From the skin to the eye
Post on 30-Dec-2016
ChurgStrauss syndrome is a rare vasculitis of the medium to smallsized vessels, ANCA-associated. Diagnosis is mainly clinical, withndings of asthma, rhinosinusitis, eosinophilia and signs of vasculitisin major organs. We report a case of a 28-year-old Caucasian male,with frequent emergency room visits due to a difcult to treatasthma. He was admitted with dyspnoea, epigastric pain, asthenia,weight loss and myalgias of the lower limbs with motion limitation.Clinical and laboratory ndings were suggestive of ChurgStrausssyndrome. Gastric biopsy with important eosinophilic inltrationcorroborated the diagnosis. In spite of its low frequency, ChurgStrauss syndrome should be considered in the differential diagnosisof difcult to treat asthma, due to its poor prognosis, if not correctlytreated, and good response to corticoids and immunossuppression.
ID: 290Clinical characterization of annular erythema in European whitepatients with primary Sjgren syndromeS. Retamozoa, P. Brito-Zerona, M. Akasbib, M. Gandiac,M. Perez-De-Lisd, M.J. Soto-Cardenasc, B. Kostove, A. Bovea,R. Perez-Alvarezd, A. Siso-Almiralle
aSjgren Syndrome Research Group (AGAUR), Laboratory of AutoimmuneDiseases Josep Font, Institut dInvestigacions Biomdiques August Pi iSunyer (IDIBAPS), Department of Autoimmune Diseases, Hospital Clinic,Barcelona, SpainbDepartment of Internal Medicine, Hospital Infanta Leonor, Madrid, SpaincDepartment of Internal Medicine, University of Cadiz, Hospital Puerta delMar, Cadiz, SpaindDepartment of Internal Medicine, Hospital do Meixoeiro, Vigo, SpainePrimary Care Research Group, IDIBAPS, Centre dAssistncia Primria ABSLes Corts, GESCLINIC, Barcelona, Spain
Objective: To evaluate the prevalence and characterize the mainepidemiological, clinical and immunological features of annular erythe-ma (AE) in a large cohort of white patients with primary Sjgrensyndrome (SS). Methods: Three hundred and seventy-seven consecu-tive patients who fullled the 2002 classication criteria for primary SSwere evaluated between 1995 and 2012. The main epidemiological,clinical, immunological and histopathological features related to AEwere retrospectively collected. As a control group, we selectedconsecutive patients with cutaneous vasculitis diagnosed during thesame follow-up period. Results: Thirty ve (9%) patients withprimary SS were diagnosed with AE. All were white females, with amean age of 47 years at diagnosis of AE. AE preceded diagnosis of SSin 27 (77%) patients. Cutaneous AE lesions involved principally theface and upper extremities. Immunological markers consisted ofpositive ANA and antiRo/La antibodies in 31 (89%) patients. Patientswith AE had a lower frequency of glandular involvement (6% vs. 54%,p b 0.001), peripheral nerve involvement (6% vs. 61% p b 0.001),renal involvement (0% vs. 14% p = 0.042), positive RF (33% vs. 68%,p = 0.017) and cryoglobulins (8% vs. 37%, p = 0.02) in comparisonwith patients diagnosed with cutaneous vasculitis. Multivariate logisticregression analysis identied glandular involvement (p= 0.023),peripheral neuropathy (p= 0.007) and positive RF (p= 0.023) asindependent statistically-signicant variables negatively associatedwith AE. Conclusion: AE was the most frequent inammatorycutaneous involvement in a large series of white patients with primarySS and showed very specic features, including frequent developmentbefore SS diagnosis, an overwhelming association with anti-Roantibodies and a weak association with the main systemic SS-relatedfeatures and other immunological markers (cryoglobulins, RF). AE
should not be considered as a cutaneous feature exclusive to Asianpatients with primary SS.
ID: 335From the skin to the eyeD. Pinto, A.M. Grilo, M. Lopes, J. Vaz
Internal Medicine I, Jos Joaquim Fernandes Hospital, Beja, Portugal
Background: Erythema nodosum represents an early sign of avariety of infections, connective tissue diseases, and other inam-matory disorders. The majorities of patients have evidence of recentstreptococcal infection or have no identiable causes. Thus, carefuldiagnostic assessment constitutes the most important aspect ofmanagement for these patients. Methods and results: The authorspresent a case of a 49 year old female with no relevant past medicalhistory or medication. She was referred to our hospital, presentingwith painful, erythematous nodules, purple in colour, on the anteriorsurfaces of the legs, thighs and arms, with an evolution of 48 h. Shedenied other complains, such as fever, arthralgia, myalgias, xeroph-thalmia and xerostomia. She was medicated with acetylsalicylic acid,and referred to the consultation. An exhaustive differential diagnosiswas performed, demonstrating presence of ANA and anti-SSA-Roantibodies. After 6 months of follow-up, a slow regression of thelesion was observed, and the patient started referring complainssuggestive of Sjgren Syndrome. Conclusion: Erythema nodosum ispresumed to be a delayed hypersensitivity reaction to a variety ofantigens. It may occur in association with several systemic diseasesor drug therapies, or it may be idiopathic. The inammatory reactionoccurs in the panniculus. Age and sex distributions vary accordingto etiology and geographic location. After presenting the case, theauthor makes a brief theoretical review.
ID: 349Systemic sclerosis in a case of ovarian cancer Paraneoplasticphenomenon or two different entities?E. Balasescua, L.M. Rusua, C.G. Badeab, S.M. Marinescua,T. Maturac, M. Obrejad
aInternal Medicine, CMDT, Bucharest, Romaniab2nd Medical Clinic, Colentina Clinical Hospital, Bucharest, RomaniacRadiology, CMDT, Bucharest, RomaniadCardiology, CMDT, Bucharest, Romania
Although Scleroderma falls into the paraneoplastically question-able autoimmune disease group with coutaneous manifestations,medical literature mentions Scleroderma cases previously to canceroccurrence or evolving concurrently with cancer and its complicat-ions.It is worth bringing into discussion the case of a 57-year oldwoman who manifested the rst symptoms of Scleroderma a yearbefore her death. The disease had a rapidly progressive evolutionmost probably in the context of a relapse of an ovarian canceradequately treated surgically and chemotherapeutically four yearsago.
Outline placeholderClinical characterization of annular erythema in European white patients with primary Sjgren s.....From the skin to the eyeSystemic sclerosis in a case of ovarian cancer Paraneoplastic phenomenon or two different ent.....