Hereditary angioedema (HAE) is an uncommon disorder characterized clinically by recurrent episodes of nonitchy subcutaneous and/or submucosal swellings. The estimated prevalence of HAE is ~ 1: 10,000 to 1: 50,000. There are no prevalence data from India, however, estimates suggest that there are 27,000 to 135,000 patients with HAE in India at present. The majority of these, however, remain undiagnosed. Replacement of plasma-derived or recombinant C1-esterase inhibitor (C1-INH) protein, administered intravenously, is the treatment of choice during the management of acute episodes of angioedema (i.e., "on-demand treatment") and is also useful for short-term prophylaxis (STP) and long-term prophylaxis (LTP). This has been found to be effective and safe even in young children and during pregnancy. Until recently, none of the first-line treatment options were available for "on-demand treatment," STP or LTP in India. As a result, physicians had to use fresh frozen plasma for both "on-demand treatment" and STP. For LTP, attenuated androgens (danazol or stanozolol) and/or tranexamic acid were commonly used. These drugs have been reported to be useful for LTP but are associated with a significant risk of adverse effects. Intravenous pd-C1-INH, the first-line treatment option, is now available in India. However, because there is no universal health insurance, access to pd-C1-INH is a significant challenge. HAE Society of India has developed these consensus guidelines for India and other resource-constrained settings where plasma-derived C1-INH therapy is the only available first-line treatment option for the management of HAE and diagnostic facilities are limited. These guidelines have been developed because it may not be possible for all patients to access the recommended therapy and at the recommended doses as suggested by the international guidelines. Moreover, it may not be feasible to follow the evaluation algorithm suggested by the international guidelines.
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.
Introduction: Cutaneous tuberculosis is an uncommon form of tuberculosis, accounting for 1%-2% of all forms of extra-pulmonary tuberculosis. Knowledge of the dermoscopic characteristics of different clinical types of cutaneous tuberculosis can help timely diagnosis resulting in better outcomes. Objectives: To characterize the Dermoscopy findings in different clinical types of cutaneous tuberculosis in dark skin phototypes. Methods: All clinically suspected and biopsy confirmed cases of cutaneous tuberculosis seen from July 2019 through December 2021 were retrospectively recruited. Information including age, gender, disease duration, site and morphology of lesions, and presence of concomitant tuberculosis elsewhere was noted. Two investigators retrospectively reviewed the dermoscopic characteristics of these cases. Results: Twenty-two patients comprised of 12 women and 10 men met the inclusion criteria. Lupus vulgaris was the commonest presentation of cutaneous tuberculosis seen in 13 patients. Five had scrofuloderma, 2 had tuberculosis verrucosa cutis and 1 patient each had lichen scrofulosorum and papulo-necrotic tuberculid. Yellow-orange structureless areas (100%), linear/dot vessels (100%), white scales (92.3%), and white structureless areas (84.6%) were the predominant dermoscopy findings in lupus vulgaris. In scrofuloderma, linear vessels and white structureless areas were visible in all cases. Dirty white scales with a papillated surface were characteristically seen in tuberculosis verrucosa cutis, with 1 of the 2 patients each showing vessels and yellow-orange structureless areas. White globules with surrounding erythema were seen in lichen scrofulosorum and yellow-orange structureless areas with keratin plugs in papulo-necrotic tuberculid. Conclusions: A thorough understanding of the characteristic dermoscopy of cutaneous tuberculosis can help suspect the diagnosis early resulting in better management opportunity.
As we were on the road to recovery from the coronavirus disease-19 (COVID-19) pandemic, the world is waking up to yet another potential adversary. Monkeypox (or human monkeypox) caused by monkeypox virus (an orthopox virus) is fast emerging in more than 80 countries worldwide, where it has never been historically reported. We in India, have already seen the advent of this outbreak since July 2022, with a progressive rise in number of cases being seen. Though the virus is not a novel virus; it is presenting with atypical manifestations as compared to our conventional knowledge of the disease. Through this document, the Indian Association of Dermatologists, Venereologists, and Leprologists Academy aims to sensitize dermatologists toward recognizing the clinical features and responding promptly, to contain the outbreak at the earliest. In view of the non-availability of specific antiviral drugs as well as vaccines; early detection, isolation, and prevention of spread form the mainstay of our approach towards the outbreak, which has been declared to be a "Public Health Emergency of International Concern" by the World Health Organization.
