The results of this study support the use of Nikolsky's sign of the gingival mucosa as a viable test to establish the presence of oral bullous diseases.

While typically seen in patients with end-stage renal failure and renal transplantation, calciphylaxis has been reported in patients without end-stage renal disease (i.e. nonuraemic calciphylaxis). 3 In a systematic review of 36 cases of nonuraemic calciphylaxis, primary hyperparathyroidism (28%), malignancy (22%), alcoholic liver disease (17%) and connective tissue disease (11%) were the most common underlying aetiologies. 6 To date, the association between calciphylaxis and pancreatic cancer has not been reported. One possible link between nonuraemic calciphylaxis and pancreatic cancer is the elevation and release of pancreatic enzymes, which results in lipolysis of subcutaneous adipose tissue, formation of free fatty acids and the subsequent saponification of fat by calcium salts. 7 A number of treatment strategies have been proposed in calciphylaxis. However, to date, no controlled prospective studies comparing these various treatments have been conducted. Traditional therapies include wound care in combination with intravenous sodium thiosulfate (STS), cinacalcet, sevelamer, bisphosphonates and/or hyperbaric oxygen therapy. Intravenous STS has gained favour as a generally well tolerated, effective therapeutic option that works by dissolving vascular calcium deposits through the creation of highly soluble calcium thiosulfate complexes. 8-10 Intravenous STS is generally well tolerated and has been shown to decrease mortality when used in combination with cinacalcet and sevelamer. [8][9][10] Sevelamer and cinacalcet also help to mitigate the endocrine abnormalities seen in calciphylaxis by acting as noncalcium phosphate binders and calcimimetics, respectively. 10 Our patient was started on oral cinacalcet 30 mg twice daily and intravenous STS 25 mg three times weekly. After 2 weeks of treatment with intravenous STS followed by 4 weeks of topical STS 10% soaks and aggressive wound care, the patient experienced considerable improvement in his lower extremity pain and leg ulcers. The patient was subsequently started on gemcitabine and paclitaxel for his pancreatic cancer, but he passed away several months later owing to complications of pneumonia. In summary, we present a case of nonuraemic calciphylaxis in a white man with pancreatic cancer. To our knowledge, this is the first reported patient with pancreatic cancer-associated calciphylaxis in the setting of normal kidney and parathyroid function, who also responded favourably to intravenous and topical STS, as well as oral cinacalcet. References1 Ng AT, Peng DH. Calciphylaxis. Dermatol Ther 2011; 24:256-62.2 Ross EA. Evolution of treatment strategies for calciphylaxis. Am J Nephrol 2011; 34:460-7. 3 Mochel MC, Arakaki RY, Wang G et al. Cutaneous calciphylaxis: a retrospective histopathologic evaluation. Am J Dermatopathol 2013; 35:582-6. 4 Weenig RH, Sewell LD, Davis MDP et al. Calciphylaxis: natural history, risk factor analysis and outcome. J Am Acad Dermatol 2007; 56:569-79. 5 Harris RJ, Cropley TG. Possible role of hypercoagulability in calciphylaxis: review...

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FIGURE I -Lack of eruption and enamel hypoplasia.Australasian Radiology, Vol. XXXI, No. I . February, I987 osteomyeiitis were present from the age of 28 months. The bone fragility was observed from the age of 4-5 years, when an apparently sponthat of pyknodysostosis can be noted: large and prominent front in contrast with facial hypoplasia. prominent and beaked nose. Deformity of the left cheek is due to mandibular osteomyelitis.Accepted for publication on 16th December, 1985.