Hand, Foot and Mouth Disease: A Single Centre Retrospective Study of 403 New Cases and Brief Review of Relevant Indian Literature to Understand Clinical, Epidemiological, and Virological Attributes of a Long-Lasting Indian Epidemic.

Background: There have been sporadic and periodic large-scale epidemics of hand, foot, and mouth disease (HFMD) with cases at risk for significant morbidity and mortality particularly in Southeast Asia since 1997 and in India since early 2003. Method: We retrospectively studied 403 cases recorded from 2009 to 2019 and reviewed relevant Indian literature published between 2004 and 2019 to understand clinical, epidemiological, and virological attributes of this long-lasting Indian epidemic. Result: There were 96.8% children and adolescents (M:F 1.6:1) aged 2 months to 18 years and 84% were aged <5 years. Adult family contacts comprised 3.2%. Only 12 sporadic cases occurred during 2009-2011 followed by increased number from 2012 to 2015 peaking with 30.8% cases in 2013 and declining slowly until the year 2019 with small resurge in 2018. The major peaks occurred during summers with small peaks in autumns. Literature review showed 3332 cases presenting between 2004 and 2019 across Indian states with similar epidemiological trends whereas serotyping identified Coxsackievirus A16 (CV A16) in 83%, Coxsackievirus A6 (CV A6) in 17%, Enterovirus 71 in 4.1%, and multiple strains in 11.7% samples, respectively. Conclusion: The overall features of this long-lasting HFMD epidemic; affecting children aged <5 years more often than adults, none or minimum neurological or pulmonary complications in few patients, peaks occurring during summer and autumn months, and identity of the pathogenic virus coincide with global trends. However, the continuous spread of the disease across the country appears in sync with pre-epidemic periods of China and Taiwan. It calls for a continuous surveillance and making HFMD a notifiable disease in India.

The Association Between Chronic Plaque Psoriasis and Nonalcoholic Fatty Liver Disease in Indian Patients: Results of a Pilot Study.

Background: Psoriasis is a chronic dermatosis with potential to cause systemic disease by triggering dysmetabolism, such as metabolic syndrome and nonalcoholic fatty liver disease (NAFLD). We studied the relationship and associations between NAFLD and clinical features, including age, gender, disease duration, and severity of psoriasis in our patients. Methods: This cross-sectional study comprised 61 (m:f, 43:19) patients without pre-existing comorbidities and matched 24 (m:f, 16:8) healthy controls aged between 20 and 68 years. Disease severity was graded as mild, moderate, and severe by psoriasis area and severity index score and body surface area (BSA) involvement. The grades of fatty liver and liver fibrosis were assessed using liver ultrasonography (USG) and transitional vibration-controlled elastography (Fibroscan). Results: Overall, 67.2% of patients were aged >40 years, and the duration of disease was <5years in 60.7% of patients. Mild and moderate to severe psoriasis occurred in 78.7% and 21.3% of patients, respectively. BSA was >10% in 57.5% patients. The proportion of NAFLD was 27.9% and 32.8% by USG and Fibroscan compared with 20.8% in controls. Statistically, there was no significant difference or association between the prevalence of NAFLD among patients and controls, and gender, age (mean ± standard deviation, 47.5 ± 13.8 vs. 45.2 ± 15.7), duration, severity of psoriasis, and arthritis between psoriatic patients with and without NAFLD. Conclusion: This was a pilot study because of the numerosity of sample and highlights trends for possible link between psoriasis and NAFLD, but the results need cautious interpretation and clinical application. Whether NAFLD can be attributed to overall systemic inflammatory process of psoriasis or it occurs as an epiphenomenon of concurrent metabolic syndrome needs elucidation with well-designed studies. Cross-sectional study design, small number of patients, and controls remain major limitations. The study did not compare its findings with liver biopsy.

The Demographic Attributes, Clinical Features, and Optimal Management of 143 Patients with Pemphigus: A Retrospective Observational Study from a Tertiary Care Center of India.

