Search

Riga-Fede disease (RFD), also known as traumatic eosinophilic granuloma, is a benign inflammatory condition of the oral cavity that may mimic a malignant tumour. It’s a rare condition mainly reported in infants. We present a unique case report of a 57-year-old man who presented to our outpatient department with foreign body sensation in the throat and swelling over the dorsum of the tongue for a period of 1 month. He gave no history of trauma, especially due to sharp teeth. Video laryngoscopy done revealed an ulceroproliferative growth on the midline of the dorsum of the tongue. With clinical suspicion of malignancy, the patient underwent wide local excision of the tongue lesion. Histopathological examination was suggestive of RFD. The postoperative follow-up was uneventful with good wound healing. The patient was followed up with no evidence of recurrence.

Langerhans cell histiocytosis (LCH) is a proliferative disease of histiocytic cells that rarely affects adults. Different therapeutic options have been used in the treatment of this condition depending on the severity of the disease. However, the management of LCH is often controversial when a resection with clean margins is not achieved. We present a 37-year-old male who was diagnosed with LCH affecting the middle third of the clavicle. The patient was subjected to a wide resection of the lesion creating a large bone defect. The reconstruction was then performed using a 3-D printed porous pseudo-prosthesis (Ti6AL4V) which was fixed using an anatomical clavicular plate (Depuy-Synthes, Raynham, MA, USA) and locking screws. The implant trabeculae were filled with bioactive bone graft substitute (GlassBONETM, Noraker, France). After two years of follow-up the patient was pain free, had a full range of movement, and scored 100 on the Constant Scale and 2.5 points on the disabilities of the arm, shoulder and hand score (DASH). Moreover, in this case, the implantation of the 3-D printed pseudo-prosthesis avoided the use of adjuvant therapies such as radiotherapy. The use of a 3-D printed pseudo-prosthesis achieved an excellent clinical and functional outcome in the treatment of a large bone defect, following a resection of a LCH of the clavicle. 3-D printed pseudo-prostheses could be useful instruments for the treatment of bone defects following large bone resections in musculoskeletal tumors.

PURPOSE Ophthalmic histiocytic lesions comprise a heterogeneous rare group of disorders that are characterized by an abnormal proliferation of histiocytes and may affect all age groups of both sexes. The aim of this study was to highlight the basic demographic, clinical, and histopathological characteristics of this rare group of diseases in ophthalmic practice, which has not been previously studied in this area. Only individual cases have been previously reported. METHODS This was a retrospective study of all biopsied ocular and periocular histiocytic lesions from two centers, King Khaled Eye Specialist Hospital (KKESH) and King Abdulaziz University Hospital (KAUH) in Riyadh, Saudi Arabia, from January 1993 to December 2018. The histopathological diagnosis was confirmed, and the cases were re-classified by reviewing all histopathological slides. The corresponding demographic and clinical data were analyzed. A relevant literature review was also carried out for comparison of our collected analyzed data to published data and to draw our own conclusions. RESULTS A total of 34 ocular/periocular histiocytic lesions in 28 patients who were mostly Saudis (92.9%) were included. The male-to-female ratio was 4:3. The median age at presentation was 6.4 years (range: 2.8-35 years). Twenty-two patients had unilateral involvement, and six patients had bilateral lesions. In patients with Langerhans cell histiocytosis (LCH; L group), the most common presenting findings were eyelid swelling (75%), periocular tenderness (37.5%), proptosis/globe displacement (37.5%) eyelid erythema (25%), and orbital pain (12.5%). In patients with Rosai Dorfman disease (RDD; R group), proptosis/globe displacement occurred in all patients and 80% had decreased vision. Patients in the C group (Cutaneous non-LCH histiocytoses) had variable clinical features because of the different locations of the histiocytic lesions, with the majority involving the eyelids (66.7%). Diagnosis was accurately reached clinically in 38.8%, 33.7%, and 46.7% of patients in the L, C, and R groups, respectively. Overall, the clinical diagnosis was in concordance with the histopathologic diagnosis in 14 out of 34 lesions (41.2%). CONCLUSIONS Histiocytic disease is more likely to be overlooked clinically owing to its rarity. In the C group, juvenile xanthogranuloma (JXG) was the most commonly encountered histiocytic lesion and had a tendency to present at a later age with extremely rare intraocular involvement in contrast to previously published reports. The median age at presentation was higher in group R. All patients in group L had strictly unilateral disease, while RDD (group R) was most commonly bilateral. Future research on genetic aspects, management, and prognosis is necessary.

Athough few case reports of human fascioliasis have been reported from different parts of India, there is no case reported from Kashmir valley till date. Here in we report two cases of human fascioliasis. The both patients presented with fever, marked eosinophilia and liver leisions on imaging. Hepatobiliary imaging showed vague features like mild biliary dilatation and liver leisions representing burrows. Liver biopsy in one of the patients revealed eosinophilic granuloma. Both patients were diagnosed definitively with endoscopic retrograde cholangio-pancreatography (ERCP) by demonstrating live adult fasciola worms. Any patient presenting with fever, marked eosinophilia and liver leisions on imaging should be evaluated for fascioliasis.

Palisaded neutrophilic and granulomatous dermatitis (PNGD) represents a cutaneous histopathologic reaction spectrum associated with several underlying disorders. Few cases of PNGD have been associated with chronic myelomonocytic leukemia (CMML), a malignant hematopoietic disorder with features in between those of a myeloproliferative neoplasm and myelodysplastic syndrome. We present a patient with a generalized papular skin reaction involving the neck, chest, and shoulders with histomorphological features on the spectrum of PNGD. Subsequent laboratory workup demonstrated a persistent mild monocytosis, raising concern for CMML. The diagnosis was ultimately confirmed with a bone marrow biopsy and associated mutational analysis through next-generation sequencing which identified deleterious variants in SRSF2, IDH2, and ASXL1. The findings in this case strengthen the previously made association between PNGD and SRSF2-mutated CMML and may help better define a unique recognizable clinical-histopathological-molecular subtype for dermatopathologists.

