Ipoh, Malaysia
Abstract: 19 yo man with skin lesions and gastro-intestinal infarcts
History: A 19 yr old student presented with multiple discrete mildly pruritic papules on the trunk and thighs of 9 months duration. It started as papules on the trunk that become enlarged resulting a central depression. 2 months ago, he had severe abdominal pain, underwent emergency laparotomy and was told he had a "perforated appendix". The surgeon noted several shallow ulcers on the large and small intestines. He was worried about typhoid but serology was negative.
Exam: Multiple discrete papules/plaques 3-6 mm diameter with a central porcelain white atrophied depression and a raised border on the trunk and thighs. There was a rim of telangiectasia on the periphery in some of the lesions. No purpura was noted.
Clinical Photos:
Lab: His blood counts and biochemistry was unremarkable.
Diagnosis: Degos disease (Malignant atrophic papulosis) appears most likely. Could this some form of cutaneous lymphoma?
Questions: Have any of our readers seen and treated similar patients? There are only around 200 cases of Degos Disease reported in the literature. Treatment is difficult and often ineffective. Aspirin and dipyridamole has been tried to reduce the complications of vessel thrombosis. Some cases has been associated with connective tissue disease but by and large those with gastrointestinal complications have poor prognosis. IV immunoglobulins and eculizumab has been tried with some success.
Cost Considerations:
In the U.S.:
Eculizumab is ~$7000 per dose at low end
A four dose course of IVIG for a 70 kg person at 2 g/kg would cost $25 000-$26 000. Or ~ $21,000 for a 60 kg person.
While these drugs may be 40% cheaper in Malaysia can your patient's family afford a trial or does the government pick up the cost?
References:
1. Cynthia M. Magro, MD, Jonathan C. Poe, PhD,et. al. A C5b-9/Interferon-α–Mediated Endotheliopathy Syndrome. Am J Clin Pathol 2011;135:599-610
2. Scheinfeld N. Commentary on 'Degos disease: a C5b-9/interferon-α-mediated endotheliopathy syndrome' by Magro et al: a reconsideration of Degos disease as hematologic or endothelial genetic disease. Dermatol Online J. 2011 Aug 15;17(8):6. Excellent discussion and available Free Full Text
3. Zhu KJ, et al. The use of intravenous immunoglobulin in cutaneous and recurrent perforating intestinal Degos disease (malignant atrophic papulosis) Br J Dermatol. 2007 Jul;157(1):206-7.
Histopath demonstrates an atrophied epidermis with evidence of arteritis in the deeper dermis.
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