Methotrexate is widely prescribed by various specialists, including internists, dermatologists, rheumatologists, orthopedics, and oncologists. The clinical features of acute methotrexate toxicity vary and are influenced by the acute cumulative dose, duration of methotrexate, associated risk factors, comorbidities, and drug interactions. We present a series of six patients with acute methotrexate toxicity. The characteristics of their clinical presentation, diagnoses, and risk factors are highlighted. All patients were given intravenous leucovorin and other supportive treatments. This series highlights the importance of thorough counseling for patients regarding the course of disease for which methotrexate is prescribed and the dosing, schedule, and adverse effects that are associated with methotrexate. Physician awareness, diagnosis, and treatment are crucial for mitigating the complications of methotrexate toxicity. (SKINmed. 2022;20:274-281).
Drug-Related Side Effects and Adverse Reactions , Methotrexate , Humans , India , Leucovorin/therapeutic use , Methotrexate/adverse effects , Risk Factors , Tertiary Care Centers
Introduction Leprosy is a chronic, infectious disease resulting in significant physical and social morbidity. This study aims to assess the nutritional status of patients with leprosy. Methods Treatment-naïve leprosy cases seen over two years at a tertiary care center were recruited in this prospective observational study. Demographic details, type of leprosy, and presence of reactions and disabilities were recorded. Nutritional assessment was done by calculating the body mass index (BMI) and measuring the hemoglobin, serum iron, serum albumin, and serum cholesterol. Results Fifty patients were recruited with a mean age of 40.5 (15.3) years. Most patients (60%) had a normal BMI of 18.5-24.9, and 14% were underweight. Two-thirds of the patients had low hemoglobin, with 18 (36%) having low serum iron. A statistically significant association was observed between low serum iron and the multibacillary form of leprosy. Conclusion A nutritional assessment should be included in the initial evaluation of leprosy.
Trichorrhexis nodosa (TN) is a common hair shaft defect that develops as a result of excessive physical or chemical trauma. Microscopy is considered the gold standard for its diagnosis. Outpatient diagnosis thus becomes difficult in the absence of availability of microscope. Trichoscopy is emerging as an excellent tool in outpatient diagnosis of hair shaft defects. Here, we report a young girl with localized TN secondary to trichoteiromania where trichoscopy clinched the diagnosis. Classical appearance of nodes in hair shaft and transverse hair shaft fractures producing "two brooms stuck in opposite direction" was evident. This case is being reported to emphasize the advantages of trichoscopy in quick diagnosis of hair shaft defects with review of published literature.
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.
Zoon balanitis is a rare chronic inflammatory dermatosis involving genitalia. It is often misdiagnosed or diagnosed late leading to significant patient distress. Dermoscopy can act as a useful modality in prompt diagnosis of zoon balanitis. Herein, we report dermoscopic findings in twelve lesions of zoon balanitis in seven patients.
Acquired lymphangiectasia is an unique entity encountered by an urologist which is marked by occlusion of lymphatics associated with abnormal permanent dilation of cutaneous lymphatics. There is presence of thin-walled ectatic vessels in the superficial and mid dermis, which develops later in life. Trauma, infection, radiation or surgery are important causes of this rare aetiology. Diagnosis is always demanding, as it closely resembles many benign aetiology. Meticulous decision-making and appropriate treatment should be chosen for the management.
Carcinoma , Penile Neoplasms , Vascular Diseases , Disease Progression , Humans , Male , Penile Neoplasms/diagnosis , Scrotum/diagnostic imaging
