简介：Pilotspatialdisorientationisaleadingfactorcontributingtomanyfatalflyingaccidents.Spatialorientationistheproductofintegrativeinputsfromtheproprioceptive,vestibular,andvisualsystems.Vestibularneuritis(VN)canleadtosuddenpilotincapacitationinflight.VNiscommonlydiagnosedbydemonstrationofunilateralvestibularfailure,asunilaterallossofcaloricresponse.Asthistestreflectsthefunctionofthesuperiorpartofthevestibularnerveonly,casesofpureinferiornerveneuritiswillbelost.ThispaperdescribesafighterpilotwithsymptomssuggestiveofVNbutwithnormalcalorictestresults.Furthertestshowedunilaterallossofvestibularevokedmyogenicpotential.Webelievethatthepilotsufferedfrompureinferiornervevestibularneuritis.VEMPplaysamajorroleinthediagnosisofinferiornervevestibularneuritisinpilots.Aeromedicalconcernsarealsodiscussed.

简介：ＡＤＥＮＯＳＱＵＡＭＯＵＳＣＡＲＣＩＮＯＭＡＯＦＴＨＥＮＡＳＯＰＨＡＲＹＮＸＣＯＮＴＡＩＮＩＮＧＥＢＶＩＲＵＳ，ＡＰＲＯＰＯＳＯＦＡＣＡＳＥＺｈａｎｇＦｅｎｇ张锋；ＺｈａｎｇＣｈａｎｇｑｉｎｇ张昌卿；ＺｈａｎｇＪｉｎｘｉａ张锦霞；ＺｏｎｇＹｏｎｇｓｈ...

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简介：Waardenburgsyndromeisararediseasecharacterizedbysensorineuraldeafnessinassociationwithpigmentarydefects.Dependingonadditionalsymptoms,WShavebeenclassifiedintofourtypes.Waardenburgsyndrometype4,alsocalledasWaardenburgShahSyndromeisaveryrarecongenitaldisorderwithastoundingvariableclinicalexpression,characterizedbypigmentaryabnormalitiesofthehair(Awhiteforelockofhair,prematuregraying)andpigmentarychangesoftheirissuchasheterochromiaorhomochromiairides,sensorineuraldeafnessandHirschsprungdisease.ThreegeneshavebeenbestowedsofarinconsociationwithEDNRB,EDN3,andSOX10genes.ThepatternofinheritanceismultifariouswiththeSOX10mutationaffiliationwithautosomaldominantinheritancewhereastheEDNRBandEDN3genesarepasseddowninanautosomallyrecessivepattern.

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简介：Littoral-cellangioma(LCA),aprimaryangiomawhichclinicallybelongstosplenichemangioma,canbemostlyfoundinnormalspleenredsinusshorecellsofreticuloendothelialcellsystem.ThecellsofLCAstronglyexpressendothelialandtissuecellassociatedantigensthatindicateadualdifferentiationcharacteristic;whereasonlyendothelialcellmarkersarepositiveinnormalspleenredsinusshorecells.DiagnosisofLCAreliesonhistopathology.Regularfollow-upisneededtomonitorrecurrenceandmetastasis.

