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Rare Manifestation (rare + manifestation)
Selected AbstractsMultiple hepatic nodules: Rare manifestation of clonorchiasisJOURNAL OF GASTROENTEROLOGY AND HEPATOLOGY, Issue 9 2006Wei-Chih Liao Abstract A 38-year-old man was admitted due to intermittent right upper quadrant pain for 1 month. Leukocytosis with marked eosionphilia and elevated serum alkaline phosphatase were noted. Stool examinations revealed no parasites or ova. Ultrasonography and computed tomography disclosed multiple hepatic tumors. Biopsy of the hepatic tumor was performed due to non-conclusive imaging studies and revealed eosinophil infiltration in portal areas only. Endoscopic retrograde cholangiography showed mild dilatation with irregularity of bilateral intrahepatic ducts, compatible with chronic cholangitis. Bile was aspirated and biliary lavage with normal saline was performed during endoscopy-guided biliary cannulation. Microscopic examination of the aspirate showed the characteristic ova of Clonorchis sinensis. The patient received Praziquantel therapy for 1 day. Abdominal pain reduced in intensity gradually. Eosinophilia and multiple hepatic lesions resolved after adequate treatment of Clonorchis sinensis. The rare manifestation of multiple hepatic tumors in Clonorchis sinensis should be differentiated from other primary or metastatic neoplasms, while biliary lavage for parasite ova is a valuable diagnostic tool when stool examination is negative. [source] Disseminated intravascular coagulation in acute leukemia: clinical and laboratory features at presentationEUROPEAN JOURNAL OF HAEMATOLOGY, Issue 4 2006Masamitsu Yanada Abstract:,Background:,Although there are two major scoring systems for the clinical diagnosis of disseminated intravascular coagulation (DIC), the validity of these systems for leukemia-associated DIC remains to be confirmed. Methods:,By analyzing 125 newly diagnosed acute leukemia patients, we investigated clinical and laboratory features of leukemia-associated DIC, and determined the validity of the two established criteria. Results:,A total of 36 patients (29%) were diagnosed with DIC according to expert opinion, a method regarded as the de facto gold standard. Leukemia-associated DIC is characterized by rare manifestation of organ failure because of thrombosis and no relevance of the platelet count for the diagnosis. The results of receiver operating characteristics analysis favored fibrin degradation product (FDP) rather than D-dimer as the fibrin-related marker test. Although prothrombin time, plasma fibrinogen, and serum FDP levels were significantly different for patients with and without DIC, multivariate analysis identified FDP levels to be the only factor associated with DIC diagnosis. The cut-off level of 15 ,g/mL for FDP was found to be the most effective to differentiate DIC from non-DIC, resulting in diagnostic sensitivity and specificity of 92% and 96%, respectively. The diagnostic results for our patients produced with this FDP-based system were at least comparable with or superior to those obtained with the two currently available scoring systems. Conclusions:,Our findings suggest that an FDP-based criterion may be applicable for the diagnosis of leukemia-associated DIC. Although it appears to be simple and practicable enough for clinical use, prospective validation of this criterion is needed. [source] PET-CT imaging of combined brachial and lumbosacral neurolymphomatosisEUROPEAN JOURNAL OF HAEMATOLOGY, Issue 1 2005Pazit Kanter Abstract:, Neurolymphomatosis is a rare manifestation of progressive non-Hodgkin's lymphoma. A 44-yr-old man with diffuse large B-cell lymphoma presented with unilateral progressive peripheral sensorimotor neuropathy after the 7th cycle of R-CHOP (rituximab, cyclophosphamide, doxorubicin, vincristine and prednisone) therapy. No pathology in the nervous system was evident by computerized tomography (CT), magnetic resonance imaging (MRI) of the head, spinal axis and plexuses and by repeated analysis of cerebrospinal fluid. However, the hybrid modality of positron emission tomography (PET) of fluorinated deoxyglucose (FDG) combined with CT scan (PET-CT) showed unilateral involvement of both the brachial and lumbosacral nervous plexuses. A complete recovery of neurological manifestations and normalization of PET-CT followed intensive chemotherapy with autologous stem cell transplantation. The diagnosis and localization of neurolymphomatosis may be supported by PET-CT imaging. [source] Dry type leishmanial lymphadenitis presented as two large parotid and cervical massesINTERNATIONAL JOURNAL OF DERMATOLOGY, Issue 7 2007I. Esfandiarpour MD Background, Cutaneous leishmanisis (CL) is a common disease in Iran, particularly in Kerman and Bam and Kerman province. Lymphadenitis resulting from leishmania tropica (dry type) with, or without, cutaneous lesion is rare. Localized leishmanial lymphadenitis (LLA) is a specific clinico-pathologic presentation of inflammatory changes caused by leishmanial parasites or antigen within an isolated lymph node without any systemic manifestation. Case report, A 55-year-old Iranian woman presented with two slow growing large nodules (masses) on the left preauricular and the left cervical areas. The nodules were large, painless, mobile, multilobulated, and associated with a small skin papule on the left-side of the cheek distal to the masses. Results, Histopathologic examination of both