Prevalence and Types of Mucocutaneous Disorders, Their Correlation to CD4 Count and Their Impact on Quality of Life in Adults with HIV Infection

Background@#Data on prevalence and type of mucocutaneous diseases in HIV-positive patients and their impact on quality of life (QoL) are sparse. We aim to determine prevalence and type of mucocutaneous disorders, their correlation to CD4+ counts and impact on QoL for adults with HIV, using the Dermatology Life Quality Index (DLQI).@*Methods@#A cross-sectional study of HIV-infected adults seen in HIV and Dermatology Clinic.@*Results@#The majority (90%) of 174 participants recruited was male. Median age at diagnosis of HIV infection was 29 years (IQR 10). Mucocutaneous disorders were present in 90.2%, out of which 58.6% had two or more mucocutaneous disorders. Mean CD4+ count was significantly lower in patients with, compared to those without mucocutaneous disorders (363 vs 548 cells/µL; p=0.030). Infections accounted for 67.2% of all mucocutaneous disorders seen, followed by inflammatory dermatoses (51.7%), cutaneous adverse drug reactions (17.8%) and neoplasm (2.3%). The five most frequent manifestations were eczema (22.4%), anogenital warts (21.2%), candidiasis (16.7%), dermatophytosis (15.5%) and secondary syphilis (12.0%). Oral candidiasis, pruritic papular eruption, drug-induced maculopapular eruption and drug rash with eosinophilia and systemic symptoms were significantly more prevalent in patients with CD4+ counts <200 cells/µL but anogenital warts were more prevalent in patients with CD4+ counts ≥200 cells/µL. The mean DLQI score was 8.39 (SD ± 6.83). QoL was severely impaired (DLQI >10) in 34.4%.@*Conclusion@#Mucocutaneous disorders were common in HIV patients causing significant impairment in quality of life. Prevalence co-related with low CD4+ counts. Adequate management of HIV may reduce the prevalence of mucocutaneous disorders and improve QoL.

Validation of the Malay version of Autoimmune Bullous Disease Quality of Life (ABQOL) questionnaire

@#Autoimmune blistering diseases (AIBD) represent a group of rare and chronic disorders with significant impact on quality of life (QoL). The aim of this study was to assess the validity and reliability of the Malay translation of the autoimmune bullous disease quality of life (ABQOL) questionnaire.

Consensus Guidelines in Usage of Biologics in Dermatology during COVID-19 Pandemic: Biologic Advisory Group Malaysia

@#The aim of this Biologic Advisory Group (BAG) Malaysia consensus guideline is to provide clinicians managing cutaneous diseases with biologics relevant parameters to consider prior to initiating or stopping or continuing any biologic treatment in the current landscape of the COVID-19 pandemic. Besides reviewing the medical literatures on COVID-19 and evidences related to other human coronavirus or influenza, expert opinions and clinical experiences are shared and debated in formulation of this biologic consensus guideline.

Serum 25-Hydroxyvitamin D deficiency in Malaysian children with severe atopic dermatitis

@#Background: Vitamin D deficiency has been shown to be a determinant of disease severity in patients with atopic dermatitis (AD). There is a lack of information on the prevalence of vitamin D deficiency in Malaysian children with AD. The objective of this study was to determine the association of vitamin D deficiency with AD severity, to compare vitamin D deficiency between children with and without AD and to determine prevalence of vitamin D deficiency in children with AD. Methods: A case-control study to examine serum 25- hydroxyvitamin D [25(OH)D] levels in children with and without AD was done. Serum 25-hydroxyvitamin D [25(OH)D] level was measured by immunoassay. AD severity was evaluated using the SCORing Atopic Dermatitis (SCORAD) index. Results: The serum levels of 25(OH)D, measured in 135 children with AD was not statistically different from 65 children without AD [median (IQR): 25.2ng/mL (15.45) vs 25.9ng/mL (15.87), p=0.616]. However, serum vitamin D levels were significantly lower in children with severe AD compared to those with mild-to-moderate AD [median (IQR): 16.0ng/mL (19.32) vs 26.3ng/mL (15.56), p=0.021]. The odds of having vitamin D deficiency in children with severe AD was 3.82 times that of children with non-severe AD (95% confidence level: 1.13, 12.87). Conclusion: This study suggests that there is an inverse association between vitamin D level and the severity of AD in Malaysian children.

