Sino-nasal malignancies are extremely rare accounting < 1

We are reporting a rare case of primary inner ear schwannomas (PIES) with an interesting clinical presentation, its diagnostic challenge, surgical management, and follow-up. A case report and review of the literature concerning PIES is presented. Key role of otolaryngologist is symptomatic management and hearing preservation or rehabilitation. This was achieved in the present case by complete resolution of her symptoms and hearing rehabilitation with cochlear implantation. Early diagnosis and management of PIES carry a good prognosis. There are no guidelines for the management of this kind of tumor due to its rarity. This case mimicking labyrinthitis, however, raised suspicion of PIES on initial imaging. The patient underwent serial imaging which revealed similar findings which were confirmed intraoperatively on tumor identification and complete excision leading to successful diagnosis at an early stage.

Lyme borreliosis is a multisystem disease transmitted by the bite of vector, Ixodes tick and caused by the strains of spirochete Borrelia burgdorferi sensu lato. The Centers for Disease Control and Prevention has listed it as a reportable disease since 1991. Although the three classical cutaneous manifestations are primary erythema migrans, borrelial lymphocytoma, and acrodermatitis chronica atrophicans, secondary disseminated erythema migrans is a rare presentation. Reported atypical erythema chronicum migrans (ECM) lesions include vesicles, erythematous papules, purpura, and lymphangitic streaks. We report a case of a female patient who was clinically, serologically, and histopathologically diagnosed as a case of Lyme disease (LD) with disseminated secondary erythema migrans with atypical edematous lesions. Only four confirmed cases of ECM have been reported in India before this report. The case highlights this atypical and rare presentation of LD in India.

Leak of urine from allograft kidney is a rare surgical complication post kidney transplant, generally occurring in the immediate post-op period and usually from the lower ureter.We present an unusual case where it occurred from upper ureter and 6 weeks posttransplant in the background of acute rejection.A 25-year-old male suffering from stage 5 CKD on maintenance hemodialysis thrice a week for an year, underwent living donor ABO-compatible kidney transplant with mother as donor.He had low immunological risk, hence started on triple immunosuppression without any induction.Laparoscopic donor nephrectomy was performed uneventfully.The immediate postoperative recovery was uneventful with progressive decline in creatinine.He suffered an acute cellular rejection a week after transplant which resolved with methylprednisolone IV pulse and anti-thymocyte globulin therapy.His creatinine settled to 1.4 mg/dL.Double-J (DJ) stent removal was done at 4 weeks posttransplant.Three days later his creatinine started rising again with preserved urine output.Repeat allograft biopsy showed acute cellular and antibody mediated rejection.He underwent five sessions of plasmapheresis along with low dose IVIg.He became oliguric with progressive abdominal distension and scrotal swelling.Examination was suggestive of free fluid in abdomen.Creatinine increased to 9 mg/dL.Ultrasonography showed massive ascites and normal graft kidney.Ascitic fluid was clear and transudative with a creatinine of 7 mg/dL.A dynamic nuclear medicine scan was done for evaluation of possible urine leak.The scan revealed ongoing urine leak from the upper ureter and renal pelvis.A DJ stent was re inserted followed by brisk diuresis and decline in creatinine to 1.0 mg/dL.The ascites resolved spontaneously and DJ stent was removed after 6 weeks.The case highlights the important role of a nuclear medicine scan in suspected urine leak and delineating the location.

