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.

Oral aphthosis: management gaps and recent advances.

INTRODUCTION: Though oral aphthosis is common, it has a significant impact on the quality of life in the patients. It is the most common oral ulcerative condition encountered in clinical practice. This study describes the characteristics and patterns of oral aphthosis seen at a tertiary dermatological centre in Singapore, with emphasis in evaluating the management gaps and in identifying underlying systemic diseases and nutritional deficiencies. MATERIALS AND METHODS: This is a retrospective review of medical records over a 10-year period between June 2000 and June 2010. Two hundred and thirteen patients were identified using the search terms 'oral ulcers', 'aphthous ulcers', 'oral aphthosis', and 'Behcet's disease'. Patients with Behcet's disease without oral ulcers and other diagnoses such as pemphigus vulgaris, lichen planus and herpes simplex were excluded. The remaining patients were evaluated with regard to demographic characteristics, characteristics of oral ulcers, associated connective tissue disorders and nutritional deficiencies, diagnostic tests results, treatment response as well as follow-up duration. RESULTS: One hundred and seventy-fi ve patients were included in this study. One hundred and one patients had recurrent oral aphthosis, with 77 having simple aphthosis and 24 having complex aphthosis. Fourteen patients (8%) fulfilled the International Study Criteria (ISG) for Behcet's disease, of which, 85.71% had complex aphthosis. The therapeutic ladder for such patients ranged from topical steroids and colchicine through to oral corticosteroids and/or dapsone therapy. CONCLUSION: Recurrent oral aphthosis is a niche condition in which dermatologists are well-poised to manage. This study demonstrates that a more definitive management and therapeutic algorithm for oral aphthosis are needed for better management patients in the future. In particular, complex aphthosis needs to be monitored for progression onto Behcet's disease.

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.

A rare connexin 26 mutation in a patient with a forme fruste of keratitis-ichthyosis-deafness (KID) syndrome.

BACKGROUND: Keratitis-ichthyosis-deafness (KID) syndrome is a rare ectodermal dysplasia characterized by generalized erythrokeratotic plaques, sensorineural hearing loss, and vascularizing keratitis. Cutaneous changes and hearing loss typically present in early childhood, whereas ocular symptoms present later. Mutations in the connexin (Cx) 26 gene, GJB2, are now established to underlie many of the affected cases, with the majority of patients harboring the p.D50N mutation. METHODS: A rare patient demonstrating features of incomplete KID syndrome associated with an uncommon Cx26 gene mutation is described. RESULTS: The patient presented late in adolescence with partial features of KID syndrome. There was limited cutaneous involvement and the rare association of cystic acne. Both hearing impairment and ophthalmic involvement were mild in severity. Genetic mutation analysis revealed a previously described, rare mutation in GJB2, resulting in a glycine to arginine change at codon 12 (p.G12R). CONCLUSIONS: This report describes a patient exhibiting characteristics suggestive of a late-onset, incomplete form of KID syndrome with the GJB2 mutation (p.G12R). The p.G12R mutation has only been described in one other patient with KID syndrome, whose clinical presentation was not characterized.

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.