Background: This retrospective study was to understand the clinico-epidemiologic and therapeutic aspects of pemphigus patients attending our clinic. Methods: We analyzed charts of 143 (M: F; 51:92) pemphigus patients having variable severity recorded between 2009 and 2019. Therapies were customized based on patient's age, disease severity, comorbidities, compliance prospects, and affordability. The patients were monitored monthly and as needed for therapeutic outcome in terms of disease control, reduced hospitalization, remission/relapse, and drug toxicity. Results: These patients were aged 15 to 86 years, the majority, 68 (47.5%), was 41 to 60 years of age. The pemphigus vulgaris in 83.9% patients was the commonest variant. Treatment regimens were; dexamethasone-cyclophosphamide-pulse (DCP) therapy in 51.2%, dexamethasone-azathioprine-pulse (DAP) therapy in 11%, dexamethasone-pulse (DP) therapy in 5.5%, rituximab in 24.4%, IVIg in 5.5% patients, and oral corticosteroids with or without adjuvant. Remission occurred after 2-17 (mean 5.8) DCP doses; 14 and 7 patients achieved remission for ≥2 y and ≥5 y, respectively. Rituximab was effective to treat both new and relapsed cases (n = 31). Additional treatment with another adjuvant prolonged remission in seven patients relapsed 12-16 months after treatment with rituximab alone. Overall, oral corticosteroids alone and DAP therapy showed unsatisfactory response. Adverse effects seen in 41.9% of patients were mainly corticosteroids related. Conclusion: The overall clinico-epidemiologic spectrum of pemphigus and therapeutic efficacy of DCP, DAP, or corticosteroids in this study was in sync with the literature. Combining rituximab and corticosteroids plus an immunomodulator initially (phase-1), followed by immunomodulator alone for one year (phase-2) will improve long-term (phase-3) therapeutic outcome. IVIg was effectively useful in patients with concurrent infections.

The Clinical Characteristics, Putative Drugs, and Optimal Management of 62 Patients With Stevens-Johnson Syndrome and/or Toxic Epidermal Necrolysis: A Retrospective Observational Study.

BACKGROUND: Case reviews of severe cutaneous adverse drug reactions (ADRs) such as SJS/TEN provide useful insights for clinical characteristics, putative drugs, and management protocols. PATIENTS AND METHODS: Medical charts of 62 (m:f- 20:42) patients with SJS/TEN hospitalized between 2010 and 2019 were analyzed retrospectively for clinical attributes, putative drugs and their indications, extracutaneous complications, and therapeutic outcome. The diagnosis was clinical based on established criteria. WHO-UMC scale for reporting ADR and ALDEN algorithm score were used for causality assessment. Therapies were customized based on in-house resources and affordability. RESULTS: Cases included were SJS (41.9%), SJS/TEN overlap (33.9%), and TEN (24.2%) aged 4-85 years. Complications included transaminitis (69.4%), lymphadenopathy (15.5%), septicemia (11.3%), and wound infections (4.8%). Aromatic anticonvulsants (37.1%), disease-modifying antirheumatic drugs (25.8%), antiretroviral drugs (12.9%), non-steroidal anti-inflammatory drugs (8.1%), antimicrobials (4.8%), and trihexyphenidyl (3.2%) were major putative drugs. The mean latent period was 16.6 days. The observed 8% mortality was because of primary comorbidities or multiorgan failure. Addition of fresh blood transfusion (BT, n = 11) or IVIg (n = 7) to systemic corticosteroids showed early relief in skin tenderness, improvement in general condition, and re-epithelialization. Only 16% of patients developed sequelae. CONCLUSION: Aromatic anticonvulsants, allopurinol, nevirapine, cotrimoxazole, paracetamol, and diclofenac remain the most implicated drugs. Sulfasalazine, leflunomide, ethambutol, and trihexyphenidyl were uncommon additions. A short course of high-dose dexamethasone in the early stage was useful. Addition of BT or IVIg provided rapid relief. Preexisting HIV disease, kidney disease, and sepsis remain important for in-hospital deaths. Retrospective study design and small number of cases remain major limitations.

The Association of Alcohol Use Disorder and Chronic Plaque Psoriasis: Results of a Pilot Study.