Langerhans cell histiocytosis (LCH) is a rare enigmatic disease that pre-dominantly affects children under 5 years of age. We report an interesting case of a 5 month old female diagnosed with multisystem LCH. Her disease process included osseous, pulmonary, gastrointestinal, cutaneous, hematopoietic and neurologic involvement. This case highlights the varying clinical symptoms, risk factors, pathogenesis, and management of multisystem LCH. This case also emphasizes the role of diagnostic imaging in this multifaceted disease.

Emergency service physicians have forensic physician duties in addition to providing treatment and protective services. Errors in forensic report writing could affect the judicial process negatively.A 16-year old male presented at the Emergency Department (ED) following an assault. With a diagnosis of epidural hematoma on the computed tomography taken because of head trauma, the patient was transferred to the brain surgery ward. In the forensic report written in ED, there was stated to be a danger to life. In the repeated evaluation, there was seen to be a mass in the calvarium and surgery was performed. In the histopatholgical evaluation, the diagnosis was made of Langerhans Cell Histiocytosis. It must not be forgotten that errors or ommissions in a forensic report constitute malpractice. The necessary care and attention must be paid to forensic reports prepared in the Emergency Department.

This paper presents two clinical cases of eosinophilic granuloma of bone diagnosed by CT, MRI, and 18F-FDG PET/CT. In both cases the patients were admitted to the clinic with suspected primary malignant bone tumor and the diagnosis of a solitary eosinophilic granuloma was made based on the results of comprehensive radiological diagnostic examination and histological verification. Solitary eosinophilic granuloma of bone is an infrequent condition, occurring in less than 1% of cases of skeletal tumor masses. The most common eosinophilic granuloma is found in the parietal and frontal bones of the skull and is an osteolytic volumetric mass that gradually increases in size. Although most bone tumors can be detected by radiography, computed tomography is preferred, primarily because of its superior ability to detect cortical bone destruction. The diagnostic accuracy of computed tomography and magnetic resonance imaging may be different. The combined use of radiological and radionuclide methods allows us to narrow the spectrum of differential diagnosis. Unfortunately, relatively low specificity of existing radiological diagnostic studies in most cases does not allow to establish a precise diagnosis, and biopsy with subsequent pathological examination remains the method of choice. These clinical observations demonstrate the need to include eosinophilic granuloma in the differential diagnosis when a solitary osteolytic focus is detected.

Introduction: Traumatic ulcerative granuloma with stromal eosinophilia is an uncommon condition of the oral mucosa with a chronic course, usually affecting the tongue. Case Report: Clinically it presents as a chronic ulcer, with raised and indurated borders, rarely presented as a tumor. Histologically it shows a diffuse mixed inflammatory infiltrate, rich in eosinophils. The etiology of this lesion is still unclear; however, chronic irritation from traumatic agents is considered a major initiating factor. In some cases, the presence of CD30+ mononuclear cells within the lesions suggest the possibility of a CD30+ lymphoproliferative disorder. This article presents a case of a traumatic ulcerative granuloma with stromal eosinophilia manifested in a 56-year-old female with a solitary ulcerated tumor inside the right cheek. Conclusion: It was diagnosed based on clinical data and histopathological features. In a brief literature review, the entity has been characterized, analyzing its etiology and nature.

The study aimed to identify, enlist, and analyze cases of unisystem LCH in the maxillofacial pediatric population to understand the clinical presentation and encourage the consideration of this rare disease in the differential diagnosis. Langerhans cell histiocytosis (LCH) is an aggressive benign condition affecting mainly the pediatric population. It can be easily masked as periodontal disease in the maxillofacial region. Early diagnosis and a systemic evaluation are of utmost importance.We are presenting a complete review of literature in the pediatric population according to PRISMA guidelines for clinicopathologic, histopathological, immunohistochemistry, and treatment for unisystem LCH. The risk of bias assessment across studies was done using a Case series appraisal checklist by Guo et al. 53 RESULTS: Forty-nine articles (152 cases) were selected which met our inclusion and exclusion criteria to be included in our review. Most of the patients fall in 6-12 years of age with the involvement of the mandibular body region in 40.79% cases. This disease mainly presents as erythematous gingiva, pain, swelling, and mobile teeth. Management can range from minimal intervention to chemotherapy and surgery.Although this is a rare condition, it should be considered especially in the pediatric population with periodontitis type lesions and floating teeth and comprehensive management should be followed. Early diagnosis of the disease is very important.

We report the case of an otherwise healthy 17-year-old female who presented for surgical removal of an enlarging “atypical cyst” on her scalp. During subtotal excision, only friable serosanguinous translucent ribbons of tissue were found. A histopathologic diagnosis of Langerhans cell histiocytosis (LCH) was rendered and imaging studies revealed extradural invasion of the tumor. Within weeks, the patient also developed progressive lymphadenopathy with grossly elevated levels of Epstein-Barr virus viral capsid antigen antibody levels. This report aims to highlight a unique presentation of LCH with discussion of workup, management, and avoidance of potential surgical complications.

BACKGROUND Hyaluronic acid filler reactions have been increasingly observed in recent years. Our study investigates whether the increased number of filler reactions observed since 2014 is associated with a specific histopathologic inflammatory pattern and type of filler. METHODS The institution's dermatopathology electronic database was retrospectively searched for histopathologic reactions to hyaluronic acid from January 2014 to December 2019. The age, sex, type of filler, procedure, location, and histopathologic patterns were recorded. RESULTS From 2014 to 2019, there were 15 cases of granulomatous reactions to hyaluronic acid filler. In 10 of these cases, there was a characteristic inflammatory pattern characterized by tightly cuffed palisades of histiocytes with varying numbers of eosinophils. Of the 11 cases in which the type of filler was known, all used Vycross technology, a novel manufacturing process in the production of hyaluronic acid filler. CONCLUSION A characteristic histopathologic pattern of discrete foci of tightly cuffed palisaded granulomas with eosinophils is associated with fillers manufactured using Vycross technology.

