ABSTRACT
Background Autoimmune pemphigus is a potentially life threatening bullous disease. The cornerstone of treatment is systemic corticosteroids. However, adjuvant therapy with immunosuppressant drugs is commonly used to improve disease control and alleviate the high morbidity and mortality associated with the use of corticosteroids. Adjunctive treatment with pulse intravenous cyclophosphamide may be more efficacious and less toxic than other immunosuppressants. Objective To retrospectively review the clinical outcome of 18 patients with recalcitrant pemphigus who were treated with cyclophosphamide over the past 10 years. Methodology A retrospective study was conducted between 1985 and 2009 in thirteen Malaysian dermatology centres. Data collected were analysed for comparison of relapse rates, compliance rates and adverse drug effects between the 2 regimes. Results Eighteen patients were included in this audit of which 12 patients had pemphigus vulgaris and 6 patients had pemphigus foliaceous. Prior to treatment with cyclophosphamide, fourteen patients were on azathioprine, three were given intravenous immunoglobulin, and two were prescribed dapsone; however all these patients were either unresponsive, intolerant or suffered serious side-effects with these drugs. Subsequently, 7 patients (median age: 31 years) received a combination of pulse intravenous cyclophosphamide and either intravenous dexamethasone or methylprednisolone. These seven patients received between 2 to 21 pulses of intravenous cyclophosphamide and steroids at monthly intervals with oral prednisolone and cyclophosphamide (50-100mg) in between pulses. The remaining 11 patients (median age: 46 years) received oral cyclophosphamide and corticosteroids. Of the 18 patients in our cohort, 15 achieved control and consolidation of disease activity at an average of 4 weeks and 10 weeks respectively. The remaining three patients are yet to achieve disease control. The total duration of treatment with cyclophosphamide ranged from 2 to 62 months with a cumulative dose ranging from 2.95g to 93.55g. Four patients achieved partial remission on minimal therapy and 3 achieved complete remission. None of patients experienced serious side effects. Conclusion Cyclophosphamide may be an alternative treatment option in patients in patients with pemphigus who fail to respond to standard therapy. Controlled trials are needed to further evaluate the efficacy and safety of this therapy.
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Background Antibiotic therapy directed against Propionibacterium acnes (P. acnes) has been a mainstay of treatment in acne vulgaris for more than 40 years. Prolonged antibiotic usage has been associated with emergence of antibiotic-resistant P. acnes and is linked to treatment failure. Little work has been done in Malaysia on drug resistance in P. acnes and there is no surveillance data on this aspect to guide the clinical decision. Objective This study aims to evaluate antibiotic sensitivity of P. acnes isolated from patients with acne vulgaris in Kuala Lumpur Hospital, Malaysia. Methods This is a non interventional, single centered, cross-sectional hospital-based survey of antibiotic sensitivity of P. acnes isolated from patients with acne vulgaris in Kuala Lumpur Hospital from January 2010 to June 2010. Results A total of 100 patients were recruited in our study. P. acnes was isolated in 53% of patients and 11% had gram negative organism. Antibiotic resistant P. acnes was found in 15.1% of positive isolates. Clindamycin resistance was the highest (15.1%) followed by erythromycin (7.5%), doxycycline (5.7%), tetracycline (1.9%) and minocycline (0%). Isolates of antibiotic resistant P. acnes was significantly higher in patients treated with antibiotics within the last 6 months (29%) as compared with non antibiotic treated patients (0%) (p<0.05).The mean duration of prior antibiotic treatment was significantly longer in the group of antibiotic resistant P. acnes as compared with antibiotic sensitive P. acnes (17.13 weeks vs 5.74 weeks, p<0.05). Conclusion Antibiotic resistant P. acnes is present locally with clindamycin and erythromycin accounting for the highest resistance. Longer duration of antibiotic treatment predisposes to antibiotic resistant P. acnes and may also induce emergence of gram negative organisms. Strategies to reduce antibiotic resistance should be emphasized when prescribing antibiotic for acne vulgaris in order to achieve optimal therapeutic results while reducing the potential for antibiotic resistance.
