Effect of adjunct Vitamin D treatment in vitamin D deficient pulmonary tuberculosis patients: A randomized, double blind, active controlled clinical trial.

BACKGROUND: Since, Vitamin D [1α,25(OH)2D)] enhances antimicrobial activity of Innate immunity and modulate Adaptive immune responses, simultaneously, so it play a potential role for balanced immune activity against Mycobacterium tuberculosis and restricting tissue injuries within the TB patients.(Chun et al., 2011) 9 We aimed to determine the role of adjunct Vitamin D treatment on the outcome of pulmonary tuberculosis patients and evaluated the effect of Vitamin D administration on Differential Leucocyte Count, Erythrocyte Sedimentation Rate, serum Adenosine deaminase, serum C- reactive protein, Oxygen saturation (SpO2) and Body Weight in Vitamin D deficient pulmonary tuberculosis patients. METHODS: We conducted a prospective, interventional, randomized, double blind, parallel group, active controlled clinical trial. Newly diagnosed Vitamin D deficient pulmonary tuberculosis patients were randomly assigned to intervention group (received standard anti-tubercular treatment with adjunct Vitamin D3) and control group (received standard anti-tubercular treatment without adjunct Vitamin D3). Total four doses [each dose of 2.5 mg (100000 IU)] of Vitamin D3 were given, orally. First dose was given within 7 days of starting anti-tubercular treatment and second, third, fourth dose were given at 2, 4 and 6 weeks respectively. At the time of enrollment, we measured all baseline characteristics. During follow-up, we measured the study variables and monitored adverse events at 2, 4, 6, 8 and 12 weeks. Our safety parameter was serum corrected calcium level to assess the risk of hypercalcemia. RESULTS: Total 130 pulmonary TB patients, 65 patients in each group, were analyzed. Our study results showed that decrease in Neutrophil count was statistically significant with small effect sizes at every time point of measurement and increase in Lymphocyte count was statistically significant with small and moderate effect sizes at 4, 6 and 8 week for intervention group than for control group. Decrease in erythrocyte sedimentation rate was statistically significant with small effect sizes at 6 and 8 week, decrease in serum adenosine deaminase and serum C- reactive protein was statistically significant with moderate effect sizes at 4, 6 and 8 week for intervention group than for control group. Increase in Oxygen saturation was statistically significant at 4 week with small effect size and increase in body weight was statistically significant with small effect sizes for intervention group than for control group. No case of hypercalcemia was reported. CONCLUSION: Our findings suggest a potential role of adjunctive Vitamin D3 to accelerate resolution of inflammatory responses and improvement in clinical outcomes of pulmonary TB patients. TRIAL REGISTRATION: This trial is registered with Clinical Trials Registry - INDIA (http://ctri.nic.in) with CTRI Number - CTRI/2021/11/037914. PLACE OF STUDY: Room Number 27, first floor out-patients department (OPD) and inpatient Wards, fourth floor, Department of Respiratory Medicine, Uttar Pradesh University of Medical Sciences, Saifai, Etawah (U.P.), INDIA.

Immune reconstitution inflammatory syndrome following disseminated TB with cerebral venous thrombosis in HIV-negative women during their postpartum period: a case report.

Immune reconstitution inflammatory syndrome (IRIS) is characterized by exuberant and dysregulated inflammatory responses to invading microorganisms. IRIS associated with tuberculosis (TB-IRIS) is widely known in HIV-positive patients after starting highly active antiretroviral therapy (HAART) therapy. However, IRIS has also been observed in solid organ transplant recipients, neutropenic patients, tumor necrosis factor antagonist recipients, and women during the postpartum period despite their HIV status. Case Presentation: We report an exceptional case of a 19-year-old HIV-negative lady who presented as IRIS following disseminated TB with cerebral venous thrombosis during her postpartum period. After 1 month of starting anti-TB therapy, we found a paradoxical worsening of her symptoms and further deterioration of radiological features showing extensive tubercular spondylodiscitis involving almost all the vertebral with extensive prevertebral and paravertebral soft tissue collections. Significant improvement was observed after 3 months of continuation of steroids along with an adequate dose of anti-TB therapy. Discussion: The possible mechanism that could explain this dysregulated and exuberant immune response in HIV-negative postpartum women would be rapidly changing immunologic repertoire, as the recovery of the immune system causes an abrupt shift of host immunity from an anti-inflammatory and immunosuppressive status toward a pathogenic proinflammatory state. Its diagnosis mostly depends on having a high degree of suspicion and ruling out alternative etiology. Conclusion: Therefore, clinicians should be aware of the paradoxical worsening of TB-related symptoms and/or radiological features at the primary site of infection or new location following an initial improvement in adequate anti-TB therapy despite HIV status.