BACKGROUND: Association between alcohol consumption, alcohol use disorder, and clinical features of psoriasis patients has not been adequately studied in the Indian context. OBJECTIVES: To study the frequency of alcohol consumption, alcohol use disorder, and its association with age, gender, duration, and severity of psoriasis. MATERIALS AND METHODS: One hundred and forty-six (M: F 6.3:1) patients completed the Alcohol Use Disorder Identification Test (AUDIT) questionnaire by World Health Organization (WHO). Excessive drinkers, occasional drinkers, and abstainers were defined. AUDIT provided a measure of alcohol consumption, its dependence, and its impact on daily life. The severity of psoriasis was graded as mild, moderate, and severe. RESULTS: Seventy-four (50.7%) patients were aged ≤40 years and 51.4% of patients had the disease for <5 years. Psoriasis was mild in 48.6% and moderate to severe in 51.4% of patients, respectively. Only males (32.9%) were consuming alcohol in varying amounts; 19.9% were occasional drinkers (AUDIT score <8). Other 67.1% of patients completely abstained from alcohol consumption (AUDIT score 0). The remaining 13% were regular drinkers (AUDIT score >8) and had more severe psoriasis compared to patients having AUDIT score <8 (P < 0.05). A high level of alcohol use disorder and alcohol dependence was present in one patient each. LIMITATIONS: Few patients, particularly females may not have disclosed their alcohol consumption due to fear of stigmatization. Small number of patients, hospital-based cross-sectional study design, and no follow-up for clinical improvement after cessation of alcohol are other limitations. CONCLUSIONS: Alcohol consumption was associated with alcohol use disorder in 32.9% of patients (AUDIT score >8) and significantly severe psoriasis compared to 67.1% abstainers. Whether increased alcohol consumption is a consequence or a risk factor for chronicity of psoriasis needs large linear studies for confirmation.

Cardiomyopathy and echocardiographic abnormalities in Indian patients with psoriasis: Results of a pilot study.

BACKGROUND: The relationship between psoriasis and cardiomyopathy is understudied in Indian patients. OBJECTIVE: We evaluated psoriasis patients for cardiomyopathy and other echocardiographic abnormalities. METHODS: About 98 (M:F = 67:31) patients with mild to moderate psoriasis aged 18-75 years (mean ± SD = 42.12 ± 12.79 years) having no pre-existing metabolic syndrome and cardiovascular disorders were studied. X-ray chest, electrocardiogram and echocardiography were performed and interpreted by cardiologist for size of the left and right ventricles, left ventricle ejection fraction, diastolic function, pulmonary artery pressure and valve abnormality/regurgitation and their severity as per current guidelines/recommendations. The cardiomyopathies were defined according to standard diagnostic guidelines. RESULTS: Echocardiographic abnormalities were noted in 13 (13.3%) patients aged 19-75 years (mean ± SD = 43.30 ± 15.71 years). The left ventricular diastolic dysfunction (grade 1) was observed in nine patients (moderate severe psoriasis in four patients) and one of them also had concentric left ventricular hypertrophy; a precursor of restrictive cardiomyopathy. Mild tricuspid valve regurgitation was present in other four patients. There was no statistically significant difference in age, gender, duration and the severity of psoriasis when compared with patients having normal echocardiography. The mitral or aortic valves, pulmonary artery pressure, mid-right-ventricular diameter and the left atrial volume showed no abnormality. CONCLUSIONS: Psoriasis perhaps plays a role in left ventricular dysfunction and possibly cardiomyopathy even with moderately severe disease and in the absence of clinical symptoms. However, these observations need to be interpreted with caution in the absence of any statistically significant difference between age, gender, duration and severity of psoriasis in the patients having normal and abnormal echocardiography.

Therapeutic efficacy and safety of oral tranexamic acid 250 mg once a day versus 500 mg twice a day: a comparative study.

Oral tranexamic acid (TXA) 250 mg twice daily has been used effectively for 4 weeks to 6 months to treat melasma. As relapses are frequent on discontinuation, a minimum effective dose of TXA that can be used safely for long time remains unknown. We compared the efficacy of oral TXA 250 mg once daily and 500 mg twice daily given for 16 weeks in 132 (m:f 23:109) adults with melasma. 42 patients in Group-A (TXA 250 mg/d) and 46 patients in Group-B (TXA 500 mg twice/d) completed the study. They were followed up at 4-week interval for percentage reduction in baseline Melasma Area Severity Index (MASI) and at 24 and 28 weeks for relapse. Therapeutic response, for both as per-protocol and intention-to-treat analysis, was scored as very good (> 75% reduction), good (51-75% reduction), moderate (25-50% reduction), mild (< 25% reduction) or no improvement. Reduction in mean MASI score at 4 weeks was not statistically significant in Group-A but it decreased significantly 8 weeks onwards and was comparable with that in Group-B. The relapse rate was higher in Group-B (10.8%) than Group-A (4.7%) at the end of 28 weeks. Oligomenorrhoea and abdominal discomfort in few patients did not necessitate treatment discontinuation. TXA 500 mg twice daily showed early reduction in mean MASI score compared to 250 mg given once daily with comparable safety and therapeutic efficacy at 16 weeks. Open-label cross-sectional design, no control arm, small number of patients in each group, MASI score being subjective assessment tool, short duration of treatment and follow-up are study limitations.