Descriptive, retrospective. Scientific level of evidence IV. The aim of this study was to evaluate a consecutive case series of 50 pediatric patients with LCH of the spine. Langerhans cell histiocytosis (LCH) is a rare disease characterized by abnormal proliferation of Langerhans cells in different organs. Incidence in children range from 2 to 10 cases per million. In the current literature, few series evaluate LCH in the pediatric spine. A consecutive case series of 50 pediatric patients with LCH of the spine treated at our hospital between 1984 and 2016, with a follow-up of at least 2 years, was analyzed. Sex, age, clinical and radiographic presentation, number of lesions, treatment, complications, and outcome were assessed. Fifty patients, 26 boys and 24 girls, were evaluated. Mean age was 5 years and 2 months (6 months to 13 years and 3 months). 27 patients had a single spinal lesion while 23 had 2 or more lesions. A total of 100 vertebrae were involved. The thoracic spine was the most affected. The most frequent lesion location was in the vertebral body in 88% of the cases. The symptoms were pain (87%), reduced range of motion, deformity, and neurologic deficit. Biopsy was performed in 48 patients. Thirty-nine patients received medical treatment, 28 used orthoses and six required surgery. Six patients (12%) recurred at a mean of 3 years and 5 months (range 2–12 years). In all cases, neurological symptoms, torticollis, and deformities resolved after medical or surgical treatment. Because of the variable presentation of the disease, ranging from a solitary isolated vertebral lesion to polyostotic and multisystemic involvement, a multidisciplinary team is required to have an adequate management of these patients and to obtain good results.

Background: Langerhans cell histiocytosis (LCH) is a rare nonmalignant disease characterized by a clonal proliferation of mononuclear cells called Langerhans histiocytes and infiltrates surrounding tissues, mostly self-limiting and usually occurring in the first two decades of life. Vertebral involvement is rare, mostly seen in the thoracic region, and involves the anterior elements of the corpus. In the literature, several treatment options and surgical approaches have been reported concerning the treatment of this disease and surgery. Case Presentation: We report an 18-month-old male with thoracic LCH who underwent surgery due to progressive neurological deficit. Gross total removal of the tumor with one level corpectomy in this patient was achieved via a posterolateral approach with postoperative functional improvement. The surgical cavity was supported by corpectomy cage and unilateral screw-rod fixation system at the same stage. Conclusion: Gross total tumor removal, corpectomy, and 360° stabilization via posterolateral approach at a single stage are safe, effective, and definite neurosurgical methods in terms of providing neurological recovery, long-term tumor-free survival, and spinal stability.

INTRODUCTION Heart involvement in vasculitis is rare, but potentially severe. The ascertainment of cardiac disease in vasculitis is complex and requires an integrated multidisciplinary approach involving the Rheumatologist, Radiologist, Cardiologist, and Heart surgeon. AREAS COVERED the authors searched PubMed using the keywords 'heart'[Mesh] and vasculitis"[Mesh]. EXPERT OPINION Virtually any vasculitis can affect the heart, but cardiac involvement is more common in some vasculitides such as Takayasu arteritis, polyarteritis nodosa, and eosinophilic granulomatosis with polyangiitis. Immunosuppressive treatment and when indicated surgery can improve the prognosis.

BACKGROUND This study aimed to evaluate the long-term clinical and radiologic follow-up results of eosinophilic granulomas (EGs) of the axial and appendicular skeleton managed with biopsy alone. METHODS Fifty-five patients with unifocal osseous EGs of the axial and appendicular skeleton were followed after biopsy. Patients were divided into 2 groups on the basis of localization of the lesions. In group 1, there were 32 (58.2%) children with extremity long bone involvement: femur, tibia, humerus, ulna, and radius. Group 2 included 23 (41.8%) patients with lesions located in other appendicular and axial skeleton bones: pelvis, scapula, clavicle, lumbar, and thoracic vertebrae. After confirming the diagnosis by a closed biopsy, no further surgical intervention was performed. Clinical recovery included regression of the localized symptoms, mainly pain resolution. Functional improvement was assessed by Musculoskeletal Tumor Society (MSTS) scoring. Radiologic healing was defined as ossification of the entire lesion with cortical thickening in long and flat bones, and restoration of vertebral body height in the spine. Complications, including local recurrence, were determined. RESULTS The patients comprised 28 boys and 27 girls with an average age of 9.2 years (range, 3 to 16 y). The average follow-up was 76 months (range, 28 to 132 mo). The median time from biopsy to clinical recovery was 17 days [95% confidence interval (CI), 13.3-20.6] and 36 days (95% Cl, 32.8-39.1) in group 1 and group 2, respectively. MSTS scores increased progressively till the end of 12 months in both groups. The median time from biopsy to radiologic healing was 16 months (95% CI, 11.5-20.4) and 42 months (95% Cl, 39.3-44.6) in group 1 and group 2, respectively. Both clinical recovery (P=0.021) and radiologic healing (P=0.009) were significantly faster in group 1 compared with group 2. No major complication was seen after biopsy. All lesions regressed without a local recurrence. CONCLUSIONS Unifocal osseous EGs have spontaneous healing potential and confirming the diagnosis by biopsy is enough to obtain good clinical and radiologic results without any additional surgical intervention. TYPE OF STUDY This was a therapeutic study. LEVEL OF EVIDENCE Level IV.

Eosinophilic granuloma (EG) is a rare histiocytic disorder resulting from clonal proliferation of Langerhans cells. Eosinophilic granuloma, the most benign of the three entities of Langerhans cell histiocytosis, may be multiple or solitary. This lesion can affect any bones but commonly involves the mandible when the jaws are affected. It is difficult to make a correct diagnosis on it without proof of a pathological diagnosis, which correlates with the diverse clinical and radiographic presentations of EG in the jaws. This report describes a case of unifocal EG of the mandible occurring in a 6-year-old boy whose initial presentation led to confusion of any solitary bony lesion. A final diagnosis of EG was established after histopathological examination of the biopsy specimen.

