The Australasian journal of dermatology
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The mortality rate of Stevens-Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN) is high; approximately 5% for SJS and 25% for TEN. It is therefore vital for the treating physician to recognise SJS and TEN promptly through the identification of these diseases' characteristic clinical features so that the offending drug is promptly withdrawn, supportive therapy is administered and adjunctive therapies are considered. Supportive therapy addressing the manifestations and complications of acute skin failure include monitoring the fluid - electrolyte balance and providing enteral or parenteral nutrition, wound care and treatment of sepsis. ⋯ Some earlier studies have shown a lack of efficacy or increased mortality in their use but the use of high doses early in the course of disease may actually reduce morbidity and mortality. The role of plasmapheresis, anti-tumour necrosis factor (TNF) biologics and N-acetylcysteine is promising but further studies are required to elucidate their benefit. Preventative strategies such as pharmacogenetic screening needs to be strongly considered, with the provision of cost-effective assays with a rapid turn-around time.
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Australas. J. Dermatol. · May 2012
Case ReportsBirt-Hogg-Dubé syndrome with a renal angiomyolipoma: further evidence of a relationship between Birt-Hogg-Dubé syndrome and tuberous sclerosis complex.
Birt-Hogg-Dubé syndrome (BHDS) is an autosomal dominant condition caused by mutations in the gene which codes for folliculin (FLCN). It is characterised clinically by fibrofolliculomas, trichodiscomas, pulmonary cysts, spontaneous pneumothoraces and renal cancers. ⋯ Recent studies suggest that clinical similarities between BHDS and TSC may be explained by FLCN and TSC proteins functioning on a common pathway, mammalian target of rapamycin. This case adds to the literature of cases with clinical similarities.
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Australas. J. Dermatol. · May 2012
Dermoscopy: Ex vivo visualization of fleas head and bag of eggs confirms the diagnosis of Tungiasis.
Tungiasis, caused by the impregnated female sand flea Tunga penetrans, is increasingly common in returned travellers from endemic areas. Clinical suspicion is raised by the clinicodermoscopic correlation, leading to rapid treatment which involves extraction of the intact flea. Ex vivo dermoscopy demonstrates the parasite's head and distended abdomen full of eggs, confirming the diagnosis.
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Australas. J. Dermatol. · Feb 2012
Case ReportsTreatment-resistant elephantiasic thyroid dermopathy responding to rituximab and plasmapheresis.
A 44-year-old woman was diagnosed with Graves' disease in 1995 and over the following 12 months developed thyroid dermopathy (pretibial myxoedema). Despite being trialled on multiple recognized therapies over the course of 11 years, the patient's dermopathy progressively worsened. ⋯ In mid 2006, the patient was started on rituximab and plasmapheresis, with rapid response. The patient's condition stabilized and in October 2009 at the age of 58 years she was able to cease therapy.
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Australas. J. Dermatol. · Nov 2011
Skin disease in the first two years of life in Aboriginal children in East Arnhem Land.
The most common skin infections affecting children in remote Aboriginal communities are scabies and impetigo. Group A streptococcal skin infections are linked to the high rates of heart and renal disease occurring in Aboriginal Australians. ⋯ Skin infections are a major reason for presentation to primary health clinics and contribute to the high disease burden experienced by children in the first 2 years of life. This high frequency of presentation provides multiple opportunities for intervention and monitoring.