We present a case of a 69-year-old woman, with a previous history of arterial hypertension and dyslipidemia. Her medication included amlodipine 5 mg, candesartan 8 mg, hydrochlorothiazide 12.5 mg and nebivolol 5 mg. She presented with a 1-year history of left-side supraclavicular swelling associated with progressive fatigue, anorexia, and weight loss (body weight: 74 to 63 kg). Cervical ultrasound documented multiple cervical lymphadenopathies with the largest lymph node (measuring 3.1 × 1.7 cm) being localized on the left supraclavicular region; and she was referred to the Department of General Surgery. A left supraclavicular lymph node excisional biopsy was performed. The histopathological examination of the specimen was consistent with the diagnosis of herpes virus-8-positive CD (HHV-8 CD, ).\nShe was then forwarded to the Department of Internal Medicine upon lymph node biopsy showed CD. Additional clinical history inquiry was attempted and revealed that, during the last few months, she noted progressive diffuse skin darkening and symmetrical paresthesia of the lower limbs. She denied fever, night sweats, gastrointestinal or respiratory complaints. Neurologic review of symptoms was negative for dysphagia, dysarthria, ocular changes, and bowel and bladder dysfunctions. She had no history of smoking, alcohol abuse, or illicit-drug use. She had no contact with animals or any sick person and did not have any recent travel outside Portugal. She had no family history of neurologic or oncologic diseases.\nOn physical examination, she was afebrile. Her blood pressure was 151/77 mm Hg and heart rate 62 beats/min, and peripheral oxygen saturation was 97% while on room air. She had diffuse cutaneous hyperpigmentation and palpable lymph nodes in her cervical and supraclavicular regions, with the largest measuring 3 × 2 cm. Abdominal examination revealed an enlarged liver and no other abnormality. She had bilateral edema of lower limbs. Neurological examination revealed hypoesthesia below the ankles and tendon hyporreflexia; her muscle strength was decreased in upper and lower extremities with muscle strength grade 4-/5 proximal and grade 4+/5 distal.\nBlood tests showed normal complete blood count, coagulation tests, serum bilirubin (total and conjugated), aspartate and alanine aminotransferases and lactate dehydrogenase. Erythrocyte sedimentation rate and C-reactive protein were within normal range. Renal function, serum calcium and total serum protein were normal. Endocrine screening showed normal thyroid function, a normal glycemic profile (fasting plasma glucose and hemoglobin A1c), and a normal cortisol dosage. Hepatitis B and C, toxoplasmosis, tuberculosis, human immunodeficiency virus (HIV), cytomegalovirus (CMV) and Epstein-Barr virus (EBV) infections were ruled out. Auto-immune screening (antinuclear antibodies (ANA), anti-double-stranded deoxyribonucleic acid (dsDNA) and rheumatoid factor) was negative with no complement consumption. Albumin was 3.2 g/dL (reference range (RR): 3.4 - 5) and serum protein electrophoresis (SPEP) with immunofixation demonstrated a monoclonal immunoglobulin G (IgG)/kappa peak with IgG level of 2,120 mg/dL (RR: 650 - 1,600). Serum free light chains ratio was normal. Bence-Jones proteins were not detected. Bone marrow biopsy showed the presence of reactive-appearing lymphocytes B and T and 5% of plasma cells ().\nPlasma vascular endothelial growth factor (VEGF) measurement was elevated (644 pg/mL, RR: < 115). Human herpes virus-8 (HHV8) was not detected in peripheral blood by polymerase chain reaction.\nThoraco-abdominopelvic computed tomography scan revealed small bilateral pleural effusion, multiple enlarged supra and infra-diaphragmatic lymphadenopathies, hepatosplenomegaly and multiple osteosclerotic lesions (). On transthoracic echocardiography there was a normal systolic function without pulmonary hypertension or pericardial effusion. Electromyography was consistent with sensorimotor peripheral polyneuropathy.\nThus, our patient meets the criteria for the diagnosis of POEMS syndrome in association with CD.\nShe was referred to the Hematology Department, and she started treatment with a combination of bortezomib, cyclophosphamide, and dexamethasone.\nAfter the first cycle, she noticed improvement of paresthesia and IgG level reduced from 2,120 to 960 mg/dL (RR: 650 - 1,600). She has tolerated treatment well without any clinically significant adverse effect.
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.