Vaccines and therapeutics for immunocompromised patients with COVID-19.

The COVID-19 pandemic has disproportionately impacted immunocompromised patients. This diverse group is at increased risk for impaired vaccine responses, progression to severe disease, prolonged hospitalizations and deaths. At particular risk are people with deficiencies in lymphocyte number or function such as transplant recipients and those with hematologic malignancies. Such patients' immune responses to vaccination and infection are frequently impaired leaving them more vulnerable to prolonged high viral loads and severe complications of COVID-19. Those in turn, have implications for disease progression and persistence, development of immune escape variants and transmission of infection. Data to guide vaccination and treatment approaches in immunocompromised people are generally lacking and extrapolated from other populations. The large clinical trials leading to authorisation and approval of SARS-CoV-2 vaccines and therapeutics included very few immunocompromised participants. While experience is accumulating, studies focused on the special circumstances of immunocompromised patients are needed to inform prevention and treatment approaches.

Partial Sheehan's syndrome with abdominal tuberculosis presented with pancytopenia and fluctuating thyroid profile: a case report.

Sheehan's syndrome is a well-recognized cause of panhypopituitarism secondary to pituitary apoplexy, followed by postpartum hemorrhage. Depending upon the degree of ischemic injury, it can be either partial or complete. Case presentation: We report an interesting case of a 35-year-old woman admitted to our hospital with complaints of abdominal distension, which was later presumed to be due to disseminated tuberculosis (TB) after excluding the possible differentials. During the treatment course, she was going through repeated attacks of hypovolemic shock and hypoglycemia due to adrenocortical insufficiency. This, along with the history of prolonged amenorrhea 4 years back due to severe postpartum hemorrhage in her last pregnancy, has led us to our diagnosis of partial Sheehan's syndrome. After 1 month of starting steroid and anti-TB therapy, it was quite surprising when she presented with features of pancytopenia and antitubercular drug-induced hepatitis. Discussion: Sheehan's syndrome may have a varying degree of presentation depending upon the degree of damage to the pituitary gland, which includes amenorrhea, lactation failure, adrenocortical insufficiency, hyponatremia, hypoglycemia, as well as pancytopenia in some rare instances The hormone panel especially the thyroid profile should be monitored carefully. Such cases are often challenging to deal with because of their varying degrees of presentation and the delay in diagnosis due to a lack of clinical suspicion. Conclusion: Therefore, we believe that this rare presentation of pancytopenia in Sheehan's syndrome with fluctuating thyroid profile and abdominal TB in the background will let clinicians approach such a rare disease differently.

Floppy Eyelid Syndrome: an Overlooked Comorbidity Among Bariatric Patients.

PURPOSE: Floppy eyelid syndrome (FES) is a clinical entity characterized by palpebral hyperlaxity and chronic conjunctivitis. Patients' eyelids evert ("flip inside out"), leading to eye irritation, dryness, grittiness, and tearing. More severe cases can lead to significant ocular complications, such as keratoconus and impaired eyesight. Research has revealed an association between FES and obstructive sleep apnea syndrome (OSAS). OSAS is also one of the most common comorbidities among patients with obesity and an indication for bariatric surgery. This is one of the first studies to explore FES in a group of patients who have undergone bariatric surgery. MATERIALS AND METHODS: This was a retrospective study. A total of 88 patients completed a survey by mail or telephone. Additional data on demographics and baseline preoperative clinical information was extracted from the online medical records and the MBSAQIP database. RESULTS: Thirty-nine patients (44%) recalled having chronic ocular symptoms before their bariatric surgery, among whom six reported palpebral laxity and/or an established diagnosis of FES. The majority of them (67%) rated their symptoms postoperatively as "somewhat" or "significantly improved." The patients that reported improvement in their ocular symptoms also experienced an improvement in their OSAS severity. CONCLUSION: Bariatric surgery might affect the clinical course of FES and the severity of symptoms. Treating OSAS, the underlying mechanism of FES, is a possible mechanism of how bariatric surgery can help patients. It is also critical for bariatric surgeons to consider FES when patients with obesity, particularly those with OSAS, present with chronic eye symptoms.

A 15 year-old-girl with persistent leg pain diagnosed as a giant cell tumor of the tibial diaphysis: A case report and review of the literature.

