Linear Immunoglobulin A Dermatosis: A Rare Case Illustrating Successful Treatment With Dapsone.

This is the case report of a previously healthy four-year-old girl with a history of upper airway infection that was treated with a ß-lactam antibiotic. She was seen in the emergency department one month later with vesiculobullous lesions with clear content that were isolated or grouped in rosettes. Direct immunofluorescence showed baseline linear positivity for immunoglobulin A (IgA) (+) and fibrinogen-positive bullous content with absent remaining immunosera expression. The observed results were compatible with linear IgA bullous dermatosis. After confirming the diagnosis and excluding glucose-6-phosphate dehydrogenase (G6PD) deficiency, dapsone was added to the initial treatment with systemic and topical corticosteroids. This case report is a reminder of the importance of a high index of clinical suspicion for this condition to reach a timely diagnosis.

Large-vessel vasculitis induced by pegfilgrastim.

Granulocyte colony-stimulating factor (G-CSF) is increasingly being used to prevent febrile neutropenia associated with chemotherapy. Large-vessel vasculitis (LVV) has been recognized as a rare side effect of G-CSF treatment. We report a case of G-CSF associated LVV in a patient with breast cancer. While clear pathogenic mechanisms remain unknown, G-CSF may cause vasculitis due to inflammatory cytokines production. This adverse reaction should be recognized in patients with suggestive symptoms following the administration of pegfilgrastim. A 56-year-old woman with luminal B breast cancer who had undergone surgery and adjuvant chemotherapy, initially with paclitaxel, was started on a doxorubicin plus cyclophosphamide protocol, followed by supportive use of long-acting G-CSF pegfilgrastim. Following the administration of pegfilgrastim, the patient developed intermittent fever and was given empiric antibiotics in the outpatient setting with no improvement. There were no signs of cancer progression, and the contrast-enhanced CT scan highlighted wall thickening of the aortic arch and the proximal segment of the subclavian artery, which was not present in previous imaging studies. The patient was diagnosed with LVV, and a differential diagnosis was performed to rule out paraneoplastic setting, immune-mediated diseases, infection or other drug-induced vasculitis. Treatment with steroids was initiated and tapered with significant improvement and resolution of the radiological signs of aortitis.

Heterotopic gastric mucosa in the gallbladder-a rare find.

It is universally known and accepted that the development of a certain type of tissue outside its usual location, like in the gastrointestinal tract, can occur. This is a relatively common situation in the upper region of the gastrointestinal tract. However, the development of gastric mucosa in the gallbladder is a rare find. The following is the case of a 22-year-old male with an 18 mm gallbladder polyp, who electively underwent a laparoscopic cholecystectomy, having been diagnosed at a histopathological level with heterotopic gastric mucosa in the gallbladder. This brief article also aims to provide a reflection on the possible evolution of neoplasms from this histological change, based on the doubts raised in literature.

Facial Paralysis as Initial Manifestation of Light-chain Amyloidosis.

Light-chain (AL) amyloidosis is a systemic disease capable of damaging virtually all body tissues. Neurologic involvement is commonly manifested by dysautonomia and peripheral nervous system affection. However, from 1970 to 2018, only 12 cases of cranial nerve injury associated with AL amyloidosis were identified. Eight months before hospital admission, a previously healthy 61-year-old man complained to his general practitioner of episodes of lipotimia while walking and, three months later, he developed a left facial nerve paralysis assumed, at that time, to be idiopathic. After two months, he started complaining of dyspnea and lower limb edema. Physical examination at admission revealed hypotension, exuberant peripheral edema, jugular venous distention, periorbital purpura and left peripheral facial paralysis. He had elevated troponin and brain natriuretic peptide, mild proteinuria and a monoclonal gammopathy IgG/lambda. Bone marrow biopsy revealed 20% plasmocytes and cardiac ultrasound showed diffuse hypokinesia and restrictive filling pattern. AL amyloidosis with major cardiac involvement was considered and a rectal biopsy revealed amyloid protein. Chemotherapy protocol to AL amyloidosis was initiated but cardiac disease progressed leading to death. Persistent facial nerve paralysis should be considered as a rare initial manifestation of AL amyloidosis allowing an earlier diagnosis.

