A retrospective review of the therapeutic response with remission in patients with newly diagnosed bullous pemphigoid.

We reviewed the clinical characteristics and therapeutic response in cases of newly diagnosed bullous pemphigoid at the National Skin Centre between June 2009 and December 2010. Most (76%, n = 68/90) achieved clinical remission within 6 months of commencement of therapy. Oral mucosal involvement was identified as a risk factor associated with a prolonged duration of treatment beyond 6 months.

A ten-year retrospective study on livedo vasculopathy in Asian patients.

INTRODUCTION: This study aims to analyse the clinico-epidemiological characteristics of Asian patients diagnosed with livedo vasculopathy (LV). MATERIALS AND METHODS: We performed a retrospective analysis of all patients diagnosed with LV from 1997 to 2007 at our centre. RESULTS: Seventy patients were diagnosed with LV with a mean age of 39 years, female: male ratio of 3:1 and no racial predilection. Most cases remained purely cutaneous, presenting with painful leg ulcers and atrophie blanche. Peripheral neuropathy was the only extra-cutaneous complication (9%). In patients who were screened, associations included hepatitis B (7%) and hepatitis C (4%), positive anti-nuclear antibody (14%), positive anti-myeloperoxidase antibody (5%), positive anti-cardiolipin antibodies (7%) and positive lupus anticoagulant (2%). In 49 patients who achieved remission, 55% required combination therapy, most commonly with colchicine, pentoxifylline and prednisolone. In those treated successfully with monotherapy, colchicine was effective in 59% followed by prednisolone (17.5%), pentoxifylline (17.5%) and aspirin (6%). Mean follow-up period was 50 months. CONCLUSION: LV in Asian patients is a high morbidity, chronic relapsing ulcerative skin condition. Most patients require induction combination therapy for remission. As further evidence emerges to support a procoagulant pathogenesis, a standardised protocol is needed to investigate for prothrombotic disorders during diagnosis.

Improving adherence to safe prescription guidelines for Dapsone: harnessing an enhanced electronic medical records system and a team approach.

BACKGROUND: Dapsone is a commonly prescribed medication in dermatological practice. Its use is associated with a broad spectrum of adverse effects. Careful selection and monitoring of patients on dapsone are paramount in the prevention and early recognition of adverse effects. OBJECTIVE AND METHODS: We designed a risk-management program for dapsone at National Skin Centre, Singapore, enhancing an existing electronic medical records system and harnessing a team approach involving the nurses. This includes the performance of key laboratory tests before and after starting dapsone, ensuring adequate counseling before starting dapsone and screening for adverse effects using a questionnaire every visit. RESULTS: This system of dapsone prescription efficiently improved the adherence to safe prescription and monitoring guidelines. Average adherence rates for key safety parameters improved from 61.4% pre-implementation to 95.3% at six months and were sustained at 12 months at 91.3%. Percentage of follow-up cases in which all three key monitoring parameters were fulfilled increased from 9.5% to 79.6% (p=0.0001) after 12months. The percentage of new patients in which all four key monitoring parameters were met increased from 50% to 80%. It was not statistically significant possibly because of small patient numbers. This project has also translated into enhanced patient safety with dapsone dosages adjusted in 17 patients who experienced mild adverse effects. No severe adverse effects to dapsone were observed in the 12-month period. CONCLUSION: This example of risk management for dapsone may serve as a model for institutions looking at harnessing information technology and a team approach for safer prescription of high-alert medications.

Fas-ligand staining in non-drug- and drug-induced maculopapular rashes.