INTRODUCTION: Giant cell tumor (GCT) is a benign bone tumor typically seen in epiphysis or metaphysis of mature long bones. Multiple large multinucleated giant cells dispersed among mononuclear spindle cells and monocytes constitute characteristic histological background of GCT of bone (GCTB). CASE PRESENTATION: A 15-year-old girl was admitted to our hospital with the complaint of pain and swelling in the left leg with difficulty in walking for 2 years. On X-ray of the left leg, osteolytic, expansile, eccentric lesion with sclerotic bone margin on the diaphysis of the tibia was seen suggesting oesteofibrous dysplasia. MRI demonstrated findings compatible with adamantinoma. The subsequent histology report was rather surprising, consistent with giant cell tumor of the bone. Extended intralesional curettage was done with the help of a high-speed burr followed by chemical cauterization and bone grafting. The patient was followed up for 2 years. The patient could walk normally without assistance or any signs of a recurrence. DISCUSSION: GCTB commonly affects people in their third and fourth decades of life and involves epiphysis of the long bone, but this is a case of diaphyseal GCT, at an age of 15 years. It is challenging to diagnose GCT, if present in an unusual location, unless confirmed by histopathological examinations. CONCLUSION: A multi-disciplinary approach is required to correctly reach the diagnosis of GCT when it happens to be in an uncommon location(s). Early diagnosis with appropriate treatment and long-term follow-up is mandatory for the successful outcome of the treatment.

Cefixime induced Steven Johnson syndrome: A case report from Bangladesh.

Introduction: Stevens Jonson syndrome, a type IV mediated hypersensitivity reaction is a rare mucocutaneous disorder accounting for <10% of TBSA. It affects skin, oral mucosa, eyes, esophagus, mouth, pharynx, larynx, skin and genitals. SJS is caused mainly due to drugs, infectious agents, immunization, and radiation therapy. Presentation of case: We present a case of a 40 years old male who developed SJS after being administered cefixime for a short period. Given the patient's past profile, he was admitted due to RTA and was under treatment with cefixime. Irrespective of any symptoms of SJS in the past, he started developing symptoms soon after being treated with cefixime giving us a clue about cefixime-induced SJS. Discussion: Steven-Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN) are opposite ends of a spectrum of diseases arising usually from an adverse reaction to medications. The most common drug reactions include penicillin in antibiotics, carbamazepine in antiepileptics and allopurinol in gout treatment in the Asian community. In our case, the patient was under Cefixime for 6 days after which cutaneous manifestations were seen. SJS is a fatal condition, with a global mortality rate stretching between 10% and 34%. The first step in its management is to identify the culprit drug and stop its use. Other is symptomatic, with special attention to airway and hemodynamic stability, wound care, and pain alleviation measures. Medical therapy include corticosteroids, cyclosporine, intravenous immunoglobulin (IVIG), and TNF- α inhibitors. Conclusion: Cephalosporin group, like cefixime, is a commonly prescribed drug in developing countries due to its efficacy and cost-effectiveness. Therefore, physicians must beforehand be mindful of the consequences of its use and advice patients to visit the hospital with even the slightest cutaneous manifestation.

Perforator-based propeller flap for coverage of lower leg: Single centre experience.

This study was conducted to assess outcomes of propeller flaps for reconstruction of small- to medium-sized defects in the distal third of the leg. Of 53 lower third leg defects covered using the propeller flap, 43 survived without complications. Only minor complications were seen and no flap was lost completely. The propeller flap is thus a safe option for medium-sized defects of the lower leg.

Polyalthia longifolia leaves methanolic extract targets entry and budding of viruses-an in vitro experimental study against paramyxoviruses.

