A 61-year-old male patient was admitted to our hospital with a prior one-year history of cutaneous hyperpigmentation. Loss of appetite, abdominal distension, constipation, dry skin, less sweat, and insomnia were concomitant symptoms. Ten months prior to admission, his symptoms became severe and were accompanied by symmetrical pitting edema, lower extremity numbness, and weakness in the left lower limbs. Brain MRI showed cerebral infarction, and the patient was treated appropriately. One month later, he was diagnosed with hypothyroidism and Addison’s disease (AD) for severe edema of the lower extremities, unexplained cutaneous pigmentation, and higher ACTH levels (Tables and ). Hydrocortisone 20 mg and Levothyroxine 12.5 μg per day as well as diuretic therapy were administered, and the symptoms mildly improved. After discharge from the hospital, he gradually stopped the diuretic drugs and the doses were adjusted to hydrocortisone 40 mg and Levothyroxine 200 μg per day based on the lab tests. Concomitantly, he experienced pain and numbness in his lower limbs. Since the onset of illness, his general condition was poor. The patient suffered from decreased appetite, poor sleep, weight loss of 15 kg, and hyposthenia (Fig. ).\nThe patient’s past history showed he was a carrier of hepatitis B virus for 60 years, psoriasis for 40 years with external steroid use, had a 10-year history of type 2 diabetes mellitus controlled by insulin glargine and voglibose, and had hypertension for 2 months. In addition, he was diagnosed with depression two months prior and was treated with flupentixol and melitracen tablets without obvious improvement.\nExamination showed T 36.1°C, P 75 bpm, R 16 tpm, BP 140/85 mmHg, H 176 cm, W 62 kg, and BMI 20 kg/m2. The patient had diffuse cutaneous pigmentation of his skin and mucous membranes, especially the areolas and armpits. His superficial lymph nodes were not palpable. He had edema of the eyelids and pitting edema of the upper and lower extremities (Figs. , , , , ). Neurological examination showed grade IV muscle strength in his left lower limbs. His physiological reflexes were normal, and his pathological reflexes were not elicited. His thyroid function and adrenal function were abnormal (Tables and ).\nMild anemia was noted, with HBsAg(+), HBsAb(−), HBeAg(−), HBeAb(+), and HBcAb(+). Rheumatoid factor, anti-nuclear factor, and anti-HIV were negative. Serum electrolytes revealed normal sodium levels. BNP was 2010 ng/L (450–900), and TNI < 0.012 ng/ml (0–0.12). Albumin was 34 g/L (35–55), and fasting blood glucose (FBG) was 5.3–6.5 mmol/L (3.6–5.8). Electroneuromyography disclosed polyneuropathy of the upper and lower extremities. The results of an adrenal CT scan and a head MRI were negative. Abdomen B-ultrasound presented splenomegaly (4.8 × 13.8 cm) and ascites. UCG indicated pericardial effusion with 59% EF. Bone marrow aspirate showed medullary phagocytosis. No M protein was found via serous protein electrophoresis but an immunofixation examination revealed a monoclonal IgAλ peak. The diagnosis POEMS syndrome was made.\nThe patient was initially treated with a very expensive drug, Revlimid (lenalidomide capsules 25 mg), which is generally used for multiple myeloma (MM) patients. He then underwent autologous stem cell transplantation (ASCT) and experienced significant improvement of his hyperpigmentation and weight loss(Fig. ). Hydrocortisone and Levothyroxine were gradually decreased. His diabetes was also relieved.
Woman, 65-year-old, with sensory-motor polyneuropathy for 15 years, worsening in the\nprevious 6 months. The patient had diffuse hyperpigmentation, dry skin and hemangiomas\n( and ). She reported hypertrichosis, edema of the legs, facial lipoatrophy,\nleukonychia and weight loss ( and ). Analytically, she presented polyglobulia,\nthrombocytosis, hypotestosteronemia, hyperalbuminemia and elevation of IgG in the\nliquor. The immunochemical study emphasized IgG monoclonal gammopathy of lambda\nchains.\nInvestigations showed small adenopathies, cardiomegaly, hepatosplenomegaly, generalized\nedema and extensive lytic lesion of L5. A biopsy of this lesion showed a lytic\nproliferation of plasma cells, CD138 +, К chains - chains λ +. The myelogram showed\nhypercellularity and eosinophilia. The electromyogram showed slowing of motor and\nsensory nerve conduction of several nerves. An excision of the right breast angioma was\nperformed and was compatible with capillary hemangioma. The patient has begun treatment\nwith dexamethasone, radiotherapy, bortezomib and is waiting for a bone marrow\ntransplant.