A Unique Case of Multicentric Infantile Myofibromatosis with Radiologic-Pathologic Correlation

Infantile myofibromatosis (IM) is a rare condition with a variable clinical presentation that characteristically affects young children. Most frequently it presents as one or more benign nodules of the skin, bones, soft tissues, or, rarely, visceral organs. According to the location and number of lesions, there are three different forms: solitary, multicentric without visceral involvement, and multicentric with visceral involvement (generalised), with the latter having the least favourable prognosis. We present a unique case of severe congenital generalised IM in a new-born male who required intubation and mechanical ventilation immediately after the birth due to respiratory distress. A chest radiograph showed numerous tumours involving the entire lung, resembling a metastatic lung disease. Additionally, the neonate had multiple, bluish, papular skin nodules and a biopsy of a skin nodule ultimately led to the diagnosis of IM. Diffuse lung involvement prevented adequate ventilation which resulted in multiorgan failure and death before targeted treatment could have been initiated. The presented case is unique, as such atypical extensive involvement of the lung and leptomeninges in IM has not been reported before. In this brief report, we present the findings of radiographic and ultrasonographic examinations in correlation with autopsy and histopathology.

How skin and liver can lead to diagnosis

A 73-year-old woman was referred by her hematologist for cholestasis of unknown origin. She was recently diagnosed with chronic myelomonocytic leukemia grade 0 in a context of fatigue, night sweats, weight loss and monocytosis. A PET-CT showed hepatosplenomegaly and multiple centimetric adenopathy. The diagnosis was confirmed by a bone marrow aspiration and biopsy. Interestingly, the evolution of the patient is marked by the appearance of cholestasis and an erythematous firm skin nodule of the right forearm. The skin lesion was biopsied to rule out a cutaneous localization of the patient’s known hemopathy.

Traversing the Spectrum of Non-Langerhans Cell Histiocytosis: A Case of Rosai-Dorfman Disease with Features of Necrobiotic Xanthogranuloma

Introduction. Non-Langerhans cell histiocytoses (non-LCH) are a group of rare diseases with varied clinical manifestations and overlapping features seen among the subtypes. Here, we present a case of Rosai-Dorfman disease with features of necrobiotic xanthogranuloma. Case. A 45-year-old female presented with a 10-year history of an enlarging neck mass with normal overlying skin accompanied by dysphagia and multiple asymptomatic pink to yellowish-brown papules, nodules, and plaques on the face, trunk and extremities. Biopsies of a skin nodule and plaque revealed granulomatous dermal infiltrates (lymphocytes, foamy histiocytes, and Touton giant cells), emperipolesis and areas of necrosis. CD1A and Fite-Faraco staining showed negative results while CD68 and S100 positively stained the tissues of interest. Histopathology of the neck mass paralleled these findings in addition to being negative for lymphoid markers. Patient had monoclonal gammopathy and thyromegaly with enlarged cervical lymph nodes on further tests and imaging. Intralesional and systemic steroids were given which led to flattening of skin lesions and improvement in dysphagia, respectively. Conclusion. Diagnosis and classification of a particular type of non-LCH may be difficult due to similarities across its subtypes. Hence, it is our belief that these diseases may occur on a spectrum. Treatment involves a multidisciplinary approach for the best possible care.

Diagnosis of naturally occurring lumpy skin disease virus infection in cattle using virological, molecular, and immunohistopathological assays

Background and Aim: Lumpy skin disease (LSD) is a contagious viral disease that has great economic losses among Egyptian breeding flocks. The present study was designed to compare the results of different diagnostic approaches used for the diagnosis of LSD virus (LSDV). Materials and Methods: A total of 73 skin nodule samples were collected from suspected infected cattle with LSDV from some Egyptian governorates during 2019 and 2020. Trials for virus isolation (VI) and identification on embryonated chicken eggs (ECEs) were conducted. Molecular detection, histopathological, and immunohistochemical examination were also conducted. Results: The virus was isolated into ECEs, and 58 samples of 73 were positive and gave a characteristic pock lesion on the chorioallantoic membrane. Twenty-two representative nodular skin specimens of the 58 positive samples were selected to be used for molecular, histopathological, and immunohistochemistry (IHC) diagnosis. Conventional polymerase chain reaction succeeded in detecting LSDV DNA in all tested 22 skin nodule samples. Histological examination of skins of different cases revealed various alterations depending on the stage of infection. IHC was used as a confirmatory test for detecting LSDV antigen in the tissues of the skin nodules of infected cattle using specific anti-LSDV antibodies. Lumpy skin viral antigen was detected within the cytoplasm of the epidermal basal cells layer and prickle cell and within the cytoplasm of the hair follicles' epithelial outer and inner roots. Conclusion: This study confirmed the prevalence of LSDV infection in different Egyptian governorates during 2019 and 2020. In addition, histopathology and IHC could be potential methods to confirm Lumpy skin disease infection besidesVI and molecular detection.

