An Unusual Case of Anderson-Fabry Disease: Case Report.

Angiokeratoma is a group of capillary malformations characterized by the formation of variably sized dark red hyperkeratotic papules. Initially, it was believed that angiokeratoma corporis diffusum was a telltale sign of Anderson-Fabry disease; however, current consensus states that it is also seen in various other lysosomal enzymatic deficiencies. In this report, we present the case of a 12-year-old boy who developed angiokeratoma corporis diffusum with sensorineural deafness, acroparesthesias, and renal involvement.

Assessing the Association between Psoriasis and Cardiovascular Ischemia: An Investigation of Vascular Endothelial Growth Factor, Cutaneous Angiogenesis, and Arterial Stiffness.

Background: Vascular endothelial growth factor (VEGF)-mediated angiogenesis's role in developing psoriasis and cardiovascular events has been established. However, the interplay between the two diseases regarding this cytokine remains an understudied area. Aim and Objectives: This case-control study aimed to investigate the relationship between VEGF-mediated angiogenesis and cardiovascular ischemia in patients with psoriasis. Materials and Methods: The study included 200 clinically diagnosed treatment-naïve cases of psoriasis and 200 controls. The VEGF level, cutaneous vascularity, and cardiovascular ischemia were measured between cases and controls. Cutaneous vascularity was assessed using non-invasive imaging technique such as laser doppler imaging (LDI) and measuring skin blood flow measurement (SBFM). Cardiovascular ischemia was evaluated using noninvasive techniques by measuring carotid intima-media thickness (CIMT) and pulse-wave velocity (PWV). The arterial vasa vasorum was evaluated using ultrasound imaging. Results: The study found a significant correlation between psoriasis severity and levels of VEGF (P < 0.001). Cases had significantly higher CIMT and PWV levels (P = 0.001 and <0.001, respectively). There was a significant positive correlation between the severity of psoriasis and the levels of cutaneous angiogenesis (r = 0.7, P < 0.001). Conclusion: According to this study, patients with psoriasis are at a higher risk of developing cardiovascular ischemia due to excessive angiogenesis associated with the condition. VEGF plays a key role in atheroma formation in psoriasis patients.

Histopathological Spectrum of Cutaneous Reactions following COVID-19 Vaccine - An Observational Study.

Background: Owing to the myriad clinical presentations of COVID-19 vaccine-induced adverse events, clinicopathological correlation is the key to understanding the underlying pathophysiology of these reactions. However, there is still a dearth of such systematic studies across a wide spectrum of vaccine reactions. Aim and Objectives: This study provides a clinical and histopathological correlation of COVID-19 vaccine-induced mucocutaneous reactions. Materials and Methods: The study population included all the individuals developing any form of self-reported mucocutaneous adverse events within 4 weeks of receiving the COVID-19 vaccine. The clinical and histopathological features were recorded. Results: A total of 126 cases were identified. Seven histopathological patterns were recognized. The most common histopathological feature was spongiotic dermatitis, seen in all the patients with "vaccine-related eruption of papules and plaques" (VREPP). Other patterns included lichenoid or interface dermatitis, dermal hypersensitivity reaction, leukocytoclastic vasculitis, subepidermal blistering, psoriasiform hyperplasia, and dermal granulomatous reaction. Conclusion: Owing to such myriad clinical presentations, utilizing a histopathological classification could ease categorizing the vaccine-induced mucocutaneous eruptions.

Evaluation of the Knowledge, Attitude and Practice of Patients on Immunosuppressive Drugs Towards COVID-19 Attending Dermatology Department- A Multicentric Cross-Section Study.

BACKGROUND: The deadly COVID-19 (Coronavirus Disease 2019) or SARS-CoV-2 (severe acute respiratory syndrome coronavirus 2) was identified for the first time in December 2019 from Wuhan, China, and by the beginning of March 2020, it was declared a pandemic by the World Health Organization (WHO). Despite so many government regulations and awareness measures, there is still a lacuna between the gravity of illness and the knowledge of the average person toward it. OBJECTIVE: We aimed at assessing the knowledge, attitude, and behavioral practice (KAP) of patients on immunosuppressive medication attending the dermatology department toward, COVID-19. MATERIAL AND METHODS: A self-designed printed/digital questionnaire consisting of 30 questions (Hindi and English) was supplied to patients being treated with any systemic immunosuppressives (for at least 3 weeks), for any dermatological ailment. The questionnaire consisted of 12 questions assessing the knowledge, 11 for attitude, and 7 for practices of patients toward COVID-19. RESULTS: The study included 237 patients with a mean age of 44.57 ± 13.72 years. The correct knowledge toward COVID-19 was present in 126 (53.16%) patients with a mean score of 7.79 ± 3.08 out of 13. The mean attitude score was 8.35 ± 2.16 (out of 11) while the mean score of practice was 5.64 ± 2.03 (out of 8). Increased hygiene levels were seen in 220 (92.83%) patients. Sixty-six (27.85%) patients admitted to stopping their prescribed immunosuppressives by themselves during COVID-19 and a significant proportion agreed to the use of alternative medicines with questionable efficacy (n = 91; 38.39%). A significant difference in KAP was found across various strata of society like gender, age, socioeconomic status, literacy, and residence (P < 0.001). LIMITATIONS: Our study was limited by small sample size, absence of a control group with healthy individuals, and short duration of the study. CONCLUSIONS: Most of the participants had poor knowledge, a positive attitude, and good practices toward COVID-19. Proper counseling of patients and the use of telemedicine could help combat the gap in KAP without compromising the healthcare facilities needed for the management of such patients.

An Observational Study of Mucocutaneous Manifestations among SARS-CoV-2 Patients from Three COVID-19 Dedicated Tertiary Care Centers.

INTRODUCTION: Ever since the outbreak of COVID-19, the respiratory system has been the chief focus of researches, however, understanding the impact of this disease on the integumentary system is just as essential. OBJECTIVES: We aimed at collecting data on any cutaneous manifestation arising in patients with active and recovering COVID-19 infection, or a direct consequence of the infection's treatment, and correlating these findings with systemic disease severity and duration. MATERIALS AND METHODS: A prospective observational study was conducted in three tertiary care centers from Rajasthan, India, to acquire data of laboratory-confirmed cases of COVID-19 presenting with any mucocutaneous manifestation. RESULTS: Eight predominant patterns of dermatological involvement were seen, namely, maculopapular (14.59%), urticarial (13.17%), perniotic (12.1%), pityriasis rosea (11.74%), acral erythema/edema (10.3%), petechial (4.63%), vesicular (2.49%), and livedo (1.78%). Rare findings included eruptive pseudoangioma, eruptive hypomelanosis, alopecia parvimaculata, geographic tongue, chikungunya-like hyperpigmentation, and nail changes. On correlating these findings with the gradient of the disease, livedo, vasculitis, exfoliation, and erythroderma were associated with severe disease, whereas perniosis and eruptive pseudoangioma were seen in mild illness. CONCLUSION: We reported a few previously unpublished skin manifestations of COVID-19, namely, geographic tongue, chikungunya-like pigmentation, eruptive hypomelanosis, and alopecia parvimaculata. This study provides a visual description of the muco-cutaneous manifestations of COVID-19 disease which could aid a dermatologist or physician in early diagnosis of this novel infection, especially in a resource-poor setting.