We performed gross and histological examinations of the livers of sika deer (Cervus nippon yesoensis) in Hokkaido, Japan. Out of 1,381 deer slaughtered for venison production, thickening and dilation of the large intrahepatic bile ducts and Fasciola flukes in the duct lumens were detected in 621 deer (45.0%). Furthermore, 107 non-bile lesions (75 intrahepatic and 32 capsular lesions) were detected during gross examinations. Histologically, the bile duct lesions included chronic proliferative cholangitis, papillary hyperplasia, goblet cell and pyloric gland metaplasia, and periductal fibrosis. Many of the intrahepatic non-bile duct lesions (53/75, 71%) were considered to be Fasciola fluke migration-associated lesions, including two lesion types: necrosis, hemorrhage, and eosinophilic granuloma formation (29 lesions), and lymphoid tissue formation (24 lesions). Lymphoid tissue formation was considered to result from the persistent immune responses against dead Fasciola flukes. An epidermoid liver cyst was found incidentally, which has not been reported in the veterinary literature. In summary, this study demonstrated the predominance of fascioliasis-associated lesions in sika deer livers. The gross and histological lesions caused by Fasciola flukes in sika deer were similar to fascioliasis in other animals. Moreover, we described lymphoid tissue formation as a fascioliasis-associated lesion for the first time. The fact that bile duct lesions (45.0%) had a markedly higher prevalence than fascioliasis-associated parenchymal lesions (53/1,381, 3.8%) indicated that sika deer are a permissive host for fascioliasis. Our results provide information that will aid pathological examinations of sika deer.

Langerhans’ cell histiocytosis (LCH) is a rare disease of the reticuloendothelial system in which there are abnormal proliferation and accumulation of histiocytes, abnormal cells deriving from bone marrow that can migrate from the skin to the lymph nodes. Langerhans’ cell histiocytosis has three variants: unifocal (eosinophilic granuloma), multifocal unisystem (Hand–Schuller–Christian triad), and multifocal multisystem (Letterer–Siwe disease). We present a case of oral lesions associated with LCH in a young male aged 15 years. The history, radiological appearance, histopathology, and treatment options of the patient are discussed. How to cite this article: Soangra R, Kapoor A, Meena D, et al. Langerhans’ Cell Histiocytosis Diagnosed through Periodontal Lesion in a 15-year-old Child: A Case Report. Int J Clin Pediatr Dent 2020;13(S-1):S115–S118.

Langerhans cell histiocytosis (LCH) is a rare idiopathic disease that is characterized by the clonal proliferation of bone marrow-derived Langerhans cells. It usually occurs in children at an incidence of 2.6–5.4/10,00,000 children/year. The disease occurs with a slight male predilection. The peak age ranges from 1 to 4 years. The common oral findings include the gingival enlargement, oral ulcers, and loosening of teeth. The variable radiographic appearance mimicking odontogenic cyst, osteomyelitis, or a malignancy poses a diagnostic challenge. This rare case report emphasizes on the differential diagnosis of LCH.

Background: Anisakiasis-related gastric eosinophilic granuloma is rare. Case Report: Herein, we report a patient with anisakiasis-related gastric eosinophilic granuloma who was treated with laparoscopic and endoscopic cooperative surgery (LECS). A 59-year-old woman was presented to our hospital for further examination of a gastric lesion that was initially diagnosed by a local medical doctor. Esophagogastroduodenoscopy showed a submucosal tumor-like lesion in the lower body of the stomach. Endoscopic ultrasonography showed a heterogeneous hypoechoic submucosal mass lesion in the submucosal layer measuring 10 mm, without evidence of deep involvement. Under a clinical diagnosis of gastrointestinal stromal tumor, the patient underwent LECS. Gross appearance of the resected specimen revealed a 1.5×1.0 cm submucosal tumor-like lesion. Microscopic examination revealed necrosed insects consistent with the characteristics of gastric anisakiasis, around which prominent eosinophilic infiltration and granulomas were observed. This prompted a diagnosis of gastric eosinophilic granuloma related to anisakiasis. Conclusion: To the best of our knowledge, this is the second case of gastric eosinophilic granuloma related to anisakiasis resected by LECS in the English medical literature. LECS might be a useful procedure for minimally invasive therapeutic diagnosis.

Background Non-traumatic acquired spinal deformities represent a separate entity within the axial pathologies in children. Their causes are variable (neurological, muscular, infectious, especially Pott's disease, inflammatory or tumoral). The etiological diagnosis can be clinically oriented by pain, medical history, especially rheumatic. The contribution of the CT scan is crucial, as it localizes the lesion, specifies its nature, but above all it helps the histological diagnosis by guided biopsies. Our case illustrates the difficulties in diagnosing the aetiology of spinal acquired deformity in a girl with a multi-evocative context. Observation A 14 years-old girl complains for a few months of a dorso-lumbar spinal pain and a vicious attitude. Her medical history includes femoral thrombophlebitis and psoriasis. She had no trauma or fever. Clinically, the general state is preserved, with no local inflammatory signs, a kyphotic attitude with an analgesic lumbar support was noted. The rest of the locomotor exam was normal. CT scan shows cancellous body geodes with a double mirror. Discussion Acquired spinal deformities are always secondary to general disease. The fever may point to bacterial infections, especially tuberculosis (Pott's disease), but also non-infectious causes such as a rheumatic disease. For our patient, psoriasis argues for the latter possibility. However, the rarity of isolated disco-vertebral involvement and the severity of the lytic lesions are against this diagnosis. The histopathological exam showed an eosinophilic granuloma, confirming the histiocytosis X diagnosis. The cortico-response strengthen the diagnosis. The course was marked by the bone remodeling with the posture correction. Conclusion A clinical, radiological, and histological analysis allow the etiological orientation in front of acquired spinal deformity.

We herein report a case of coagulation necrosis with granulation and eosinophilic infiltration of the liver. A 37-year-old woman was diagnosed with a new mass lesion in the liver 1 month after breast cancer surgery and admitted for a further examination. Because the tumor occurred immediately after surgery, it was considered essential to determine whether or not it was a metastatic liver tumor from breast cancer. A percutaneous liver tumor biopsy revealed eosinophilic granuloma of the liver, which is considered to have a high possibility of visceral larva migrans with suspected gnathostomiasis infection. A detailed medical history and histological diagnosis are important for making a differential diagnosis.