Adults with a more extensive body involvement, moderate to extremely severe vitiligo and a prolonged clinical course have an early onset in childhood in addition to other prognostic factors as compared to individuals with later-onset vitiligo.

BACKGROUND: The extent and disease severity, duration and other associated prognostic cofactors in vitiligo in adults may vary with the age of onset (before or after 10 years of age). OBJECTIVES: To compare extent and disease severity, duration and other cofactors in adults with early-onset and late-onset vitiligo. METHODS: The medical records of 408 (M:F 1:1.1) adults aged 20-75 years diagnosed with vitiligo between January 2016 and December 2019 were examined retrospectively. The extent and severity of vitiligo were defined. Characteristics of vitiligo with early onset and late onset were compared statistically and odds ratios calculated for risk assessment. RESULTS: 31 (7.6%, M:F 1:2.4) patients had early-onset vitiligo, and 377 (92.4%, M:F 0.8:1) patients had later-onset vitiligo. Compared to late onset, patients with early-onset vitiligo had a significant number of males (71% vs 45.9%), higher percentages of body surface area involvement and moderate to extremely severe disease (29% vs 10.6%), longer duration of disease (41.9% vs 9%), Koebner's phenomenon (48.4% vs 15.6%) and halo nevus (9.7% vs 1.9%). Differences between the two groups were not significant for types of vitiligo, family history of vitiligo and presence of cutaneous and systemic/autoimmune diseases. CONCLUSION: The adults, males in particular, with generalised vitiligo (>10% BSA involvement) appear to have an early onset and a prolonged clinical course. The presence of Koebner's phenomenon and halo nevus in patients with early-onset vitiligo was other poor prognostic factors compared to patients with late-onset vitiligo. The retrospective, hospital-based cross-sectional design and small sample size for stratification remain major limitations.

The clinico-epidemiological characteristics and therapeutic experience of 152 patients with cutaneous sporotrichosis: a 10-year retrospective study from India.

BACKGROUND: Cutaneous sporotrichosis, a subcutaneous mycosis because of Sporothrix schenckii, is sporadic worldwide with local hyperendemic pockets. OBJECTIVES: To study clinico-epidemiological and therapeutic aspects of sporotrichosis in our clinic. METHODS: We retrospectively analyzed medical records of 152 (M:F 52:100) patients with cutaneous sporotrichosis managed during 2010-2019. RESULTS: All patients were involved in agricultural activities, and 63.2% were aged 21-60 years. Women outnumbered men by nearly two times. Fixed and lymphocutaneous sporotrichosis occurred in 54.6% and 43.4% patients, respectively. Only 2% of patients had multifocal sporotrichosis. Only 48% of patients imputed their disease to prior injuries. Extremities, upper in 53.9% and lower in 21% of patients, were mostly involved. Scrotum involvement in one patient was unusual. A mixed inflammatory infiltrate in 38.7%, chronic granuloma formation in 35%, and presence of spores in 48.9% biopsies was noted. S. schenckii grew on Sabouraud's dextrose agar in 40.2% of cases. Treatment with saturated solution of potassium iodide was curative in 76.8% patients, and lesions healed in 2-9 months (average 5.2 months). Metallic taste was experienced by 42.9% of patients. Itraconazole therapy was safe and effective in seven patients, and the response was better when combined with SSKI compared to either drug used alone. CONCLUSION: Cutaneous sporotrichosis mostly affects persons during active years of life. The injuries predisposing to infection are mostly forgotten. Both fixed and lymphocutaneous sporotrichosis involving extremities remain common forms. SSKI alone or in combination with itraconazole is safe and effective treatment. Itraconazole is preferable in patients having preexisting hypothyroidism or intolerance to SSKI.

The Association of Thyroid Dysfunction with Chronic Plaque Psoriasis: A Hospital-Based Retrospective Descriptive Observational Study.