Clinical characteristics, culprit drugs and outcome of patients with Acute Generalised Exanthematous Pustulosis seen in Hospital Sultanah Aminah, Johor Bahru

Background: Acute generalised exanthematous pustulosis(AGEP) is a rare, cutaneous reaction characterised bysudden onset of numerous, non-follicular, sterile pustuleson oedematous erythematous skin, accompanied by feverand neutrophilia. AGEP is predominantly drug-induced. Skinlesions appear rapidly within 1-3 days of drug exposure andupon drug withdrawal, resolve rapidly within 15 days.Objective: To determine the clinical characteristics, culpritdrugs and outcome of patients with AGEP.Methods: A retrospective note review of all AGEP patientsseen from 2001-2015.Results: Among 21 AGEP patients, 76% were Malays, 9.5%Chinese, 9.5% Indians, and 5% Iban. Sixteen were femalesand 5 were males. Median age of patients was 40 years (IQR:26). The main culprit drug was amoxicillin (10 cases),followed by cloxacillin (three cases), phenytoin (two cases)and one case each of carbamazepine, sulphasalazine,allopurinol, cephalexin, ceftriaxone, celecoxib and herbalproduct. The median time from drug initiation to onset ofAGEP was 3 days (IQR: 5.5). Fever was documented in 52.4%, mucosal involvement 9.5%, purpura 4.7% and blisters4.7%. Neutrophilia was observed in 63.6% of patients andeosinophilia in 28.5%. While most patients requiredadmission (67%), all achieved complete recovery within 15days without any sequela.

A Case Report of Acne Agminata

Granulomatous facial skin lesions are a rare and challenging clinical problem. Differential diagnoses include cutaneous tuberculosis, sarcoidosis, granulomatous rosacea and acne agnimata. We reported a case of acne agminata presented with granulamatous facial papules.

Clinical features and prognostic factors of cutaneous vasculitis among dermatology patients in Johor Bahru, Malaysia

Background: Cutaneous vasculitis is common, yet the riskfactors for its chronicity have not been established.Objective: To describe the clinical spectrum and identify riskfactors for chronicity of cutaneous vasculitis.Methods: Retrospective data analysis of 275 patientsdiagnosed with cutaneous vasculitis from January 2008 toDecember 2013.Results: The mean age was 33.7 (±17.89) years, with femalepredominance. The majority of patients were Malays (67.3%).Skin biopsy was performed in 110 (40%) patients. Thecommonest sign was palpable purpura (30.6%). Theaetiology remained elusive in 51.3% of patients. Commonidentifiable causes include infection (19.7%) and connectivetissue disease (10.2%). Extracutaneous features were notedin 46.5% of patients. Erythrocyte sedimentation rate andantinuclear antibody were raised in 124 of 170 and 27 of 175patients with documented results respectively. Cutaneousvasculitis was the presenting symptom in seven patientswith newly diagnosed systemic lupus erythematosus. AntiStreptolysin O Titre was positive in 82 of 156 patients withdocumented results. Despite antibiotics, 31.7% of them hadchronic lesions. Prednisolone alone was used in 20% ofpatients while 16.4% needed steroid-sparing agents. Mostpatients who needed systemic therapy (62%) hadunidentifiable aetiology. Among the 155 patients whoremained under follow up, 36.4% had chronic disease, onepatient succumbed due to septicaemia, and the rest fullyrecovered within three months. The presence of ulcerativelesion was significantly associated with developing chronicvasculitis (p=0.003).Conclusion: The clinical spectrum of cutaneous vasculitis inour population was similar to other studies. Ulcerativelesion predicts a chronic outcome

Generalized Pustular Psoriasis

Generalized Pustular Psoriasis (GPP) is a dermatological emergency that often requires hospitalizationbecause of possible life-threatening complications, including heart failure, renal failure and sepsis. Itis a chronic recalcitrant disease in which acute pustular flares frequently recur on exposure to classictriggers. This review article is aimed to update the new insights into the genetic basis of GPP andhighlighted the central role IL1 and IL36 in the pathogenesis of GPP.

