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Background: Syringocystadenoma papilliferum is a benign adnexal neoplasm. Contiguous squamous proliferation has been rarely described in syringocystadenoma papilliferum. Aims: This study aimed to evaluate the spectrum and pathogenesis of contiguous squamous proliferation in syringocystadenoma papilliferum. Materials and Methods: All cases of syringocystadenoma papilliferum diagnosed over the past 12 years were screened for contiguous squamous proliferation. Cases with associated nevus sebaceous were excluded from the study. Immunohistochemistry for GATA3, CK7, BRAFV600E and p16 was performed. PCR for human papilloma virus, type 16 and 18, was carried out. Results: Of a total of 30 cases, 14 cases showed associated contiguous squamous proliferation which included four cases of verrucous hyperplasia, six cases with papillomatosis, two cases with mild squamous hyperplasia and one case each of Bowen’s disease and squamous cell carcinoma. In the cases with non-neoplastic contiguous squamous proliferations, the squamous component did not express CK7 or GATA3. However, the squamous component of premalignant and malignant lesions expressed CK7 and GATA3 concordant with the adenomatous component. BRAF was positive in adenomatous component in five cases while the contiguous squamous proliferation component was negative for BRAF in all but one case. p16 was negative in both components of all cases and PCR for human papilloma virus was negative in all cases. Limitations: Due to the rarity of disease, the sample size of our study was relatively small with two cases in the 2nd group, that is, syringocystadenoma papilliferum with malignant contiguous squamous proliferation. Detailed molecular studies such as gene sequencing were not performed. Conclusion: Syringocystadenoma papilliferum with contiguous squamous proliferation is underreported, and most commonly displays verrucous hyperplasia. The premalignant and malignant contiguous squamous proliferations likely arise from syringocystadenoma papilliferum while the hyperplastic contiguous squamous proliferations likely arise from the adjacent epidermis. Relationship with high-risk human papilloma virus is unlikely. However, further molecular analysis of larger number of cases is required to establish the pathogenesis.

Palmoplantar involvement has been infrequently reported in leprosy and is an easily misdiagnosed entity. The institutional database of leprosy clinic from 2015 to 2018 was accessed. Details pertaining to demography, clinical presentation, comorbidities (if any), treatment received, and outcome were analyzed in leprosy patients with palmoplantar involvement. Among the 520 patients studied, the involvement of palms and/or soles was reported in 49 (9.4%) patients. Isolated palm involvement was the most frequent (26/49, 53.1%), followed by both palm and sole involvement (12/49, 24.5%) and sole involvement alone (11/49, 22.4%). A higher incidence of lepra reactions and disabilities was noted in patients with palmoplantar involvement than in those without (P < 0.0001). Palmoplantar involvement in leprosy, although uncommon, is associated with a higher risk of reactions and disabilities. A knowledge of this aspect of leprosy can help in maintaining a high index of suspicion and reduce misdiagnosis.

This study reports a rare case of cryptococcoma of the brain and review the related literature. An intracranial lesion, in a healthy, middle-aged adult who underwent surgery with a preoperative diagnosis of neoplastic/metastatic lesion, which turned out to be cryptococcoma, is being reported. Histopathological examination revealed that this immunocompetent patient had cryptococcoma of the cerebellum. He responded to antifungal chemotherapy (amphotericin B initially for 2 weeks and later oral fluconazole for 10 weeks) very well. Occurrence of cryptococcomas in immunocompetent patients is rare and responds to antifungal chemotherapy very well. Only six cases (including this) have been reported in literature till now.

Introduction: Acquired dermal hyperpigmentation (ADMH) presenting on periorbital region has been described as individual case reports. We tried to characterize the features of periorbital ADMH. Materials and Methods: This was a retrospective case-control study among our patients who attended the pigmentary clinic during January 2016–December 2017. Clinical, dermoscopic, and histopathological features of subjects who were recruited during the study period were prospectively evaluated. Results: Total 19 subjects (11%) were identified among 177 ADMH patients. Periorbital ADMH patients had a relatively younger age of onset (23.26 ± 11.06 vs. 36.16 ± 13.41, P < 0.001). Dermoscopy of early periorbital ADMH showed only imperceptible speckled blue-gray dots that accentuated at outer-corner creases of eyes (the “outer-corner crease sign”). Clinicopathological features and prognosis of periorbital ADMH were similar to that of ADMH per se. Conclusion: Periorbital ADMH should be considered as a differential diagnosis of periorbital hyperpigmentation in children and young adults. Outer-corner crease sign on dermoscopy may help to rule out other differentials in its early presentation.