the skin lesion and the lymph nodes suggested the leishmanial etiology of skin papule and lymphadenitis. The Leishman-bodies (amastigotes) were demonstrated in two lymph nodes and a skin lesion. The clinical picture plus pathological finding and the response to meglumine-antimoniate confirmed LLA. Conclusion, Lymph node involvement is another rare manifestation of dissemination of infection with dermotropic leishmania. This presentation of CL should not be treated with the ordinary local treatments such as curettage, cryotherapy or surgical excision. [source] Bile duct varices presenting as a porta hepatis massJOURNAL OF MEDICAL IMAGING AND RADIATION ONCOLOGY, Issue 5 2005MK House Summary Bile duct varices are a rare manifestation of portal vein cavernous transformation. They can present as an apparent enhancing, vascular mass in the porta hepatis and are readily diagnosed using ultrasound and CT modalities. Diagnosis is important to avoid unnecessary intervention and concomitant risk of haemobilia. [source] Trichotillomania ± trichobezoar: revisitedJOURNAL OF THE EUROPEAN ACADEMY OF DERMATOLOGY & VENEREOLOGY, Issue 8 2006VN Sehgal Abstract Trichotillomania is an intriguing psychosomatic entity in which there is an irresistible desire to pull out the hair from the scalp, eyelashes, eyebrows and other parts of the body. The process results in an instant release of tension, a sense of relief and security. However, non-scaring alopecia is its clinical presentation. The development of trichobezoar following ingestion of the pulled hair is its salient complication in a few cases. Subsequently, it may cause symptoms pertaining to the gastrointestinal tract culminating in intestinal obstruction, perforation, pancreatitis and obstructive jaundice. The Rapunzel syndrome (trichobezoar) may occur when gastrointestinal obstruction is produced by a rare manifestation of a trichobezoar with a long tail that extends to or beyond the ileocecal valve. In most cases in children, trichotillomania ± trichobezoar is a habit disorder and thus has a better prognosis. However, in adults the psychopathology is usually deeper and thus entails a poor prognosis. The diagnosis is made after taking a thorough history, noting the clinical features and evaluating a hair-root examination, where telogen hair is (almost) completely lacking, which distinguish trichotillomania from other hair disorders. Treatment modalities vary in childhood and adult varieties. Apart from psychotherapy, the drug treatment involves several agents including selective serotonin reuptake inhibitors (SSRIs) and domipramine. Trichobezoar/Rapunzel syndrome requires surgical intervention. [source] Laryngeal histoplasmosis in an immunocompetent patient from a non-endemic region: case reportMYCOSES, Issue 6 2009Teresa C. A. Ferrari Histoplasma capsulatum infection involving the larynx is a rare manifestation, especially in immunocompetent individuals and a high index of suspicion is needed to establish the diagnosis correctly. We report a case of a 50-year-old Brazilian man who presented with progressive hoarseness and throat pain for 4 months. Laryngoscopy showed a supraglottic vegetant lesion, and the biopsies chronic granulomatous inflammation without any specific agent. A second laryngoscopy with biopsies was performed and after 17 days of incubation in specific medium, H. capsulatum was isolated. The patient was successfully treated with amphotericin B. [source] Fournier's gangrene and scrotal ulcerations during all-trans-retinoic acid therapy for acute promyelocytic leukemiaPEDIATRIC BLOOD & CANCER, Issue 2 2008Rahul Naithani MD Abstract Scrotal ulcers are a rare manifestation in patients with acute promyelocytic leukemia. Fournier's gangrene (FG) is even rarer. We describe three adolescents and young adults who developed scrotal ulcerations during induction with all-trans-retinoic acid. One patient developed FG. These lesions are predominantly seen in Asian population. A good outcome with supportive management occurred in all the cases. Pediatr Blood Cancer 2008;51:303,304. © 2008 Wiley-Liss, Inc. [source] Amoebiasis cutis: Clinical suspicion is the key to early diagnosisAUSTRALASIAN JOURNAL OF DERMATOLOGY, Issue 1 2010Ghanshyam K Verma ABSTRACT Amoebiasis cutis is a rare manifestation of Entamoeba histolytica, primarily an intestinal pathogen, which occurs as a complication of amoebic dysentery. Primary cutaneous amoebiasis occurs from contamination of pre-existing wounds. A high degree of clinical suspicion and demonstration of trophozoites from lesions are important for making an early diagnosis lest these patients should suffer significant morbidity. A HIV-negative and otherwise healthy 40-year-old man presented with a well-defined, indurated, painful, progressively enlarging plaque with overlying ulcers and pus discharging sinuses involving buttocks, perianal/perineal area and part of the left thigh of 3 years' duration. A wide array of investigations was unhelpful but demonstration of Entamoeba histolytica trophozoites in wet-drop preparation from the ulcer margin was diagnostic. The trophozoites were also visualized both in H&E and periodic acid Schiff-stained histological sections. Resolution of lesion was observed 2 weeks after treatment with oral metronidazole 800 mg three times a day and wound care. [source] Dystrophic epidermolysis bullosa pruriginosa is not associated with frequent FLG gene mutationsBRITISH JOURNAL OF DERMATOLOGY, Issue 