Subcorneal Pustular Dermatosis Occuring in Association with Pyoderma Gangrenosum and Rheumatoid Arthritis: A Triple Whammy!

Neutrophilic dermatoses are a wide group of disorders encompassing indolent to severely disabling conditions. A co-existence of two such conditions, pyoderma gangrenosum (PG) and subcorneal pustular dermatosis, necessitates a thorough investigation for IgA dysglobulinemia. We report a middle-aged woman who developed PG following 18 years of (undiagnosed) subcorneal pustular dermatosis, along with rheumatoid arthritis, a known association of PG.

A real-world study of low-dose thalidomide in severe erythema nodosum leprosum highlighting its mechanistic rationale in a resource-constrained target population.

BACKGROUND: Corticosteroids remain the main therapy in erythema nodosum leprosum (ENL), and long-term usage in chronic or recurrent ENL is a cause of significant morbidity and mortality. Thalidomide exerts dramatic effect in controlling ENL and helps reduce the dose of steroids, but the cost is a hindrance to its usage. METHODS: Patients of ENL (steroid naïve and steroid-dependent) were recruited over a 1-year period. An escalating dose of low-dose thalidomide with a reducing dose of prednisolone was titrated depending on the control of disease activity. The primary aim was to reduce the dose of steroids to the lowest effective dose, and the secondary aim was to stop. RESULTS: Sixteen patients of ENL were studied (mean duration of ENL 22.1 months, 15 severe ENL), and a majority (11/16, 68%) were on steroids with a mean duration of 11.27 months. All patients had steroid-related side effects (cushingoid habitus 81.8%, weight gain 54.5%, diabetes mellitus 9%, hyperlipidemia 18.18%, cataract 18.1%, osteoporosis 36.3%, striae 36.3%, acneiform eruptions 18.1%, and myopathy 9%). Steroids could be tapered in a majority of patients (n = 9) within 3 months (mean 2.44 months) with a low dose of thalidomide (25-150 mg/day, mean 78.3 mg) achieving a significant reduction in prednisolone dose (33.16 mg at baseline; 4.28 mg at 3 months, P < 0.05). Steroids could be stopped in 92% of patients by 3.03 months, and both drugs could be stopped in 80% of cases by 5.83 months. CONCLUSION: The rapid and effective control of ENL with low-dose thalidomide in our series is comparable to the historical efficacy of high-dose thalidomide regimens, making it an affordable therapy in resource-constrained settings and an excellent steroid-sparing agent. The rapid onset of disease control is likely attributable to its action via neutrophils.

A prospective real-world study evaluating serum levels of arginine and cysteine in women with chronic telogen effluvium: do we really need blanket prescription of amino acid supplementation?

Amino acids form a major component of hair fibres and are prescribed routinely in the form of nutritional supplements in patients with chronic telogen effluvium (CTE). Such a practice is based on assumption of a nutritional deficiency state in such patients. In this prospective study, we evaluated the serum levels of cysteine and arginine in 30 women with CTE and in healthy controls. We found no significant difference between the two groups in terms of serum arginine levels. Cysteine levels were higher in patients with CTE (P < 0.001). No correlation was found between levels of serum amino acids (cysteine and arginine) and either diet type (vegetarian or not) or body mass index, and no significant correlation between levels of the two amino acids and severity of disease. Our work suggests that arginine and cysteine deficiency is not present in women with CTE. Supplementation is unlikely to be of any benefit in nutrient-replete populations and only adds to the cost of therapy.

Intestinal mucormycosis in a patient with COVID-19: A case report.

The novel coronavirus disease (COVID-19) has spread to all the continents posing a serious threat to global health. It can present with myriad symptoms and complications including susceptibility to fungal co-infections. We hereby describe a case of mucormycosis in a 17 year old COVID-19 positive female with no known comorbidities presenting with abdominal pain and distention and ultimately developing intestinal perforation. Early identication of the symptoms is essential to make a correct and early diagnosis to prevent complications.

Exostosis of Rib: Case Report of an Extremely Rare Giant Osteochondroma at an Unusual Site, Radiopathological Correlation with Brief Review of Literature.

