One of the most visible and potentially disfiguring cutaneous manifestations of tuberous sclerosis complex is the development of multiple facial angiofibromas, present in over 80% of patients. Topical rapamycin has been shown in many reports to be a safe and effective treatment for facial angiofibromas. In February 2012 we reported the results of a pilot study of four patients undertaken at a paediatric tertiary hospital in Australia. Since then, we have continued to refine the optimal formulation and concentration of topical rapamycin and expanded our selection of patients. We present an update on our current cohort of treated patients, discuss the optimal formulation of topical rapamycin and include a literature review on all published cases to date. Although topical rapamycin is not a curative treatment, we have demonstrated that its early institution significantly reduces both the vascularity and palpability of angiofibromas and prevents their progression with age. It is well tolerated and now a cost effective option.
Bowel bypass syndrome, also known as bowel-associated dermatitis arthritis syndrome, has been described after a range of intestinal bypass procedures. With the increasing trend in laparoscopic gastric bypass surgery, we report an interesting case of bowel-associated dermatitis arthritis syndrome that developed 12 months following this procedure. A 49-year-old woman presented with ulcerating lesions and pustules on the upper and lower limbs, polyarthralgia, fevers and joint effusions. Before the development of these symptoms she was well, with no significant past medical or family history. A skin biopsy taken from the left shin showed superficial to mid-dermal neutrophilic dermatosis, consistent with bowel-associated dermatitis arthritis syndrome. The patient received corticosteroids, antibiotics and colchicine treatment, with control of disease after continuing with colchicine. This case may illustrate another possible complication following bariatric surgery. The significant time period between the initial surgery and the development of bowel-associated dermatitis arthritis syndrome may mean that more cases of this condition will continue to emerge.
NB-UVB and UVA/UVB phototherapy significantly increased 25(OH)D serum level in patients with psoriasis and atopic dermatitis in Western Australia. Our study cohort had a higher baseline vitamin D level and a lower percentage increase of serum 25(OH)D post-phototherapy than the increases reported in the literature from cohorts in the northern hemisphere.
Malignant melanoma has previously been reported with paraneoplastic syndromes including dermatomyositis. We report a case involving a 34-year-old woman who presented with a persistent skin rash over her neck and upper body, with clinical features suggestive of dermatomyositis. The patient had a history of a right shoulder lesion that was previously removed; a solitary axillary lymph node was detected on additional imaging and biopsy results were consistent with malignant melanoma. The following year, surveillance positron emission tomography scan detected a solitary lung metastasis, leading to a partial lobectomy and confirmation of further spread of the malignant melanoma.
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