311keywords = oral cavity, cavity (Clic here for more details about this article) |
4/15. cephalexin-induced stevens-johnson syndrome.OBJECTIVE: To report a case of cephalexin-induced stevens-johnson syndrome (SJS), a devastating adverse drug reaction that involves the entire skin surface and mucosal areas of the body. DATA SOURCES: medline search (key terms cephalosporins, stevens-johnson syndrome, erythema multiforme, and systemic lupus erythematosus) and references identified from bibliographies of pertinent articles. DATA SYNTHESIS: Clinical presentation and manifestations of SJS include the skin, eyes, gastrointestinal tract, and pulmonary system. Infectious complications are the leading cause of mortality. Early intervention is important to prevent progression of SJS. The case described is consistent with the features of this syndrome. The patient presented with fever, arthralgias, and malaise. skin lesions included a diffuse violet macular rash with erythema and multiple bullous lesions on her neck and abdomen. The skin biopsy was consistent with SJS. Multiple mucocutaneous ulcers were found in her mouth, but no evidence of lower gastrointestinal tract involvement was documented. She remained relatively free of pulmonary complaints except for the presenting bronchitis. CONCLUSIONS: cephalexin should be added to the list of agents to consider as iatrogenic causes of SJS.- - - - - - - - - - ranking = 22.693595387191keywords = mouth (Clic here for more details about this article) |
5/15. Steven Johnson syndrome due to I.V ceftriaxone--a case report.Steven-Johnson syndrome (SJS) is a rare vesiculobullous disease characterized by an acute cutaneous eruption that ivolves the skin and mucous membranes including those of the oral cavity. A rare case of Steven-Johnson syndrome, an unexpected treatment response, in a 25-year-old female patient due to administration of intravenous Cefriaxone (1 gm), a third generation cephalosporin has been reported and literature reviewed.- - - - - - - - - - ranking = 35.615155310311keywords = oral cavity, cavity (Clic here for more details about this article) |
6/15. Dacryocystoceles in the aftermath of stevens-johnson syndrome.Although stevens-johnson syndrome has been reported to result in canalicular and nasolacrimal duct obstruction, reports of dacryocystocele formation are lacking. We describe the occurrence and management of bilateral dacryocystoceles related to stevens-johnson syndrome. A 45-year-old man, recovered from stevens-johnson syndrome, presented with bilateral medial canthal masses and intermittent overlying facial cellulitis. Examination revealed severe keratitis sicca with extensive corneal scarring, cicatricial occlusion of all puncta, and palpable medial canthal masses bilaterally. Computed tomography demonstrated bilateral cystic masses consistent with dacryocystoceles. Bilateral lacrimal sac/cyst excision was attempted, but the left cyst recurred within 6 months after surgery. A dacryocystorhinostomy was then performed, effectively marsupializing the cyst. The puncta were not canalized and stents were not placed. The masses have not recurred with 2 years of follow-up. Dacryocystoceles may arise in the setting of stevens-johnson syndrome. Successful management may be achieved with marsupialization to the nasal cavity (dacryocystorhinostomy).- - - - - - - - - - ranking = 1keywords = cavity (Clic here for more details about this article) |
7/15. stevens-johnson syndrome induced by mizoribine in a patient with systemic lupus erythematosus.A 32-year-old Japanese woman, who had a treatment history of systemic lupus erythematosus (SLE) with lupus nephritis world health organization class IV for 11 months, visited our hospital due to fever, facial erythema, and erosion of the oral cavity on November 10, 2003. Her mucosal erosion and facial skin erythema progressed over the following week, and stevens-johnson syndrome was diagnosed due to pathological findings of the skin. Among the administrated drugs, only mizoribine, started 6 months earlier, produced a positive reaction in the drug lymphocyte stimulation test. Increased prednisolone and high dose intravenous gamma-globulin were given successfully. cyclosporine at 50 mg was administered to control the SLE, followed by an increase to 100 mg on January 7, 2004. She suffered from abdominal pain, blindness, and convulsion on January 9. The magnetic resonance image of her brain prompted a diagnosis of reversible posterior leukoencephalopathy syndrome. After withdrawal of cyclosporine and control of hypertension, symptoms disappeared rapidly. cyclophosphamide pulse therapy was successfully administrated to control lupus nephritis. This is the first report describing the relationship between stevens-johnson syndrome and mizoribine. Although the use of mizoribine is thought to be safe, careful observation is necessary.- - - - - - - - - - ranking = 35.615155310311keywords = oral cavity, cavity (Clic here for more details about this article) |