Disseminated Nocardiosis: A Case Report.

Disseminated nocardiosis is a rare infection associated with underlying immunosuppression, and patients usually have some identifiable risk factor affecting cellular immunity. Due to advances in taxonomy and microbiology identification methods, infections by Nocardia species are more frequent, making the discussion of its approach and choice of antibiotherapy increasingly relevant. A 77-year-old man presented to the emergency department with marked pain on the right lower limb, weakness, and upper leg edema. He had been diagnosed with organized cryptogenic pneumonia one year before and was chronically immunosuppressed with methylprednisolone 32 mg/day. Blood cultures isolated Nocardia cyriacigeorgica. Computed tomography revealed a gas collection in the region of the right iliacus muscle with involvement of the gluteal and obturator muscles upwardly and on the supragenicular plane inferiorly. Triple therapy with imipenem, amikacin, and cotrimoxazole was started, and the patient was submitted for emergent surgical decompression, fasciotomy, and drainage due to acute compartment syndrome. The patient had a good outcome and was discharged from the hospital after 30 days of intravenous therapy. This case illustrates the severity of Nocardia infection and highlights the need for a meticulous approach in the diagnosis and treatment of these patients.

Incidence, predictive factors and clinical significance of development of portal vein thrombosis in cirrhosis: A prospective study.

BACKGROUND AND AIMS: The role of portal vein thrombosis (PVT) in the natural history of cirrhosis is controversial. There are few prospective studies validating risk factors for development of PVT. We analysed the incidence, factors associated with PVT development and its influence on cirrhosis decompensations and orthotopic liver transplant (OLT)-free survival. METHODS: In this prospective observational study between January 2014 and March 2019, 445 consecutive patients with chronic liver disease were screened and finally 241 with cirrhosis included. Factors associated with PVT development and its influence on cirrhosis decompensations and OLT-free survival by time dependent covariate coding were analysed. RESULTS: Majority of patients belonged to Child-Pugh class A 184 (76.3%) and the average MELD score was 10 ± 5. Previous cirrhosis decompensations occurred in 125 (52.1%), 63 (26.1%) were on NSBB and 59 (27.2%) had undergone banding for bleeding prophylaxis. Median follow-up was 29 (1-58) months. Cumulative incidence of PVT was 3.7% and 7.6% at 1 and 3 years. Previous decompensation of cirrhosis and low platelet counts but not NSBB independently predicted the development of PVT. During follow-up, 82/236 (34.7%) patients developed cirrhosis decompensations. OLT-free survival was 100% and 82.8% at 3 years, with and without PVT respectively. MELD score, but not PVT, independently predicted cirrhosis decompensations (HR 1.14; 95%CI:1.09-1.19) and OLT-free survival (HR 1.16;95%CI:1.11-1.21). CONCLUSION: Previous decompensations of cirrhosis and thrombocytopenia predict PVT development in cirrhosis suggesting a pathophysiologic role for severity of portal hypertension. PVT development did not independently predict cirrhosis decompensations or lower OLT-free survival.

Anticoagulation in Cirrhosis and Portal Vein Thrombosis Is Safe and Improves Prognosis in Advanced Cirrhosis.

BACKGROUND: The role of portal vein thrombosis (PVT) in the natural history of cirrhosis is controversial. AIMS: We analyzed the safety and effect of anticoagulant therapy (AT) on PVT recanalization and orthotopic liver transplant (OLT)-free survival. METHODS: Eighty consecutive patients from a prospective registry of cirrhosis and non-tumoral PVT at a tertiary center were analyzed. AT effect on PVT recanalization and OLT-free survival was determined by time-dependent Cox regression analysis. RESULTS: Average MELD score was 15 ± 7. Portal hypertension-related complications at PVT diagnosis were present in 65 (81.3%) patients. Isolated portal vein trunk/branch thrombosis was present in 53 (66.3%) patients. AT was started in 37 patients. AT was stopped in 17 (45.9%) patients, in 4 (10.8%) due to bleeding events. No variceal bleeding occurred while on AT. Anticoagulation was restarted in 6/17 (35.2%) patients due to rethrombosis. In 67 patients with adequate follow-up imaging, AT significantly increased the rate of PVT recanalization compared with those who did not receive anticoagulation [51.4% (18/35) vs 6/32 (18.8%), p = 0.005]. OLT-free survival after a median follow-up of 25 (1-146) months was 32 (40%). Although there was no significant effect of AT on overall OLT-free survival, OLT-free survival was higher among patients with MELD ≥ 15 receiving AT compared to those who did not (p = 0.011). Baseline MELD at PVT detection independently predicted PVT recanalization (HR 1.11, 95% CI 1.01-1.21, p = 0.027) and mortality/OLT (HR 1.12, 95% CI 1.05-1.19, p < 0.001). CONCLUSIONS: Although AT did not improve overall OLT-free survival, it was associated with higher survival in advanced cirrhosis. Anticoagulation increased PVT recanalization and should be maintained after PVT recanalization to avoid rethrombosis.