Chronic myeloid leukemia (CML) is the most common leukemia presenting with priapism. Due to social stigma or shyness, the real incidence of priapism in our population is difficult to estimate. Since the successful development of tyrosine kinase inhibitors, the life expectancy of CML-chronic phase (CML-CP) has been comparable to the healthy population, and the importance of priapism has further magnified. As of now, the long-term outcome and fertility issues are not very well known. We present the case of a 24-year-old previously healthy gentleman who presented with priapism and was diagnosed as CML-CP. He achieved major molecular response at 18 months. On follow-up at 2 years since the diagnosis, he offers no complaint except moderate erectile dysfunction (International Index of Erectile Function score 11), compromising his quality of life.

Langerhans cell histiocytosis (LCH) is a rare histiocytic disorder characterized by clonal proliferation of neoplastic Langerhans cells (LCs). LC proliferation can also be seen in different reactive dermatosis. CyclinD1 is a downstream marker of mitogen-activated protein (MAP) kinase pathway, which is often activated in LCH. This study aimed to evaluate the role of cyclinD1 to differentiate reactive LC proliferation from LCH. All cases of cutaneous LCH diagnosed by biopsy in the past 3 years (n = 13) were immunostained with CD1a, p53, CD31, and cyclinD1. Seven cases each of discoid lupus erythematosus (DLE) and lichen planus (LP) were taken as control. Presence of p53, CD31, and cyclinD1-positive LCs (CD1a-positive) were compared in the dermis. In all LCH cases, dermal neoplastic LCs showed diffuse CD1a positivity and 12 cases (92.3%) showed variable (30%-70%) cyclinD1 expression. Weak p53 and CD31 expression were seen in 61.5% and 46.1% of LCH cases, respectively. In the control group, 5 cases of LP and 4 cases of DLE showed variable LC proliferation, highlighted by CD1a positivity. However, no case of reactive dermatosis showed cyclinD1 or p53 expression by the reactive LCs. Weak and patchy CD31 expression by the reactive LCs were found in 1 (25%) and 2 (40%) cases of DLE and LP, respectively. To conclude, cyclinD1 is frequently expressed in neoplastic LCs in LCH. It is an efficient marker to differentiate neoplastic from reactive LC proliferation, and can be used as a surrogate marker in LCH.

INTRODUCTION Pineal parenchymal tumor of intermediate differentiation (PPTID) is an uncommon tumor of the pineal gland. Although this behaves as a grade II/III tumor, the exact clinical behavior is not well known. There is no well-established pathologic factor that can predict the behavior of PPTID. AIM AND OBJECTIVE The aim of this study was to determine the pathologic prognostic factors in PPTID. MATERIALS AND METHODS All PPTID cases diagnosed between 2006 and 2016 were analyzed retrospectively. Immunohistochemistry for synaptophysin, neurofilament protein (NFP), glial fibrillar acid protein, NeuN, and Ki-67 were performed in all cases. Cases were classified arbitrarily into low grade (mitosis

OBJECTIVE To characterize glioma preoperatively using quantitative 99mTc-methionine SPECT and comparison with MR-perfusion/spectroscopy and histopatholgical/Ki-67 scoring. METHODS Twenty-nine patients (21M: 8F; mean age 42.3 ± 10.5 years) with clinical and radiological suspicion of glioma assessed by 99mTc-MDM/SPECT and ceMRI. Additionally, 12/29 patients underwent dynamic susceptibility contrast-enhanced (DSCE) MRI and magnetic resonance spectroscopy (MRS) examination. Three patients with benign pathologies were recruited as controls. Histopathological tumor analysis was done in all (n = 29) the patients, and the Ki-67 index was evaluated in 20/29 patients. The target-to-nontarget (T/NT) methionine tumor uptake ratios, normalized cerebral blood volume (nCBV) and metabolites [choline/N-acetyl aspartate (Cho/NAA), Cho/creatine (Cr), Cr/NAA and Cr/Cho) ratios were measured in tumor areas. RESULTS On histopathological analysis, 26/29 patients had glioma (G IV-13; G III-04; G II-09). The mean T/NT ratio in G-II was significantly lower (2.46 ± 2.3) than in G-III (7.13 ± 2.2) and G-IV (5.16 ± 1.2). However, the mean ratio was highest (15.9 ± 6.8) in meningioma (n=3). The T/NT cutoff ratio of 3.08 provided 100% sensitivity, 87.5% specificity for discriminating high-grade glioma (HGG) from low-grade glioma (LGG) disease. Likewise, the nCBV cutoff of 2.43 offered 100% sensitivity and 80% specificity. Only the Cho/NAA cutoff value of greater than 3.34 provided reasonable sensitivity and specificity of 85.7% and 80.0% respectively for this differentiation. T/NT ratio correlated significantly with nCBV and Cho/NAA, Cho/Cr ratios but not with Ki-67. CONCLUSION Quantitative 99mTc-MDM -SPECT provided high sensitivity and specificity to differentiate HGG versus LGG preoperatively and demonstrated a potential role for the differential diagnosis of glial versus nonglial tumors.