Bullous lichen planus (BLP), a variant of lichen planus (LP), and lichen planus pemphigoides (LPP), a variant of bullous pemphigoid (BP) manifest with diverse clinical and histopathological features and hence, pose difficulty in diagnosis. The case is being reported to highlight important clues in history, examination, and investigations, which aid in differentiating these two close mimickers. A 49-year-old man presented with multiple, itchy raised lesions over upper and lower limbs of 6 weeks duration. Two months later, he developed multiple blisters over the pre-existing lesions, predominately over ankles and wrists. There was no history of any oral lesions/weight loss/fever/any drug intake/aggravation of lesions on sun exposure. On examination, there were multiple discrete, erythematous papules with a violaceous hue, multiple vesicles, and tense bullae on top of these lesions. Koebner's phenomenon was also present [Figure 1-3]. Nails, hair, and mucosa did not reveal any abnormality. The patient was evaluated further considering BLP and LPP as two possibilities. Two skin biopsies from a plaque and a bulla were done for HPE and DIF. Histopathology of both was consistent with LP, whereas additionally, the bulla showed a prominent subepidermal blister containing fibrin and mixed inflammatory cells [Figures 4 and 5]. Direct immunofluorescence was negative for IgG, IgM, IgA, or C3 deposits along the dermo-epidermal junction confirming the diagnosis of BLP. The patient was managed by tapering doses of oral steroids and topicals to which he responded well with significant resolution in 3 weeks.Figure 1: Multiple violaceous to erythematous papules and plaques and a few ruptured bullae over both handsFigure: s 2. Multiple violaceous to erythematous papules and ruptured bullae with surface crusting over both feet and ankle regionFigure: s 3. Multiple violaceous to erythematous papules and ruptured bullae with surface crusting over both feet and ankle regionFigure 4: Sub-epidermal bulla with necrotic keratinocytes (H&E 40×)Figure 5: Epidermis showing hypergranulosis, interface dermatitis, and necrotic keratinocytes (black circles) in subepidermal space (H&E 100×)BLP is a rare variant of LP. Both familial and sporadic forms are known to occur, with the former occurring early with prominent nail involvement. BLP is considered as a hyper-reactive form of LP, where Th1 and Th2 immune reactions mediate the pathogenesis.[1] Clinically, BLP is characterized by vesicles and tense bullae over pre-existing LP lesions and in the near vicinity with less severe pruritus compared to classical LP. Mucosal lesions usually manifest as shallow erosions as the bullae rupture very fast. Nail involvement in BLP is also frequent and shows characteristic LP features. The course of the disease is variable, and recurrence tends to occur, which can present with classical LP and rarely BLP lesions.[2] BLP must be clinically and histopathologically differentiated from LPP, which is a sub-epidermal blistering disorder resulting from IgG and C3 autoantibodies directed against basement membrane antigens. First described by Kaposi, LPP was initially considered as a separate entity; however, later it was included as a variant of BP. Prodromal urticarial wheals and pruritus are prominent in LPP, whereas the bullae appear on normal skin not harboring LP lesions. Nail involvement and mucosal LP lesions additionally aid in distinguishing BLP from LPP.[3] On histopathology, a subepidermal separation is evident, which may be accompanied by interface dermatitis. Colloid and civatte bodies are prominent in BLP, whereas they are rarely seen in LPP. Eosinophils are prominent in LPP, which is a mixed infiltrate in BLP. Direct immunofluorescence is pivotal in distinguishing LPP from BLP and shows C3 and IgG deposits along the basement membrane zone. Immunoelectron microscopic studies show deposition of IgG and C3 in the base of bulla and not in the roof as found in BP. On serology, circulating antibodies against BPAG 1 and 2 can also be detected.[4] The course of the disease is less severe than that of BP with less recurrence. Prognosis is good and so is the response to conventional therapy.[5] It is difficult to distinguish between BLP and LPP solely based on clinical presentation. However, reaching a correct diagnosis is crucial to decide upon the correct management and predicting the clinical course. The aim of this case report was to highlight overlapping features of two rare variants of two very common dermatoses leading to diagnostic dilemmas. The report also emphasizes the importance of DIF studies to differentiate the two entities. Ethics approval and consent to participate Not applicable. Consent for publication Obtained. Financial support and sponsorship Nil. Conflicts of interest There are no conflicts of interest.

Erythema nodosum leprosum (ENL) forms a part of type 2 leprosy reaction characterized by crops of tender evanescent erythematous nodules that appear on extensors accompanied by systemic symptoms like fever and arthritis. Bullous ENL is a rare variant of the type 2 reaction characterized by multiple vesicles and flaccid bullae at sites of classic ENL with severe systemic symptoms. Other atypical variants include necrotic, hemorrhagic, purpuric, Sweet syndrome-like and erythema multiforme-like ENL. Nowadays, dermoscopy is routinely employed in the diagnosis of leprosy, and it shows features akin to granulomatous dermatoses, explicitly, yellow-orange background reminiscent of underlying granulomas and few specific features. Specific features in the tuberculoid pole include loss of appendages and in the lepromatous pole xerosis, scaling, and hypopigmentation. Leprosy reactions are characterized by vascular changes in the form of arborizing blood vessels in ENL and diffuse erythema in type 1 reaction [1]. We report dermoscopic findings of bullous ENL lesions in a patient, findings that revealed both typical and atypical features.