Osteochondromas are common benign tumors developing as an abnormal bony growth in the metaphyseal region. Being more of a developmental anomaly rather than a true neoplasm, they are usually found around the growth plates of long bones such as the knee, hip, and shoulder. These are typically managed conservatively if they are asymptomatic; however, they require excision in symptomatic patients. A 38-year-old woman presented with a huge swelling causing disfigurement measuring 16 × 16 cm on the left side of chest wall. Radiographs and computed tomography scan showed a bony outgrowth at costochondral junction of second rib which was in continuity with the periosteum. Excision via mediastinal sternotomy and left thoracotomy was done. Histopathological features corroborated with the radiological diagnosis of osteochondroma. Osteochondroma should be considered in the differential diagnosis of chest wall tumors. Rib is an extremely rare site of presentation. The cartilaginous cap becomes fully ossified and is lost in longstanding lesions. Huge tumors at such a location can cause irritation of adjacent viscera which can lead to pleural effusion or hemothorax; therefore, a cautious and logical approach to diagnosis is warranted for appropriate therapeutic management.

Coronary arteritis as a cause of sudden cardiac death in a young girl.

A case of probable coronary arteritis in a young girl who died suddenly and unexpectedly is presented. The histologic presentation of the disorder is discussed, especially the differential diagnosis of arteritis of the coronary arteries with an emphasis on tuberculosis (TB). TB myocarditis with or without concomitant lung involvement is rare, and tubercular coronary arteritis without underlying pulmonary Koch's disease is all the rarer. We herein describe a case where the cause of death was ascertained on post-mortem examination.

Liposomal amphotericin B-induced reversible ototoxicity in a patient with disseminated histoplasmosis.

Amphotericin B (AmB) is a polyene antifungal agent used widely for systemic fungal infections. Infusion-related side effects, nephrotoxicity, and dyselectrolytemia are well-known adverse effects with the use of this drug. Liposomal preparation of AmB has less adverse events. However, ototoxicity due to AmB is scarcely mentioned in the literature. We report a case of disseminated histoplasmosis who was treated with liposomal amphotericin B (LAmB) and developed hearing loss during the treatment, which recovered after discontinuing the drug. This is a rare adverse effect of LAmB and reported only once from India, to the best of our knowledge.

A clinicopathological study of peripheral schwannomas.

AIM AND OBJECTIVE: Schwannomas are benign neoplasms of neural origin with sporadic or syndromic occurence. They are commonly seen in cranial nerves. Peripheral schwannomas occur rarely and may have unique presentations. The aim of this study is to evaluate the clinico-pathological characteristics of peripheral schwannomas. METHODS: A retrospective cross sectional study of peripheral schwannomas excluding head neck region was conducted. The study group consisted of 18 cases which were recorded over a period of seven years. The corresponding data were collected from the archives of the Department of Pathology. RESULTS: Male to female ratio was 1:1. The average age of the cases was 47 years. The most common site was the upper limbs (55.55%) followed by lower limbs, chest and penis. The lesions mostly presented as painless swellings (62%). Histopathological examination revealed classic features of schwannoma. Secondary changes included cystic degeneration, foam cells, epitheloid cells, hyalinization, microcystic change and collection of plasma cells. All cases were confirmed by positive S100 staining. CONCLUSION: Peripheral schwannomas may be missed due to its rarity and atypical presentations. Both clinicians and pathologists should be aware of this common entity at unusual sites for the proper management of the patients. Surgery is usually the treatment of choice.

A Rare Case of Epithelioid Myofibroblastoma of Breast Mimicking Lobular Carcinoma on Trucut Biopsy: a Diagnostic Pitfall and Literature Review.

Epithelioid myofibroblastoma of the breast is a rare benign stromal tumor occurring in the postmenopausal females and elderly males. We report one such case in a 65-year-old female who underwent trucut biopsy from a well-defined right breast lump, which was misinterpreted as invasive lobular carcinoma on histology. Tumor showed mild to moderately pleomorphic tumor cells arranged in single file pattern, loosely dispersed and focally in sheets with inconspicuous nucleoli embedded in a fibrotic and hyalinized stroma. Immunohistochemistry for E-cadherin was negative further substantiating the morphological diagnosis. However, mastectomy specimen revealed it to be a case of epithelioid myofibroblastoma with the help of a large panel of immunohistochemical stains. In this report, we emphasize on the challenges of establishing this rare diagnosis mimicking lobular carcinoma, a diagnostic pitfall on trucut biopsy.

Severe type 2 leprosy reaction with COVID-19 with a favourable outcome despite continued use of corticosteroids and methotrexate and a hypothesis on the possible immunological consequences.