简介：AbstractBackground:Endolymphatic sac tumor (ELST) is one of neuroectodermal tumor which arising from endolymphatic sac and duct. It is actually quite rare, with less than 200 cases reported. Although ELST presents benign appearance in histopathology, it can present aggressive destructive behavior in clinical. The cornerstone of treatment for ELST is complete surgical excision. However, it is almost impossible to completely resect the advanced stage tumor. There is still controversy about other treatments, such as radiotherapy and gamma knife surgery.Case presentation:A 47-year-old man was admitted in The First Affiliated Hospital of Fujian Medical University with a 7-year history of progressive hearing loss and near 6-month repeated attacks of headache. Preoperative CT revealed a massive intracranial lesion and associated hydrocephalus. MR scanning demonstrated a 7.2 cm × 4.6 cm × 4.2 cm bulky mass located in left-sided posterior cranial fossa and temporo-occipital region which showed hyperintensity on T1-weighted images and mixed signal intensity on T2-weighted images. There was no neither clinical manifestation nor family history of Von Hippel-Lindau syndrome (VHL).Due to the mass that was large and invading the bone of skull base, it was difficult to extirpate surgically, so the ventriculoperitoneal shunt combined with local biopsy was performed. The postoperative pathology and immunohistochemical findings confirmed the lesion was an endolymphatic sac tumor. After operation, the patient regularly received radiotherapy.Conclusion:The widely accepted management of ELST is complete surgical resection. However, it is difficult for surgeons to achieve radical resection with late-stage ELST. Currently, there is much dispute about the role of radiotherapy for the management of ELST in academic circles. In this case where the mass cannot be surgical removed, radiotherapy has the curative effect for ELST in terms of disease control and quality of life.

简介：A78-year-oldwomanpresentedwithfever,severeabdominalpain,anddistension.Shehadbeeninstitutionalizedfordepressionandseniledementia.Laboratoryexaminationsdisclosedaleucocytosis(WBC:12500/μL)andelevatedlevelsofserumC-reactiveprotein(2.8mEq/L).Diagnosisofacutececalvolvuluswasmadefroma'coffeebeansign'onanabdominalcomputedtomographyanda'beaksign'onagastrographinenema.Anemergentlaparotomyconfirmedthediagnosisandanileo-colectomywithprimaryanastomosiswascarriedout.Thepatientrecoveredafterintensiverespiratorycareandfluidtherapy,andthenreturnedtoherformerinstitution.AreviewofJapaneseliteraturedisclosedthat:(1)amarkedincreaseofagedpatientswithmentaldisabilitypresentingwithcecalvolvulus,(2)adoptionofileo-colectomyasthestandardsurgicalprocedure,and(3)improvedsurvivalofthepatients,wereobservedinthelastdecade.

简介：Congenitalcholesteatoma(CC)isararelyseenbenigntumorofthetemporalbone.Therearefivegeneralsitesofextraduraloccurrence:themiddleear,externalauditorymeatus,mastoid,squamousportionandthepetrousapexofthetemporalbone.CCgrowsslowlyandpresentsnosymptomsattheearlystage.Delayedandmis-diagnosisarecommonwiththiscondition~1.CasereportA10-year-oldboypresentedwitha3-monthhistoryofhearinglossonrightside.Therewasnohistoryofotorrhea,facialpalsy,previousotologicalproceduresortrauma.Otoscopyrevealedabulgingposterosuperiorquadrantintheotherwiseintactrighttympanicmembrane(Fig.1).Puretoneaudiometryshowedanaveragethresholdof51dBfor500,1000,2000and4000Hz,witha40dBair-bonegap,suggestingamoderateconductivehearingloss(Fig.4).CTscanofthetemporalboneshowedanisolatedsofttissuedensitylesioninthemiddleear(Fig.2).

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简介：AbstractIntroduction:Nicolau syndrome is a rare complication following intramuscular injections of various drugs. It is characterized by severe pain, skin and sometimes muscle necrosis after drug injection. However the pathogenesis of Nicolau syndrome remains unclear.Here, we report a case of Nicolau syndrome following metamizole injection.Case presentation:A 35-year-old female presented with an painful necrotic ulcer who developed Nicolau syndrome after an intramuscular injection of metamizole in her right thigh due to reccurent episodes of back pain. This reaction is typical for Nicolau syndrome.Discussion:The most common finding of Nicolau syndrome in the early period was severe pain at the injection site. Other common findings were skin discoloration, tenderness, and swelling; indurated livedoid plaques; and erythematous, ecchymotic and purpuric patches. The histopathological results of the case with Nicolau syndrome showed formation of ulceration and fat necrosis.Conclusion:Although Nicolau syndrome is an uncommon cutaneous adverse reaction, clinicians should be aware of this complication to prevent or minimize the severity of Nicolau syndrome.