Stevens–Johnson syndrome (SJS) is a delayed type hypersensitivity reaction to drugs and less commonly infections. The entity represents one end of the spectrum of SJS-TEN (Toxic Epidermal Necrolysis) complex. Together, both conditions contribute to the majority of Severe Cutaneous Drug Reactions (SCARs) with high mortality and morbidity. Clinically, it presents with widespread painful erythematous macules, targetoid lesions, full-thickness or focal epidermal necrosis while mucosal involvement involves oral, genital, and ocular mucous membranes along with preceding prodromal flu like symptoms. While there is no consensus on the treatment of choice, systemic steroids, intravenous immunoglobulin and cyclosporin are most often employed for specific management of SJS-TEN. We report a case of SJS managed with a single dose of etanercept monotherapy.

Anti-tubercular therapy (ATT) drugs are the mainstay of management of tuberculosis worldwide. These medicines are used extensively across the globe in treating tubercular infections of any organ. The life-threatening complications of pseudomembranous enterocolitis (PMC) associated with ATT, especially with capsule Rifampicin is not known to most practitioners. An early suspicion during the onset of loose stools in a patient on ATT is essential for managing this life-threatening condition. This fatal case report is to create awareness among the healthcare professionals and sensitize the clinicians, about the possibility of PMC in patients on ATT. We discuss our case and the lessons learnt in this case report.

Drug reaction with eosinophilia and systemic symptoms (DRESS) is a severe adverse cutaneous drug reaction with mortality up to 10%. It is a rare condition with risk varying between 1 in 1000 and 1 in 10 000 drug exposures. The aim of the study was to describe clinical features, management and drugs responsible for causing DRESS. The study was retrospective, observational study. The data of patients admitted to hospital with diagnosis of DRESS during study period (March 2018 to February 2020), were retrieved and analyzed. The descriptive data of patients were summarized. The continuous variables were summarized as mean +/- SD and/or median, depending on the skewness of the data. The categorical variables were expressed as absolute numbers, frequency, and proportions (%). The data was tabulated and analyzed in Microsoft Excel 2019 version. A total of 20 patients who met inclusion criteria (probable or definite DRESS as per RegiSCAR criteria) were included in the study. The mean age of the patients was 41.2 +/- 15.7 years. The average latency period was 26.45 +/- 5.65 days (range: 7-60). The commonest culprit drugs were dapsone and phenytoin, each in five (25%) patients. Commonest morphology of rash was morbilliform in 13 (65%) patients. One patient with targetoid rash had multi-organ involvement. Facial edema, periorbital edema, and conjunctival injection were seen in 17 (85%), seven (35%), and six (30%) cases, respectively. Eosinophilia was present in 18 (90%) patients with mean (+/- SD) value of 1976 +/- 840 cells/mu l. Liver was the commonest internal organ involved in 14 (70%) patients and kidney in three (15%) patients. The initial dose of prednisolone for treatment varied from 0.75 to 2 mg/kg/day. The mean duration of steroid treatment was 64 +/- 21 days. Two patients were treated with intravenous methylprednisolone and one with intravenous immunoglobulin. Two patients (10%) had recurrence of adverse drug reaction >6 months after completion of initial treatment and two (10%) developed autoimmune thyroiditis during follow-up. Small sample size and retrospective nature of the study were main limitations. Selection bias is a possibility as study was carried out in tertiary care center. Tests for incriminating culprit drugs such as patch test, intradermal test, and lymphocyte transformation test were not performed. DRESS is a rare disease that can be diagnosed early with high index of suspicion and treated successfully with steroids. The internal organ involvement is common in DRESS and requires a thorough evaluation.

Overview Mesenchymal tumors of the breast are rare. Few epithelial tumors also have mesenchymal components. It is crucial to identify these as per histogenesis. This can be facilitated by markers of epithelial-mesenchymal transition (EMT). Objectives The aim of this study was to categorize the breast lesions with mesenchymal morphology and to study EMT on immunohistochemistry (IHC). Materials and Methods This is a retrospective study of 5-year duration from January 2015 to December 2019. Inclusion criteria: all breast lesions showing mesenchymal/nonepithelial morphology, complete or partial, on histology. Exclusion criteria: Mammary carcinomas without any mesenchymal/nonepithelial morphology, fibroadenomas, and lymphomas. Demographics, clinical, gross examination, histology, and IHC findings of selected cases were reviewed and recorded. Three additional markers p53, E-cadherin, and β-catenin were performed. Statistical Analysis Used Frequency calculation for each variable (IHC). Results Thirteen (2.5%) out of total 510 breast specimens showed mesenchymal histology. Of these, five (38.5%) were metaplastic breast carcinomas (MBC), four (31%) were phyllodes tumor (PT), and one (7.7%) case each of malignant peripheral nerve sheath tumor, primary stromal sarcoma of breast, pseudoangiomatous stromal hyperplasia, and myofibroblastoma. Loss of E-cadherin was seen in 4/5 (80%) MBCs and was retained in ductal component of PTs. p53 was not expressed in any of the tumors except 3/5 (60%) MBCs. β-Catenin was aberrant in all MBCs. Conclusions Primary breast tumors with mesenchymal morphology present a spectrum ranging from benign mesenchymal, fibroepithelial neoplasms to malignant tumors of mesenchymal and epithelial origin. Loss of E-cadherin, expression of p53, and aberrant expression of β-catenin are suggestive of EMT and molecular heterogeneity of MBCs.

