Clinical features, pathogenesis, pathology, neuroimaging, clinical course and outcome of measles inclusion-body encephalitis: a systematic review of published case reports and case series.

Measles inclusion-body encephalitis (MIBE) is rare, with insights largely from case studies. We systematically analyzed subacute Sclerosing Panencephalitis (SSPE) cases in immunocompromised patients, identifying distinctive clinical and neuroimaging features. These findings could facilitate MIBE diagnosis without the need for brain biopsies. Our systematic review on MIBE and HIV-related SSPE adhered to PRISMA guidelines and was registered with PROSPERO. We searched multiple databases and followed a detailed inclusion process with independent reviews and quality assessment. Data on patient demographics, clinical features, and outcomes were compiled. A review of 39 studies on 49 MIBE patients and 8 reports on HIV-positive SSPE patients was conducted. Acute lymphoblastic leukemia, HIV, organ transplants, and malignancies were common precursors to MIBE. Perinatal HIV was prevalent among SSPE cases. Seizures were the primary symptom in MIBE, often drug-resistant and progressing to status epilepticus or epilepsia partialis continua, whereas periodic myoclonus was universal in SSPE. Neuroimaging showed distinct patterns for each group, and histopathology confirmed measles virus presence in 39% of MIBE cases. MIBE patients typically progressed to coma and death. In conclusion, MIBE and SSPE in HIV-infected patients present with distinct clinical pictures but identical brain pathological abnormalities.

The etiological spectrum of miliary brain lesions: A systematic review of published cases and case series.

OBJECTIVE: This systematic review aimed to evaluate the published cases with miliary brain lesions and their etiological factors, clinical manifestations, diagnostic procedures, and outcomes. METHODS: A comprehensive search of PubMed, Scopus, Embase, and Google Scholar was conducted using the specified search strategy. Eligibility criteria included cases with miliary lesions in the brain confirmed through neuroimaging and various diagnostic procedures. The PRISMA guidelines were followed, and the PROSPERO registration number for the protocol is CRD42023445849. RESULTS: Data from 130 records provided details of 140 patients. Tuberculosis was the primary cause in 93 cases (66.4%), malignancies in 36 cases (25.7%), and other causes accounted for the remaining 11% cases. Tuberculosis patients averaged 35.7 years old, while those with malignancies averaged 55.44 years. Tuberculosis symptoms primarily included fever, headache, and altered sensorium, whereas malignant cases often exhibited progressive encephalopathy, headache, and specific neurological deficits. Distinctive indicators for CNS tuberculosis were choroidal tubercles and paradoxical reactions. Additionally, 63 tuberculosis patients showed miliary lung shadows and 49 had abnormal CSF findings. For the malignancy group, 13 exhibited miliary lung lesions, and 8 had CSF abnormalities. Regarding outcomes, a significant mortality disparity was observed, with 58.3% in the malignancy group, compared to 10.8% in the tuberculosis group and 27.3% in other cases. CONCLUSION: Miliary brain lesions are a crucial imaging abnormality that necessitates prompt work up. In an immunocompromised state, diagnostic possibilities of miliary brain lesions are more varied and often pose a bigger challenge.

Association of pruritus with sleep in patients with psoriasis and chronic spontaneous urticaria: A cross-sectional study.

Background: Pruritus is a frequent complaint associated with various inflammatory dermatoses. Sleep is often disturbed because of pruritus but the impact of severity and diurnal pattern of pruritus has not been studied so far. Objectives: To estimate the prevalence of nocturnal itch (NI) and its association with itch severity, sleep disturbance and quality of life (QoL) compared with non-NI in chronic plaque psoriasis (CPP) and chronic spontaneous urticaria (CSU). Methods: We performed a cross-sectional study in patients aged ≥18 years with CPP or CSU for at least 6 weeks. A comprehensive in-house questionnaire designed for study formed the basis for categorizing patients into NI and non-NI. Validated instruments like visual analog scale, pruritus grading system, General Sleep Disturbance Scale, and Dermatology life quality index were used to assess itch severity, sleep, and QoL. Results: A total of 255 patients (CPP: 131; CSU: 124) were included in this study. Prevalence of NI was 43.5% (95% confidence interval: 34.9%-52.4%) in CPP and 29% (95% confidence interval: 21.2%-37.9%) in CSU. NI was strongly associated with higher pruritus grading system scores in CSU and CPP (regression coefficient = 1.5, P =0.004 and regression coefficient = 1.3, P =0.004, respectively), with impaired sleep (OR = 2.97, P = 0.025) in CPP and with itch-affected sleep in CSU. Itch severity was associated with impaired sleep; however, the association was modified by the presence of NI in CSU patients. Conclusion: Nocturnal itch is prevalent in chronic dermatoses and significant for sleep deficit and impaired QoL. Early screening and management of sleep disturbance among patients presenting with nocturnal itch should be routinely undertaken.

