Extracerebral choroid plexus papilloma in a newborn: a rare presentation.

Choroid plexus papillomas (CPPs) are extremely rare lesions that originate in the central nervous system. Still rarely, these can occur in heterotopic locations. We report a case of aneonate who presented at fourth week of life with a small swelling in the left side of the oropharynx. There were no other symptoms. MRI scan revealed a cystic lesion with the possibility of lymphatic malformation or teratoma. Swelling which was gradually increasing in size was electively excised. Histopathological examination revealed it to be an extracerebral CPP. Postoperative period was uneventful, and baby had no further complications. CPP is a rare lesion and, almost always, cannot be anticipated before surgery. Imaging only helps in surgical planning. Complete excision is curative.

Evaluation of Accuracy of Intraoperative Frozen Section and Imprint Cytology in Gynecological Neoplasms-A Descriptive Cross-Sectional Study of 50 Cases in Tertiary Care Center.

Aims and Objectives Gynecological neoplasms are among the most common cancers in female population of India and worldwide. Various new advances have been made to diagnose gynecological pathologies which include imprint cytology and frozen sections in addition to the histopathological techniques. The aim of the study was to assess the diagnostic accuracy of intraoperative diagnostic procedure with gold standard histopathology. Method The study included 50 patients who had suspected gynecological neoplasm in a time period of 12 months. Their intraoperative diagnosis was made on frozen section and imprint cytology to rule out benign or malignant lesions and compared with gold standard histopathology. Results Our study concludes that maximum cases of female genital tract neoplasms belonged to the age group of 19 to 76 years, with 60% cases in postmenopausal age group. Overall diagnosis of 62, 52, and 76% malignancies were made on imprint cytology, frozen section, and histopathological examination, respectively. Conclusion The study concludes that diagnostic accuracy by intraoperative imprint cytology is higher (80%) than frozen section (76%). True positive cases were maximally reported by histopathology. True negative and false positive cases were equally reported by both frozen and imprint cytology. False negative cases were reported by frozen section more than by imprint cytology. The kappa statistical value was lesser in frozen versus histopathology and more in imprint versus histopathology. The sensitivity, specificity, positive predictive value, and negative predictive value for imprint cytology were 77.5, 90.0, 96.9, and 50%, respectively, whereas for frozen section, it was 72.5, 90.0, 96.7, and 45.0%, respectively.

Dermoscopy in the Diagnosis of Palmoplantar Eczema and Palmoplantar Psoriasis: A Cross-Sectional, Comparative Study from a Tertiary Care Centre in North India.

Introduction: Palmoplantar hyperkeratotic lesions pose a diagnostic difficulty when differentiating clinically between palmoplantar psoriasis (PPP) and palmoplantar eczema (PPE). Dermoscopy can provide valuable clues in reaching the final diagnosis. Objectives: To identify and compare dermoscopic findings seen in PPP and PPE. Methods: This was a cross-sectional, comparative study carried out prospectively between March 2019 and June 2020. All adult, consenting patients visiting the dermatology outpatient department who were clinically diagnosed and histopathologically confirmed as PPP and PPE were recruited into two groups. The dermoscopic examination was done, and images were taken from the representative area. The findings were analysed by two dermatologists who were unaware of the diagnosis. The sensitivity, specificity, positive predictive value and negative predictive value of these findings were assessed. Result: A total of 81 patients were recruited (39 patients of PPP and 42 patients of PPE). Significant findings seen in psoriatic plaques were the presence of regular vessels (84.6%, P < 0.001), with diffuse scales (87.2%) over a dull red/pink background (69.2%, P < 0.001). Dermoscopy of PPE displayed a significant presence of yellowish scales (76.2%, P < 0.001), with focal (61.9%) or absent vessels (35.7%), brown/orange-brown dots and/or globules (66.7%), yellow/yellow-orange crusts (57.1%) over a yellow/yellow-brown background (88.1%). Conclusion: Distribution of vessels, distribution of scales and the colour of scales, presence of brown/orange-brown dots and/or globules, yellow/yellow-orange crust and background colour (dull red vs. yellow/yellow-brown) of the plaques can be useful clues in the diagnosis of PPP and PPE.