Type 2 leprosy reaction (T2LR), or Erythema Nodosum Leprosum (ENL), often poses a therapeutic challenge to clinicians and commonly requires long courses of steroids for control. While immunosuppressants are known to achieve control and lower steroid dependence in T2LR, the prospect of managing a severe T2LR in conjunction with COVID-19, with the concern of worsening COVID-19 with long-term immunosuppression has not previously been encountered. We report a case of severe T2LR treated with oral steroids and methotrexate, with COVID-19 infection acquired during hospital stay, and a favourable outcome achieved despite the continued use of immunosuppressants. We discuss the possible reasons for this both in terms of the drug pharmacodynamics and the immunological profile of T2LR.

Coronary arteritis as a cause of sudden cardiac death in a young girl

A case of probable coronary arteritis in a young girl who died suddenly and unexpectedly is presented. The histologic presentation of the disorder is discussed, especially the differential diagnosis of arteritis of the coronary arteries with an emphasis on tuberculosis (TB). TB myocarditis with or without concomitant lung involvement is rare, and tubercular coronary arteritis without underlying pulmonary Koch's disease is all the rarer. We herein describe a case where the cause of death was ascertained on post-mortem examination.

Long segment cervicothoracic intramedullary dermoid with concomitant conal lesion - A case report.

BACKGROUND: Spinal dermoid cysts are benign tumors that result from congenital or acquired ectodermal inclusions. Long segment intramedullary involvement of the spinal cord is exceedingly rare, and there are only a handful of case reports found in the literature. CASE DESCRIPTION: A 30-year-old female presented with a 3-month history of myelopathy characterized by progressive quadriparesis and urinary incontinence. Magnetic resonance imaging revealed multifocal heterogeneous intramedullary masses extending from C2 to T4 and at T12-L1 with similar intensity lesions seen within the central cord from T5 to T11 level. Following tumor decompression, she showed significant improvement in neurological function 1 month later. The histopathological examination confirmed the diagnosis of a multifocal intramedullary dermoid cyst. CONCLUSION: Partial surgical extirpation is a reasonable treatment for long segment intramedullary dermoid cysts, particularly when the tumor capsule is adherent to critical adjacent neural tissues.

A rare case of intramedullary schwannoma at conus medullaris: A case report with review of literature.

BACKGROUND: Intramedullary spinal schwannomas constitute only 0.3% of primary spine tumors. We could identify only 13 such cases involving the conus that were not associated with neurofibromatosis (NF). Here, we report a 70-year-old male without NF who was found to have a paraparesis due to a schwannoma of the thoracolumbar junction/conus (D11-L2). CASE DESCRIPTION: A 70-year-old male presented with an L1-level paraparesis with urinary incontinence. The magnetic resonance showed an intramedullary mass of 85 × 10 mm extending from D11 to L2; it demonstrated significant patchy enhancement. The patient underwent a D12 and L1 laminectomy with gross total excision of the mass that proved to be a schwannoma. Three months postoperatively, he was able to ambulate with support, and regained sphincter function. CONCLUSION: Intramedullary schwannomas involving the conus/thoracolumbar junction are rare, and can be successfully excised resulting in good outcomes.

Epithelial mesenchymal transition in urothelial carcinoma: twist in the tale.

CONTEXT: Epithelial to mesenchymal transition (EMT) is a process involving conversion of cells from an epithelial to mesenchymal phenotype. The role of candidate genes promoting EMT and favoring a promigratory phenotype has been demonstrated in epithelial cancer. Existing scientific research has not yielded a clinically relevant biomarker with predictive capacity beyond grade and stage in bladder cancer. AIM: The purpose of this study is to evaluate the immunohistochemical expression pattern of a panel of epithelial and mesenchymal markers in paraffin-embedded archival material of primary urothelial carcinoma as evidence of EMT. MATERIALS AND METHODS: Immunohistochemical expression of transcription factor twist, epithelial (E-cadherin, cytokeratin) and mesenchymal (vimentin, N-cadherin) markers was analyzed on archival paraffin-embedded tissue samples from 48 patients with diagnosis of primary urothelial carcinoma of bladder. STATISTICAL ANALYSIS: Karl Pearson's χ2 test was used to evaluate the association between the expression of immunohistochemical markers and various clinico-pathologic variables. Non-parametric Kendall's tau-b statistics was used to determine the correlation between categorical variables. RESULTS AND CONCLUSION: The study demonstrated statistically significant association of cytokeratin, E-cadherin, vimentin, and twist with stage and grade of bladder cancer. Since these markers form part of the spectrum of changes associated with EMT, the study establishes proof of concept of the existence of this process in vivo. A significant negative correlation was noted between the expression of twist and E-cadherin. Exploiting its role as a transcriptional repressor of E-cadherin, twist may prove to be a useful candidate for targeted therapy in urologic oncology.