8/15. verapamil-associated stevens-johnson syndrome.stevens-johnson syndrome (SJS) is a complication infrequently associated with the use of calcium-channel blocking agents. A 59-year-old woman developed symptoms typical of SJS approximately ten days following the initiation of sustained-release verapamil for hypertension management. Symptoms consisted of a generalized red macular rash associated with swelling of the face, tongue, lips, and mouth as well as a fever of 40 degrees C. Resolution of symptoms began approximately four days after verapamil discontinuation. Rechallenge with verapamil was not performed. Although SJS appears to be a rare adverse effect of this drug, the potential complications warrant the inclusion of SJS in the differential diagnosis of patients presenting with malaise, fever, rash, and a history of verapamil ingestion.- - - - - - - - - - ranking = 22.693595387191keywords = mouth (Clic here for more details about this article) |
9/15. vitamin a in stevens-johnson syndrome.A 9-year-old boy was admitted to the Pediatric Department with high fever, severe photophobia, and a rash on his face, eyelids, and neck. Two days before admission he received injections of penicillin. During the night his general condition worsened, and his fever rose to 40 degrees C. He began to hallucinate, and clear fluid-filled blisters appeared on his skin and mouth. Severe photophobic epiphora, conjunctival injection, and punctate keratitis were also observed. Several hours after local administration of corticosteroids and antibiotic eye drops, his eyelids swelled severely, the corneas became covered by pseudomembranes, and later, in attempting to open the eyelids, the skin peeled off and the eye lashes fell out. Treatment consisted of artificial tear eye drops, and intravenous antibiotics and steroids. His condition improved gradually, visual acuity became 6/15 in both eyes, and a superficial punctate staining of the cornea was observed. The Schirimer test showed lacrimal hyposecretion. A tarsal conjunctival biopsy showed a complete absence of goblet cells. vitamin a was administered topically and systemically. After three months of treatment with vitamin a, tear secretion was almost normal, and conjunctival biopsy indicated a regeneration of goblet cells.- - - - - - - - - - ranking = 22.693595387191keywords = mouth (Clic here for more details about this article) |
10/15. Management of stevens-johnson syndrome.A patient with stevens-johnson syndrome is described, and the literature concerning the etiology, pathophysiology, clinical manifestations, and management of stevens-johnson syndrome is reviewed. A 2 1/2-year-old girl was treated with phenobarbital and i.v. ampicillin, followed by oral amoxicillin, for an upper-airway infection, otitis media, and febrile seizures. The fever returned, and she was treated unsuccessfully with penicillin and cefaclor. She was admitted to the hospital and treated with i.v. ampicillin. Within 24 hours an erythematous maculopapular rash developed. phenobarbital was discontinued and phenytoin was begun. Four days later bullous lesions developed; ampicillin and phenytoin were discontinued, and cefazolin and phenobarbital were given. By the eighth day severe sloughing of the skin occurred over 75% of her body, and mucosal sloughing was apparent. The patient's condition was diagnosed as stevens-johnson syndrome. Porcine xenografts were immediately grafted to 75% of her total body surface. Severe lesions of the mouth and pharynx made parenteral nutrient therapy necessary, and ocular complications required the care of an ophthalmologist. Although the skin had healed by 14 days after grafting, another 14 days of treatment for respiratory complications was required. stevens-johnson syndrome is a severe exfoliative dermatitis accompanied by fever, inflammation of the gastrointestinal mucosa, and severe purulent conjunctivitis. It is associated with high morbidity and mortality. The etiologic factors may be iatrogenic (e.g., various antibiotics and anticonvulsants), infectious, or idiopathic. Respiratory complications, leukopenia, infections, erosion of the gastrointestinal mucosa, fluid and electrolyte disturbances, and chronic ocular complications may occur.(ABSTRACT TRUNCATED AT 250 WORDS)- - - - - - - - - - ranking = 22.693595387191keywords = mouth (Clic here for more details about this article) |
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