Background Defect in internal elastic lamina, defect in tunica media, and the amount of collagen and elastin play a role in vessel wall weakening leading to aneurysm formation. A similar picture may be found in connective tissue disorders, such as Marfan syndrome, Ehlers-Danlos syndrome (EDS), neurofibromatosis type 1, and Loeys-Dietz syndrome (LDS), where there is a predominant disorder of collagen formation/maturation. Methods Histopathology of skin and the superficial temporal artery (STA) was done. All specimens were obtained during craniotomy for ruptured aneurysm clipping or other indicated procedures (for control subjects). Parameters in skin biopsy seen were epithelial thickness, dermal collagen thickness, and so forth. For the STA, parameters such as intimal thickness, intimal proliferation, thickness of media, and so forth were studied. Results Twenty cases and twenty control subjects were studied. The mean age of the study population was 40.5 years. Salient findings on skin biopsy in patients of intracranial aneurysms (IAs) (cases) were haphazard orientation of collagen, inflammation in the subepidermal layer, increased dermal collagen thickness, and reduced and/or fragmented elastic fibers. Prominent findings on vessel wall biopsy were intimal proliferation, reduplication of internal elastic lamina, reduced and/or fragmented elastin, and vacuolation of smooth muscle cells. The average number of aberrations per patient was significantly higher in cases than control subjects. Conclusions The histologic changes seen in skin and the STA in cases of IAs signify a weak connective tissue. Some of these findings are also seen in known connective tissue disorders such as Marfan syndrome, EDS, neurofibromatosis type 1, and LDS. The connective tissue abnormalities found in patients with IAs may be congenital, which gets further accentuated by known risk factors leading to weak vessel wall and subsequent aneurysm formation.

The pituitary function is regulated by a complex system involving the hypothalamus and biological networks within the pituitary. Although the hormones secreted from the pituitary have been well studied, comprehensive analyses of the pituitary proteome are limited. Pituitary proteomics is a field of postgenomic research that is crucial to understand human health and pituitary diseases. In this context, we report here a systematic proteomic profiling of human anterior pituitary gland (adenohypophysis) using high-resolution Fourier transform mass spectrometry. A total of 2164 proteins were identified in this study, of which 105 proteins were identified for the first time compared with high-throughput proteomic-based studies from human pituitary glands. In addition, we identified 480 proteins with secretory potential and 187 N-terminally acetylated proteins. These are the first region-specific data that could serve as a vital resource for further investigations on the physiological role of the human anterior pituitary glands and the proteins secreted by them. We anticipate that the identification of previously unknown proteins in the present study will accelerate biomedical research to decipher their role in functioning of the human anterior pituitary gland and associated human diseases.

We present the case of a 36-year-old woman who underwent (18)F-FDG PET/CT with suspicion of a primary breast malignancy. However, PET/CT detected an occult renal cell carcinoma with metastases to the thyroid, breast, lungs and lymph nodes. Thyroid and breast metastases are atypical metastatic sites of renal cell carcinoma. Breast metastases from extra mammary tissue are extremely rare, more so from renal cell carcinoma. Histopathologic confirmation of the breast lesions is imperative to avoid unnecessary mastectomy and imaging can help in raising the suspicion of metastatic involvement versus primary breast malignancy.

