Letters to the Editor

A recent study has demonstrated that plain radiographs reveal a fine, ‘net-like’ calcification pattern with a 90% specificity.4 While biopsy may only show some features of calciphylaxis, such as ischaemic necrosis and thrombosis, radiography has consistently demonstrated arteriolar calcification. Imaging may also reveal the calcifications prior to manifestation on the skin.4,5 CT with 3-dimensional image reconstruction and mammography are other potential methods for diagnosis, but cost and availability are factors to consider.4 Sodium thiosulphate has been used successfully to treat penile calciphylaxis. STS acts as a reactive oxygen species scavenger, chelates calcium and may create hydrogen sulphide, which contributes to its ability to work as a vasodilator, analgesic and anti-inflammatory. Treatment with STS should be started as soon as the diagnosis is made. Pain and skin ulceration is typically relieved within a few weeks. Side-effects of infusing STS too rapidly include diarrhoea, nausea and vomiting.6 In our case, the additional use of radiographic imaging aided in the diagnosis and treatment of penile calciphylaxis, suggesting that radiography evaluation should be considered as a supportive, and in some cases, alternative diagnostic tool for dermatologists to utilize when evaluating for calciphylaxis. In addition, our case supports the safety and efficacy of STS as a treatment option for treating penile calciphylaxis. M. Morrison,1,* M. Merati,2 J. Ramirez,3 H.C. Cha,1 A. LaFond1


POEMS syndrome (polyneuropathy organomegaly endocrinopathy M-protein skin changes) with xanthoma – a case report Editor POEMS syndrome is a rare multisystemic and paraneoplastic disease which is characterized by polyneuropathy, organomegaly, endocrinopathy, M protein and skin changes. This syndrome is usually associated with plasma cell dyscrasia. The most common cutaneous manifestations include hyperpigmentation, hypertrichosis, skin thickening, multiple glomeruloid angiomata, acrocyanosis, white nails and clubbing.1 Herein, we report a case of POEMS syndrome with xanthomatous infiltration within the hyperpigmented lesions of patient. A 50-year-old man who was diagnosed with POEMS syndrome at the department of internal medicine presented a 2years history of hyperpigmentation on his face and neck. He did not take any dermatological treatment for the hyperpigmentation. He was hospitalized in the internal medicine department for congestive heart failure, chronic kidney failure and monoclonal gammopathy of undetermined significance (MGUS). He was prescribed ramipril 5 mg, carvedilol 2 9 6.25 mg, furosemide 2 9 40 mg, spironolactone 50 mg, digoxin capsule 1 9 0.125 mg, and lansoprazole 1 9 30 mg daily for 1 year.


Department of Dermatology, St. Joseph Mercy Hospital, Ypsilanti, 2 Michigan State University, East Lansing, 3Department of Dermatopathology, St. Joseph Mercy Hospital, Ypsilanti, MI, USA *Correspondence: M. Morrison. E-mail: [email protected]

References 1 Auriemma M, Carbone A, Di Liberato L et al. Treatment of cutaneous calciphylaxis with sodium thiosulfate. Am J Clin Dermatol 2011; 12: 339–346. 2 Sandhu G, Gini MB, Ranade A, Djebali D, Smith S. Penile calciphylaxis: a life-threatening condition successfully treated with sodium thiosulfate. Am J Ther 2012; 19: e66–e68. 3 Cimmino CB, Costabile RA. Biopsy is contraindicated in the management of penile calciphylaxis. J Sex Med 2014; 11: 2611–2617. doi: 10. 1111/jsm.12390. [Epub ahead of print]. 4 Shmidt E, Murthy NS, Knudsen JM et al. Net-like pattern of calcification on plain soft-tissue radiographs in patients with calciphylaxis. J Am Acad Dermatol 2012; 67: 1296–1301. 5 Vedvyas C, Winterfield L, Vleug R. Calciphylaxis: a systematic review of existing and emerging therapies. J Am Acad Dermatol 2012; 67: e253– e260. 6 Hayden MR, Goldsmith D, Sowers J, Khanna R. Calciphylaxis: calcific uremic arteriopathy and the emerging role of sodium thiosulfate. Int Urol Nephrol 2008; 40: 443–451. DOI: 10.1111/jdv.12764

Figure 1 Diffuse hyperpigmentation is noticed on his face.

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© 2014 European Academy of Dermatology and Venereology

Letters to the Editor




Figure 2 Dense infiltration of xantomatous histiocytes within the upper dermis (a, H&E, 2009; b, H&E, 4009).

Physical examination revealed diffuse hyperpigmentation on his forehead, bilateral periorbital area, jaw and nose without affecting fold regions, also yellow-brownish, soft nodular lesions besides ala nasi, more sparse hyperpigmentation on the neck as compared to the face (Fig. 1). On systemic examination 3 degrees/6 murmur in the mesocardiac region, crepitant rales in bilateral basal region of lungs and hepatosplenomegaly were detected. Two skin biopsies were taken from the edge of the nose and neck. Histopathological examination revealed a thin epidermis and there were sparse lymphocytes and xanthomatous foamy cells that completely infiltrated into the upper papillary dermis (Fig. 2). Laboratory examination results were as follows: hemoglobin: 7.3, Hct: 23.1, Plt: 42.000, ESR: 89 mm/h, urea: 114, creatinine: 1.82, AST: 56, ALT: 114, LDH: 430, and serum cholesterol and triglyceride levels were normal. In immunoelectrophoresis: IgG kappa paraproteinemia was detected positive. Electromyography detected sensorimotor axonal type polyneuropathy in bilateral lower extremities. Computed tomography scannings of the chest and abdomen revealed bilateral pneumonic infiltrations and pleural effusion. The diagnosis of POEMS syndrome (Crow-Fukase Syndrome) comprises three major criteria, two of which include polyradiculoneuropathy and clonal plasma cell disorder (PCD), and at least one minor criteria. The major criteria for this syndrome are polyradiculoneuropathy, clonal PCD, sclerotic bone lesions, elevated vascular endothelial growth factor, and the presence of Castleman disease. Minor features include organomegaly, endocrinopathy, characteristic skin changes, papilloedema, extravascular volume overload, and thrombocytosis.1,2