Angiosarcoma Mimicking Rhinophymatous Rosacea

Cutaneous angiosarcoma is a rare, highly malignant vascular tumor. More than 50% of them arelocalized to the skin of the head and neck regions.It usually present as nodules with ulceration, plaques,or bruise-like lesions. However, the clinical features may vary. We reported this case due to its atypicalclinical presentation, which presented with rhinophyma-like features, making it a diagnostic challengeto the clinicians.

Incidence of cutaneous adverse drug reactions among medical inpatients of Sultanah Aminah Hospital Johor Bahru

Introduction: Cutaneous adverse drug reactions (cADRs) are common. There are only few studies on the incidence of cADRs in Malaysia. Objective: To determine the incidence, clinical features and risk factors of cADRs among hospitalized patients. Methods:A prospective study was conducted among medical inpatients from July to December 2014. Results: A total of 43 cADRs were seen among 11 017 inpatients, yielding an incidence rate of 0.4%. cADR accounted for hospitalization in 26 patients. Previous history of cADR was present in 14 patients, with 50% exposed to the same drug taken previously. Potentially lifethreatening severe cutaneous adverse reactions (SCAR), namely drug reaction with eosinophilia and systemic symptoms (DRESS: 14 cases) and Stevens-Johnson Syndrome/Toxic Epidermal Necrolysis (SJS/TEN: 6 cases) comprise almost 50% of cADRs. The commonest culprit drug group was antibiotics (37.2%), followed by anticonvulsants (18.6%). Cotrimoxazole, phenytoin and rifampicin were the main causative drugs for DRESS. Anticonvulsants were most frequently implicated in SJS/TEN (66.7%). Most cases had “probable” causality relationship with suspected drug (69.8%). The majority of cases were of moderate severity (65.1%), while 18.6% had severe reaction with 1 death recorded. Most cases were not preventable (76.7%). Older age (> 60 years) and mucosal involvement were significantly associated with a more severe reaction. Conclusion: The incidence of cADRs was 0.4%, with most cases classified as moderate severity and not preventable. The commonest reaction pattern was DRESS, while the main culprit drug group was antibiotics. Older age and mucosal membrane involvement predicts a severe drug reaction.

The MARCOPOLO Study of Ustekinumab Utilization and Efficacy in a Real-World Setting: Treatment of Patients with Plaque Psoriasis in Asia-Pacific Countries

BACKGROUND: Ustekinumab is a fully human monoclonal antibody approved for the treatment of chronic moderate-to-severe plaque psoriasis in adults. However, factors including efficacy, tolerability, ease of use, and cost burden may affect ustekinumab utilization. Noncompliance may, in turn, affect treatment response. OBJECTIVE: To evaluate ustekinumab utilization in the real-world setting in Asia-Pacific countries. METHODS: In this phase 4 observational study conducted in Indonesia, Malaysia, Singapore, Korea, and Taiwan, adults with plaque psoriasis receiving ustekinumab were followed for up to 52 weeks. Study endpoints were the proportion of all patients using ustekinumab according to label-recommended intervals and the proportion of Korean patients who achieved a psoriasis area severity index 75 response at week 16. Safety was assessed by monitoring adverse events. RESULTS: Overall, 169 patients received ustekinumab (Korea, n=102; other countries, n=67). Just over half (56.2%) of patients used ustekinumab with the label-recommended interval from baseline to week 40; the proportion was higher in Korea (73.5%) than in other countries (29.9%), probably because ustekinumab was provided without charge for Korean patients up to week 40. Noncompliance increased after week 40 in Korea and from week 28 in other Asia-Pacific countries, with cost cited as the most common reason. At week 16, 56.9% of Korean patients achieved a Psoriasis Area Severity Index 75 response. Safety results were in line with those seen in previous studies. CONCLUSION: More than half of all patients in Asia-Pacific countries used ustekinumab as per the label-recommended dose interval, but reimbursement variations between countries may have confounded overall results.