Convexity subarachnoid hemorrhage (SAH) represents 6% to 7% of all cases of subarachnoid hemorrhage, with cerebral amyloid angiopathy and reversible cerebral vasospasm accounting for 55% to 70% of all cases.

Background: Previous studies have tried to relate magnetic resonance (MR) characteristics of meningiomas with its intraoperative features and histopathology with varying results. This is a prospective study to assess the independent predictive value of conventional MR signals [T1, T2, and fluid attenuated inversion recovery (FLAIR)] in relation to consistency, vascularity, operative plane, Simpson excision, and histopathology of intracranial meningiomas in a multivariate model. Materials and Methods: Seventy patients underwent T1, T2, FLAIR, postcontrast sequences followed by excision of their meningiomas. Tumor signal intensity in various sequences was studied in relation to the above-mentioned variables. Multivariate analysis to find their independent association was performed. Results: T1 images showed no correlation with any of the variables. FLAIR hypointensity and inhomogeneous enhancement had significant association with tumor hardness. FLAIR hypointense tumors were associated with low vascularity. FLAIR hypointensity, skull base location, and recurrence were significantly related to the subpial or mixed operative plane. The meningioma–brain interface on T2 sequence was significantly related to the operative plane. Only skull base location had significant impact on the extent of excision. T2 and FLAIR hypointensity had significant association with fibroblastic or psammomatous meningiomas. On multivariate analysis, FLAIR hypointensity and skull base location had a significant independent relationship with suboptimal operative plane, while skull base location had association with the extent of excision. Among MRI sequences, FLAIR hypointensity showed high specificity (94%) in predicting the suboptimal operative plane. Conclusions: FLAIR hypointensity of meningiomas appears to have a significant independent association with the suboptimal operative plane with high specificity.

Low-grade epithelial tumor of pituitary region with dominant papillary architecture is extremely rare. We describe a case of 20-year female who had a recurrent nonfunctioning pituitary tumor. Histologic examination revealed a low-grade epithelial tumor with predominant papillary architecture, lined by cuboidal to columnar epithelial cells. The tumor cells were immunpositive for cytokeratin (CK), CK7, epithelial membrane antigen, carcinoembryonic antigen and showed diffuse and strong nuclear positivity for thyroid transcription factor 1. They were negative for neuroendocrine markers and pituitary hormones. Ki-67 proliferation index was low (1%). Ultrastructural examination revealed presence of microvilli, intercellular tight junctions, and keratin filaments within the tumor cells and lack of neurosecretory granules. No lesion was identified in thyroid or lung on systemic evaluation. On the basis of the morphology, immunophenotype, ultrastructural findings, and diffuse thyroid transcription factor 1 positivity, this tumor may represent an epithelial variant of pituicytoma with dominant papillary architecture. This type of differentiation is extremely rare, and to the best of our knowledge, has not been described previously in the literature.

Background Granulomatous dermatitis due to noncontiguous involvement of Crohn disease (CD) of the gut has been described as metastatic CD (MCD). MCD is the rarest form of cutaneous manifestations of CD. This study aims to analyze the clinicohistological features of MCD in a tertiary care center of India. Materials and methods A retrospective review of patients diagnosed clinically and histologically with MCD over past 5 years was performed. Data on cutaneous features, histological findings, and response to treatment were collected. Results Twelve patients (3 men and 9 women) with a mean age of 29 years were identified. All women had vulval involvement in the form of edema (80%), ulceration (60%), and fistula (20%). Among the 3 men, 2 had perineal and scrotal swelling and ulcer, whereas the third patient presented with leg ulcer. Intestinal CD was already diagnosed in 50% patients (5/10) at cutaneous presentation, whereas it was diagnosed subsequently in 30% (3/10) cases. Histological examination revealed nonnecrotizing granulomatous inflammation in the dermis in 11 patients (92%). Additional histological features included eosinophilic infiltrate (58%), panniculitis (33%), and vasculitis (33%). The patients were treated with various combinations of oral prednisolone, metronidazole, minocycline, azathioprine, and subcutaneous adalimumab with partial relief. Conclusion MCD shows a wide spectrum of clinical presentation, with anogenital involvement being the most common. Histology reveals nonnecrotizing granulomas in the dermis in majority of the cases. The diagnosis is extremely challenging in patients without gastrointestinal involvement at presentation, and thus, a high index of suspicion is imperative.