A retrospective study was performed on skin biopsy specimens from horses and cats having eosinophilic granulomas with ‘collagen degeneration’, eosinophilic skin diseases without ‘collagen degeneration’ and pyogranulomas without ‘collagen degeneration’. Neither the appearance of collagen fibres nor the dominant polymorphonuclear cells, as seen in H & E-stained sections, were predictive of Masson’s trichrome findings. Hence, a Masson’s trichrome staining abnormality of collagen fibres was no more likely to be present in lesions with ‘collagen degeneration’ than in those lesions without ‘collagen degeneration’. In addition, evaluation of trichrome-stained specimens suggested that there is no collagen degeneration in equine and feline eosinophilic granulomas and that the areas previously referred to as collagen degeneration are more appropriately called flame figures.

Eosinophilic granuloma is a localized, non-invasive form of Langerhans cell histiocytosis. It usually develops in the long bones and is more frequent in children under the age of 10 years. It is very rare in adults. We present the case of a young woman admitted to hospital for persistent refractory left parietal headache, later revealed to be caused by an eosinophilic granuloma. LEARNING POINTS: Eosinophilic granuloma is a localized benign form of Langerhans cell histiocytosis. It is essential to exclude the presence of bone lesions in other locations and/or systemic involvement. The prognosis is good, with surgical resection, when necessary, being a curative intervention, although sometimes (as in this case) a conservative approach is sufficient.

— Mast cells were found in the surface epidermis and outer root sheath epithelium in 37 of 338 cats (11.6 per cent) with non-neoplastic skin disorders. Epidermal mast cells were most commonly found in the eosinophilic plaque and the eosinophilic granuloma. Thevast majority of dermatoses wherein epidermal mast cells were found were characterised by: (1) a presumed immune-mediated pathogenesis, and (2) tissue eosinophilias. Resume— Des mastocytes ont ete observes dans l'epiderme superficiel ainsi que dans l'epithelium folliculaire chez 37 chats parmi 338 (11,6%) atteints de dermatoses non neoplasiques. Les mastocytes epidermiques ont ete le plus souvent trouves dans la plaque eosinophilique et le granulome eosinophilique. La grande majorite des dermatoses presentant des mastocytes epidermiques etait caracterisee par: (1) la presomption d'une pathogenie immunodependante et (2) une eosinophilie tissulaire. Zusammenfassung— Mastzellen konnten in der oberflachlichen Epidermis und in den Epithelien der auseren Wurzelscheide bei 37 von 338 untersuchten Katzen (11.6%) mit nicht-neoplastischen Plaques oder eosinophilem Granulom gefunden werden. Die uberwiegende Zahl der Dermatosen, bei denen epidermale Mastzellen auftraten, war durch die beiden Kriterien 1) vermutlich immunvermittelte Pathogenese und 2) Gewebs-Eosionophilie charakterisiert. Resumen En el presente trabajo se describe la presencia de mastocitos en la epidermis superficial y en la vaina folicular externa de 37 gatos de 338 examinados (11,6%) ninguno de los gatos examinados padecia enfermedades neoplasicas. Los mastocitos epidermicos fueron mas frecuentes en animates que padecian placas eosinofilicas y granulomas eosinofilicos. La gran mayoria de las dermatosis en las cuales se detectan mastocitos epidermicos cuales se se caracterizan por: 1. Una posible patogenia inmunomediada y 2. eosinofilia tisular.

Introduction and importance Langerhans cell histiocytosis (LCH) is a rare haematological disorder affecting infants and young children and has an estimated incidence of 2-5 cases per million people per year. LCH invades the reticuloendothelial system and causes the proliferation of Langerhans cells and mature eosinophils. LCH involving the temporoparietal bone has rarely been reported in the literature. Presentation of case A ten-year-old boy presented to the Neurosurgical outpatient clinic with a swelling on the right temporoparietal region following a fall from his bicycle. Local examination revealed a single, 3 × 3 cm, non-tender, cystic, immobile swelling in the right temporoparietal region. On evaluation for recent head trauma, an incidental finding of eosinophilic granuloma was discovered on a CT scan. The FNAC was suggestive of a histiocytic lesion pertaining to a diagnosis of LCH. The patient underwent wide excision of the mass and cranioplasty. A one-month follow-up CT scan of the head had no evidence of residual or recurrent disease. Discussion Eosinophilic granuloma is one of the three variants of LCH and has a relatively better prognosis. Clinical diagnosis can be challenging and mandates tissue sampling for histopathological examination. Treatment modalities including surgery, radiotherapy, chemotherapy, and steroid injection are used alone, or in combination, depending on the extent and severity of the disease. Conclusion Examining a swelling in the temporoparietal region with no other characteristic symptoms could be a case of LCH. The timely diagnosis and surgical excision with other adjuvant treatment options of this rare pediatric disease would help in a better outcome., Highlights • Langerhans cell histiocytosis can present as a mass in the temporal or parietal region with no symptoms otherwise. • The etiology of Langerhans cell histiocytosis is still unknown and diagnosis can be challenging. • The prognosis depends greatly on early identification and prompt surgical treatment.

Background Langerhans cell histiocytosis (LCH) is a rare disorder characterized by abnormal proliferation and infiltration of histiocytic cells. Methods This review focused on the main aspects associated with LCH. Results LCH can involve single or multiple organs and systems, with bone and skin being the most commonly affected sites. Regarding skeletal manifestations, the jawbones are involved in about 20%-30% of all cases. Such lesions may present as unilocular or multilocular images mainly affecting the posterior mandible. Oral soft tissue lesions may also occur, with the gingiva and hard palate being the most frequently affected sites. Conclusion The diagnosis and management of LCH are challenging, requiring a multidisciplinary approach, with dentists playing a central role since oral manifestations can be the first sign of the condition.

Intestinal anisakiasis is not only a rare but also a difficult to diagnose parasitic disease. The symptoms are not specific and are often severe and abrupt; therefore, patients are sometimes diagnosed as having surgical abdomen. The clinical imaging findings are remarkable, including ascites, enteritis, ileus, eosinophilic granuloma and sometimes perforation. We experienced a case of intestinal anisakiasis diagnosed on the basis of the Anisakis-specific immunoglobulin A level from paired sera and treated successfully with conservative therapy, although ileum perforation was complicated by a 50-mm abscess. Even the large abscess could be treated without drainage in thiscase.

