INTRODUCTION
A 56-year-old African American female presented to
the hospital with a chief complaint of severe pain in
her body. She has a history of having painful subcutaneous
nodules for the past 8 years, which had become
exponentially more painful over the course of a few months.
She had a recent hospitalization and was thought to have
calciphylaxis, but biopsy revealed dermal edema and slight
subcutaneous fat necrosis and the diagnosis of Dercum’s
disease (adiposis dolorosa) was given instead. Her past
medical and surgical history includes diabetes mellitus, hypertension,
coronary artery disease, chronic kidney disease
(dialysis once for acute kidney injury two months prior to
hospitalization), gout, pulmonary hypertension, hyperlipidemia,
pancreatic divisum, gallstone pancreatitis, secondary
hyperparathyroidism, coronary artery bypass grafting, and
hysterectomy. Her medications include aspirin, cinacalcet,
citalopram, colchicine, hydroxyzine, insulin glargine, insulin
NPH, oxycodone, simvastatin, torsemide, tramadol, amitriptyline,
trazodone, febuxostat, esomeprazole, metolazone,
and spironolactone. In review of symptoms, she described
severe (10/10) pain and an unintentional rapid weight loss of
100 lbs in the last 5 months.
On physical examination, she had multiple painful and firm
nodules in varying sizes on the abdomen, bilateral lower
legs, chest, and bilateral upper extremities. She also had
some areas of hyperpigmentation and the formation of a
bullae was present on the right medial thigh. The pertinent
laboratory findings on admission include blood urea nitrogen/
creatinine ratio of 14.05 mg/dl:3.99mg/dl; phosphorus:
5.9 mmol/l; calcium: 9.8 mg/dl; anti-nuclear antibody/extractable
nuclear antigens: negative; hepatitis panel: negative;
albumin: 3.1g/dl; human immunodeficiency virus antigen/
antibody: non-reactive; vitamin D, total (25OH): 14 ng/ml;
parathyroid hormone: 196 pg/ml; thyroid stimulating hormone:
1.69 microIU/ml. A punch biopsy of the left medial
thigh revealed deposition of calcium in subcutaneous septae
and lobules, and in the walls of large subcutaneous vessels.
There is also an accompanying lobular lymphocytic panniculitis
with fat necrosis.
After the diagnosis was made, the patient was given a course
of cephalexin for prophylaxis of secondary infection. Her pain
was controlled with a fentanyl patch and oxycodone. Intravenous
sodium thiosulfate 25 grams was initiated with MWF
dosing and is planned for the next 3 months. She was also
scheduled for a parathyroidectomy and continued cinacalcet
to normalize her calcium levels.
Calciphylaxis is also known as calcific uremic arteriolopathy,
which results in painful ischemic purpura and necrotic ulceration.
The distribution of the subcutaneous nodules may be
similar to adiposis dolorosa, which is a rare condition char-