Nevoid hyperkeratosis of the nipple and/or areola (NHNA) is an uncommon disease with no definite etiology. NHNA of the male breast is rare in clinical practice. Despite being a benign disease, it is distressing for patients and therapeutically challenging for clinicians. We report a male patient with NHNA who responded favorably to minocycline treatment.

Background Kikuchi-Fujimoto disease (KFD) is a rare disorder described mostly in adolescents and young adults. It is a great mimicker, has a diverse clinic-laboratory profile and appears to be an under-recognized disease entity, especially in children. Methods We report 6 children who presented with fever and lymphadenopathy and had findings on fine needle aspiration cytology (FNAC) and/or histopathology that were compatible with a diagnosis of KFD. Two amongst these 6 patients developed lupus. Results Case 1: A 7 year old boy was admitted with complaints of high-grade fever and left submandibular lymph node swelling for 2 weeks. A possibility of suppurative lymphadenitis was kept and he was treated with antimicrobials. He was the youngest born to a consanguineously married couple who had lost two children previously to a lupus-like illness. The striking family history suggested the possibility of a monogenic form of lupus (early complement deficiency). C1q levels were found to be significantly low (C1q level: 0.27 mg/L; normal (102–170 mg/L)) Genetic analysis revealed a nonsense mutation in the C1QA gene (c.622C>T Q208X). Investigations revealed mild anemia (hemoglobin:107 gm/L) and elevated erythrocyte sedimentation rate (ESR:68 mm) in 1 st hour and C-reactive protein (CRP:8 mg/dl). An excision biopsy of the lymph node showed necrotizing histiocytic lymphadenitis consistent with KFD. Immunological work-up revealed Antinuclear antibodies (ANA) 2+speckled pattern on immunofluorescence; no anti ds-DNA antibodies; no antiphospholipid antibodies. He was treated with immunosuppressants and twice-daily fresh frozen plasma. Fever subsided in 5 days and lymph nodes regressed in 2 weeks. Case 2: A 12-year-old boy presented with fever of 7 months duration and generalized seizures followed by altered sensorium. He was treated in lines of subacute meningo-encephalitis. As there was no response to treatment he was referred to our institute. On examination, he had malar rash, frontal alopecia and bilateral, multiple enlarged cervical lymph nodes. Laboratory investigations revealed anemia, elevated ESR, high CRP and low levels of C3 and C4. ANA was positive (3+speckled); anti-dsDNA titers were 130 IU/ml (normal- Conclusions The association of KFD with childhood onset lupus is unusual. Early recognition of this entity can prevent diagnostic and therapeutic errors Funding Source(s): nil. Kikuchi-Fujimoto disease in childhood onset lupus: a case series from a tertiary care center in North India

Background: Thyroid-stimulating hormone (TSH)-secreting pituitary adenoma (TSHoma) is the rarest functioning pituitary adenoma. Methods: A retrospective analysis of eight patients of TSHomas to highlight the presentations, diagnostic challenges, and treatment outcomes. Results: Median age at diagnosis was 42 years, median latency to diagnosis was 2.5 years, and thyrotoxic and compressive symptoms were the most common presenting symptoms. At presentation, three cases were plurihormonal, six cases were on medical treatment including thyroxine, and two cases were incidentally discovered. Imaging revealed macroadenoma in all cases. Seven cases underwent pituitary surgery, after which three achieved remission. Another case entered remission after adjunctive radiotherapy. Thyrotropin (TSH) immunostaining was demonstrated in six out of seven adenomas. Conclusion: TSHoma is a rare functioning pituitary tumor with both silent and symptomatic presentations. Diagnosis can be established with biochemical and imaging features, even without dynamic tests.

IgG4-related disease is relatively new disease entity and a rare one, and our knowledge of this entity continues to evolve. It was first described in the pancreas and since then has been described in virtually every organ. Spinal involvement resulting in pachymeningitis is rare, and there are only 8 reported cases of the same to date, with the cervicothoracic spine being the most commonly affected region. The authors describe 2 cases in which the patients presented with spinal compression resulting in myeloradiculopathy (Case 1) and radiculopathy (Case 2). Imaging of spine in both cases revealed an ill-defined contrast-enhancing lesion at the lumbar level. Preoperatively, a diagnosis of spinal tumor was made, but intraoperatively no spinal tumor was found. The diagnosis was established histopathologically. The disease has no particular defining features clinically or radiologically and can mimic common spinal tumors. It is important to accurately diagnose this rare entity because of its multisystem involvement and progressive course. Strict treatment guidelines have yet to be formulated. Although histologically this disease can mimic other inflammatory conditions, the presence of storiform fibrosis and an increased number of IgG4-positive plasma cells can help in clarifying the diagnosis.

