Castleman hastalığı, nadir görülen bir lenfoproliferatif patolojidir. Localize ve yay- gın olmak üzere iki farklı klinik tablo ile ortaya çıkabilmektedir. Histopatolojik ola- rak ise hyaline-vasküler tip, plazma hücreli tip ve mikst tip olmak üzere 3 grup- ta değerlendirilir. Olgular sıklıkla asemptomatik seyretmekte olup radyolojik bul- gularla şüphe edilerek tanı konulur. Radyolojik olarak en sık prezentasyonu medi- astinal alanda dev lenf nodu şeklindedir. Lenfadenomegali en sık paratrakeal lenf nodlarında izlenir. Bu nadir görülen patolojiyi vurgulamak ve farklı radyolojik gö- rünümü ile özellik arz eden bir olgu ile bu hastalığı hatırlatmak istedik.
ABSTRACT – Bowen's disease consists of a squamous cell carcinoma in situ and the pigmented form of the disease is anunusualpresentation. The authors describe a case of pigmented Bowen's disease in a male patient, 56 years, withan asymptomatic hyperpigmented plaque on the anterior surface of the right thigh for 10 years. Histopathological examination showed atypical keratinocytes on the entire epidermis with loss of polarity, melanin pigmentation in the basal layer and lichenoid lymphocytic infiltrate. A complete excision of the lesion with wide margins was made with no recurrence. Although relatively rare, pigmented Bowen's disease should always be remembered and included in the differential diagnosis of melanoma and other pigmented lesions.
He was born at 39 week’s gestational age with a birth weight of 3.5 kg. The pregnancy was uncomplicated. Vomiting began from ten days of age. By the time of admission, at 12 months of age, the patient had intermittent non projectile, non bilious vomiting that occurred 1h to 2h after feeding. She appeared hungry immediately after vomiting. The patient had been offered several different formulas and anti reflux treatments, but these did not result in symptomatic improvement. Physical examination on admission revealed slight upper abdominal distention. The infant admission weight was 8.5 kg, and otherwise was healthy. Laboratory studies were within normal limits.
A second skin biopsy was repeated and showed dermis with mild fibrosis and lympho-histiocytic infiltrates, focal aspects compatible with vasculitis, sparse cells CD117 and tryptase positive (20/HPF) located pre- dominantly in perivascular seat and eosinophils (Figu - re 2). A bone marrow biopsy was repeated and allowed us to definitively rule out the diagnosis of mastocitosis. On the basis of the patient’s history, laboratory tests and skin biopsy results, which were compatible with cuta- neous eosinophilic vasculitis, a diagnosis of EGPA was made. Specific therapy with methotre xate was under- taken obtaining a complete remission of the patient’s skin lesions. After an 18 month follow-up the patient is on maintenance therapy with weekly do ses of methotrexate (10 mg/week). She is still asympto matic and her blood tests are completely normal. ANCA- -MPO remain positive, but at a borderline level.
The pathology of the case at issue has a very suggestive clinical history, confirmed by the copper and ceruloplasmin serum dosage and hair analysis. The skull tomography displayed considerable white matter lesion at two months of life, and included a differential diagnosis regarding other leukodys- trophies. Brain magnetic resonance at four months showed intense bilateral damaging of white matter, very similar to diseases primarily affecting white matter. Usually radiological studies of patients with Menkes disease show cerebral atrophy, large sub- dural collections, and tortuosity of cerebral blood vessels. The neuroimaging study using brain magne- tic resonance shows local hypodensity areas, atrophy, necrosis, and gliosis of white matter that may be identified before the phenotypic alterations 1,11,12 . In
Introduction. Modern knowlegde defines Mikulicz´s dis- ease as a part of immunoglobulin G4-related disease. The main feature is the presence of lymphoplasmacytic infil- trates, immunoglobulin G4 plasma cells positivity, distinc- tive storiform fibrosis and moderate eosinophilia. Case re- port. A 59-years old male presented with a mild keratocon- juctivitis sicca and enlarged lacrimal and salivary glands dur- ing the last two years. Althought clinical presentation of the patient was typical, earlier testing did not pinpoint Miku- licz´sdisease. By typical clinical presentation, elevated se- rum immunoglobulin G4 level and histopathological finding of lacrimal glands tissue we diagnosed Mikulicz´sdisease successfully treated with corticosteroid therapy. Conclu- sion. We reported the first case of IgG4-related Mikulicz´sdisease in Serbia. Our report highlights IgG4-related Miku- licz`sdisease as an important differential diagnosis with Sjögren`s syndrome and lymphoproliferative disease in rheumatological practice.
this condition is serious and olmesartan is commonly pre- scribed for the treatment of hypertension. Negative serology for celiac disease and absence of response to a gluten-free diet on patients taking olmesartan should aware clinicians for the possibility of this condition. We show iconogra- phy namely capsule endoscopic images, that document the endoscopic ﬁndings associated with this entity and can add information on the hallmarks of this entity. Increasing reports of this drug-induced enteropathy may lead to the identiﬁcation of more cases, a better characterization of this entity and an earlier diagnosis, with obvious beneﬁts to patients.