Mucocutaneous manifestations of COVID-19-associated mucormycosis: A retrospective cross-sectional study.

Background Cutaneous mucormycosis has shown a significant upsurge during the COVID-19 pandemic. Due to the rapid progression and high mortality of cutaneous mucormycosis in this context, it is important to identify it early. However, very few studies report detailed clinical descriptions of cutaneous mucormycosis in COVID-19 patients. Objectives To describe mucocutaneous lesions of COVID-19-associated mucormycosis based on clinical morphology and attempt to correlate them with radiological changes. Methods A retrospective cross-sectional study was conducted at a tertiary care centre from 1st April to 31st July 2021. Eligibility criteria included hospitalised adult patients of COVID-19-associated mucormycosis with mucocutaneous lesions. Results All subjects were recently recovering COVID-19 patients diagnosed with cutaneous mucormycosis. One of fifty-three (2%) patients had primary cutaneous mucormycosis, and all of the rest had secondary cutaneous mucormycosis. Secondary cutaneous mucormycosis lesions presented as cutaneous-abscess in 25/52 (48%), nodulo-pustular lesions in 1/52 (2%), necrotic eschar in 1/52 (2%) and ulcero-necrotic in 1/52 (2%). Mucosal lesions were of three broad sub-types: ulcero-necrotic in 1/52 (2%), pustular in 2/52 (4%) and plaques in 1/52 (2%). Twenty out of fifty-two patients (38%) presented with simultaneous mucosal and cutaneous lesions belonging to the above categories. Magnetic resonance imaging of the face showed variable features of cutaneous and subcutaneous tissue involvement, viz. peripherally enhancing collection in the abscess group, "dot in circle sign" and heterogeneous contrast enhancement in the nodulo-pustular group; and fat stranding with infiltration of subcutaneous tissue in cases with necrotic eschar and ulcero-necrotic lesions. Limitations The morphological variety of cutaneous mucormycosis patients in a single-centre study like ours might not be very precise. Thus, there is a need to conduct multi-centric prospective studies with larger sample sizes in the future to substantiate our morphological and radiological findings. Conclusions COVID-19-associated mucormycosis patients in our study presented with a few specific types of mucocutaneous manifestations, with distinct magnetic resonance imaging findings. If corroborated by larger studies, these observations would be helpful in the early diagnosis of this serious illness.

An overview of mycetoma and its diagnostic dilemma: Time to move on to advanced techniques.

The neglected tropical disease mycetoma can become extremely devastating, and can be caused both by fungi and bacteria; these are popularly known as eumycetoma and actinomycetoma respectively. The classical triad of the disease is subcutaneous swelling, multiple discharging sinuses and the presence of macroscopic granules. The present study aims to highlight the existing diagnostic modalities and the need to incorporate newer and more advanced laboratory techniques like pan fungal/pan bacterial 16S rRNA gene polymerase chain reaction (PCR) and sequencing, Matrix-assisted laser desorption/ionization-time of flight mass spectrometry (MALDI-TOF MS), rolling circle amplification (RCA), loop-mediated isothermal amplification (LAMP) and recombinase polymerase amplification (RPA). It is important for the medical team to be aware of the various diagnostic options (both existing and future), so that diagnosis of such a debilitating disease is never missed, both by clinicians and microbiologists/pathologists. The newer diagnostic methods discussed in this article will help in rapid, accurate diagnosis thus facilitating early treatment initiation, and decreasing the overall morbidity of the disease. In the Indian context, newer technologies need to be made available more widely. Making clinicians aware and promoting research and development in mycetoma diagnostics is the need of the hour.

Nerve biopsies in patients with peripheral neuropathy: A prospective evaluation.