Dowling–Degos disease (DDD) is a late-onset genodermatosis characterized by hyperpigmented macules on the flexures along with scattered comedo-like lesions and pitted acneiform scars. Follicular Dowling–Degos is a rare type of DDD, with only two reports so far. It presents with follicular papules and comedo-like lesions predominantly on the face and trunk. Dermoscopy of follicular DDD shows irregular star-shaped/Chinese letter pattern pigmentation along with comedo-like lesions. Herein, we describe diagnostic clues including dermoscopy in three patients of follicular DDD which can help in differentiating it from other disorders presenting with comedo-like lesions .

Background Low-grade fibromyxoid sarcoma (LGFMS), an uncommon soft tissue tumor, generally occurs in extremities and is characterized by an aggressive clinical course with frequent recurrences. Primary intracranial occurrence of LGFMS is rare. The few existing descriptions present the clinicopathologic aspects of LGFMS and lack information from a radiologic perspective. In these reports, the lesions appeared solid on imaging. Case Description In a 45-year-old woman, a falx-based lesion with solid-cystic radiologic appearance that turned out to be LGFMS on histopathology. The patient's clinical follow-up (2 years) is presented along with a literature review of other primary central nervous system cases. Conclusions This report emphasizes the radiologic aspects of LGFMS and describes an atypical solid-cystic appearance of this uncommon central nervous system tumor. This adds another facet to the imaging of LGFMS. Although rare, LGFMS can form a differential diagnosis of dural-based lesions.

Background: Imatinib, a tyrosine kinase inhibitor, causes growth failure in children with chronic myeloid leukemia probably by targeting the growth hormone (GH)/insulin like growth factor-1 (IGF-1) axis. We aim to explore the imatinib targets expression in pituitary adenomas and study the effect of imatinib on GH secretion in somatotropinoma cells and GH3 cell line.Materials and Methods: The expression pattern of imatinib's targets (c-kit, VEGF, and PDGFR-α/β) was studied using immunohistochemistry and immunoblotting 157 giant (≥4 cm) pituitary adenomas (121 non-functioning pituitary adenomas, 32 somatotropinomas, and four prolactinomas) and compared to normal pituitary (n = 4) obtained at autopsy. The effect imatinib on GH secretion, cell viability, immunohistochemistry, electron microscopy, and apoptosis was studied in primary culture of human somatotropinomas (n = 20) and in rat somato-mammotroph GH3 cell-line. A receptor tyrosine kinase array was applied to human samples to identify altered pathways.Results: Somatotropinomas showed significantly higher immunopositivity for c-kit and platelet-derived growth factor receptor-β (PDGFR-β; P < 0.009 and P < 0.001, respectively), while staining for platelet-derived growth factor receptor-α (PDGFR-α) and vascular endothelial growth factor (VEGF) revealed a weaker expression (P < 0.001) compared to normal pituitary. Imatinib inhibited GH secretion from both primary culture (P < 0.01) and GH3 cells (P < 0.001), while it did not affect cell viability and apoptosis. The receptor tyrosine kinase array showed that imatinib inhibits GH signaling via PDGFR-β pathway.Conclusion: Imatinib inhibits GH secretion in somatotropinoma cells without affecting cell viability and may be used as an adjunct therapy for treating GH secreting pituitary adenomas.

