Extensive Skin Necrosis Associated with POEMS Syndrome: A Case Study of a Rare Disease

Extensive skin necrosis, such as with Staphylococcal Scalded Skin Syndrome (SSSS), toxic epidermal necrolysis (TEN) or burns, are commonly managed with debridement and split thickness skin grafting techniques.  However when that massive tissue loss occurs in a patient who is underweight and has severe protein malnutrition, thrombocytopenia, aberrant coagulopathy and a rare disease known as POEMS syndrome, options for skin closure become very limited.  The acronym POEMS encompasses the features of this syndrome: polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy, and skin changes (specifically pigmentation changes).  KM experienced the features common to POEMS including recurring ascites, pleural effusions, renal failure, hypothyrioidism, neuropathy, an elevated vascular endothelial growth factor (VEGF) and pigmentation skin changes.  However the severity of the skin changes in this case were intensified by the patient’s progressive microvascular subcutaneous bleed that resulted in hemorrhagic skin infarction involving the lower abdomen bilaterally, circumferential thighs, back, buttocks and both calves.  The patient was managed in the hospital for over 3 months with oral nutritional supplementation, chemotherapy for POEMS, dermal templates, periodic negative pressure wound therapy, nonadherent silver impregnated foam dressings, and hydrofiber dressings.  Because skin grafting was not a safe option in a patient with this disease and with such extensive skin involvement, once the wound beds were filled with granulation tissue, epithelial cell transplantation was applied by the WOC nursing staff at three-week intervals using conscious sedation. This presentation will describe the pathophysiology of POEMS as a rare disease, the conditions leading to the extensive skin necrosis and the subsequent course of topical therapies used by the WOC nursing staff in conjunction with the hospitalist and plastic surgeon to achieve an epidermal cover to the massive wounds that eventually enabled a transfer to a transitional care unit and then home with home care nursing support.