Background Traumatic ulcerative granuloma with stromal eosinophilia (TUGSE) is a rare self-limiting condition of the oral mucosa. The lesion manifests as an isolated ulcer that can be either asymptomatic or associated with mild to severe pain, and in most cases, it affects the tongue. TUGSE lesions may mimic malignancy such as squamous cell carcinoma, CD30 positive lymphoproliferative disorder, or infectious diseases such as primary syphilis, tuberculosis, or Epstein-Barr virus mucocutaneous ulcer. Histologically dominating cells are lymphocytes, histiocytes, and eosinophils. Case presentation We describe a TUGSE case of a patient with a solitary ulcer on the lower left retromolar buccal plane. Upon presentation, the patient reported a swelling on the buccal mucosa of the left lower jaw since 1 year with rapid growth over the last days and mild pain while chewing. The diameter of the intraoral lesion on the lower left retromolar buccal plane was approximately 4 × 3 cm; the lesion presented as indurated base with a central superficial ulceration of 2 × 1 cm, indicative for a malignant process. Histologically, the ulceration showed an expanding, infiltrative, and vaguely granulomatous morphology, involving the superficial mucosa and the fatty tissue, and extended between the deep striated muscle fibers. The lesion was rich in lymphocytes, histiocytes, and eosionophils intermingled with activated T-blasts without phenotypic abnormalities. TUGSE was then diagnosed based on the phenotype (especially the lacking expression of CD30, the retained T-cell phenotype, and the absence of Epstein-Barr virus), the clinical presentation, and the morphology. Twenty-six months after diagnosis, no recurrence of the ulceration was seen. Conclusions As TUGSE may mimic malignancy or infectious diseases, biopsy is mandatory and should be combined with thorough clinical examination. A screening for infectious diseases (mainly syphilis, Epstein-Barr virus, and HIV infections) must be performed routinely. In most cases, the lesions resolve spontaneously, obviating the need of further actions other than clinical follow-up. The pathogenesis of TUGSE lesions is still under debate, although local traumatic events and a locotypic immune response have been suggested to be major contributing factors.

Background Langerhans cell histiocytosis (LCH) is a rare group of disorders without a well understood etiology. Known formerly as histiocytosis X, the disease has a wide spectrum of clinical presentations, including eosinophilic granuloma (solitary bone lesion), diabetes insipidus, and exophthalmos. Many of these patients initially present to orthopaedic surgeons, and misdiagnosis is frequent. Methods We deliver a case of a 10-month-old boy who consulted to our department. Previously misdiagnosed as a Kawasaki syndrome, TORCH, and osteomyelitis. He had undergone several examinations and had been discussed in clinocipathological conference (CPC) to narrow down the diagnosis. Result After serial examinations, the diagnosis of Langerhans Cell Histiocytosis was confirmed and chemotherapy was initiated. And after 6 cycles of chemotherapy, with 1-week interval of each therapy, the clinical appearance of this patient significantly improved. Conclusion Despite major advances in our understanding and management of LCH, it remains one of the most challenging diagnoses for the orthopedic surgeon. By doing a comprehensive examination, it is possible to narrowing down the diagnosis and planning the accurate treatment., Highlights • Langerhans cell histiocytosis (LCH), also known as histiocytosis X, is a rare disorder resulting from an abnormal clonal proliferation of macrophages. • Despite major advances in our understanding and management of LCH, it remains one of the most challenging diagnoses. • In diagnosing LCH a thorough physical examination, Laboratory studies, and radiological studies are mandatory. • Many different treatments for multisystem LCH, from minimal therapy to intensive chemotherapy, have been tried. • Vinblastine and etoposide, have been identified as effective single agents. Both have activity against diverse disorders of the monocyte-macrophage system and combination chemotherapy including them had promising results in multisystem LCH.

Langerhans cell histiocytosis (LCH) is characterised by tissue destruction caused by the abnormal proliferation of pathogenic dendritic cells. We report a rare case of multi-system LCH with local invasion of the orbital apex.A 56-year-old woman suffered from a decrease of visual acuity in the left eye caused by central scotoma and the limitation of eye movement in all directions. Magnetic resonance imaging revealed an enhanced lesion in the left orbital apex, suggesting optic nerve compression. She had been diagnosed with eosinophilic granuloma 24 years previously. Two weeks after the current presentation, we admitted the patient for optic canal and orbital apex decompression and subtotal tumour resection. Histopathological analysis confirmed the diagnosis of LCH. Post-surgical treatment with low-dose cytarabine was initiated for the residual tumour. However, it was ceased because of myelosuppression-induced pyelonephritis. After surgery, the central scotoma disappeared on day 5 and eye movement palsy resolved by 6 months. After the cessation of cytarabine, she has received low-dose steroid therapy for 2 years with no recurrence. Early surgical intervention with low-dose steroid therapy can lead to recovery of visual acuity and resolve eye movement palsy in patients with lesions of the orbital apex caused by multi-system LCH.

Author(s): Johnson, Lynelle R; Johnson, Eric G; Hulsebosch, Sean E; Dear, Jonathan D; Vernau, William | Abstract: BackgroundEosinophilic lung disease is a poorly understood inflammatory airway disease that results in substantial morbidity.ObjectiveTo describe clinical findings in dogs with eosinophilic lung disease defined on the basis of radiographic, bronchoscopic, and bronchoalveolar lavage fluid (BAL) analysis. Categories included eosinophilic bronchitis (EB), eosinophilic granuloma (EG), and eosinophilic bronchopneumopathy (EBP).AnimalsSeventy-five client owned dogs.MethodsMedical records were retrospectively reviewed for dogs with idiopathic BAL fluid eosinophilia. Information abstracted included duration and nature of clinical signs, bronchoscopic findings, and laboratory data. Thoracic radiographs were evaluated for the pattern of infiltrate, bronchiectasis, and lymphadenomegaly.ResultsThoracic radiographs were normal or demonstrated a bronchial pattern in 31 dogs assigned a diagnosis of EB. Nine dogs had intraluminal mass lesions and were bronchoscopically diagnosed with EG. The remaining 35 dogs were categorized as having EBP based on radiographic changes, yellow green mucus in the airways, mucosal changes, and airway collapse. Age and duration of cough did not differ among groups. Dogs with EB were less likely to have bronchiectasis or peripheral eosinophilia, had lower total nucleated cell count in BAL fluid, and lower percentage of eosinophils in BAL fluid compared to dogs in the other 2 groups. In contrast to previous reports, prolonged survival (g55 months) was documented in dogs with EG.Conclusions and clinical importanceDogs with eosinophilic lung disease can be categorized based on imaging, bronchoscopic and BAL fluid cytologic findings. Further studies are needed to establish response to treatment in these groups.