Unilocular macrocystic serous cystadenoma of the pancreas--atypical features: a case report.

The authors report and discuss an exceedingly rare case of a unilocular macrocystic serous cystadenomas diagnosed in a 63-year-old female patient, which was preoperatively misdiagnosed as a mucinous cystic neoplasm, due to the atypical magnetic resonance (MR) imaging features shown at presentation and the misleading results obtained through cystic fluid analysis. This manuscript overviews the typical and atypical manifestations of this entity and highlights the advantages, potential limitations and pitfalls of both MR imaging and cystic fluid analysis.

Dermatology missed appointments: an analysis of outpatient non-attendance in a general hospital's population.

BACKGROUND: Non-attendance of scheduled appointments is a serious issue in dermatology hospital practice with implications in patient care. Herein we aim to characterize the population of non-attendees of dermatology appointments at a general hospital. MATERIAL AND METHODS: A prospective study was carried out of the reschedule requests received from January to December 2009 at a dermatology outpatient department of a general hospital. Variables such as gender, age, type of consultation, justification for non-attendance, timing between missed appointments and the reschedule request as well as rates of other missed appointments were analyzed. RESULTS: Throughout the study period, 265 patients filed a written request to reschedule a missed appointment, of whom 165 were female and 100 were male. The age distribution displayed two peaks, one in the second and the other in the sixth decade. Regarding the type of consultation, there was a clear predominance of missed follow-up consultations. Confusion and illness were the most frequent excuses for non-attendance. CONCLUSION: We propose to identify patients at risk of missed appointments and suggest interventions to overcome this pitfall and improve patient outcomes.

Invasive lymphatic malformation (gorham-stout) of the pelvis with prominent skin involvement.

Gorham-Stout syndrome is a rare disease characterized by progressive osteolysis leading to disappearance of the bone. Vascular proliferations have been implicated in the pathogenesis of this syndrome. The case of a 7-year-old girl with a prominent invasive lymphatic malformation on the lumbosacral area and massive osteolysis of the pelvic girdle is reported.

Papillary carcinoma arising in struma ovarii versus ovarian metastasis from primary thyroid carcinoma: a case report and review of the literature.

We present a case of a postmenopausal woman diagnosed with an ovarian mass containing thyroid follicles and foci of papillary thyroid carcinoma during pathological examination. This patient referred having had a metachronous thyroid malignancy 10 years before. The differential diagnosis between a thyroid malignancy arising from a struma ovarii and a metastatic ovarian tumor originating from thyroid-cancer is challenging. Struma ovarii should be considered when thyroid components are the predominant element or when thyroid malignant tissue is identified within an ovarian lesion. Thyroid carcinoma arising from a struma ovarii is reported to occur in a minority of cases. Of these, papillary carcinoma is the most frequent subtype encountered. Regarding primary thyroid carcinomas, papillary carcinomas have a lower metastatic potential when compared to follicular carcinomas, and most of the metastases occur in the cervical lymph nodes. Ovarian metastases are exceedingly rare and generally associated with widespread disease. However, they must be considered in the presence of previous history of malignant thyroid carcinoma. The authors review the main clinical, imaging and therapeutic aspects of both these entities and present the most likely diagnosis.