BACKGROUND: Associations among thyroid dysfunction, thyroid autoimmunity, and clinical features including age, gender, disease duration, and severity of psoriasis is less studied. OBJECTIVES: To study frequency of thyroid dysfunction and thyroid autoimmunity and examine association among thyroid dysfunction, thyroid autoimmunity, and clinical features including gender, age, duration, and severity of psoriasis. MATERIAL AND METHODS: The medical records of 290 (m:f 2.15:1) patients aged 13-75 years with plaque psoriasis were analyzed for thyroid dysfunction and thyroid autoimmunity. Thyroid dysfunction was defined as 10% variation in any thyroid hormone levels. Thyroid autoimmunity was diagnosed from presence of antithyroid peroxide (anti-TPO) antibodies. RESULTS: The majority, 57.9% patients, was aged ≥41 years (Type-2 psoriasis) and duration of disease was <5 years in 58.6% patients. Mild and moderate to severe psoriasis was present in 58.3% and 41.7% patients, respectively. Deranged thyroid functions were present in 29 (10%) patients. Hypothyroidism and hyperthyroidism occurred in 5.4% and 2.7% patients, respectively. Anti-TPO antibodies were observed in 13.5% patients; 11had hypothyroidism. There was no statistically significant difference in gender, age, duration, and severity of psoriasis when compared with patients having normal thyroid function tests. CONCLUSION: The study suggests possible thyroid dysregulation and thyroid autoimmunity in psoriasis but results need careful interpretation and clinical application. Their significance as standalone risk factor for the chronicity, severity, and relapses in psoriasis or whether thyroid hormone replacement or antithyroid drugs become a useful therapeutic option remains tenuous at best for need of more robust evidence. Retrospective, observational, cross-sectional study design, small number of patients, and lack of controls remain major limitations.

Association of Psoriasis with Autoimmune Disorders: Results of a Pilot Study.

BACKGROUND: Association of psoriasis with other autoimmune diseases remains an ongoing research subject. OBJECTIVES: To investigate the association of psoriasis with other autoimmune disorders. MATERIALS AND METHODS: We studied 80 (M: F 57:23) psoriasis patients aged 13-75 years for concurrent autoimmune disorders. After clinical examination, hemogram, fasting blood sugar, HbA1c, thyroid function tests, anti-TPO antibody, rheumatoid factor, anti-tTG antibody, anti-CCP antibody, ANA, anti-dsDNA antibody, anti-Ro antibody, and fecal calprotectin were estimated. RESULTS: Mild-to-moderate and severe psoriasis was present in 86.3% and 13.8% patients, respectively. Psoriatic arthritis was present in 3.8% patients, all of whom also had severe psoriasis. Only 37 (46.3%) patients had clinical and/or sero-abnormality suggestive of autoimmune disorders; vitiligo in 3.8%, type-1 diabetes mellitus (DM) in 1.3%, and type-2 DM in 6.3% patients. Sero-positivity reflecting subclinical autoimmunity was noted for anti-CCP antibodies (in 2.5%), rheumatoid factor (in 2.5%), hypo- or hyper-thyroidism (in 8.8%), anti-TPO antibodies (in 5.0%), anti-tTG antibody (in 1.3%), ANA (in 5.0%), anti-dsDNA antibody (in 2.5%), and anti-Ro antibody in 11.3% patients. Elevated fecal calprotectin levels suggestive of inflammatory bowel disease (IBD) occurred in 11.2% of 27 patients. Multiple abnormalities happened in 2.5% patients. CONCLUSION: Apparently psoriasis patients seem to have a predilection for other autoimmune disorders particularly for vitiligo, diabetes mellitus, autoimmune thyroiditis, rheumatoid arthritis, and IBD. However, association between psoriasis and other autoimmune disorders at best remains tenuous for want of strong evidence. Nevertheless, screening for them will improve overall management of these patients. Cross-sectional study design and small number of study subjects remain important limitations.

Characteristics of Vitiligo in Children and Adolescents.

Vitiligo in children and adolescents displays some distinct features, which may affect its clinical course, therapeutic outcome, and prognosis. We studied 579 children and adolescents with vitiligo, comprising 275 (47.5%) boys and 304 (52.5%) girls (male:female ratio [m:f], 1:1.1) aged between 2 and 19 years (mean ± SD 11.13 ± 4.23 years). The majority of children (301, 52%) were aged >5-12 years, and 221 (38.2%) were adolescents; onset of vitiligo in the above groups occurred between the ages of 2 and 19 years (mean ± SD 9.18 ± 4.08 years). The majority of patients (337, 58.2%) had developed vitiligo between 5 and 12 years of age, and 332 (57.4%) patients had a medical consultation within 1 year of the onset of the disease. The involvement of up to 10% of body surface area in 569 (98.3%) patients, generalized vitiligo in 328 (56.7%) patients, and focal vitiligo in 158 (27.3%) patients were the major presentations. Only 150 (15.9%) patients had family members affected by vitiligo, and associated systemic disorders, predominately thyroid abnormalities, occurred in three (0.5%) patients. Vitiligo was more frequent in children aged 5-12 years, and it affected girls with a slight preponderance, commonly presenting as generalized vitiligo and focal/localized vitiligo. Patients with a family history of vitiligo had an earlier onset but without a statistically significant difference. Local trauma is an important trigger, and screening for thyroid disorders appears important.