Skin is commonly affected by vasculitic process and often subjected to biopsy. Cutaneous vasculitis can be either primary or part of a systemic vasculitic process. This study was conducted to evaluate the diagnostic utility of direct immunofluorescence (DIF) in determination of etiology of cutaneous vasculitis. All histologically proven cases of cutaneous vasculitis over the past two and half years were retrospectively analyzed along with their clinical and DIF findings (IgG, IgA, IgM, and C3). Within this study period, a total of 198 cases of small-vessel vasculitis were diagnosed based on skin biopsy and DIF findings. The mean age of patients was 31.2 years (range 1-84 years) with slight male dominance (M:F ratio 1.06:1). Henoch-Schonlein purpura/IgA vasculitis was the commonest clinical diagnosis (31%), followed by urticarial vasculitis (11%) and others. Idiopathic vasculitis was suspected in 33% cases. Overall, DIF was positive in 60% (119/198) cases, with vascular deposition of IgA being commonest, followed by C3. The clinical diagnosis of Henoch-Schonlein purpura could be confirmed in 61.5% (40/65) cases by DIF, whereas another 20 unsuspected cases were picked up as IgA vasculitis based on DIF findings. DIF findings confirmed lupus vasculitis in 50% cases. Other cases showed variable nonspecific deposition of C3 and IgM in 42% cases. DIF can be highly useful to classify cutaneous vasculitis, with maximum efficacy for diagnosis of IgA vasculitis and lupus vasculitis. It can aid in the accurate diagnosis even when the histological changes are minimal. All cases of suspected cutaneous vasculitis should be subjected to DIF.

Mucormycosis, a rare opportunistic infection seen in immunocompromised hosts, is caused by fungi of Mucorales family. It may be confined to the organs, such as rhinocerebral and pulmonary mucormycosis, or may cause disseminated infection. A 14-year-old boy presented to our clinic with fever and left upper quadrant abdominal pain, and on evaluation was found to have pancytopaenia, and imaging revealed ill-defined splenic collection with thrombus in the splenic vein. He was started on empirical intravenous antibiotics, followed by antifungals empirically as he did not show any improvement clinically. Eventually, splenectomy was done, which on histopathological examination revealed mucormycosis. The patient finally succumbed to his illness as he developed peritonitis and refractory shock. To date, only two cases of isolated splenic mucormycosis have been reported. Aggressive treatment is needed, which includes the use of antifungals (amphotericin B) and surgical debridement or resection of the involved tissues or organs.

Plasma cell vulvitis (PCV) is a rare benign inflammatory condition of the female genitalia. Its equivalent condition in males is plasma cell balanitis, which is a well-known entity and was first de...

Dear Editor, Ticagrelor is a recently introduced antiplatelet drug that has shown superiority over clopidogrel in reducing overall mortality in acute coronary syndrome (ACS). The drug is an ADP P2Y12 receptor inverse agonist.1 We report here a patient who developed neutrophilic eccrine hidradenitis (NEH) 3 days after initiating ticagrelor. A 66-year-old farmer was hospitalised after he was diagnosed with an ST elevation ACS. He was a known smoker, and had been receiving aspirin for the past 2 years. During his stay in hospital, he was administered loading doses of aspirin and ticagrelor, before stenting was performed in the left anterior descending branch of the left coronary artery. He was subsequently discharged on ticagrelor 90 mg twice daily (orally). The rest of the medications were continued. Following hospital discharge, after 3 days he developed multiple painful erythematous papulonodules on both palms. When he presented to us he had multiple dusky, erythematous to violaceous, tender papulonodules and plaques symmetrically …

Hemorrhage as a presenting feature in pilocytic astrocytoma is an extremely rare phenomenon. When seen in children, most of such tumors exist in the cerebellum. Rarely, a supratentorial pilocytic astrocytoma can present with bleeding. We present similar two cases and discuss the pathophysiology of such hemorrhage and histopathological changes in thinned hyalinised vessels of this tumor. The presence of calcifications in the peri-hemorrhagic areas and the presence of mass effect disproportionate to the size of the bleeding are harbingers of the presence of a benign neoplasm that may have bled.

Background: Extraskeletal mesenchymal chondrosarcoma (ESMCS) is an unusual pathologic variant of chondrosarcoma. There are no specific clinicoradiographic parameters to distinguish it from other intracranial pathologies. The diagnosis can be established only on the basis of histopathology, which may pose significant challenges in certain unusual locations. Purpose: In this case, we discuss the pitfalls in diagnosis, management, and major characteristics of ESMCS with a review of current literature. Methods and Results: A 47-year-old female patient presented with a jugular foramen tumor which was misdiagnosed as glomus jugulare tumor for which she received primary gamma knife radiosurgery at 2 instances. But unfortunately, the patient died because of the increase in size of the lesion associated with necrosis. Autopsy analysis revealed a highly cellular tumor, rich in sarcomatous cells, and well-differentiated cartilages consistent with ESMCS. Conclusion: A definite preoperative diagnosis of ESMCS is crucial though difficult especially when located at complex sites like jugular foramen and clinicoradiological mimicry. The most crucial step in the management of ESMCS is accurate diagnosis with critical evaluation of clinical, radiological, and histopathological parameters and realization of highly variable clinical course of the disease.