Histopathological Characterization of Drug Rash with Eosinophilia and Systemic Symptoms (DRESS) and Comparison with Maculopapular Drug Rash (MPDR).

INTRODUCTION: Drug rash with eosinophilia and systemic symptoms (DRESS) is a severe cutaneous adverse drug reaction (cADR) associated with significant systemic involvement and greater mortality. Variable patterns of inflammation are reported in the histopathology of DRESS. However, the role of histopathology in predicting systemic involvement and thus final outcome remains elusive. In the present study, we aim to review clinical and histopathological characteristics of patients with DRESS and compare their histopathology with that of maculopapular drug rash. MATERIALS AND METHODS: A retrospective analysis of cases of cADRs diagnosed from July 2014 to July 2020 at a single tertiary care institute was performed. A RegiSCAR score of ≥4 was used to recruit patients as DRESS. Patients with a probable/definite diagnosis of cADR on the basis of Naranjo criteria and presenting with exanthem attaining a RegiSCAR score of ≤3 were categorized as MPDR. Correlation of histopathology characteristics with the investigative profile of patients with DRESS was done. MPDR and DRESS were also compared for histopathological characteristics using Chi-square test. Further histopathology of patients with drug rash (both DRESS and MPDR) having systemic involvement was compared with those without systemic involvement to identify specific predictors. RESULTS: Eighteen patients of DRESS and 20 of MPDR fulfilled the inclusion criteria. Most common drugs implicated were anticonvulsants (27.8%). Characteristic findings seen on histopathology in patients with DRESS were epidermal spongiosis (94.5%), epidermal dyskeratosis (33.3%), lymphocytic exocytosis (88.9%), interface vacuolization (77.8%), papillary dermal edema (100%). and perivascular lymphocytic infiltrate (100%). Findings in favor of DRESS compared to MPDR were lymphocytic exocytosis (P < 0.001), interface vacuolization (P = 0.002), severe spongiosis (P = 0.046), severe papillary dermal edema (P = 0.018), and higher density of dermal infiltrate (P = 0.005). Lymphocyte exocytosis and distribution and density of dermal inflammatory infiltrate correlated significantly with deranged kidney function. CONCLUSION: Histopathology revealing prominent basal vacuolization, spongiosis, and dense dermal infiltrate suggests DRESS. Lymphocyte exocytosis and distribution and density of dermal inflammatory infiltrate predict renal involvement.

Polymyositis Presenting with Respiratory Symptoms.

Polymyositis is a group of muscle disease characterised by progressive muscle inflammation and predominantly muscle weakness. It usually presents subacutely with proximal weakness and mild diffuse muscular pain. Some patients have atypical presentation like early respiratory difficulty, Motor neuron disease (MND), or isolated dysphagia which leads to delay in diagnosis and treatment. We present one such case.

Ectopic Cervical Deciduosis: A Rare Cause of Antepartum Hemorrhage in Mid Trimester.

Decidual change is a key process required by the uterus to make itself ready for implantation. Presence of ectopic decidual tissue outside the uterine cavity is known as deciduosis. The clinical presentation can vary from being totally asymptomatic and subtle to presenting in the form of life-threatening emergencies like hemoperitoneum, recurrent pneumothorax, or even bowel perforation. Here, we present a case of ectopic deciduosis of cervix presenting in the form of severe life-threatening antepartum hemorrhage in second trimester of pregnancy.

Idiopathic perniosis presenting as acral purpuric lesions: Clustering of cases before COVID-19 pandemic and their comparison with chilblain like lesions reported in the literature.

