Cutaneous manifestations in patients with POEMS syndrome

Miest, Rachel Y.; Comfere, Nneka I.; Dispenzieri, Angela; Lohse, Christine M.; el-Azhary, Rokea A.

doi:10.1111/j.1365-4632.2012.05648.x

Cited by 48 publications

(47 citation statements)

References 37 publications

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“…The IL-6 gene is related to plasmocyte proliferation, monoclonal gammopathy, thrombocytosis, Castleman disease, hemangiomas and microangiopathic glomerulopathy. VEGF induces an increase in capillary permeability and angiogenesis; it is implicated in the development of hydric overload, pulmonary hypertension, macular compromise, papilledema, glomeruloid hemangiomas and nail clubbing [12].…”

Section: Discussionmentioning

confidence: 99%

POEMS Syndrome: A Multidisciplinary Diagnostic Challenge

Enciso¹,

Aponte²,

Rodríguez³

et al. 2017

J Clin Case Rep

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POEMS syndrome is a paraneoplastic phenomenon associated with polyneuropathy and paraproteinemia that arises out of the clonal expansion of plasma tumor cells. Its other distinguishing features include sclerotic bone lesions, increased vascular endothelial growth factor levels, Castleman disease alterations in lymph node biopsy and association with organomegaly, endocrinopathy, cutaneous lesions, papilledema, extravascular volume overload and thrombocytosis. Although established diagnostic criteria exist, the fact that the disease is rare and shares similar clinical manifestations with non-neoplastic conditions means that in most cases patients are diagnosed at latestage, when multiple complications have appeared and treatments for other conditions have already been given. Therefore, a high degree of suspicion combined with a multidisciplinary approach are requisites for attaining precise diagnosis and providing timely treatment. Due to the wide differential diagnosis that the syndrome´s clinical features encompass as well as its subsequent favourable responses to bone marrow transplant and diverse chemotherapy treatments, clinicians from diverse fields should include the POEMS syndrome within polyneuropathy differential diagnoses that require specific diagnostic tests. Herein, we present our case study in which we emphasize diagnosis and treatment of patients treated at our institution.

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Section: Discussionmentioning

confidence: 99%

POEMS Syndrome: A Multidisciplinary Diagnostic Challenge

Enciso¹,

Aponte²,

Rodríguez³

et al. 2017

J Clin Case Rep

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“…Diğer bulgular arasında çomak parmak, Raynaud fenomeni, sklerodermatöz deri kalınlaşması, hiperhidroz, kilo kaybı, pulmoner hipertansiyon/restriktif akciğer hastalığı, trombotik diyatez, daire ve B12 vitamin düşüklüğü belirtilmiştir 34,35 . Hemanjiyomlar hastaların yaklaşık 1/3-1/2'sine varan oranlarda görülür [34][35][36] . Gövde ve ekstremitelerin proksimal kısımlarında daha sık olmak üzere vücudun değişik bölgelerinde yerleşen, değişken sayıda, kırmızı küçük vasküler tümörler şeklindedir.…”

Section: Glomeruloid Hemanjiyomunclassified

“…POEMS sendromunun seyrinde çeşitli plazma hücreli diskraziler ortaya çıkabilir. Hastaların çoğunda lambda gammopati ve yaklaşık yarısında serumda monoklonal IgG veya IgA artışı saptanır 36 . Bu nedenle POEMS sendromlu hastalara plazma hücreli diskrazilere yönelik başlangıçta ve daha sonra aralıklarla serum protein elektroforezi ve immunfiksasyon, etkilenmiş immunglobulin düzeyine yönelik inceleme, 24 saatlik idrarda total protein, elektroforez ve immunfiksasyon, kemik iliği aspirasyon ve biyopsisi (immunhistokimyasal inceleme ile kappa/lambda için test) yapılması önerilmektedir 35 .…”