Sudden unexpected death caused by infantile acute lymphoblastic leukaemia

ABSTRACT A 7-week-old girl with a normal birth history suddenly developed respiratory distress while feeding. Cardiopulmonary resuscitation was initiated at home after she had a cardiac arrest and was continued in the emergency room but all efforts at resuscitation proved unsuccessful and she died 2 h after presentation. Investigations performed in the emergency room revealed that she had a significantly high white blood cell count and severe anaemia. The cause of death was identified as KMT2A-rearranged infantile acute lymphoblastic leukaemia based on cytogenetic tests. She had no abnormalities at the 4-week check-up; however, she developed a skin nodule on her abdomen thereafter, and the family did not consult a doctor for fear of contracting COVID-19. Early detection and diagnosis could have changed the prognosis of the patient. The present case highlights the negative impact of the reduction of outpatient consultations during the COVID-19 pandemic.

Unusual Presentation of Feline Leprosy Caused by Mycobacterium lepraemurium in the Alpine Region

A 9-year-old cat was referred with multiple, raised, ulcerative and non-ulcerative nodules in the periocular area, sclera and ear-base region, and on the ventral aspect of the tongue. In addition, a progressive ulcerative skin nodule on the tail was observed. Fine-needle aspirations of multiple nodules from the eyelid and sclera revealed the presence of histiocytes with numerous acid-fast intracellular bacilli. The replication of slowly growing mycobacteria in liquid media was detected from biopsied nodules after three months of incubation. The molecular characterization of the isolate identified Mycobacterium (M.) lepraemurium as the cause of the infection. The cat was treated with a combination of surgical excision and a four-week course of antimicrobial therapy including rifampicin combined with clarithromycin. This is an unusual manifestation of feline leprosy and the first molecularly confirmed M. lepraemurium infection in a cat with ocular involvement in Europe. The successful combination of a surgical and antimycobacterial treatment regimen is reported.

LEPROSY MASQUERADING AS DEEP FUNGAL INFECTION

Leprosy exhibits a wide spectrum of presentations, varying from the tuberculoid to the lepromatous pole, with immunologically unstable reactional states in-between, depending on the immune status of the individual. The reactional states of leprosy itself maybe the initial presentation of leprosy in some cases. We hereby report an untreated case of lepromatous leprosy in reaction mimicking deep fungal infection. Our patient is a 58 year old male, who presented with multiple episodes of painful raised lesions all over the body associated with fever and generalized malaise since 2 months. Lesions that initially developed on the trunk, later progressed to involve face and bilateral extremeties, became pustular followed by ulceration and crusting. History of signicant weight loss over the past 6 months. Cutaneous examination revealed multiple pustules, nodules over the face, ears, trunk, abdomen, both extremeties. Multiple ulcerated plaques with necrotic crusting were also present. DDs of deep fungal infection, and leprosy in erythema nodosum leprosum were considered . KOH mount for fungal elements came negative. Slit skin smear showed a bacillary index of 4+. Skin biopsy from the skin nodule revealed thinned epidermis with attened rete ridges, clear grenz zone and multiple foamy macrophages and neutrophilic inltrate in the dermis. So we conrmed it as a case of lepromatous leprosy in erythema nodosum leprosum necroticans and as the patient had no previous evidence suggestive of leprosy it was difcult to diagnose the condition clinically. Hence it is important to have a high index of clinical suspicion of leprosy and initiate prompt treatment to prevent further disabilities and reduce the morbidity associated with leprosy.

Not Just Uterine Adenocarcinoma—Neoplastic and Non-Neoplastic Masses in Domestic Pet Rabbits (Oryctolagus cuniculus): A Review

With increasing numbers of pet rabbits living out their natural lifespan, rabbit oncology is stepping more and more into the limelight. On the other hand, rabbit tumors are less covered in recent editions of textbooks of veterinary pathology than before. We present 1238 cases with neoplastic and non-neoplastic masses in rabbit tissue, submitted from 2008 to 2019, supplemented by a review of the literature on neoplasms in rabbits. Cutaneous masses comprised 47% of submissions. Trichoblastoma was by far the most common skin neoplasm, and nodular suppurative panniculitis was the second most frequent skin nodule in this series. Epithelial as well as mesenchymal cutaneous neoplasms can be virally induced in rabbits (eg, Shope papilloma, myxomatosis) but were infrequent in the current cases. Mammary neoplasms comprised 21% of submitted masses and 94% of these had histologic features of malignancy. Tumors of the female reproductive tract were responsible for 9% of biopsies and were predominantly uterine adenocarcinoma. Polypoid proliferation of rectal mucosa was the most common lesion in the alimentary tract. A broad spectrum of other neoplasms was described, including sarcomas at vaccination sites and ocular posttraumatic sarcomas, comparable to lesions described in cats.