Perniosis/chilblains are the acral inflammatory skin lesions developing in susceptible individuals as an abnormal reaction to cold. In the absence of a discernible cause, it is labeled as idiopathic perniosis (IP). With the ongoing COVID-19 pandemic, there was an upsurge of reports of chilblain like lesions (CLL) especially in young patients possibly implicated to the SARS-CoV-2 virus. Twelve clinically suspected and histopathologically confirmed cases of IP seen from November 2019 through February 2020 were retrospectively recruited. Clinical, dermoscopic, and histopathological characteristics of these were reviewed and compared with CLL reported in the literature. Mean age of patients was 26.58 ± 15.18 years with an equal male to female ratio. Characteristic histopathology findings were spongiosis (100%), dermal edema (100%), perivascular lymphocytic infiltrate (100%) with peri-eccrine accentuation (66.7%), keratinocyte necrosis (50%), focal basal vacuolar damage (58.3%), and lymphocytic vasculitis (58.3%). Significant dermoscopy findings were variable background color ranging from dull red and violaceous to copper red and brown orange, coiled vessels (44.4%) and orange-red structureless areas (63.9%). Lesions over palms and soles preferentially had white dots/clods and lines (38.9%). There appears no exclusive histopathological as well as dermoscopy features of CLL and IP, yet certain clues can be appreciated. Keratinocyte necrosis and severe dermal edema favors IP, whereas fibrin thrombi with involvement of both superficial and deep dermal vessels favor CLL. Dermoscopically presence of irregular, linear or branching vessels, red/purple dots and clods and gray brown reticule supports CLL while white dots/clods and lines supports IP.

Human papillomavirus infection and p53 mutation in esophageal squamous cell carcinoma and its impact on treatment outcome.

INTRODUCTION: Human papillomavirus (HPV) is emerging as a risk factor for esophageal squamous carcinoma. The prognostic value of the HPV status has been investigated. However, the results are much controversial. AIM: This study aims to document the association of HPV infection and mutation of p53 gene in esophageal squamous cell carcinoma (ESCC) and its impact on treatment outcome. SUBJECTS AND METHODS: The study was conducted over a period of 12 months. A total of 30 cases of ESCC who were primarily to be treated with radiotherapy/chemoradiotherapy were included in the study. All the tissue samples for biopsy were subjected to immunohistochemistry to study p53 and p16 expression, which is a surrogate marker for HPV. The patients were treated by radiotherapy alone or concurrent chemoradiotherapy depending on performance status and stage of disease. The impact of p16 and p53 on overall survival (OS) and disease-free survival (DFS) was determined. RESULTS: The median OS of HPV-positive patients was 22 months (95% confidence interval [CI] 12-31) as compared to 13 months (95% CI 7-18) for HPV-negative patients (P = 0.298). The median DFS for HPV-positive patients was 16 months (95% CI 7-24) as compared to 5 months (95% CI 4-6) for HPV-negative patients (P = 0.048). The median OS of p53-positive patients was 13 months (95% CI 6.7-19) as compared to 22 months (95% CI 12.7-31.2) for p53-negative patients (P = 0.080). The median DFS for p53-positive patients was 5 months (95% CI 3.7-6.2) as compared to 22 months (95% CI 15.7-29.4) for p53-negative patients (P = 0.014). CONCLUSION: Clinical findings of our result can be used to sum up that both HPV infection and p53 mutation status are reliable biomarkers and can help clinicians to predict treatment outcome and prognosticate patients better.

Histopathology of morphea: Sensitivity of various named signs, a retrospective study.