An 87-year-old right-handed Afro-Brazilian male with four years of schooling, was first referred to the dementia outpatient division of the Hospital das Clínicas of the Fed- eral University of Goiás in December 2006. He presented with progressive cognitive deterioration, mainly memory impairment and executive dysfunction (difficulties in plan- ning, sequencing, abstraction and goal-directed behavior), as well as a history of personality changes and depressive traits that began in 2002. At this time, he had a CDR of 1 and no trichotillomania.
Syphilis, Treponema pallidum infection, with its numerous presenta- tions has been nicknamed “the great imitator”. Potential ophthalmic mani- festations are many and can aid in pathogen identification; however, isola- ted vitreitis has rarely been described (1-3) . Although not infrequent, move-
Finally, as previously mentioned, MBL is a precursor of virtually all cases of CLL and the diagnosis of the latter is established when >5.0 × 10 3 /L monoclonal BL are detected in PB. However, clinical data supporting this arbitrary labo- ratory cut-off value is lacking. Nevertheless, it is powerful enough to stratify patients between having the diagnosis of MBL, a benign condition despite its potential to progress to malignancy, against patients with CLL/SLL. In fact, 1–2% of “high-count” MBL patients progress to overt CLL/SLL per year. 1 In our case, although a lymph node biopsy is required
pathomechanism remain elusive. In general, the skin changes are symmetric and include an initial edema and erythema of the extremities, followed by ‘peau d’orange’ and woody skin induration in a later phase. The skin indu- ration can lead to joint contractures with limited mobili- ty. Laboratory tests may show hypergammaglobulinemia, peripheral eosinophilia and an elevated erythrocyte sed- imentation rate 2 . Clinical pictures of lower motor neu-
FIGure 2. MRI imaging from November 2019 – Definite diagnosis of Parenchymal Neuro-Behcet Diseasewith migration (development and disappearance) of lesions. A: Axial T1 - Hyperintense lesion at the left mesencephalon extending to crus cerebri and the posterior limb of capsula interna, with reduced volume and reduced mass-effect in comparison with previous images. B: Axial T1 and D:Axial T2 – Newly formed hyperintence lesion in right basal ganglia with edema and mass-effect; C: Coronal T1 – Newly formed hyperintence lesion in right basal ganglia with edema and mass-effect; new smaller lesions in left thalamus and in the right cortical area
To our knowledge, this is the first report of full thickness skin burns following ESWL for renal calculi. There have been two previously re- ported cases of partial thickness skin burns follo- wing ESWL. One was of a first degree skin burn with erythema, bruising and slight pain which was treated with Thrombocid® (0.1%) (4). The second report by Sur et al. (5) was a case of a second degree skin burn following ESWL using a Medispec® EM1000 lithotripter. The patient had two renal calculi and a total of 4,000 shocks were delivered. Conventional ultrasound gel was used instead of the Medispec-recommended coupling medium, Lithoclear® gel. The authors specula- te that the skin burns could be attributed to the
The article analysis followed previously determined eligibility criteria. The studies must have met all the following criteria for inclusion: (1) manuscripts written in English; (2) articles addressing atypical lesions of cutaneous leishmaniasis; and (3) prospective or retrospective observational (analytical or descriptive), experimental or quasi-experimental studies, or casereport. Considering the scarce of data about atypical manifestations of CL, and that the cases are publicized in the literature mainly as case reports, we decided to include this type of publication in our search in order to better explore the uncommon lesions. The full text of the selected articles (sample) were obtained directly from the aforementioned databases, when freely available, or through Coordination of Improvement of Higher Education Personnel (CAPES) Portal of Journals, a virtual library linked to Brazil’s Ministry of Education and subjected to content subscription. We adopted the following exclusion criteria: (1) repeated articles in diﬀerent databases; (2) non-original studies, including editorials, re- views, prefaces, brief communications, and letters to the editor; (3) full text not available; (4) author’s name not disclosed; and (5) out of context. Each paper in the sample was read in its entirety, and data elements were then extracted and entered into a spreadsheet that included authors, publication year, description of the study sample, and main ﬁndings.
She reported having completed the second round of PB- MDT, but the lesion only improved after using higher doses of prednisone. She was examined in a different dermatology clinic and, after a third biopsy, a diagnosis of leprosy type 1 reaction was made, and she was directed to continue with 40mg/day of prednisone. Physical examination revealed an ulcerated lesion on the face (Figure 2) and painful discrete submandibular and cervical lymphadenopathy, with no other changes, associated with low-grade fever. The following diagnostic possibilities were considered: dimorphic leprosy with necrotic type 1 reaction, co-infection (leprosy and PMC), opportunistic infection due to immunosuppression by prolonged corticosteroid therapy, or another chronic granulomatous disease that had been misdiagnosed as leprosy since the beginning.