Acute dorsal myelopathy resulting from intramedullary cysticercus: a case report

Background Neurocysticercosis is the most common parasitic infection of the central nervous system, brain being the most frequent site. Intramedullary location of cysticercus is a rarely described entity in literature. Widespread dissemination of cysticercus is also considered a rare occurrence, and only a handful of cases are documented, almost exclusively from tropical nations. Here we present a case of disseminated cysticercosis with rare initial presentation as acute dorsal myelopathy resulting from intramedullary cysticercus. Case presentation A 62-year-old male patient from India (Asian) presented with features of dorsal myelopathy as manifested by acute-onset symmetric paraparesis, sensory loss below umbilicus, and double sphincter dysfunction. General physical examination revealed pea-sized nodules in skin and tongue. On spinal cord imaging, it was found that he had intramedullary cysticercus with diffuse perilesional edema. Brain and muscle imaging showed extensive cysticercosis suggestive of dissemination. Histological examination from skin nodule and antibody assay confirmed the diagnosis of cysticercosis. Following steroid administration, our patient showed improvement with observable increase in power of the lower limbs. He was subsequently discharged with antiepileptics, symptomatic therapy, and regular physiotherapy. Antihelminthic agents were initially avoided in view of extensive cysticercosis in brain including crucial areas such as brainstem. Conclusion Rare manifestation of a rare but treatable disorder makes it an important reportable observation in the context of tropical medicine.

Anesthetic Management of a Brown Bear (Ursus arctos) Captive Undergoing Incisional Biopsy of a Skin Nodule

Background: The brown bear (Ursus arctos) is considered one of the largest terrestrial carnivores, native from temperate forest regions of North America, Europe and Asia. In Brazil, they are founded in captivity and their safe capture and immobilization are obtained with one effective anesthesia for management and surgical and diagnostic procedures. Some anesthetic protocols are described for these purposes, however, there is a lack for data on the anesthetic and adverse effects they have on bears when used. The aim of this case is to report the use and effects of the association of dexmedetomidine with tiletamine and zolazepam in the chemical containment of a captive adult brown bear.Case: A 33-year-old female brown bear, weighing 100 kg, belonging to the Zoobotanic Park of Teresina, Piauí, Brazil, was chemically immobilized for an incisional biopsy of a cutaneous nodule with 1.0 cm diameter in the right face region. The anesthetic protocol included 6 μg/kg of dexmedetomidine associated with 3 mg/kg of tiletamine and zolazepam, administered intramuscularly by dart into the gluteal region of the right pelvic limb. The animal showed moderate ataxia at 5 min and assumed sternal decubitus 7 min after anesthetic administration. The bear's degree of sedation was considered adequate and safe to perform the biopsy at 10 min after administration. Heart rate (47 ± 3 bpm), respiratory rate (17 ± 2 mpm) and rectal temperature (38.7 ± 0.1ºC) were monitored. The bear remained immobile and unconscious throughout the procedure, with intense muscle relaxation, bilateral eyeball rotation, absent lateral palpebral reflex and mild medial reflex and without nystagmus. Complementary sedative doses were not necessary. At the beginning of anesthetic recovery, the bear received 6 µg/kg of atipamezole, intramuscularly. After 25 min of administration of atipamezole, the animal showed signs of recovery in the level of consciousness and reactivity to external stimuli, and assumed the quadrupedal position at 60 min after reverser application.Discussion: Even in captivity, the bears behavior is unpredictable and attack can occur, causing trauma or death to people. For this, the chemical immobilization is important to keep safety of everyone. This procedure was performed using blowgun-assisted darts thrown by a staff member who had experience in using this method, who darted accurately and effectively. The latency time observed after administration of the anesthetic protocol used is similar to reported in other studies with bears that also received intramuscular dexmedetomidine and tiletamine and zolazepam and showed intense muscle relaxation and immobility. The doses used contributed to the absence of bradycardia and hypoventilation and, performing the procedure in the morning, when the temperature is milder in the city, minimized the chance of hyperthermia and thermal stress in the animal, not requiring body cooling. The use of dexmedetomidine in chemical containment protocols for short and minimally invasive procedures allows the subsequent use of its antagonist, atipamezole, contributing to a shorter recovery time, return of the animal’s degree of consciousness and lower incidence of ataxia after assuming a quadrupedal position. The anesthetic protocol used was considered efficient, providing a quick and gentle chemical containment, adequate anesthetic plan and good anesthetic recovery in an adult brown bear from captivity.Keywords:atipamezole, dexmedetomidine, wildlife.Título: Manejo anestésico de um urso pardo (Ursus arctos) cativo submetido a biópsia incisional de nódulo cutâneoDescritores: atipamezole, dexmedetomidine, animais selvagens.