OBJECTIVE: Spinal eosinophilic granulomas (EGs) are uncommon tumors, constituting 80% of patients and only 2 had vertebra plana morphology. The cervical lesions involved single vertebra in 42 patients (82%), whereas 9 patients (18%) had multiple vertebral involvement. Fifteen patients were managed conservatively, of whom 7 failed and required surgery later; 40 patients underwent definitive surgery and 17 received adjuvant treatment. Posterior-only surgeries were performed in 5 patients, anterior-only in 20, and circumferential in 14. The mean follow-up duration was 68 months (range, 12e 268 months). The outcome was good to excellent in all, except 1, irrespective of type of treatment received. CONCLUSIONS: Cervical spine EG is rare in adults and usually presents as an osteolytic lesion involving the vertebral body; vertebra plana is very rare. Surgery is typically reserved for patients with severe neurologic deficits and bony instability. The outcome seems good in most cases.

Shoulder lesions range from tumor-like lesions such as simple bone cysts to aggressive high-grade sarcomas. The clinical presentation is often nonspecific with shoulder pain as the primary complaint, which may lead to a delayed or missed diagnosis. Delayed diagnosis or a poorly planned biopsy of a malignant shoulder lesion can have a detrimental effect on the patient's prognosis and treatment options. Because the initial patient assessment is crucial for successful treatment, knowledge of the key features of common shoulder tumors and tumor-like conditions can help determine the diagnosis and treatment plan. This article reviews the key features and treatment options of the more commonly encountered benign and malignant shoulder bone and soft-tissue tumors and tumor-like conditions.

Langerhans cell histiocytosis (LCH) is a rare hematological disorder characterized by local or generalized, uncontrolled proliferation and infiltration of Langerhans type of histiocytic cells. It represents a spectrum of clinicopathologic disorders, ranging from a highly aggressive and frequently fatal multisystem disease to an easily cured solitary lesion of bone. Involvement of children and the younger age group is more common than the adults. Oral cavity involvement occurs early in LCH, but the initial symptoms are generally nonspecific, often causing misdiagnosis. This report describes a rare case of chronic localized LCH in an adult patient, with involvement of oral cavity. A 34-year-old male patient presented with multiple nodulo-papular, ulcerated lesions in gingiva involving both the jaws (primarily mandible) and the left buccal mucosa, in addition to regional teeth mobility. The most striking feature was that even after extraction of mobile teeth, the lesions persisted. After recording proper history, performing clinical and radiological evaluation, an incisional biopsy was performed followed by histopathology and immunohistochemistry to reach a confirmatory diagnosis of LCH, thereby implementing early and appropriate initiation of treatment.

A middle meningeal artery (MMA) aneurysm is a type of intracranial aneurysm that has been rarely documented and is not completely understood in clinical medicine and especially in pediatrics, because juvenile cases have been extremely uncommon. Vascular conditions have occasionally been diagnosed in osteoarchaeological remains, providing evidence of their occurrence in previous eras. In the present report, we have described a lesion that was highly consistent with a case of an MMA aneurysm observed in an Etruscan child, whose remains had been exhumed during archaeological excavations in the necropolis of Pontecagnano (Salerno, southern Italy). The remains date back to the seventh to sixth century BC. Macroscopic and radiological examinations revealed an oval depression in the endocranial area corresponding to the parietal branch of the right MMA. The localized thinning of the bone and the absence of a bone reaction were compatible with a long-lasting compression of vascular origin highly consistent with an MMA aneurysm. Primary malignant bone tumors, bone metastasis, benign neoplastic conditions, and other non-neoplastic conditions, including calvarial venous malformations, eosinophilic granuloma, and sarcoidosis, could be excluded from the differential diagnosis, although the occurrence of other pathologies, such as dural arteriovenous fistulas, could not be rejected. Although it was not possible to definitely establish whether the MMA aneurysm was symptomatic, it could not be ruled out that rupture and subsequent extradural hemorrhage and/or intracranial hemorrhage could have caused the death of the child. From a paleopathological viewpoint, the case illustrated provides an interesting historical perspective on a rare neurovascular disorder that continues to be debated in modern clinical research.

Prayer marks are asymptomatic nodular and cyst like swellings or callosities with or without chronic skin changes attributable to repeated, prolonged pressure and friction exerted on bony prominences when praying on hard surfaces. This study aimed to describe the clinical and pathological features of forehead prayer marks in Kano, Northwest Nigeria. A retrospective review of Muslims with regular praying habits who presented to the maxillofacial surgery clinic of Murtala Mohammed Specialist Hospital, Kano within a five year (January 2014–December 2018) period on account of central forehead nodular prayer marks was conducted. Biographic data, duration, size, consistency and histological diagnoses of forehead prayer marks were retrieved and analysed. Of the 26 patients with forehead prayer marks, there were 19 men (M: F = 2.7:1). The mean age was 42 ± 3 years and there ages ranged from 15 to 62 years. The mean duration and size of the swellings were 1 ± 5.4 years and 2.5 ± 3.1 cm respectively. The consistency of the lesions was mostly soft to firm (n = 23). Seventeen of the lesions were histologically diagnosed as lipoma. Others were fibroma, soft tissue cysts, eosinophilic granuloma and osteoma. Forehead prayer marks are more common in men than women with highest occurrence in fifth decade of life. Lipoma was the commonest post-excision histological diagnosis of forehead prayer marks.