Background: In approximately 25% of peripheral neuropathy cases, diagnosis remains obscure. In India, leprosy continues to remain one of the most frequent causes of peripheral neuropathy. We, in this prospective evaluation, performed nerve biopsies in patients with peripheral neuropathy for early confirmation of the diagnosis. Materials and Methods: A total of 55 consecutive cases of peripheral neuropathy were included in this study. All patients were subjected to clinical and electrophysiological evaluation. Sural nerve biopsies were performed in all the patients. Result: After a nerve biopsy in 29 cases, we were able to identify the underlying cause of peripheral neuropathy. In 26 cases, the diagnosis remained obscure. The most frequent histopathological diagnosis was leprosy, which was seen in 20 cases. Other diagnoses were chronic demyelinating neuropathy (four cases), vasculitis (two cases), and amyloidosis in one case. In two biopsies, the findings were consistent with hereditary neuropathies. The demonstration of lepra bacilli was the most distinctive feature. In addition, foamy macrophages (100%) and granuloma (100%) formation, epineurial (83.3%) and endoneurial infiltration (69%) along with epineurial (87.5%) and perineurial thickening (77.3%) were also noted more frequently in leprosy-associated neuropathy. Conclusion: The nerve biopsies revealed that leprosy was the most common etiology in patients with peripheral neuropathy. In approximately 47% of the cases, even nerve biopsies failed to establish a confirmed diagnosis.

Central nervous system, spinal root ganglion and brachial plexus involvement in leprosy: A prospective study.

Background: Leprosy is primarily a disease of peripheral nerves. Some isolated case reports and case series have communicated imaging changes in the central nervous system (CNS) and brachial plexus in patients with leprosy. Objectives: To study the neuroimaging abnormalities in patients with lepra bacilli-positive neuropathy in the context of CNS, spinal root ganglion, and brachial plexus. Design: Prospective observational study. Methods: We screened newly-diagnosed patients with multibacillary leprosy presenting with neuropathy. Patients with bacilli-positive sural nerve biopsies were included in the study and subjected to magnetic resonance imaging (MRI) of the brain and spinal cord. Results: A total of 54 patients with bacteriologically confirmed multibacillary leprosy were screened; Mycobacterium leprae was demonstrated in the sural nerve biopsies of 29 patients. Five patients (5/29; 17.24%) had MRI abnormalities in CNS, spinal root ganglion, and/or brachial plexus. Three patients had MRI changes suggestive of either myelitis or ganglionitis. One patient had T2/FLAIR hyperintensity in the middle cerebellar peduncle while 1 had T2/FLAIR hyperintensity in the brachial plexus. Conclusion: CNS, spinal root ganglion, and brachial plexus are involved in patients with leprous neuropathy. Immunological reaction against M leprae antigen might be a plausible pathogenetic mechanism for brachial plexus and CNS imaging abnormalities.

Reticular Skin Rash as an Adverse Effect of 5-Azacitidine.

Azacitidine is a hypomethylating agent used for the treatment of patients with myelodysplastic syndrome (MDS). It has been approved by the Food and Drug Administration (FDA) and the European Medicines Agency for the treatment of MDS and is also indicated for the treatment of acute myeloid leukemia (AML). Injection site erythema, ecchymosis, and petechiae are some of the common cutaneous adverse reactions associated with azacitidine. This article describes a rare adverse cutaneous drug reaction with azacitidine in the form of a reticular generalized skin rash in a 28-year-old female with AML.

Perineal warty plaques: A case of verrucous porokeratosis.

Nonvenereal genital dermatoses form an important category of disorders, and verrucous porokeratosis is a rare and less recognized entity among the same. We present the case of a young adult male with warty growths over scrotum and buttocks for a year. Characteristic cornoid lamellae with typical differentiating features were seen in the histopathology, establishing the diagnosis. This case emphasizes the rare nonvenereal cause for a condition clinically mimicking condyloma acuminata.

Case Report of Cutaneous Squamous Cell Carcinoma at the Wrist Joint and the Public Health Crisis of Arsenicosis.