The presently accepted classification systems of capi- tellar fractures are descriptive and not treatment-direc- ted . Bryan and Morrey classified capitellar fractures as Type 1, 2 and 3 . Type 1, often referred to as the Hahn-Steinthal fracture, is a shear fracture in the coro- nal plane involving most of the capitellum and little or none of the trochlea. Type 2, called the Kocher-Lorenz fracture, involves a variable amount of articular cartilage of the capitellum with minimal attached subchondral bone. Type 3 is a comminuted or compression fracture of the capitellum. McKee and colleagues described a fourth type, a shear fracture of the distal aspect of the humerus in the coronal plane including the capitellum and most of the trochlea (Figure 1) . Ring and col- leagues reported another descriptive classification for distal humeral articular fractures and, recently, Dubber- ley and colleagues proposed a novel classification system . As a result of the rarity of capitellar fractures in children, studies focusing on this injury are insufficient. Furthermore, the administration of treatment for this type of injury remains controversial with regards to young children [5-8,13].
Cervical adenitis >1.5cm in diameter is the less frequently observed criteria in patients with Kawasaki disease and it is usually found in association with other symptoms during the acute phase. Moreover, the finding of fever and lymphadenitis with intense local signs of inflammation and phlegmon is rarely seen as the initial manifestation of Kawasaki disease. We report the case of a 7-year-old boy who had cervical lymphadenitis with adjacent cellulitis and phlegmon mimicking bacterial adenitis as the first presentation of Kawasaki disease. The patient had fever, cervical lymphadenitis with adjacent cellulitis, and severe headache. Cefadroxil was prescribed based on the clinical diagnosis of bacterial adenitis. Because he remained febrile and phlogistic signs worsened, after 1 day of hospitalization, antibiotics were administrated intravenously (ceftriaxone and oxacillin). The computed tomography of the neck showed primary infectious/ inflammatory process. On the fourth day, the patient had dry and scaly lips, and treatment with oxacillin was replaced by clindamycin because the patient was still febrile. On the ninth day, he presented non-exudative bilateral conjunctival injection. On the tenth day of febrile disease, a rash appeared on his trunk, hands and feet. Patient’s symptoms resolved after intravenous administration of immunoglobulin (2g/kg/dose), and he was discharged 2 days later. On the 14th day, the patient had lamellar desquamation of fingers. Kawasaki disease should be considered as a differential diagnosis in children with febrile cervical lymphadenitis unresponsive to empiric antibiotics even if they have adjacent cellulitis and phlegmon.
presented with signs of hypothyroidism, complaints of erec- tile dysfunction (just like our case) or galactorrhoea and had good response to antimicrobial and hormonal replacement therapy. Pyramidal and sensation deficits are other signs of CNS derangement recorded in WD, just as myoclonus and seizures, which are noticed in almost one fourth of patients.Additionally, gait and stance instability related to cerebellar damage occur in 20% of patients, being a major source of functional impair- ment 8 . A few cases presenting w ith rapidly evolving cranial
Gallbladder rupture following blunt abdominal trauma is a rare event recognized on evaluation and treatment of other visceral injuries during laparotomy. Isolated gallbladder rupture secondary to blunt abdominal trauma is even more uncommon. The clinical presentation of gallbladder injury is variable, resulting in a delay in diagnosis and treatment. We report the case of a patient who suffered an isolated gallbladder rupture due to blunt abdominal trauma.
comprises virtually all painful conditions of the nails such as subungual warts, keratoacanthoma, subungual exostosis, enchondroma, neuroma, Pacinian neuroma, caliber-persistent artery, leiomyoma, paronychia, osteitis terminalis, subungual felon, herpetic whitlow, causalgia, gout, melanoma and several more. In contrast, glomangioma is often multiple, sometimes in linear distribution involving an extremity including the periungual skin, usually not painful or only tender on deep palpation, and the main clinical differential diagnosis is venous malformation or blue rubber bleb angiomatosis. Caliber-persistent artery is also known as Dieulafoy's lesion, cirsoid aneurysm or submucosal arterial malformation. It is an acquired or inborn lesion where the terminal artery caliber does not diminish with each branching but remains wide. It is common in the intestinal tract and may be the reason for dramatic or even fatal gastrointestinal bleeding with a lethality of 60%, but it is rare in skin. Its main cutaneous localization is the lower lip. Two lesions causing a split nail were histologically diagnosed as subungual caliber persistent artery. This again bears some clinical resemblance to Wollina's case.