Section: Glomeruloid Hemanjiyomunclassified

Selim deri tümörleri ve sistemik malinite ilişkisi

Yazganoğlu¹,

Baykal²

2013

TURKDERM

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ÖzetDerinin karmaşık histolojik görüntüsü ile ilişkili olarak çok sayıda selim deri tümörü tipi vardır. Bunlardan az bir bölümü sistemik maliniteler ile ilişki gösterebilir. Bu makalede sistemik maliniteler ile ilişkisi iyi bilinen veya tartışmalı olan selim deri tümörlerinden bahsedilecektir. Bahsi geçen tümörler ve ilişkili olduğu durumlar şunlardır: seboreik keratoz/ Leser-Trélat belirtisi, lentigo simpleks/ Peutz Jeghers ve Carney sendromu, yağ bezi tümörleri/Muir-Torre sendromu, fibrofoliküloma-trikodiskoma/ Birt-Hogg-Dubé sendromu, trikoepitelyom-silindrom-spiradenom/ BrookeSpiegler sendromu, leiomyom/ multipl kutane ve uterin leiyomiyomatozis sendromu, glomeruloid hemanjiyom/ POEMS sendromu, venöz malformasyon/ Maffucci sendromu, mukozal nörom/ multipl endokrin neoplazi tip 2B, jüvenil ksantogranülom/ jüvenil kronik miyeloid lösemi. Deri tümörleri ve ilişkili olduğu belirtilerin veya sendromların sistemik malinite riskinin bilinmesi, malinitelerin erken tanısı açısından önem taşır. Sum maryThere are various types of benign skin tumors in association with the complex nature of the skin. A minor part of them may be associated with visceral malignancies. This review focuses on the benign skin tumors that are described to have well-known associations with systemic malignancies or those with controversial associations. These tumors and the associated conditions are as follows: seborrheic keratosis/ Leser-Trélat sign, lentigo simplex/Peutz Jeghers and Carney syndrome, sebaceous tumors/ Muir-Torre syndrome, fibrofolliculoma-trichodiskoma/ Birt-Hogg-Dubé syndrome, trichoepitheliomacylindroma-spiradenoma/ Brooke-Spiegler syndrome, leiomyoma/multiple cutaneous and uterin leiomyomatosis syndrome, glomeruloid hemangioma/ POEMS syndrome, venous malformation/ Maffucci syndrome, mucosal neuroma/ multiple endocrine neoplasia type 2B, juvenile xanthogranuloma/ juvenile chronic myeloid leukemia. It is important to know the skin tumors related with visceral malignancies in part of a syndrome or in association with a sign as this may yield an earlier recognition of these malignancies. (Turkderm 2013; 47

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“…57 Cutaneous findings include flushing, hyperpigmentation, hypertrichosis, plethora and acrocyanosis of the skin are attributed to elevated vascular endothelial growth factor (VEGF) levels. [57][58][59] In a cohort of 96 patients with cutaneous skin findings, flushing was reported in 17 (16%) patients 58 and is recurrent, located on the face, trunk and abdomen with a violaceous appearance. …”

Section: Clinical Presentation and Gastrointestinal Involvementmentioning

confidence: 99%

Flushing Disorders Associated with Gastrointestinal Symptoms: Part 1, Neuroendocrine Tumors, Mast Cell Disorders and Hyperbasophila

et al. 2018

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Flushing is the subjective sensation of warmth accompanied by visible cutaneous erythema occurring throughout the body with a predilection for the face, neck, pinnae, and upper trunk where the skin is thinnest and cutaneous vessels are superficially located and in greatest numbers. Flushing can be present in either a wet or dry form depending upon whether neural-mediated mechanisms are involved. Activation of the sympathetic nervous system results in wet flushing, accompanied by diaphoresis, due to concomitant stimulation of eccrine sweat glands. Wet flushing is caused by certain medications, panic disorder and paroxysmal extreme pain disorder (PEPD). Vasodilator mediated flushing due to the formation and release of a variety of biogenic amines, neuropeptides and phospholipid mediators such as histamine, serotonin and prostaglandins, respectively, typically presents as dry flushing where sweating is characteristically absent. Flushing occurring with neuroendocrine tumors accompanied by gastrointestinal symptoms is generally of the dry flushing variant, which may be an important clinical clue to the differential diagnosis. A number of primary diseases of the gastrointestinal tract cause flushing, and conversely extra-intestinal conditions are associated with flushing and gastrointestinal symptoms. Gastrointestinal findings vary and include one or more of the following non-specific symptoms such as abdominal pain, nausea, vomiting, diarrhea or constipation. The purpose of this review is to provide a focused comprehensive discussion on the presentation, pathophysiology, diagnostic evaluation and management of those diseases that arise from the gastrointestinal tract or other site that may cause gastrointestinal symptoms secondarily accompanied by flushing. This review is divided into two parts given the scope of conditions that cause flushing and affect the gastrointestinal tract: Part 1 covers neuroendocrine tumors (carcinoid, pheochromocytomas, vasoactive intestinal polypeptide, medullary carcinoma of the thyroid), polyneuropathy, organomegaly, endocrinopathy, monoclonal protein, skin changes (POEMS), and conditions involving mast cells and basophils; while Part 2 covers dumping syndrome, mesenteric traction syndrome, rosacea, hyperthyroidism and thyroid storm, anaphylaxis, panic disorders, paroxysmal extreme pain disorder, and food, alcohol and medications.

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Cutaneous manifestations in patients with POEMS syndrome

Abstract: Background Polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy,

Cited by 48 publications

References 37 publications

POEMS Syndrome: A Multidisciplinary Diagnostic Challenge

POEMS Syndrome: A Multidisciplinary Diagnostic Challenge

Selim deri tümörleri ve sistemik malinite ilişkisi

Flushing Disorders Associated with Gastrointestinal Symptoms: Part 1, Neuroendocrine Tumors, Mast Cell Disorders and Hyperbasophila

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