The MARCOPOLO Study of Ustekinumab Utilization and Efficacy in a Real-World Setting: Treatment of Patients with Plaque Psoriasis in Asia-Pacific Countries

BACKGROUND: Ustekinumab is a fully human monoclonal antibody approved for the treatment of chronic moderate-to-severe plaque psoriasis in adults. However, factors including efficacy, tolerability, ease of use, and cost burden may affect ustekinumab utilization. Noncompliance may, in turn, affect treatment response. OBJECTIVE: To evaluate ustekinumab utilization in the real-world setting in Asia-Pacific countries. METHODS: In this phase 4 observational study conducted in Indonesia, Malaysia, Singapore, Korea, and Taiwan, adults with plaque psoriasis receiving ustekinumab were followed for up to 52 weeks. Study endpoints were the proportion of all patients using ustekinumab according to label-recommended intervals and the proportion of Korean patients who achieved a psoriasis area severity index 75 response at week 16. Safety was assessed by monitoring adverse events. RESULTS: Overall, 169 patients received ustekinumab (Korea, n=102; other countries, n=67). Just over half (56.2%) of patients used ustekinumab with the label-recommended interval from baseline to week 40; the proportion was higher in Korea (73.5%) than in other countries (29.9%), probably because ustekinumab was provided without charge for Korean patients up to week 40. Noncompliance increased after week 40 in Korea and from week 28 in other Asia-Pacific countries, with cost cited as the most common reason. At week 16, 56.9% of Korean patients achieved a Psoriasis Area Severity Index 75 response. Safety results were in line with those seen in previous studies. CONCLUSION: More than half of all patients in Asia-Pacific countries used ustekinumab as per the label-recommended dose interval, but reimbursement variations between countries may have confounded overall results.

Clinico-epidemiological profile, including body mass index of Malaysian children with psoriasis

background: Limited information exists regarding paediatric psoriasis and its association with body mass index (bMI) in Asia. Objectives: to determine the clinico-epidemiological profile and to compare the bMI of children with and without psoriasis. Methods: A case-control study of 92 children with psoriasis versus 59 with atopic eczema and 56 with non-inflammatory skin conditions. results: Psoriasis was more common in Malay and Indian children when compared to Chinese with odds ratios (Or) of 4.30 (95% CI, 1.85-9.99) and 3.00 (95% CI, 1.02-8.81) respectively. Prevalence of psoriasis was similar between Malay and Indian children (Or 1.43, 95% CI, 0.63-3.25). Male:female ratio was 1:1.09. the mean onset age of psoriasis was 7.9 years. Median onset age was earlier in males (6.5 years versus 9.0 years in females, p=0.05). Plaque psoriasis was the most common phenotype (89.1%) and 94.5% had scalp lesions. Arthritis was seen in 4.3%. Odds of excess adiposity, defined as bMI ≥85th percentile, was higher in children with psoriasis versus noninflammatory controls (Or 2.35, 95% CI 0.99-5.56, p= 0.052). No increased risk of adiposity was noted between children with psoriasis and eczema (Or 1.14, 95% CI 0.5-2.62, p=0.753). More children with psoriasis (17.4%) and eczema (20.3%) were underweight (bMI <5th percentile) compared to non-inflammatory controls (10.7%). Conclusion: Malays and Indians are three to four times more likely than Chinese to have psoriasis in multi-ethnic Malaysia. Plaque psoriasis is the most common phenotype. Odds of excess adiposity is about two times higher in children with psoriasis compared to non-inflammatory controls although this observation just missed conventional statistical significance.

Extramammary Paget's disease: a report of 2 cases and a review of the literature

Extramammary Paget's disease (EMPD) is a rare disorder and may be found in the vulva, scrotum, penile area, perianal region and the groin. Frequently, it is associated with an underlying regional neoplasm or internal malignancy. We report 2 cases of EMPD; one involving the scrotal area and the other the vulva. Both were elderly patients who presented to the dermatologists with chronic eczematous lesions in the perineum that did not respond to topical treatment. Skin biopsies confirmed extramammary Paget's disease. Investigations for internal malignancies were negative. However, one of the patients defaulted treatment before surgery. The other patient had two excision surgeries with skin grafting to try to achieve tumour free margins. A long term follow-up was planned for him to look for recurrences. These cases emphasise that EMPD can mimic exudative dermatitis and present as a chronic non-healing lesion in the perineum for many years. Clinicians should have a high index of suspicion to pick up the disease early by biopsy. Various immunohistochemical markers not only can help differentiate other histological diagnoses but also help predict the presence of underlying malignancies. Management of EMPD included thorough search for occult or underlying malignancy followed by complete excision surgery with intraoperative frozen sections. Even then, recurrences are high for this disease and long term follow-up is advocated.