ETHNOPHARMACOLOGICAL RELEVANCE: Synthetic antiviral drugs have several limitations including high cost. Thus research on antiviral property of medicinal plants is continuously gaining importance. Polyalthia longifolia possesses several medicinal properties and has been used in traditional ayurvedic medicine for treatment of dermatological ailments as kushta, visarpa/herpes virus infection and also to treat pyrexia of unknown origin as mentioned in Visarpa Chikitsa. AIM OF THE STUDY: Keeping in view the cytotoxic, anti-cancer activity and antiviral efficacy of Polyalthia longifolia against herpes, present study was undertaken to evaluate the in vitro antiviral activity of methanolic extract of Polyalthia longifolia leaves, if any, and to unravel the possible target(s)/mechanism of action. MATERIAL AND METHODS: Antiviral activity of Polyalthia longifolia methanolic extract was studied using Vero cell lines against paramyxoviruses, namely-peste des petits ruminants virus (PPRV) and Newcastle disease virus (NDV). Cytotoxicity of the test extract was evaluated employing MTT assay. Virucidal activity, and viral-attachment, virus entry and release assays were determined in Vero cells using standard experimental protocols. The viral RNA in the virus-infected cells was quantified by qRT-PCR. RESULTS: At non-cytotoxic concentration, methanolic extract of Polyalthia longifolia leaves was found to inhibit the replication of PPRV and NDV at viral entry and budding level, whereas other steps of viral life cycle such as attachment and RNA synthesis remained unaffected. CONCLUSIONS: Polyalthia longifolia leaves extract possesses promising antiviral activity against paramyxoviruses and acts by inhibiting the entry and budding of viruses; and this plant extract evidently possesses excellent and promising potential for development of effective herbal antiviral drug.

Glaucoma secondary to intraocular tumors: mechanisms and management.

PURPOSE OF REVIEW: Glaucoma secondary to intraocular tumors is important to consider in eyes with a known tumor and those with unilateral or refractory glaucoma. The purpose of this review is to discuss the mechanisms and management of intraocular tumors with related secondary glaucoma. RECENT FINDINGS: Several intraocular tumors can lead to glaucoma, including iris melanoma, iris metastasis, iris lymphoma, trabecular meshwork melanoma, choroidal melanoma, choroidal metastasis, retinoblastoma, and medulloepithelioma. The mechanisms for glaucoma include solid tumor invasion into the angle, tumor seeding into the angle, angle closure, and iris neovascularization. Management of the tumor can lead to resolution of glaucoma. Management of the secondary glaucoma may involve medical therapy, transscleral cyclophotocoagulation, laser trabeculoplasty, and potentially antivascular endothelial growth factor therapy. Minimally invasive glaucoma surgery (MIGS) can be considered for eyes with treated, regressed posterior segment malignancies if there is no iris or ciliary body involvement. Importantly, avoidance of MIGS, filtering, or shunting surgery in eyes with active malignancies is emphasized. SUMMARY: Intraocular tumors can produce secondary glaucoma. Treatment of the primary tumor can sometimes resolve the glaucoma. Topical, oral, or laser therapies can be considered. Avoidance of MIGS, filtering, or shunting surgery is advised until the malignancy is completely regressed.

Pathogenesis-related proteins and peptides as promising tools for engineering plants with multiple stress tolerance.

Pathogenesis-related (PR) proteins and antimicrobial peptides (AMPs) are a group of diverse molecules that are induced by phytopathogens as well as defense related signaling molecules. They are the key components of plant innate immune system especially systemic acquired resistance (SAR), and are widely used as diagnostic molecular markers of defense signaling pathways. Although, PR proteins and peptides have been isolated much before but their biological function remains largely enigmatic despite the availability of new scientific tools. The earlier studies have demonstrated that PR genes provide enhanced resistance against both biotic and abiotic stresses, which make them one of the most promising candidates for developing multiple stress tolerant crop varieties. In this regard, plant genetic engineering technology is widely accepted as one of the most fascinating approach to develop the disease resistant transgenic crops using different antimicrobial genes like PR genes. Overexpression of PR genes (chitinase, glucanase, thaumatin, defensin and thionin) individually or in combination have greatly uplifted the level of defense response in plants against a wide range of pathogens. However, the detailed knowledge of signaling pathways that regulates the expression of these versatile proteins is critical for improving crop plants to multiple stresses, which is the future theme of plant stress biology. Hence, this review provides an overall overview on the PR proteins like their classification, role in multiple stresses (biotic and abiotic) as well as in various plant defense signaling cascades. We also highlight the success and snags of transgenic plants expressing PR proteins and peptides.

Orbital Decompression in the Endoscopic Age: The Modified Inferomedial Orbital Strut.