The association of anti-gliadin and anti-transglutaminase antibodies and chronic plaque psoriasis in Indian patients: Preliminary results of a descriptive cross-sectional study.

BACKGROUND: Gluten sensitivity among psoriasis patients and its association with gender, age, disease duration and severity of psoriasis are under studied in Indians. OBJECTIVE: To examine association among serum levels of anti-tTG and anti-gliadin antibodies and clinical features including gender, age, duration and severity of psoriasis. METHODS: Serum levels of anti-transglutaminase and anti-gliadin antibodies were measured quantitatively in 80 (M:F 57:23) psoriasis patients aged 15 to 83 years and matched healthy subjects. RESULTS: Forty-five (56.3%) patients were aged ≥41years, duration of disease was >5years in 43(53.8%) patients, and 22 (27.5%) patients had moderate-to-severe psoriasis. Two (2.5%) patients had arthritis and elevated serum anti-gliadin antibody. Significantly more patients than controls had elevated serum anti-gliadin antibody (67.5% vs. 2.5%) and anti-transglutaminase antibody levels (62.5% vs. 0%). Two patients, each with mild and moderate-to-severe psoriasis, had highly elevated serum anti-gliadin antibody and symptoms akin to coeliac disease. Except for a longer duration of psoriasis in patients with elevated anti-gliadin antibodies, there was no statistically significant difference in gender, age, and severity of psoriasis when compared with patients having normal levels. CONCLUSION: Significant elevation of serum anti-transglutaminase and anti-gliadin antibodies levels is noted in psoriasis patients reflecting a possible link. However, results need careful interpretation for any significance of gluten sensitivity in pathogenesis of psoriasis/arthritis or as a stand-alone risk factor for chronicity/severity of psoriasis or whether gluten-free diet will be ameliorating. Small number of subjects, cross-sectional study design, lack of pathological/endoscopic diagnosis and follow-up are study limitations.

Psoriasis and Co-morbidities: Is Hyperhomocystienemia the Common Link?

BACKGROUND: Hyperhomocystienemia is a plausible common link between psoriasis and associated co-morbidities. AIM: To assess and compare serum homocystiene levels in 160(M:F 94:66) patients aged 18-70 years with chronic plaque psoriasis of varying severity with or without metabolic syndrome, cardiovascular and thyroid disorders and controls. The 155 controls (M:F 97:58) were healthy volunteers aged between 18 and 66 years. RESULTS: Overall, 123 (76.9%) psoriasis patients with or without co-morbidities and 87 (56.1%) controls had elevated serum homocystiene levels; 23.48±14.37 and 18.74±12.59 (mean±SD) µmol/L, respectively. Eighty-one (58%) patients had associated co-morbidities with mean serum homocystiene levels of 22.65±13.70 µmol/L.The difference between psoriasis patients with or without comorbidities and controls was statistically significant. CONCLUSIONS: Hyperhomocystienemia in psoriasis patients with or without comorbidities versus healthy controls suggests its possible dysregulation in psoriasis. The significance of hyperhomocystienemia as an independent risk factor for cardiovascular or other comorbidities in psoriasis patients remains tenuous at best. Well-designed studies will perhaps resolve this issue.

Cloves Syndrome: A Rare Disorder of Overgrowth with Unusual Features - An Uncommon Phenotype?

CLOVES syndrome characterized by Congenital Lipomatous Overgrowth, Vascular malformations, Epidermal nevi, and Skeletal anomalies is a recently described sporadic syndrome from postzygotic activating mutations in PIK3CA. This 3-year-old boy, born to nonconsanguineous and healthy parents, had epidermal verrucous nevus, lower limb length discrepancy and bilateral genuvalgum, anterior abdominal wall lipomatous mass, central beaking of L2 and L3, and fibrous dysplasia of the left frontal bone. Ocular and dental abnormalities (ptosis, esotropia, delayed canine eruption, dental hypoplasia), ipsilateral asymmetrical deformity of skull, and large left cerebral hemisphere with mild ipsilateral ventriculomegaly were peculiar to him denoting an uncommon phenotype. The parents did not consent for magnetic resonance imaging and genetic studies because of financial constraints. The CLOVES syndrome has emerged as an uncommon yet distinct clinical entity with some phenotypic variations. Its diagnosis is usually from cutaneous, truncal, spinal, and foot anomalies in clinical and radioimaging studies. Proteus syndrome remains the major differential.