简介：ObjectiveTostudyclinicalcharacteristicsofendolymphaticsactumor(ELST)anditsdiagnosisandtreatment.MethodsELSTwasdiagnosedin6casesbasedonsurgicalandhistologicalfindings.Thesecaseswerereviewedfortheirclinicalmanifestations,differentialdiagnosisandsurgicaltreatmenttechniques.ResultsTherewere1maleand5femalesinthisgroup,agedfrom28to59years(meanage=38.7years).Thetumorwasinleftearin4casesandinrightearintheother2cases.Diseasecoursesrangedfrom5to30years(meanduration=12.6years).Clinicalpresentationsincludedsensorineuralhearingloss(n=4),otorrheaandtinnitus(n=2),tinnitusandfacialspasm(n=1),otorrheawithfacialparalysis(n=1),andhearinglosswithtinnitus(n=2).NoneofthecaseswasdiagnosedasELSTpreoperatively.Twocasesweremisdiagnosedasglomusjugularetumor,2aschronicsuppurativeotitismedia,1assweatglandadenomaonbiopsyand1astemporalbonetumor.Tumorsweresurgicallyresectedinall6casesviathemastoidectomy(n=2)orcombinedoto-cervicalorcranio-oto-cervicalapproaches(n=4).Postoperativecerebrospinalfluidotorrheaoccurredin1case.Thetumorswereconfirmedonhistologicalexaminationtobealow-gradeadenocarcinoma.Allpatientshavesurvivedatthetimeofthispaper.ConclusionELSTisrareandcommonlymisdiagnosedandinadequatelytreated.Itsprognosisisrelativelyfavorablebecauseofitsslowgrowthrate.

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简介：AbstractIntroduction:Basal cell carcinoma (BCC) is the most common human malignancy commonly in white people, but in less than 1% of cases it may appear on unexposed areas, like the perineal and anal regions. Vulvar BCC is often diagnosed late because it grows slowly and tends to be asymptomatic, with no specific physical findings. Here, we present a rare Latin patient with a 10-year history of vulvar BCC with dermatoscopic and histopathologic findings.Case report:A 65-year-old Latin woman presented with a nodule on her left labium majus, which she first noted 10 years ago, and that had grown slowly but constantly. She also reported a 20-year history of pruritus and occasional pain. Physical examination showed a 3.5 cm × 3.0 cm erythematous lesion with a central rough without inguinal lymphadenopathy. Contact dermatoscopy (Dermalite DL3 Gen.) showed one blurred cluster of arborising vessels in a pinkish background with white homogeneous areas and lines radial converging. An incisional biopsy was realized, and histopathology diagnosed BCC.Discussion:Vulvar BCC is a rare malignancy that affects mainly Caucasian women over 70 years of age. The most common etiology for BCC is ultraviolet radiation, but as the vulva is unexposed its cause is unclear. Mutations in tumor suppressor and regulatory genes such as p53 are present in 50% of cases. Gorlin syndrome, chronic radiation, chronic arsenic exposure, xeroderma pigmentosum, and immunosuppression have been considered as risk factors.Conclusion:Vulvar BCC characteristics are the same as other cutaneous forms, featuring blue ovoid nests and arborizing telangiectasia, confused with inflammatory diseases, such as eczema, psoriasis, and chronic infection (especially if it is accompanied by pruritus), this makes diagnosis complex, with a delay of 5 to 6 years on average, with an average size of 2.1 cm. Diagnosis is enhanced with dermatoscopy, as the preferred treatment for most BCC cases is wide surgical excision.