OBJECTIVE: Postoperative diplopia occurs in up to 45% of patients following orbital decompression for exophthalmos associated with Graves' orbitopathy. We sought to describe outcomes of our balanced orbital decompression strategy that includes the preservation of a modified inferomedial orbital strut (mIOS). STUDY DESIGN: Case series with chart review. SETTING: Academic medical center. SUBJECTS AND METHODS: A total of 115 consecutive orbital decompressions were performed on 73 patients (42 bilateral) with Graves' orbitopathy. All patients underwent (1) a balanced decompression technique incorporating an endoscopic medial and external lateral decompression and (2) a mIOS technique with preservation of the anterior half of the inferomedial orbital strut. A periorbital periosteal (orbital) sling was utilized in patients (n = 54) without threatened vision loss, proptosis >28 mm, or periorbital disruption to prevent prolapse of the medial rectus muscle. RESULTS: Utilization of the mIOS technique with or without a sling did not adversely affect the reduction in proptosis (5.1 mm with sling vs 5.0 mm without sling; P = .85).The incidence of new-onset postoperative diplopia was 17% (n = 6). The sling was not associated with postoperative diplopia (odds ratio = 0.54, 95% confidence interval: 0.08-3.40, P = .51), while it was associated with resolution of preexisting diplopia (odds ratio = 6.67, 95% confidence interval: 1.06-42.06, P = .04). No intraoperative complications occurred, and no patients suffered a decrement in visual acuity. CONCLUSION: Balanced orbital decompression utilizing a mIOS in patients with Graves' orbitopathy provides a safe and effective reduction in proptosis with a low rate of new-onset diplopia as compared with historical values. Utilization of an orbital sling may be beneficial in reducing postoperative diplopia in select patients.

Melkersson-Rosenthal syndrome with isolated unilateral eyelid edema: an immunopathologic study.

Lymphedema is caused by defective drainage of the lymphatic system. In Melkersson-Rosenthal syndrome, involvement is predominantly of the lumens with blockage of lymphatic channels by histiocytic-epithelioid cell clusters accompanied by dermal granulomas and lymphocytes. It is a localized, painless, nonitching, and nonpitting form of lymphedema. Besides the eyelids, the disease can cause lip edema, facial palsy, and/or fissured tongue. It is rare and has received little attention in the ophthalmic literature, either in its complete triadic form, or more frequently, in its monosymptomatic forms. Pathogenesis is not well understood, and there is no effective therapy. The authors describe a case of Melkesson-Rosenthal syndrome in a 45-year-old Hispanic man with isolated unilateral upper eyelid edema. Histopathological and immunohistochemical evaluations of an eyelid biopsy specimen revealed intravascular and extravascular clusters of histiocytic-epithelioid cells that were CD68/163-positive. Variable numbers of mostly T-lymphocytes were found in the epidermis, dermis, and orbicularis muscle and by virtue of the associated granulomas established the diagnosis of Melkersson-Rosenthal syndrome. CD4 helper and CD8 suppressor T-lymphocytes were equally represented. CD20 B-lymphocytes were exceedingly sparse. Conspicuous CD1a-positive Langerhans' cells were present in the epidermis, sometimes formed subepithelial loose aggregates and were also incorporated in the granulomas. The differential diagnosis includes the far more common condition of acne rosacea. Management of Melkersson-Rosenthal syndrome, and of angioedema in general, is reviewed.

Cadaveric study of anatomical variations of the median nerve and persistent median artery at wrist.

BACKGROUND: Awareness of anatomical variations of the median nerve at wrist is important in repair of traumatic injuries and treatments of compression syndrome because in these situations precise dissection of the nerve is mandatory and such variations are not infrequent. MATERIALS AND METHODS: In this study, 52 hands of 52 fresh cadavers were dissected and median nerve anatomy along with the presence of persistent median artery (PMA) was noted. RESULTS: A total of 26 hands (50%) had the deviation from the standard text book anatomy of the median nerve. There was early division of the median nerve into the medial and lateral branches in 11.53% hands. There was early branching of the 2(nd) common digital nerve in 9.6% hands. The transligamentous motor branch to the thenar muscle was most prevalent (42.3% hands). The single motor branch to the thenar muscles was found in the majority of hands (84.6%). The PMA was present in 11.53% hands and it was associated with variations in the median nerve anatomy in all cases. CONCLUSIONS: This study shows a high percentage of deviation from standard anatomy as well as a high percentage of transligamentous thenar muscle motor branch. The presence of PMA was associated with variations in the median nerve anatomy in all cases. Therefore if PMA is present there are very high chances of associated median nerve anomalies.

Stereotactic microdebrider in deep lateral orbital decompression for patients with thyroid eye disease.