CONTEXT: Arsenicosis is caused by long term (6 months plus) ingestion of arsenic above a safe dose, characterized by skin lesions and possible involvement of internal organs. Arsenicosis is common in India and Bangladesh where naturally occurring high concentrations of arsenic in the earth's crust contaminate ground water, causing adverse health effects. CASE PRESENTATION: We report a case of a 55-year-old Indian male, resident of a known arsenic endemic region of Uttar Pradesh who suffered from characteristic pulmonary and cutaneous features of chronic arsenic toxicity which included radiological findings of interstitial lung disease, hyperkeratotic lesions over the palms and soles, rain drop like pigmentation over the trunk, and carcinomatous changes at the wrist joint. The patient was started on chelating agents (d-penicillamine) and oral retinoids (isotretinoin) followed by the surgical excision of the carcinoma. DISCUSSION: Environmental contamination with arsenic is a well-known health hazard in South Asian countries. The main source is consumption of contaminated ground water for domestic purposes. Cutaneous lesions, internal organ involvement including interstitial lung disease and carcinomas as observed in our patient have been reported in the literature. Various mechanisms like epigenetic changes and arsenic-induced immune suppression have been proposed for the development of cutaneous carcinomas with prolonged exposure to arsenic. RELEVANCE TO CLINICAL PRACTICE: Among the various causes of palmo-plantar hyperkeratosis, arsenicosis should be kept in mind when presenting in combination with pigmentary changes and carcinomatous growth from an arsenic-endemic region. CONCLUSIONS: People residing in arsenic-endemic regions should be made aware of arsenic-related health hazards. Rainwater harvesting and good nutrition are the simplest measures which could be adopted by the exposed population in affected areas. Several methods have also been employed by governmental and non-government organizations to separate arsenic from contaminated water to combat arsenic-related diseases and carcinomas. COMPETING INTERESTS: The authors declare no competing financial interests.

Unusual non-trophic ulcerations in leprosy: A case series of 17 patients.

Non-neuropathic ulcers in leprosy patients are infrequently seen, and atypical presentations are prone to misdiagnosis. We evaluated diagnosed cases of leprosy between January 2017 and January 2020 for the presence of cutaneous ulceration, Ridley-Jopling subtype of leprosy, reactions and histologic features of these ulcerations. Treatment was given as WHO recommended multi-bacillary multi-drug therapy. We found 17/386 leprosy patients with non-neuropathic ulcers. We describe three causes - spontaneous cutaneous ulceration in lepromatous leprosy (one nodular and one diffuse), lepra reactions (five patients with type 1; nine with type 2, further categorised into ulcerated Sweet syndrome-like who also had pseudoepitheliomatous hyperplasia, pustulo-necrotic and necrotic erythema nodosum leprosum) and Lucio phenomenon (one patient). Our series draws attention towards the different faces of non-neuropathic ulcers in leprosy, including some atypical and novel presentations.

Cyclooxygenase 2 and vascular endothelial growth factor-potential targets to manage lepra reactions: A case-control study.

Reactions in leprosy have an immune mediated pathogenesis. While type 1 reactions are delayed hypersensitivity phenomenon, type 2 reactions are immune complex mediated. Key molecules which mediate the immune insult in lepra reactions require evaluation in order to tailor their therapy and prevent disability. The objective of the study was to evaluate expressions of Cyclooxygenase 2 and Vascular Endothelial Growth Factor in skin biopsies from leprosy patients and correlate their expression with presence of either type 1 or type 2 lepra reactions. This was a case control study. Cyclooxygenase 2 and Vascular Endothelial Growth Factor expression in dermal macrophages and vascular endothelium was assessed immunohistochemically. Biopsies from patients with Non-reactive leprosy and healthy controls were used for comparison. SPSS software was used for statistical analysis. A total of 147 skin biopsies were evaluated, including 18 with Type 1 reaction, 39 Type 2 reaction, 81 non-reactive leprosy and 9 healthy controls. Both Cyclooxygenase 2 and Vascular Endothelial Growth Factor expression were significantly higher in type 1 followed by type 2 reaction as compared to controls. These results may guide us regarding use of Cyclooxygenase 2 and Vascular Endothelial Growth Factor inhibitor drugs which may be a major step in treating reactive leprosy patients and preventing nerve damage and disability.

Updates on Management of Leprosy in the Context of COVID-19 Pandemic: Recommendations by IADVL SIG Leprosy.

The Special Interest Group (SIG) on leprosy thought it to be prudent to revisit its previous practice recommendations through this update. During this period, the pandemic course shifted to a 'second wave' riding on the 'delta variant'. While the number of cases increased manifold, so did the research on all aspects of the disease. Introduction of vaccination and data from various drug trials have an impact on current best practices on management of diseases including leprosy. The beneficial results of using steroids in management of COVID-19, gives elbow room regarding its usage in conditions like lepra reactions. On the other hand, the increase in cases of Mucormycosis again underlines applying due caution while recommending immunosuppressants to a patient already suffering from COVID-19. This recommendation update from SIG leprosy reflects current understanding about managing leprosy while the dynamic pandemic continues with its ebbs and flows.