BACKGROUND: Morphea or localized scleroderma is characterized histopathologically by sclerosis, fibrosis, and atrophy of the skin and subcutaneous tissue. Various authors have named the characteristic findings seen in histopathology of morphea and have labeled them as specific signs, including line sign, cookie-cutter sign, and square biopsy sign. Besides, other findings mentioned include high eccrine glands and the presence of interstitial mucin. The present study was undertaken to assess the sensitivity of these tests in the histopathological diagnosis of morphea. METHODS: All cases clinically diagnosed and histopathologically reported as morphea in the last 3 years (September 2016 to August 2019) were included. The slides were reviewed by two independent investigators for the presence of line sign, cookie-cutter sign, square biopsy sign, high eccrine glands, and mucin. The sensitivity of these signs in accurately diagnosing morphea was assessed. Besides, specificity, positive predictive value, and negative predictive value of these signs were assessed using 40 random histopathology slides as controls. RESULTS: The highest sensitivity was of high eccrine glands (82.5%) followed by the presence of mucin in the dermis (77.5%). Cookie-cutter sign and square biopsy signs were seen in 70% and 62.5% patients, respectively. Line sign was least sensitive of all, seen in 45% of biopsy specimens, but was most specific (82.5%). CONCLUSION: A fair number of biopsies of morphea displays the presence of high eccrine glands, mucin, cookie-cutter sign, square biopsy sign, and line sign. These signs thus can be of immense help to the dermatopathology trainees.

Dermoscopic findings of a congenital smooth muscle hamartoma in an infant.

Cutaneous smooth muscle hamartoma is an uncommon, benign tumor developing in the dermis as a result of disorganized hyperproliferation of arrector pili muscle fibers. We present a 1-month-old infant with a congenital smooth muscle hamartoma together with the dermoscopic findings of the case. Dermoscopy can be a helpful non-invasive tool in diagnosing congenital smooth muscle hamartoma due to its distinct findings that help to differentiate it from close mimickers like solitary mastocytoma.

Congenital Erythropoietic Porphyria: A Rare Case of Photosensitivity with Hemolytic Anaemia and Mental Retardation.

Congenital erythropoietic porphyria, also called Gunther's disease, is a very rare genetic autosomal recessive diseaseaffecting less than 1 per 1,000,000 children. Pathogenesis involves genetic mutation encoding uroporphyrinogen-III cosynthase which leads to accumulation of porphyrin in many tissues, leading to extreme skin photosensitivity, red cell lysis, splenomegaly and reduced life expectancy. Herein, we report a 12-year mentally challenged girl with multiple blisters and scars on sun exposed sites since birth. She had hepatomegaly, erythrodontia, severe anaemia with haemolytic blood picture and mildly elevated liver enzymes. Skin biopsy showed deposition of amorphous eosinophilic porphyrins in the dermis, thus confirming a diagnosis of congenital erythropoietic porphyria.

Hereditary segmental neurofibromatosis: a report of three cases in a family.

Hereditary segmental neurofibromatosis (SNF) is an extremely rare form of SNF with nine families reported till date. SNF is considered to be a result of mosaicism and hereditary transmission is possibly explained by increased susceptibility of mutations at neurofibromatosis gene loci. We report here three members of an Indian family with SNF. Our index case, a 20-year-old woman had multiple soft to firm papules and nodules over right shoulder associated with severe intermittent pain. Her father had three similar nodules over left upper arm and elder sister had two such nodules over right upper arm. The father and elder sister were incidentally diagnosed and they had never sought medical advice considering their lesions to be a familial trait. In the absence of symptoms, many familial cases of SNF are missed thus making any systematic analysis to ascertain its genetic basis difficult.

Von Meyenburg complex clinically presenting as metastatic liver nodule: A rare finding in an elderly male.

Von Meyenburg complex is a benign biliary hamartoma observed in approximately 3% of the autopsies conducted. These hamartomas are small, multiple and can be seen anywhere in the liver. There is usually no abnormality in liver function tests, and the patient is mostly asymptomatic. They serve as a diagnostic dilemma, particularly during metastatic workup in suspected cancer patients. Imaging studies are usually noncontributory, and biopsy is necessary for a definite diagnosis. We present here a middle-aged North Indian male who underwent liver biopsy to rule out metastasis.