Malondialdehyde, lipoprotein-a, lipoprotein ratios, comprehensive lipid tetrad index and atherogenic index as surrogate markers for cardiovascular disease in patients with psoriasis: a case-control study.

Psoriasis is now recognized as an immune-mediated inflammatory dermatosis with increased risk for metabolic syndrome, its individual components, and cardiovascular disease. We quantitatively estimated malondialdehyde (MDA), lipoprotein-a (LP-a), lipoprotein ratios, comprehensive lipid tetrad index (CLTI), and atherogenic index (AI), and evaluated cardiovascular risk in 132 (M:F 94:38) patients with psoriasis aged 20-79 years with chronic plaque psoriasis and equal number of age and gender-matched controls. Lipoprotein ratios, CLTI and AI were calculated using standard formulae. Cardiovascular 10-year risk was graded by Framingham risk score (FRS) as low, intermediate and severe. Mild-to-moderate and severe psoriasis was present in 125 (94.7%), and 7 (5.3%) patients, respectively, and 19 (14.39%) patients had psoriatic arthritis. Statistically significant differences were noted for LDL, LDL/HDL, non-HDL/HDL, MDA, LP-a, AI and CLTI. There was a significantly positive correlation between PASI with LP-a (p = 0.003, r = 0.25) and AI (p = 0.012, r = 0.22). Serum levels of MDA correlated positively with LP-a (p < 0.001, r = 0.55), AI (p < 0.001, r = 0.51) and CLTI (p = 0.006, r = 0.24). FRS was low, intermediate and severe in 78%, 18.9%, and 3% patients compared to 85.6%, 13.6%, and 0.8% controls, respectively, and the difference was not statistically significant. Psoriasis appears to be an independent risk factor for elevated serum MDA, LP-a, CLTI and AI. However, whether they can be used as surrogate markers for enhanced cardiovascular risk in patients with psoriasis, remains conjectural.

Clinicoepidemiologic Features of Chronic Spontaneous Urticaria in Patients with Elevated Plasma D-Dimer Levels versus those without It: A Case-Control Cross-Sectional Study of 100 Indian Patients.

BACKGROUND: Activated coagulation cascade is implicated in urticaria pathogenesis marked by high plasma D-dimer, a marker of fibrinolysis, levels correlating with high urticaria activity score (UAS) and poor therapeutic outcome. METHODS: Quantitative plasma D-dimer levels and coagulation parameters in 100 (male:female ratio 1:3) Indian patients with chronic spontaneous urticaria and age- and gender-matched healthy controls were compared. The clinicoepidemiologic features of chronic urticaria were then compared among patients with normal (≤0.2 mg/L) and elevated (≥0.3 mg/L) plasma D-dimer levels. RESULTS: Plasma D-dimer in 23% patients and 4% controls and prothrombin time and activated partial thromboplastin time in 63% and 5% patients, respectively, were significantly higher compared with 58% and 1% of controls, respectively. About 18 of 72 (25%) patients with high UAS of ≥16-42 were compared with 5 of 28 (17.8%) patients with UAS7 of ≤15. Patients with elevated plasma D-dimer levels had significantly more systemic symptoms (86.9% vs. 81.8%) compared with patients with normal plasma D-dimer levels. CONCLUSION: A subset of patients with chronic urticaria have elevated plasma D-dimer levels and exhibit higher UAS7 and systemic symptoms that may influence long-term prognosis and therapeutic choices. Small number of patients, a cross-sectional nature of study, lack of treatment outcome measures, information on self-medication, and unavailability of specific parameters for coagulation pathway activation remain few limitations of this study.

Evaluation of Serum Vitamin D Levels in Patients with Systemic Sclerosis and Healthy Controls: Results of a Pilot Study.