Background: Medical thoracoscopy is a minimally invasive technique to inspect the pleural space and to perform biopsy of pleural lesions under direct vision. In this study, the diagnostic utility of thoracoscopy for undiagnosed exudative lymphocytic pleural effusions from a tertiary care respiratory center will be discussed. Materials and Methods: In a retrospective analysis of thoracoscopic procedures, we performed between September 2017 and August 2019, the yield of thoracoscopic pleural biopsy for achieving a diagnosis in undiagnosed exudative lymphocytic pleural effusions was evaluated. Undiagnosed exudative pleural effusions were defined as pleural effusions where an etiologic diagnosis could not be ascertained by initial pleural fluid biochemical and microbiological analysis, including protein, sugar, lactate dehydrogenase, Gram and acid-fast bacilli stains and Mycobacterium tuberculosis culture, pleural fluid adenosine deaminase levels, and at least two pleural fluid cytologies negative for malignant cells or other definite causes. We analyzed the clinical, radiological, cytological, and histopathological data of the patients and also the complications of thoracoscopy. Results: We performed thoracoscopy in 68 cases of undiagnosed exudative pleural effusions using the rigid thoracoscope. The overall diagnostic yield of thoracoscopic pleural biopsy was 95.6% in patients with undiagnosed exudative pleural effusions. Malignant pleural effusion was diagnosed in 67.6% of patients, while tuberculosis was diagnosed with pleural biopsy in 25% of patients. Three cases of subcutaneous emphysema, 2 cases of postprocedure fever were observed, and one patient had prolonged air leak. Conclusion: Medical thoracoscopy has a good diagnostic yield in patients with undiagnosed exudative lymphocytic pleural effusions and is a safe procedure as well.

The authors declare no conflict of interest. The authors declare that data supporting the findings of this case are available within the article [and its supplementary information files].

Kimura disease (KD) is a rare chronic inflammatory disorder of unknown cause, primarily seen in young Asian males. The disease is characterized by painless subcutaneous swelling in head and neck region, accompanied by regional lymphadenopathy and frequent salivary gland enlargement. Blood and tissue eosinophilia, and elevated immunoglobulin E (IgE) levels, are common associations. Characteristic histopathological findings of biopsy specimens obtained from the subcutaneous swellings or lymph nodes include eosinophilic infiltrates, follicular hyperplasia, and proliferation of postcapillary venules. The course is usually waxing and waning, albeit benign. Early diagnosis may spare the patient from unnecessary invasive procedures. We herein, describe a case of KD in an 18 years old male, who presented with subcutaneous swelling in the both cheek (right more than left) in 2014, underwent multiple surgical interventions for the same, before being reassessed and finally diagnosed as KD in 2020, based upon peripheral blood eosinophilia, raised serum IgE levels and histopathological findings. He subsequently made a good recovery on oral steroids.

IgG4-related disease (IgG4-RD) is an emerging disease concept that was first recognized in the 21st century. It has since then been attracting substantial attention in many fields of medicine as it tends to involve either synchronously or metachronously, various organs, including the pancreas, bile duct, lacrimal gland, salivary gland, and many others. This umbrella diagnosis now successfully explains a substantial number of disorders, which were previously regarded as “idiopathic.” Reports of the manifestations of IgG4-RD in the head and neck are extremely rare. Otologic manifestations have been reported, but only a handful of cases are available in literature that have been confirmed by immunohistopathology. We, herein, present a case of IgG4-RD of the middle ear, which manifested as a growth in the left mastoid and middle ear cavity, and became symptomatic with symptoms of chronic otitis media in the left ear, and was subsequently diagnosed to have IgG4-RD.