简介：AbstractIntroduction:Primary systemic amyloidosis is characterized by clonal plasma cell disorder, and its signs and symptoms are various and complex, damage to the skin and mucous membrane is often more likely to attract attention.Here we reported a case of a 61-year-old male patient who presented with topical mucocutaneous lesion, as well unusual skin vegetations.Case presentation:A 61-year-old man was hospitalized due to repeated burning sensation on his back, multiple ecchymosis, and skin vegetations. Through a series of examinations (mainly including skin histopathology, bone marrow cytology, bone marrow flow cytometry, immunofixation electrophoresis), Primary systemic amyloidosis was diagnosed, but multiple myeloma could not be diagnosed. Subsequently, he received chemotherapy. In the half-year follow-up, there was no significant change in his symptoms and signs.Discussion:In this case, in addition to the typical skin damage of primary amyloidosis, the multiple skin vegetations in the buttocks, abdomen, and arms are particularly noteworthy. According to the histopathology and Immunohistochemistry of the skin vegetation, we infer that the formation mechanism of these skin vegetation is lymphatic obstruction caused by amyloid, which leads to lymphatic dilatation, lymph leakage, and dermal edema.Conclusion:Primary systemic amyloidosis is a rare disease, which is often difficult to diagnose. We should be alert to those atypical skin features so as not to delay diagnosis.

简介：AbstractIntroduction:Basal cell carcinoma (BCC) is the most common skin cancer which mainly affects the population over 50 years of age. In addition to surgical treatment, nonsurgical treatment is also an attractive option for some patients.We herein report a case of an 82-year-old man with BCC successfully treated with acitretin.Case presentation:An 82-year-old man presented with BCC on his left nose wing more than 2 years ago. Due to his unwillingness to accept treatment that may lead to pain, discomfort, or trauma, the patient was prescribed oral acitretin 25 mg twice daily [0.8 mg/(kg·d)] and was instructed to apply 2% fusidic acid cream topically once daily for trauma protection. The lesion progressively shrank in size after 4 weeks of treatment, and was almost completely resolved after 28 weeks of follow-up. The patient reported mild adverse effects, such as mild skin fragility and cheilitis, and apparent scaling skin, which caused minor discomfort but did not affect the continuation of treatment.Discussion:The pathogenesis of BCC is still unclear, but it has been demonstrated to be linked to overactive hedgehog signaling and its crosstalk with other pathways such as phosphoinositide 3-kinase and mammalian target of rapamycin. Acitretin could obviously inhibit cell growth and proliferation and down-regulate AMP-dependent protein kinases that plays critical role in the blocking of malignant progression of several tumors including BCC.Conclusion:We provide an effective alternative for the patients with BCC who are unwilling to receive surgical therapy.

简介：AbstractIntroduction:Malignant syphilis (MS) is a rare and severe variant of secondary syphilis that is frequently associated with HIV infection. The clinical impact of HIV and syphilis co-infection is bidirectional, and the presence of MS is associated with acute HIV infection. Clinically, MS is characterized by nodular and ulcerative lesions affecting the trunk and extremities, which are covered with thick crusts. The treatment of choice for MS remains benzathine penicillin G. Herein, we report a case in which MS was the initial presentation of HIV infection.Case presentation:A 35-year-old male patient came with the chief complaint of extensive erythematous nodular rash for the past one month, with notable ulceration on some lesions with annular configuration along with fever and malaise. Based on the abovementioned clinical, laboratory, and histopathologic findings, the patient was diagnosed with MS and HIV. The diagnosis was established based on histopathological examination and syphilis serological testing.Discussion:Cutaneous disorders are a frequent presenting feature of HIV infection. The clinical manifestations of syphilis in immunosuppressed patients are often severe and/or atypical. The patient was then treated with weekly intramuscular administration of 2.4 million units of benzathine penicillin G for 3 weeks and achieved rapid and significant clinical improvement, with no Jarisch-Herxheimer reaction.Conclusion:Co-infection with syphilis and HIV alters the course of both diseases, and most HIV-infected patients with syphilis have typical disease manifestations associated with decreased CD4+ T-cell counts.