BACKGROUND: The anti-inflammatory, immunomodulatory, and anti-proliferative effects of vitamin D in pathogenesis of autoimmune diseases have been highlighted in recent years but implications of vitamin D deficiency in systemic sclerosis (SSc) remain understudied. OBJECTIVES: To evaluate serum vitamin D levels in SSc patients and matched controls. MATERIALS AND METHODS: Serum vitamin D levels were estimated in 38 (M:F 5:33) patients aged 23-70 years of untreated SSc and age and gender matched healthy controls. Clinical and investigative evaluation for skin sclerosis by modified Rodnan skin score (mRSS), presence of digital ulcers, Raynaud's phenomenon, type of auto-antibodies, systemic involvement, and serum vitamin D levels were performed. Serum vitamin D levels were defined as normal (30-100 ng/ml), insufficient (10-30 ng/ml), and deficient (<10 ng/ml). RESULTS: Serum vitamin D levels (median ± IQR) were 19.5 ± 77.8 ng/ml in 38 patients and 100 ± 31.3 ng/ml in controls each. Vitamin D deficiency in 13 (34.2%) and insufficiency in 10 (26.3%) patients were identified. Only 2 (5.3%) controls had vitamin D insufficiency and the difference was statistically significant (P = 0.001). An inverse relationship was observed between mRSS and serum vitamin D levels. CONCLUSIONS: Patients with SSc have significantly lower serum vitamin D levels than healthy controls. Serum vitamin D levels do not correlate well with age, gender, disease duration or its variants, type of auto antibodies, presence of digital ulceration, or systemic involvement but has inverse correlation with skin sclerosis. Better-designed studies will perhaps resolve issues of potential benefits of vitamin D supplementation in modification of disease activity or severity in SSc.

Pesticide contact dermatitis in agricultural workers of Himachal Pradesh (India).

BACKGROUND: Common pesticides used in the region by agricultural workers may cause contact allergy. METHODS: Thirty agricultural workers with a history of pesticide exposure and dermatitis involving the face, neck, trunk or extremities, and 20 controls comprising 2 groups of 10 subjects each, group 1 with dermatitis and no exposure to pesticides, and group 2 with neither exposure to pesticides nor dermatitis, were patch tested with 10 pesticides commonly used in the region by use of the Finn Chamber method. RESULTS: The 30 patients, 20 of whom were male, aged 30-77 years, had dermatitis for 1 month to 18 years, with relapses and remissions. Seasonal exacerbation was present in 18 patients. Six patients attributed aggravation of their dermatitis to pesticide exposure, and 2 of these reacted positively to propiconazole. Positive patch test reactions to pesticides occurred in 10 patients, but not in controls. Thiuram was the commonest sensitizer (4 patients). Three patients were sensitized to propiconazole, and 2 patients reacted positively to metaldehyde. Formaldehyde, mercaptobenzothiazole, cypermethrin and isoproturon gave positive reactions in 1 patient each. CONCLUSION: The sensitizing potential of pesticides remains a concern. Apparently, pesticide contact dermatitis is more common than expected, but remains under-reported, as the implicated pesticides vary across regions and according to the crop patterns.

Mucocutaneous Manifestations in Patients with Chronic Kidney Disease: A Cross-sectional Study.

BACKGROUND: Chronic kidney disease (CKD)-associated mucocutaneous manifestations significantly impair the quality of life but often remain understudied. They may also vary across regions, socioeconomic and nutritional status, and racial differences. OBJECTIVES: To study the patterns of mucocutaneous disorders and their prevalence in CKD patients irrespective of clinical stage or dialysis status. MATERIALS AND METHODS: 122 (M:F = 77:45) patients aged 21‒85 (Mean ± SD = 57.5 ± 14.0) years having CKD for 3 month to 5 years were studied for mucocutaneous manifestations. Fifty (41%) patients were on hemodialysis for 1‒42 months. Detailed medical history, clinical and mucocutaneous examination, and lab investigations were performed. KOH mounts, skin biopsy, Gram's and Giemsa staining, bacterial or fungal cultures were performed as required. RESULTS: Xerosis in 93 (76.2%), skin pallor in 61 (50%), pruritus in 57 (46.7%), pigmentation in 47 (38.5%), and purpura in 18 (14.8%) patients were the major dermatoses. Bullous lesions and perforating folliculitis occurred in 3 (2.5%) patients each. Major nail abnormalities were pallor (in 35.2%), absent lunula (in 23.8%), nail discoloration (in 18%), and "half-and-half nails" in 16.4% patients, respectively. Hair abnormalities included sparse scalp and body hairs (in 35.2% and 13.1%, respectively) and lusterless hair in 12.3% patients. Coated tongue (in 14.8%), xerostomia (in 12.3%), and macroglossia with teeth indention (in 7.4%) patients were the mucosal manifestations. CONCLUSIONS: Xerosis, pruritus, skin pallor/pigmentary changes, nail pallor, absent lunula, nail discoloration, sparse hairs, coated tongue, xerostomia, macroglossia, and infections were the most common mucocutaneous manifestations in the studied patients irrespective of hemodialysis status. Cold and dry climates might be additional aggravators for xerosis/pruritus. Lifelong follow-up may be needed to reduce the morbidity associated with CKD/hemodialysis specific dermatoses appearing over a period.