Introduction: Corona Virus Disease-19 (COVID-19) caused by the Severe Acute Respiratory Syndrome-Corona Virus-2 (SARS-CoV-2) has led to a global pandemic. Currently, the standard method for the diagnosis of COVID-19 is the detection of SARS-CoV-2 by Reverse Transcriptase Quantitative Polymerase Chain Reaction (RT-qPCR). Lower Cycle threshold (Ct) value of PCR is found to be associated with increased probability of progression to severe disease and infectivity. Blood group A individuals have been found to be more susceptible to infection by SARS-CoV-2 while that of O blood group have lower risk of infection. There has been growing scientific interest to obtain data which may characterise the susceptibility to COVID-19 infection and determine the risk factors which may be associated with progression and severity of the disease. Aim: To study the association of RT-qPCR Ct values for SARS-CoV-2 with ABO blood group types. Materials and Methods: A prospective observational study was conducted at tertiary care centre in western Maharashtra, India where in a total of 116 symptomatic admitted patients between mid-July to mid-August 2020 and was found to be positive for SARS-CoV-2 by RT-qPCR were enrolled in the study. Ct values for pan-Sarbecovirus E-gene and SARS-CoV-2 specific RdRP gene were recorded for each patient. In addition, all the patients were typed for ABO blood grouping. Distribution of different ABO blood group types in SARS-CoV-2 positive patients were analysed and was compared with blood groups in 882 non-COVID-19 blood donors comprising control group. Ct values for E and RdRP gene were also analysed in respect to different age group, gender and blood group types. Blood group records of voluntary healthy blood donors reporting at our centre between September to November 2019 were obtained. Results: Of the 116 patients, 33 (28.45%) comprised of type A, 34 (29.31%) of type B, 41 (35.34%) were of type O and remaining 8 (6.90%) had AB blood group type. Compared to the control group, no significant difference was noted in the distribution of ABO blood types in SARS-CoV-2 positive patients. Also, no statistical significance was found in the Ct values with respect to age, gender and different ABO blood group types. Conclusion: RT-qPCR Ct values for SARS-CoV-2 infection have no association with ABO blood group types.

Graft versus host disease (GVHD) is a unique entity wherein the donated marrow cells (graft) view the hosts as foreign and attack various body organs. Skin is the most frequently affected organ followed by mucosa, eyes, gastrointestinal, respiratory, musculoskeletal system, and other organs. The incidence of GVHD varies from 25 to 80%. Cutaneous involvement can present as exanthem, epidermolysis, lichenoid eruptions, erythroderma, ichthyosis, pityriasis rubra pilaris like lesions, psoriasiform lesions or just pruritus. Asymptomatic truncal follicular eruptions as the major presentation is rare. We report a case of aplastic anemia that developed extensive truncal folliculocentric papules 10 months following an allogeneic hematopoietic stem cell transplantation. Histopathological examination of the follicular lesions revealed perifollicular inflammatory infiltrate comprising of lymphocytes, plasma cells and histiocytes at the dermo-epidermal junction. Basal cell vacuolization, pigment incontinence in the upper dermis and few apoptotic keratinocytes in the follicular epidermis were also seen. The patient responded satisfactorily to tapering doses of steroids.

A 73 years old female presented with an insidious onset asymptomatic palm‐sized ring‐shaped lesion over the neck (right) which had been progressing gradually for the past one year. There was no history of itching, altered or loss of sensation, prolonged drug intake before the onset of the lesion, any comorbidities such as diabetes mellitus or any systemic involvement. She had routine outdoor exposure to sunlight.

Context: Gastric cancer is the fifth most common malignancy, with a high incidence in Eastern Asian countries. Diet is an important risk factor in the genesis of gastric cancer, and the fact that it is modifiable warrants it to be studied extensively in relation to various clinicopathological parameters of gastric cancer. Aims: The present study was undertaken to study the dietary habits (vegetarian/nonvegetarian) among gastric carcinoma patients in the Indian context. Materials and Methods: We studied 100 consecutive cases of gastric carcinoma excluding tumors at gastroesophageal junction. Clinical data regarding food habits in the patients were collected by personal enquiry. Pathological characteristics including size, site, gross appearance, Lauren's histological type, and the World Health Organization (WHO) histological type were noted. Data analysis was done using Chi-square test. Results: Sixty-nine percent of gastric cancer patients were vegetarians and 31% were nonvegetarians. Statistically significant association between nonvegetarian diet and gastric cancer location at the lesser curvature was found (P ≤ 0.001). No statistically significant association between diet and gross appearance, Lauren's, and WHO histological type of gastric cancer was found. Conclusions: For anatomical locations, most gastric cancers located at lesser curvature had been developed among nonvegetarian patients, whereas we did not observe diet to be related to gross appearance and histological type of gastric carcinoma.