2 2008H. Schumann Summary Background, Dystrophic epidermolysis bullosa pruriginosa (DEB-Pr; OMIM 604129) is a rare manifestation of dystrophic epidermolysis bullosa (DEB), characterized by pruritus, nodular prurigo-like lesions and fragility of the skin mainly in the extremities. Fewer than 40 patients with autosomal dominant or recessive inheritance, or sporadic DEB-Pr, have been described in the literature. Objectives, To disclose mutations in DEB-Pr and to elucidate the role of other pathogenic factors which may influence the pruriginous phenotype. Methods, Seven patients with typical clinical features of DEB-Pr were studied. Results, In all patients, mutations in the gene encoding collagen VII (COL7A1) were disclosed, two of them novel (p.G2623V, p.E2736K). Three mutations were dominant, three recessive and one de novo. In the families with dominant DEB there were one or more members with DEB-Pr, but also at least one affected sibling who did not develop DEB-Pr. In six of seven patients, the clinical history revealed factors that initially induced pruritus, such as atopy, pregnancy, thyroid hormone imbalance, diabetes, infections and contact sensitization. Common filaggrin mutations were ruled out in all patients and normal filaggrin staining was found in the skin samples. Conclusions, DEB-Pr develops as a result of COL7A1 gene mutations and acquired phenotype-modifying factors. Filaggrin mutations did not contribute to the pruriginous phenotype in the present patient cohort. [source] Herpes folliculitis: clinical, histopathological, and molecular pathologic observationsBRITISH JOURNAL OF DERMATOLOGY, Issue 4 2006A. Böer Summary Background, Herpes folliculitis is a rare manifestation of herpes virus infection and it is often misdiagnosed. Diagnostic criteria are not well established, only 24 patients being reported in the literature. Recently it has been suggested that herpetic folliculitis is more common in infections with varicella zoster (VZV) than in those with herpes simplex viruses (HSV-1 and -2). Objectives, To refine diagnostic criteria for folliculitis caused by VZV, HSV-1 and HSV-2, and to study whether follicular involvement enables morphological differentiation between VZV and HSV infections. Patients and methods, Twenty-one patients with herpetic infection of follicular epithelium were assessed clinically and histopathologically. Polymerase chain reaction (PCR) studies for specific DNA of herpes viruses were performed on paraffin-embedded biopsy specimens. Results, In 17 of our cases PCR was positive for VZV, four were positive for HSV-1, none for HSV-2. The clinical presentation of herpes folliculitis often lacked vesicles or pustules (14/21). Histopathological features were often devoid of ballooning (12/21), multinucleated giant cells (12/21) and keratinocytes with steel grey nuclei (15/21). The most consistent findings were lymphocytic folliculitis and perifolliculitis (20/21) and necrotic keratinocytes in follicular epithelium (12/21). In zoster, but not in varicella eruption or HSV infections, follicular involvement was unaccompanied by marked changes in the epidermal surface. Conclusions, In biopsy specimens taken from herpes virus infections, involvement of follicular units is more commonly encountered in VZV infections compared with HSV infections. Early in the course, herpes folliculitis presents as lymphocytic folliculitis devoid of epithelial changes considered to be diagnostic of herpes virus infections. Exclusive involvement of follicles is rather typical of zoster. [source] Orthostatic hypotension associated with dorsal medullary cavernous angiomaACTA NEUROLOGICA SCANDINAVICA, Issue 1 2009J. Idiaquez Background,,, Orthostatic hypotension (OH) is a rare manifestation of medulla oblongata lesions that may be because of interruption of descending sympathoexcitatory axons. Aims,,, To illustrate the location of a medullary lesion that produced OH following resection in relationship to the location of putative sympathoexcitatory pathways. Method,,, A case with dorsal medullary cavernous angioma presenting with OH is described. The possible localization of lesion was compared with distribution of tyrosine hydroxylase (TH)-immunoreactive axons in a comparable section of the medulla of a control brain. Results,,, The patient had marked OH after partial removal of the cavernous angioma. Biopsy confirmed the diagnosis. The magnetic resonance imaging location of the lesion overlapped that of TH-immunoreactive axons of the medullary transtegmental tract. Conclusions,,, A restricted lesion of medullary lesion interrupting the catecholaminergic transtegmental tract arising from the sympathoexcitatory C1 neurons of the rostral ventrolateral medulla could result in severe OH. [source] Cutaneous manifestations of tuberculosisCLINICAL & EXPERIMENTAL DERMATOLOGY, Issue 4 2007J. E. Lai-Cheong Summary Cutaneous involvement is a rare manifestation of tuberculosis (TB). The correct diagnosis is often significantly delayed because cutaneous TB is not routinely considered in the differential diagnosis or because investigations fail to reveal the presence of Mycobacterium tuberculosis. The clinical features of cutaneous TB are diverse, and result from exogenous and endogenous spread of M. tuberculosis and from immune-mediated mechanisms. The recognition of cutaneous TB is important, as the diagnosis is frequently overlooked resulting in delayed treatment. [source] | ||||||||||||||||||||||