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Primary cutaneous anaplastic large cell lymphoma (c-ALCL) is an uncommon type of cutaneous T cell lymphoma currently classified as one of the CD30+ lymphoproliferative disorders of the skin under the WHO-EORTC classification1. We describe a series of three patients with c-ALCL from 2005-2009 in the Department of Dermatology, Hospital Kuala Lumpur.
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Introduction: Stevens Johnson syndrome (SJS), Stevens Johnson Syndrome - toxic epidermal necrolysis overlap syndrome (SJS-TEN overlap), toxic epidermal necrolysis (TEN) and drug-induced hypersensitivity syndrome (DHS) are well known severe adverse cutaneous drug reactions (SACDRs). All clinicians are responsible for the diagnosis and management of SACDR. Objective: To retrospectively review the clinical patterns, management strategies and outcome of 134 patients with severe adverse cutaneous drug reactions managed at the Department of Dermatology, Kuala Lumpur Hospital between 2006 and 2010. Results: The mean age of presentation was 44.8 years (13-83). The male: female ratio was 1:1. There were 68 cases (50.7%) of SJS, 10 cases (7.5%) SJS-TEN overlap, 32 cases (23.9%) TEN and 24 cases (17.9%) DHS. The five commonest drugs associated with SACDRs were allopurinol (26.9%), carbamazepine (13.4%), phenytoin (9.7%), non-steroidal anti-inflammatory drugs (11.2%) and co-trimoxazole (7.5%). The mean duration of drug exposure before the onset of reaction was 2.8 weeks. A hundred and thirty patients (97%) were managed as in-patient. The mean duration of in-patient stay was 12.4 days. All identified culprit drugs were withheld. Systemic corticosteroids was given to 96% cases of DHS with mean duration of 9.7 weeks; 52.9% of SJS with mean duration of 2.8 weeks; 60% of SJS-TEN overlap with mean duration of 2.3 weeks; and 62.5% of TEN with mean duration of 3.3 weeks. Thirteen patients (42%) with TEN were treated with intravenous immunoglobulin. Eight patients (6%) died, of which 7 were TEN and one DHS. Conclusion: SACDRs are life-threatening emergencies which not only results in significant morbidity and mortality; but also potentially increases the health care cost and burden. Clinicians should recognize high risk medications and prescribe them with great caution.
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Wegener’s granulomatosis is a rare multisystem necrotizing granulomatous vasculitis aff e c t i n g small - and medium-sized vessels. Its clinical manifestations can be nonspecific during the initial stages and indistinguishable from a variety of neoplastic, infectious, and inflammatory diseases. The disease may run a course from indolence to one of rapid progression leading to life-threatening multiorgan failure. We report a rare case of rapidly progressing Wegener’s granulomatosis.
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Pemphigus foliaceous (PF) is an autoimmune blistering disease resulting from acquired immunoglobulin G autoantibodies against desmoglein 1 of the skin, which is one of the adhesion molecules of keratinocytes. Clinically patients with PF develop crusted and scaly erosions mainly over the seborrhoeic distribution i.e. the face, scalp and upper trunk. Mild cases of PF may be localized but in some cases it may progress to erythrodermic exfoliative dermatitis. There is however no mucosal involvement in PF in contrast to pemphigus vulgaris and paraneoplastic pemphigus. Light microscopy of lesional biopsy shows subcorneal acantholysis. Direct immunofluorescence study of perilesional skin reveals presence of intraepithelial intercellular deposit of IgG and C3. We describe 2 cases of PF in the presence of thymoma, a relatively rare association, which could further support the fact of thymoma associated autoimmune disease.
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Herpes simplex virus (HSV) infection is one of the common opportunistic viral infections that may occur in human immunodeficiency virus (HIV) - infected patients. The natural history of HSV infection is often altered in this group of patients. Characteristically, genital herpes presents with multiple painful vesicles and erosions in immunocompetent patients. However, clinical presentations in immunocompromised patients are frequently severe and atypical which may lead to a delay in diagnosis and treatment. Genital herpes enhances transmission of HIV infection and hence early detection of this condition is important to reduce transmission of HIV and HSV.