BACKGROUND: Morphologically and histopathologically, drug- and non-drug-induced maculopapular rashes can be almost indistinguishable. It has been postulated that Fas-ligand (Fas-L) is involved in the pathogenesis of drug rashes but not in the genesis of rashes, such as viral exanthems, that are not induced by medications. AIM: This study sought to determine if epidermal Fas-L is a distinguishing feature in the pathology of drug and non-drug maculopapular rashes. METHODS: Archived skin biopsies of patients with a confirmed diagnosis of drug or non-drug maculopapular rashes (n = 10 each) and positive and negative controls were retrieved for immunohistochemical staining for Fas-L. The proportion of Fas-L-positive skin biopsies were compared. The presence of tissue eosinophilia was also evaluated. RESULTS: Ten percent of non-drug-induced rashes were Fas-L positive compared to 50% of drug rashes (p = 0.05). Twenty percent of non-drug exanthems had moderate tissue eosinophilia, while 60% from drug rashes had moderate to dense tissue eosinophilia (p = 0.17). CONCLUSION: There is a trend toward Fas-L being more prevalent in the epidermis of drug maculopapular rashes, although this did not reach statistical significance. This is possibly because of the small sample size.

An 11-year review of dermatomyositis in Asian patients.

INTRODUCTION: Dermatomyositis (DM) is a multisystem inflammatory disease with a strong association with malignancy. We aimed to describe a series of Asian patients with DM and identify any significant clinical factors associated with malignancy. MATERIALS AND METHODS: This was a retrospective review of a multi-racial cohort of 69 Asian patients diagnosed with DM over an 11-year period from 1996 to 2006. RESULTS: Malignancy was detected in 15 out of 68 patients (22%), the most common of which was nasopharyngeal carcinoma (7 cases). Compared to the non-malignancy group, the malignancy-associated group was older and had more male patients. There were no statistically significant clinical, serological or laboratory factors associated with a higher risk of malignancy. CONCLUSION: This study highlights the importance of ongoing malignancy screening especially for nasopharyngeal carcinoma in Asian patients with DM.

Delayed hypersensitivity reaction after intravenous glucagon administered for a barium enema: a case report.

INTRODUCTION: Few reports have documented allergic hypersensitivity reactions after barium gastrointestinal studies. Of these, the barium suspension, its additives or intravenous glucagon given for bowel relaxation has been implicated as possible allergens. We report a patient with delayed hypersensitivity reaction after barium enema and discuss the reasons supporting glucagon as the possible allergen. CLINICAL PICTURE: A 74-year-old Chinese woman presented with pruritic rashes, 1 day after a barium enema. Intravenous glucagon (GlucaGen, Novo Nordisk, Denmark) was administered during the barium enema. Physical examination revealed palpable purpuric rashes on the legs with erythematous papules and plaques on the arms and trunk. Skin biopsy demonstrated superficial perivascular infiltrates of lymphocytes and eosinophils, consistent with a drug eruption. TREATMENT AND OUTCOME: The rashes resolved with antihistamines and topical corticosteroids. CONCLUSION: This report highlights the potential of glucagon to cause hypersensitivity reactions. Awareness of this entity is important for the prevention and recognition of complications during barium gastrointestinal studies.

Dermatofibrosarcoma protuberans: a clinicopathological analysis of 10 cases in Asians.

Dermatofibrosarcoma protuberans (DFSP) is a locally aggressive cutaneous neoplasm that exhibits a marked tendency for recurrence after local excision. This case series aims to study the clinical, histological and immunohistochemical features of DFSP in Asians. Ten patients with DFSP diagnosed between 1992 and 2001 were reviewed. There were more women than men in a ratio of 4:1. There were six Chinese, two Malays, one Indian and one Eurasian. The mean age was 38 years. The duration of each lesion before excision varied from 6 months to 27 years. Fifty per cent of tumours occurred on the trunk. On histology, all the lesions were dermal-centred spindle cell tumours, extending to the subcutis, and exhibited the characteristic storiform pattern. One tumour also demonstrated fibrosarcomatous changes. Two tumours were of the rare pigmented variant (Bednar tumour). Immunohistochemistry with CD34 was positive in all cases, except the fibrosarcomatous area of one tumour, which was negative for CD34. For comparison, six cases of deep-penetrating dermatofibroma were stained for CD34 and all showed an absence of CD34 expression. Wide excision of the tumour was performed in all cases of DFSP. There was no recurrence after mean follow up of 6 years (range 2.25-9.5 years).