Introducción: El granuloma eosinófilo es una enfermedad benigna poco usual que suele afectar a la población pediátrica y adultos jóvenes. Es la afección más benigna de las enfermedades tradicionalmente conocidas como histiocitosis X que en la actualidad reciben la denominación de histiocitosis de células de Langerhans. Caso Clínico: Paciente pediátrico con bultoma doloroso en región temporal. Las pruebas de imagen realizadas ponen de manifiesto la existencia de una lesión osteolítica con patrón de agresividad compatible con granuloma eosinófilo. El paciente es intervenido mediante cirugía con diagnóstico histológico definitivo concluyente de granuloma eosinófilo. Discusión: El granuloma eosinófilo puede afectar a uno o múltiples huesos, de los cuáles los más frecuentes son los huesos craneales, epífisis de huesos largos y costillas, precisando estrategias de diagnóstico y tratamiento individualizadas para un manejo y resultado óptimo, siendo el tratamiento quirúrgico de primera elección. Conclusión: El granuloma eosinófilo es un cuadro benigno infrecuente que requiere de una correcta anamnesis y exploración clínica del paciente, así como la demostración de las imágenes radiológicas características, permitiendo llegar a un diagnóstico de presunción generalmente certero que en la mayoría de ocasiones se puede considerar definitivo.

Study Design: Retrospective case series. Objectives: To evaluate the efficacy of growth-preserving posterior spinal column reconstruction surgery in children with collapsed vertebral eosinophilic granuloma (EG). Methods: We retrospectively reviewed 15 confirmed cases of vertebral EG who met the inclusion and exclusion criteria from March 2015 to March 2018 in our hospital. The recovery of diseased vertebrae was assessed. Results: The cases were composed of 11 males and 4 females with a mean age of 74.3 ± 38.8 months. Nine cases presented with thoracic vertebral destruction, 6 cases with lumbar vertebral destruction. Under Garg’s classification, 10, 3 and 2 cases were classified as Grade IIA, IB and IIB lesions, respectively. All patients had mild to moderate night-aggravated back pain and recovered at 1-week after surgery. Three patients had Frankel D neurologic symptoms and recovered at 1-week, 1-week and 4-weeks after surgery, respectively. Follow-up time after surgery ranged from 1.9 years to 4.5 years, with a mean of 2.9 years. The median vertebral height at 12 months after surgery was significantly higher than the preoperative height. Furthermore, the vertebral heights of all diseased vertebrae displayed significant recovery trends at 3, 6 and 12 months after surgical treatment. The percentages of vertebral height recovery to references at 12 months after surgery ranged from 34.7% to 92.5%, with an average of 71.2%. Conclusions: In children with collapsed vertebral EGs, active surgical treatment is necessary to retain the ability to restore height and to reconstruct the spine stability.

Capillaria hepatica (syn. Calodium hepaticum) is a parasitic nematode of rodents, rarely infecting humans. An asymptomatic Israeli adult male with extensive travel history was diagnosed with a liver mass on routine post-thymectomy follow-up. Imaging and computer tomography (CT) guided biopsy were inconclusive. Surgical excision revealed an eosinophilic granuloma with fragments of a nematode suspected to be C. hepatica. Molecular methods verified the diagnosis, and the patient was treated empirically. This is the first case of hepatic capillariasis described in Israel, and the first to be diagnosed using molecular methods.

Background A histiocytosis is a group of immunoproliferative disorders of clonal cells. The management protocols are still evolving, with chemotherapy as the mainstay of treatment. Objective This study aims to evaluate the feasibility, safety, efficacy, and complication profile of stereotactic radiosurgery for intracranial histiocytosis. Methodology The authors reviewed PubMed, Scopus, Web of Science, and Embase for “radiosurgery” and “histiocytosis” in the English/Japanese language following preferred reporting items for systematic reviews and meta-analyses (PRISMA) guidelines. The patient profile, radiosurgical parameters (dose and isodose), target volume, and mode of radiosurgery (Gamma knife, LINAC radiosurgery, etc.) were collected. Its use as primary or adjuvant therapy, clinical and radiological outcome was also evaluated. Results We identified 7 studies (9 patients); mean age: 41.9 years (24–57 years). Six patients received Gamma-knife radiosurgery, whereas 3 received CyberKnife radiosurgery. The Langerhans cell histiocytosis variants were eosinophilic granuloma in 3, whereas 4 were not defined. Two cases had Rosai-Dorfman disease, and 2 different yet pathogenetically related histiocytic disorders. Four patients harbored lesions in the pituitary stalk and posterior pituitary, 2 patients in the petrous region, 1 patient had a pontine lesion, and 2 patients had multiple lesions. The dose delivered ranged from 8 to 28 Gy. A total of 18 lesions (9 patients) were followed for 81.67 patient-years: 7 (39%) disappeared, 8 (44.4%) showed radiological reduction, and 2 (11%) remained stable. One lesion (5%) showed an increase in size needed surgical excision. There were no adverse effects. Conclusion The role of stereotactic radiosurgery needs to be further evaluated as the current cohort with only 9 cases (2 are Rosai-Dorfman disease) is insufficient to make conclusions. It may be a viable alternative in localized disease, along with chemotherapy and targeted surgery.

The patient is a 17-year-old female. She presented with right upper arm pain for more than 2 months. She has a history of bone biopsy.

The various presentations of osseous Langerhans cell histiocytosis (LCH) make it difficult to distinguish from other bone diseases. In addition, there is no universally accepted protocol for managing osseous LCH for single non-central nervous system-risk lesions. Here, the rare cases of two paediatric patients, aged 1 and 2 years, who presented with a solitary tibial lesion at time of LCH diagnosis, are reported. One patient progressed to multiple lesions after curettage of the original lesion. Subsequently, both patients received preventive chemotherapy using the Taiwan Paediatric Oncology Group (TPOG) revised protocol for treating low risk patients with LCH, namely, TPOG LCH2002-LR. After receiving this treatment, which included a schedule of prednisolone and vincristine for 6 weeks, followed by prednisolone, vincristine and 6-mercaptopurine for a further 48 weeks, both patients are free from recurrence or progression.