PURPOSE: Stereotactic navigation systems have been used in neurosurgery and otolaryngology with great success. The current investigation illustrates the novel use of a microdebrider with built-in stereotactic guidance in a series of thyroid orbitopathy patients who underwent deep lateral orbital wall decompression surgery. METHODS: A noncomparative, interventional, retrospective case series of patients who underwent deep lateral deep orbital wall decompression from 2006 to 2013 was conducted in accordance with Institutional Review Board policy and the Declaration of Helsinki. Patient demographics, indications for surgery, pre-, intra-, and postoperative findings along with complications were recorded. RESULTS: One hundred eight deep lateral orbital decompression surgeries were performed in 69 patients using the Straightshot M4 Microdebrider with built-in stereotactic guidance (Medtronics). Seventy-eight cases were in women and 30 cases were in men. The average age was 50.4 years (SD = 11.9 years). Indications for surgery included proptosis, exposure keratopathy, or compressive optic neuropathy. No patient experienced intraoperative complications. Specifically, cerebrospinal fluid leak, visual loss, infection, or unanticipated inflammation were not encountered. The average postoperative follow-up time was 5.35 months. Mean reduction in proptosis was 3.72 mm (SD = 2.1). Visual acuity improved in 32.4% (35/108) of cases. CONCLUSIONS: This surgical instrument combines a single handpiece locator, microdebrider, irrigator, retractor, and suction device into one. It enhances anatomical localization during orbital decompression and, with an integrated tissue guard, may decrease the risk of injury to orbital soft tissues. Stereotactic navigation enhances the surgeon's ability to determine the maximal limits of decompression in real time by confirming depth of bone removal and may potentially increase surgeons' confidence in orbital decompression surgery.

Extruded, partially disintegrated, poly-HEMA orbital implant (AlphaSphere).

A 54-year-old diabetic man underwent enucleation for endophthalmitis. Secondary implantation of a 2-hydroxyethyl methacrylate (HEMA) sphere (AlphaSphere, Addition Technology) was performed 2 weeks later. Six weeks after insertion, noninfectious disintegration of sutured tissue planes represented by Tenon's capsule, rectus muscle, and conjunctiva occurred, requiring removal of the fragmenting implant before uncontrolled extrusion occurred. Histopathologic analysis revealed an absence of infectious pathogens and no tissue necrosis, but rather breakup of the implant material that elicited a granulomatous response with sparse T-lymphocytes and almost no polymorphonuclear leukocytes. This distinctively designed poly-HEMA orbital implant incited a dramatic and irreversible host tissue response. Investigation of other cases will be necessary to determine the frequency of such a complication and should include rigorous histopathologic techniques.

Vascular anomalies of the head and neck: a review of genetics.

PURPOSE: Vascular anomalies comprise malformations, hemangiomas, and rare tumors. The commonality among these lesions is their origin in vascular endothelia. Most occur sporadically, but occasional inheritance is observed and thus allows genetic research and insight into etiology. This review highlights those vascular anomalies in which genetic inheritance has been demonstrated. METHODS: A comprehensive literature search was performed on PubMed. Fifty-five full-length articles were reviewed. RESULTS: Five categories of vascular anomalies with patterned inheritance were identified: arteriovenous malformation (AVM), capillary malformation (CM), lymphatic malformation (LM), venous malformation (VM), and infantile hemangioma (IH). Capillary and arteriovenous malformation subtypes are associated with a RASA-1 gene mutation and show autosomal dominant inheritance. VEGFR3 mutations have been associated with generalized forms of LM and lymphedema. Mutations in TIE2/TEK genes cause inherited forms of venous malformations also with autosomal dominant inheritance. Familial clustering and atopic disease are associated with infantile hemangioma, and gene expression varies with the developmental stage of these lesions. CONCLUSION: Most vascular anomalies occur sporadically, but several genes and genetic disorders have been associated with them. Specific forms of capillary malformation appear to be most convincingly associated with genomic errors. Further research promises new insights into the development of this diverse group of disorders.

Palmar well differentiated spindle cell liposarcoma: presentation of a rare tumor at a rare site.

The most common soft tissue sarcomas of hand are epitheloid sarcomas, synovial sarcomas and malignant fibrous histiocytomas which are high grade, aggressive tumors. Liposarcomas of the hand are extremely rare and to the best of our knowledge less than 20 cases have been reported so far in the literature. Well differentiated spindle cell liposarcoma is an extremely rare subtype of well differentiated liposarcoma/atypical lipomatous tumor which is different from the other subtypes clinicopathologically, genetically and prognostically. Palmar localization of this rare variant of liposarcoma at this site makes ours only the second case reported in the world literature so far. This recently described entity is low grade lesion and the hand surgeons need to adopt a less aggressive approach in the treatment of this subtype.