[Double aortic arch: incidental cerebral angiography finding in an adult patient with headache--embrionary cardiovascular morphogenic pattern review]. / Duplo arco aórtico: achado acidental em angiografia cerebral de um paciente adulto com cefaleias--revisão do padrão de morfogénese embrionária cardiovascular a propósito de um caso clínico.

INTRODUCTION: Cerebral angiography is a technique used to detect cerebral vessel anomalies. It is critical that a neuroradiologist is familiar with the normal anatomic development and its anomalies as, during an angiography, they may influence the way the procedure is done. METHODS: Regarding an incidental finding of a double aortic arch during a cerebral angiography in an adult patient, the authors proceeded to a bibliographic review of the normal embriogenic cardiovascular morphogenesis patterns and its anomalies. DISCUSSION: The existence of a double aortic arch constitutes an embrionary pattern typical at six weeks of gestation that results from the process of progression/regression of the six pairs of pharyngeal arches; usually the double aortic arch regresses during the seventh week of gestation. When the fourth right pharyngeal arch and the right dorsal aorta do not regress, this double aortic pattern persists after birth. There are several symptoms related to this anomaly but in some rare cases, like the one presented, they might be absent and are only identified accidentally. CONCLUSION: Studying the pattern of cardiovascular morphogenesis and its anomalies helps the neuroradiologist performing a cerebral angiogram.

Ulcerative Necrobiosis Lipoidica: Is There a Place for Anti-TNFα Treatment?

Necrobiosis lipoidica is a rare granulomatous and inflammatory disease. Its management is particularly difficult when ulceration is present. The authors describe the clinical case of a 65-year-old female patient with necrobiosis lipoidica, who had been submitted in the past to several topical and systemic treatments with little or no improvement. She started treatment with subcutaneous etanercept and showed significant improvement without adverse events until today. The aim of this article is to report a valid and efficient alternative treatment to recalcitrant cases.

Granuloma faciale of the scalp.

Granuloma faciale (GF) is an uncommon dermatosis with characteristic clinicopathological features. Extrafacial isolated GF is extremely rare. Pulsed dye laser (PDL) is a treatment option for GF to minimize the risk of scarring. We report a case of a 78-year-old male with an extensive GF of the scalp successfully treated with pulsed dye laser (PDL).

Kawasaki disease associated with parainfluenza type 3 virus infection.

Kawasaki disease (KD) is an acute, febrile and multisystem vasculitis of early childhood with a striking predilection for the coronary arteries. In developed countries, the incidence of KD has replaced acute rheumatic fever as the leading cause of acquired heart disease in children. The etiologic agent of KD remains unknown, although clinical and epidemiologic features strongly indicate an infectious cause. Parainfluenza viruses are the major cause of laryngotracheobronchitis (croup), but they also commonly cause upper respiratory tract infection, pneumonia, or bronchiolitis. Types 1 and 2 viruses are the most common pathogens associated with croup, and type 3 viruses are associated with bronchiolitis and pneumonia in infants and young children. Rarely, mumps, aseptic meningitis, and encephalitis have been associated with type 3 infections. We report a patient with typical KD during parainfluenza type 3 infection.

Surgical treatment of rhinophyma using carbon dioxide (CO2) laser and pulsed dye laser (PDL).

Rhinophyma is a slowly progressive, benign dermatological disorder of the nose. The most widely accepted theory is that rhinophyma is the end stage of chronic rosacea. The primary reason for its excision is cosmetic deformity. Many treatment modalities have been described, including CO(2) laser. This method provides a very dry surgical field, which allows the sculpting of the hypertrophic areas. Pulsed dye laser (PDL) is a safe and effective treatment, resulting in a significant improvement in erythema, telangiectases, symptoms and quality of life. We report the case of a 63-year-old Caucasian man with a 2-year history of rapid progression rhinophyma. The patient was submitted to five CO(2) laser sessions, followed by three PDL sessions. Favourable re-epithelization of the surfaces treated with CO(2) laser was achieved within a very short period of time. PDL post-treatment purpura lasted a medium of 12 days. After 12 months of follow-up the patient remains without evidence of relapse. To our knowledge, this is the first case described of treatment using a combination of the CO(2) laser and PDL.