History: The patient is a 34 year old white female who presents to dermatology clinic with complaints of dark spots on her face and darkening of the skin on her upper and middle back for approximately five years. She notes that during this time, she has also suffered from a multitude of other medical problems including intracranial hypertension with papilledema, numbness and weakness of arms and legs with foot drop bilaterally, splenomegaly and thrombocytosis, edema, lymphadenopathy, and depression.
Past medical history: as above
Social history: non-smoker but has a five pack year history, social alcohol use, married with three children, currently unemployed but worked in a Pepsi factory prior
Family history: Mother alive with hepatic steatosis and hypertension, father alive with diabetes and hypertension, sister alive with hepatic steatosis, brother alive with no medical problems. Children are all in good health.
Medications: Diamox, Celexa, klonopin, folate, vitamin D, methotrexate, hydroxyzine, aspirin
Physical exam: General: alert, oriented and in no acute distress, somewhat ill-appearing, walks with a cane
Skin; Back & Chest: hyperpigmentation most prominent over shoulders, upper back, and along spine (Figs. 1-2). Face: brown homogenous macules on bilateral malar eminences, forehead and nasal dorsum (not shown)
Lymph nodes: axillary, cervical, and inguinal lymphadenopathy
Upper extremities: weakness of flexion and extension of wrists bilaterally
Back: hyperpigmentation most prominent over shoulders, upper back, and along spine
Abdomen: splenomegaly present
Lower extremities: weakness of dorsiflexion of feet bilaterally
Digits/nails: within normal limits
WBC 12, Hgb 13.3, Platelets 591,000
Vitamin B12 low
ANA 1:320 with a speckled pattern
Rheumatoid factor negative
Kappa/Lambda FLC ratio 1.11 (0.26 - 1.65 ratio)
Kappa Free Light Chain (FLC) 3.60 (0.33 - 1.94 mg/dl)
Lambda Free Light Chain (FLC) 3.25 (0.57 - 2.63)
VEGF 6,240 (upper limit of normal 707)
Skeletal survey: Sclerotic lesions on T5 and T9
CT Chest/Abdomen/Pelvis: 1. Prominent lymph nodes as described, most notable in the small bowel mesentery and retroperitoneum. Differential diagnosis would include chronic inflammatory/infectious process or low-grade neoplasm.
2. Diffuse retroperitoneal, mesenteric and body wall edema.
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