A 30-year-old woman, with no morbid history, who was diagnosed with peripheral motor neuropathy after consultation for a bilateral gait disorder (“steppage”), which was confirmed by nerve conductive measurement.
Skin hyperpigmentation was evident.
She returned 6 years later reporting that she had stopped working due to weakness of the lower limbs.
Hyperpigmentation had increased, her weight decreased, she was in amenorrhea and suffered chronic diarrhea.
Physical examination revealed a hard skin, acartonated, dark and desquamative without hyperpigmentation of the folds; white nails in clock glass, diffuse axillary goiter with bilateral hard lymphadenopathy, parotid hypertrophy
Differential diagnoses of Addison's disease, scleroderma, autoimmune liver disease and hemochromatosis were raised, which were reasonably ruled out.
The laboratory revealed hypothyroidism with absent antithyroid antibodies, normal basal cortisol with insufficient elevation of ACTH stimulus, hypogonadotrophic hypogonadism, hypogonadotrophic hyperprophylaxis, renal failure, microscopic haematuria,
The images showed hepatosplenic enlargement, ascites, echogenic kidneys with poor corticomedullary differentiation and an intrasellar aracnoidocele.
Liver biopsy was normal.
Thyroxine 50 ug/day and cortisol 20 mg/day were prescribed, with mood improvement.
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The following year she presented tension ascites with umbilical eversion.
The fluid was exudate with negative Koch culture and normal ADA.
There was bilateral papilledema.
Proteinuria persisted and renal function deteriorated.
The scanner showed thickening of the skin and subcutaneous cellular tissue; axillary, mediastinal and retroperitoneal lymphadenopathy; scarce elimination of the right kidneys contrast and signs of chronic nephropathy with reduction of volume of the right sclerilla.
A PS was diagnosed.
There was a monoclonal component in plasma protein electrophoresis with IgA concentration 4 times normal.
Myelogram was nonspecific, costal biopsy showed an osteosclerotic myeloma and lymph node biopsy showed a Castleman's disease.
Twelve monthly cycles of melphalan and prednisone were prescribed.
In the seven years after chemotherapy, the patient was regularly monitored.
Ascites did not recur, the monoclonal component disappeared, the costal lesion showed no variations and renal function and proteinuria stabilized.
The patient was lost from the controls for 4 1⁄2 years and reappeared due to thrombosis of the right common iliac artery.
She was revascularized surgically, suffering acute non-oliguric renal injury in the postoperative period.
Plasma protein electrophoresis did not show the monoclonal component.
The patient was again excluded from the controls, and died of an acute myocardial infarction 2 years later, at 50 years of age, 20 years after the onset of PS, 12 years after the start of chronic dialysis and no longer treated.
