HPI: The patient is a 77 yo woman with diabetes and ESRD on hemodialysis for a number of years. She had a diagonsis of porphyria cutanea tarda over four years ago, but no lab studies are available and it's unclear if this was a clinical or laboratory diagnosis. For over a year she has had painful skin ulcers which are located on the abdomen, scalp, breast and hands. These do not heal with good wound care. Her medications include Lantus insulin, sevelamer, cinacalcet (used to treat secondary hyperpartahyroidism), midodrine, risedronate, pravastatin, omeprazole, fenofibrate, famotadine.
O/E: This is a chronically ill-appearing woman. She has a periungual ulceration on her right ring finger. There are ulcers with escars on the abdomen, buttock, sccalp and extremities. The ulcers vary from one to 4 cm in diameter.
Clinical photos (presented with patient's permission)
Photo above is L. buttock and to right is abdomenLabs; Although a diagnosis of PCT was made four years ago, the only reference to porphyrin levels is that they were "low." We do not have parathyroid hormone or Ca++ levels. These should be available from her nephrologists.
Pathology: Bx. 11/20/11 Read by Dan Carter, M.D.
Histologic changes consistent with "acquired perforating dermatosis of dialysis."
Diagnosis: Atypical Skin Ulcers in a Renal Transplant Patient. The etiology of her ulcers is unclear at this time.
Discussion: The patient is anemic with iron deficiency and her nephrologists are reluctant to treat with i.v. iron because of the past diagnosis of PCT. The current lesions do not look like classic PCT. Nor do they look like perforating dermatosis. They are also quite atypical for calciphylaxis.
Questions: What are your thoughts? Serum porphyrin levels could be done prior to and after iron infusion. Perhaps a deeper biopsy. Has she been checked for hyperparathyroidism? Has anyone seen a case like this?
References: I could find no helpful references for this case.
Update from her nephrologist: After this posting, this woman had a GI bleed and developed very painful decubitus ulcers. Last Friday I spoke with her while she was on dialysis. Her quality of life has been poor and no reasonable expectation that things were going to improve for her. She and I agreed that dialysis was no longer allowing her to have the acceptable quality of life she has had on dialysis for over 12 years. She terminated her dialysis treatment and passed away about 48 hours later.
from Larry Eron (I.D. Specialist): I don't know what your patient has. Interestingly, a female patient, recipient of a renal transplant, with skin ulcers over her mons and scalp, was presented at a meeting I have been attending this week. The skin bx, just showed chronic inflammatory cells. Turned out, she had M. tbc skin ulcers without granulomata (because of her immunosuppressive agents). The case reflects the fact that it was an ID meeting.
ReplyDeleteFrom Sam Moschella: I think she has calciphylaxis. I would do a calcium and phosphorus determination and parathyroid test. She may be a candidate for local and systemic sodium thiosulfate or cinacalcet depending on the lab studies.
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