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Background Atopic eczema is a common dermatological condition seen in our practice in which the mainstay of treatment is topical medications. One of the main reasons for poor clinical response to therapy in atopic eczema is the lack of understanding of topical preparation usage and thus poor adherence to treatment. Objectives The aim of this study is to determine the effect of explanation and demonstration of topical medication on the clinical response of atopic eczema. Methodology Twenty newly diagnosed patients with atopic eczema who fulfilled the study criteria were recruited and randomized consecutively into 2 groups - A & B. All patients were assessed on the severity of the eczema using the six area, six sign atopic dermatitis severity score (SASSAD) and patients’ assessment of itch, sleep disturbance and irritability were recorded on 10-cm visual analogue scales. They were also assessed on their level of understanding on the proper usage of topical medications using a questionnaire. Group A then received explanation and demonstration on how to apply the topical medications while Group B was not educated on these. They were followed up 2 weeks after treatment and were re-evaluated on their understanding and the severity of their skin condition. This was followed by education by a dermatology nurse on the proper usage of topical medications for both groups. A third evaluation was done 2 weeks later. Results At baseline, 70% of the patients did not understand the potency of topical corticosteroid and between 20-30% of them did not know the correct sites, frequency, time and duration of each topical application prescribed. About two thirds of the patients claimed that they did not receive any explanation or demonstration from either their doctors or the pharmacy dispensers. After education on the proper usage of topical medications, the level of understanding improved to 100% for group A at visit 2 and group B at visit 3. A clinical improvement as measured by SASSAD score reduction was seen in both groups. In group A, a significant SASSAD score reduction of 49.5% (P=0.003) was seen after 2 weeks and it was sustainable, as evidenced by a further reduction to 67% (p=0.001) by week 4. In group B, a significant SASSAD score reduction (64.8%; p=0.002) was seen only at week 4 after patient education and demonstration. The magnitude of improvement in patients’ symptoms which included itch, sleep disturbance and irritability, measured by the patient using visual analog score, were only significant for group A after 4 weeks. Conclusions This study reinforces the importance of explanation and demonstration on the proper usage of topical medications in achieving better clinical response. Failure to explain on the use of topical medications may lead to patient dissatisfaction, poor compliance and lack of treatment efficacy.
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Background Autoimmune bullous diseases (ABD) represent a group of chronic blistering dermatoses in which management is often challenging. Epidemiologic data on these diseases in Malaysia has been limited. Objectives Our purpose was to study the spectrum of the various ABD presented to the Department of Dermatology, Ipoh Hospital, and to determine the clinico-epidemiological pattern of the 2 main ABD, namely pemphigus and bullous pemphigoid. Methodology We performed a retrospective review of records for all patients who were diagnosed with ABD confirmed by histopathology and direct immunofluorescence test in this centre between 2001 and 2005. The data were analyzed with regard to age, sex, ethnicity, subtypes of ABD, treatment provided and outcome. Results There were a total of 79 cases of ABD presented to us during this period. Bullous pemphigoid was observed to be the commonest (60.8%) followed by the pemphigus group (36.7%) with the mean incidence of 0.45/100,000/year and 0.28/100,000/year respectively.44% of patients were of ethnic Chinese origin. There was an overall female preponderance. The mean age of presentation was 65.5 years for bullous pemphigoid and 55 years for pemphigus group. The mean duration of disease before presentation was 1.6 months for bullous pemphigoid and 6.3 months for pemphigus. Various combinations of immunosuppressive agents were used to treat the patients. 48% of bullous pemphigoid cases were controlled with prednisolone alone while 67.9% of pemphigus group required at least 2 immunosuppressive agents to achieve disease control. Conclusion In our study population, bullous pemphigoid was more frequently seen than pemphigus.