Introduction: Ishikawa and Jefferson are the two most commonly used systems used for the classification of cavernous sinus syndrome (CSS). However, relative utilities of these two classification systems have not been evaluated in detail in developing countries. In this study, we compared relative utilities of these two classification schemes in the evaluation of CSS. Objective: To compare the utility of Jefferson and Ishikawa classifications in the evaluation of CSS. Patients and Methods: A total of 73 consecutive patients of CSS were prospectively classified using either Ishikawa or Jefferson classification and relative utility of these two classification schemes in determining etiology of CSS was compared. Results: While only 46.6% of patients could be classified using Jefferson classification, 95.5% of patients could be classified using Ishikawa scheme. CSS was classified as anterior, middle, and posterior in 17.8%, 21.9%, and 8.2% of patients, respectively, as per the Jefferson classification. As per the Ishikawa classification, 37% of patients each showed anterior and posterior CSS, 16.4% showed middle CSS, whereas 4.1% had whole CSS. Middle CSS was significantly associated with the presence of fungal infections (P = 0.045) as per Jefferson classifications, and anterior CSS was significantly associated with a vascular etiology (P = 0.005) as per Ishikawa classification. Overall, inflammatory causes were the most common cause for anterior CSS, while tumors accounted for maximum cases of posterior CSS. Conclusion: Although more number of patients could be classified using Ishikawa classification, there was no advantage of Ishikawa classification over Jefferson with regard to determination of etiology of CSS.

Primary central nervous system lymphomas (PCNSL) usually present as single or multiple lesions with mass effect involving the cerebral hemispheres or basal ganglia. An extremely rare pattern of involvement termed "Lymphomatosis cerebri" (LC) presents as diffuse, non-enhancing infiltrative lesions without mass effect. We describe the clinical, radiological, and autopsy findings of one such rare example with a patient presenting with a short history of fever, memory loss, and progressive cognitive decline. Because of subtle yet rapidly progressive clinical symptoms and overlapping neuroimaging features, the diagnosis of LC is challenging with wide ranging differential diagnoses. The dilemma in diagnosing such lesions can lead to delay in diagnosis and institution of appropriate management. Thus, knowledge about its imaging and morphological features is very critical for correct categorization and to avoid potential misdiagnosis of this often-missed disease.

Spinal metastases from esthesioneuroblastoma (ENB) might have variable presentations. Discrete lesions, even when multiple, warrant radical excision, followed by radiotherapy. The authors present a case of anterior skull base ENB, metastasizing to spine at muliple levels. Clinical and radiological pictures are described with intraoperative findings.

Sarcina is a Gram-positive anaerobic organism, having exclusively fermentative metabolism and has been associated with gastric outlet obstruction. We demonstrate the present case to highlight the presence of Sarcina with a coexisting gastric adenocarcinoma diagnosed on fine needle aspiration cytology.

We report a 66-year-old man with a spinal, extradural solitary fibrous tumor showing unique retiform and papillary architecture. The patient presented in May 2008 with worsening right-sided lower back pain and urinary frequency. Magnetic resonance imaging of the spine documented a heterogeneously enhancing dumbbell-shaped extradural lesion causing cord compression at T11/12 level. The tumor extended to the paravertebral soft tissue and invaded the right adjacent vertebral pedicles and laminae. An angiogram showed prominent vascular supply mainly from the right T11 radicular artery. The patient underwent surgery to relieve cord compression in May 2008 and a second operation following embolization with coils in October 2009. No recurrence was observed at the last neuroimaging follow-up in June 2012. The tumor was composed of vimentin, CD34, Bcl-2, and CD99-positive rounded or slightly elongated cells with scant cytoplasm and oval to spindle nuclei. Several pseudovascular spaces reminiscent of the rete testis were present, and several of them contained papillary projections. Cytologic atypia was minimal, and mitotic activity was low. Focal infiltration of the paraspinal adipose tissue was seen at microscopic level. To our knowledge, retiform and papillary features have never been reported in a solitary fibrous tumor.