A 40-year-old male presented with progressive weakness, tingling and numbness in lower limbs for 2 years. He noted lower limbs swelling and skin changes over the face, hands and feet for 1 year. He also gave a history of erectile dysfunction and loss of libido for past 6 months. There was no history of bone pain or drug abuse. He had no previous history of tuberculosis, diabetes mellitus or hypertension.\nOn physical examination, bilateral pedal edema was present (Fig. a and b). Skin was thickened and hyperpigmented over the face, fingers of the hands and shin (Fig. a–d). Bilateral gynecomastia and testicular atrophy were also present. Abdominal examination showed hepatomegaly and ascites. Higher mental functions were normal. Examination of the fundi revealed bilateral papilledema, but otherwise cranial nerve examination was unremarkable. Muscle power in upper limbs was normal and predominant distal weakness [Medical Research Council (MRC) grade 4/5 at hip joints and 4-/5 at ankle joints] was present in lower limbs. Deep tendon reflexes in the upper limb were diminished (+1) including biceps, triceps and supinator and absent in lower limbs (knee and ankle). All modalities of sensations including pain, touch, temperature, vibration and joint position senses were impaired below knees. Romberg's sign was positive.\nFull blood count, liver and renal function tests, muscle enzymes (creatine phosphokinase), serum ferritin and vitamin B12 level were normal. Serum total protein was 7.1 g/dl, albumin 3 g/dl, globulin 4.1 g/dl and A : G ratio 1 : 1.3. Fasting and postprandial blood sugar levels were normal. Thyroid function tests revealed hypothyroidism (TSH level: 16.62 mIU/l and normal TSH level 0.4–4 mIU/l). Luteinizing hormone and testosterone levels were 15 (1.8–8.6 IU/l) and 111 (300–1000 ng/dl), respectively. Antinuclear antibody, rheumatoid factor, enzyme-linked immunosorbent assay test for human immunodeficiency virus, serology for hepatitis B and C were negative. Abdominal ultrasonography showed hepatomegaly (16 cm), moderate ascites and multiple enlarged mesenteric lymph nodes along the iliac vessels. Fine-needle aspiration cytology of mesenteric lymph node was inconclusive. Ascitic fluid was an exudate (SAAG < 1.1). Ascitic fluid adenosine deaminase level was normal and no malignant cells were observed. Nerve conduction studies showed sensorimotor demyelinating and axonal type polyneuropathy in both upper (median and ulnar nerves) and lower limbs (peroneal, tibial and sural nerves). Cerebrospinal fluid (CSF) analysis was normal. X-ray of the pelvis showed multiple osteosclerotic lesions involving head of left femur and right iliac crest (Fig. ). X-ray of the skull and spine was normal. The serum protein electrophoresis showed gamma globulinemia with no monoclonal M spike. Immunoelectrophoresis using the immunofixation method revealed a monoclonal IgG lambda band. The bone marrow biopsy from right iliac crest showed large atypical plasma cells in the range of 6–8% (Fig. ). The patient was diagnosed with POEMS syndrome. He was given radiotherapy, followed by a combination therapy of melphalan (16 mg/m2) and dexamethasone. At 6 months and 1-year follow-up, the patient has no worsening of symptoms.
The authors observed a 66-year-old woman with a previous history of paresthesia of distal prevalence in the upper limbs and the lower right limb associated with pain in the palmar and plantar region, with onset 1 year before, followed by reduction in strength, mainly in hands and feet, of slow and progressive evolution. During this period the patient noted diffuse skin darkening and appearance of papular lesions on the cervical and right infraclavicular region, abdomen and dorsum. She suffered a 20-kg weight loss during evolution of the picture, besides diabetes mellitus and hypothyroidism. Examination showed diffuse cutaneous hyperpigmentation, atrophy of the interosseous muscles (fig. , fig. ), sclerodermiform cutaneous thickening and normochromic, papular lesions (fig. ) in the cervical region, abdominal and right infraclavicular region. At dermatoscopy of the infraclavicular lesions there was presence of red lakes (fig. ). Neurological examination showed grade 3 strength of distal prevalence in the upper and lower limbs and hypopallesthesia in ‘glove’ and in ‘boot’. These alterations were suggestive of sensorimotor polyneuropathy.\nOphthalmological examination did not reveal presence of papillary edema. Concerning laboratory examinations, thrombocytosis was noted, with rheumatoid factor <10; anti-nuclear factor, VDRL, anti-HIV, anti-HTLV and serology for hepatitis B and C were negative. Prolactin and cortisol dosages were within normal range. Electroneuromyography was compatible with polyneuropathy with predominance of demyelination and axonal degeneration. Bone marrow aspirate presented medullary plasmacytosis (9% plasmacytes). Serous protein electrophoresis demonstrated a monoclonal peak in the gamma fraction and in immunofixation examination: monoclonal IgG/lambda peak.\nSkeleton radiography revealed a lithic lesion with sclerotic borders in the left femur (fig. ); histopathologic examination confirmed it to be a plasmocytoma. Histopathologic examination of the right infraclavicular lesion showed an exophytic lesion containing dispersed vascular connective structures (fig. ), and increased magnification showed both thick- and thin-walled vascular structures, compatible with arteriovenous hemangioma (fig. ). The lesion on the dorsum presented histopathologic characteristics of hemangioma in tufts and the lesion on the abdomen cherry hemangioma.\nThe patient was treated with radiotherapy of the femur due to the osteosclerotic lesions and showed improvement of the neurological picture as well as of hyperpigmentation and weigh loss.