May 2014

 

Fig.1

Fig.2

 

 


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

Allergies: NKDA

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

Chest: hyperpigmentation

Back: hyperpigmentation most prominent over shoulders, upper back, and along spine

Abdomen: splenomegaly present

Lower extremities: weakness of dorsiflexion of feet bilaterally

Mood: pleasant

Digits/nails: within normal limits

 

Laboratory data: 

WBC 12, Hgb 13.3, Platelets 591,000

Vitamin B12 low

LDH normal

ANA 1:320 with a speckled pattern

ENA negative

dS-DNA normal

SMA negative

Rheumatoid factor negative

ESR 9

HIV 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)

 

Imaging:

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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