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Common skin lesions are hyperpigmentation (93–98%), skin thickening (77–85%), hypertrichosis (78–81%), peripheral oedema (90%), clubbing (56%), verrucous angiomata, telangiectasia and acrocyanosis. Also, there have been case reports of urticaria pigmentosa like lesions (oedema and mast cell infiltration),3 xanthomatous infiltration in hyperpigmented patches,2 facial lipodystrophy,4 white nails, erythematous papules with enduration and excoriation, glomeruloid haemangiomas, cicatricial alopecia,5 multiple eruptive angiomatous lesions6 and sclerodermoid changes.7 Earlier, Chang et al. reported a case of foamy histiocytes without xanthomatousappearing skin lesions in POEMS syndrome .2 Diffuse normolipemic plane xanthomas are uncommon lesions associated with blood dyscrasias especially monoclonal gammopathy.8 Pathogenesis of this disorder in monoclonal gammopathy is related to the formation of a complex between lipoprotein and abnormal paraprotein that is deposited on the skin.9 Lipid profiles of the patients are normal as in our case. The main point here is that the cutaneous xanthomas with normal lipid profile might be rarely the first sign of a haematologic malignancy. We want to present that rare cutaneous manifestation of POEMS syndrome herein. Our patient is diagnosed with polyneuropathy, hypogonadotropic hypogonadism, MGUS and hiperpigmentation with underlying xanthomatous cells. This skin disorder has been reported earlier only by Chang et al.. Z. Kutlubay,1,* B. Engin,1 T.K. Uzuncakmak,1 C. Demirkesen,2 M.M. Altiti,3 Y. Karter,3 Y. Tuzun1 1

Departments of Dermatology, 2 Pathology, 3 Internal Medicine, Istanbul University, Cerrahpasa Medical Faculty, Istanbul, Turkey *Correspondence: Z. Kutlubay. E-mail: [email protected]

© 2014 European Academy of Dermatology and Venereology

Letters to the Editor

References 1 Dispenzieri A. POEMS syndrome: update on diagnosis, risk-stratification, and management. Am J Hematol 2012; 87: 804–814. 2 Chang SE, Choi JH, Sung KJ, Moon KC, Koh JK, Ro JY. POEMS syndrome with xanthomatous cells. Polyneuropathy Organomegaly Endocrinopathy M-protein Skin changes. Am J Dermatopathol 1999; 21: 567– 570. 3 Jackson A, Burton IE. A case of POEMS syndrome associated with essential thrombocythaemia and dermal mastocytosis. Postgrad Med J 1990; 66: 761–767. 4 Vourc’h-Jourdain M, Clairand R, Masseau A, Barbarot S, Hamidou M. [Partial lipodystrophy and POEMS syndrome: a case report]. Ann Dermatol Venereol 2008; 135: 773–775. 5 Weichenthal M, Stemm AV, Ramsauer J, Mensing H, Feller AC, Meigel W. POEMS syndrome: cicatricial alopecia as an unusual cutaneous manifestation associated with an underlying plasmacytoma. J Am Acad Dermatol 1999; 40: 808–812. 6 Granel B, Serratrice J, de Roux-Serratrice C, Ene N, Disdier P, Weiller PJ. Multiple cutaneous angiomas and Poems syndrome. Presse Med. 2006; 35: 430–432. 7 Ishikawa O, Nihei Y, Ishikawa H. The skin changes of POEMS syndrome. Br J Dermatol 1987; 117: 523–526. 8 Morrison LK, Lopez IE. Unknown: yellow plaques on the chest and arms present for more than 6 years in an 80-year-old male. Diffuse normolipemic plane xanthomas presenting in association with myelodysplastic syndrome. Dermatol Online J 2011; 17: 11. 9 Bayer-Garner IB, Smoller BR. The spectrum of cutaneous disease in multiple myeloma. J Am Acad Dermatol 2003; 43: 497–507. DOI: 10.1111/jdv.12766

Severe exacerbation of psoriasis after intravenous immunoglobulin in patient with multiple sclerosis that started during biologic therapy Editor Psoriasis is well known to be worsened or triggered by a number of endogenous and exogenous factors, e.g. stress, infection and drugs. These include modern biological treatment modalities that interfere with complex immunological cycles in human body such as interferon-beta or TNFa inhibitors in the management of neurological, rheumatological, gastrointestinal or paradoxically dermatological diseases.1 Likewise, systemic treatments of severe psoriasis can often trigger various comorbidities and complications. Those facts bring challenging demands to every dermatologist and often require profound interdisciplinary cooperation. We present a case of a 30-year-old male with severe treatment-resistant erythrodermic psoriasis that first manifested at the age of 21. The patient was a smoker, but otherwise healthy and his mother suffered from palmar psoriasis. In the past, he

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was treated with numerous therapeutical modalities. UVB phototherapy wasn’t tolerated, acitretine was stopped for liver toxicity, methotrexate and cyclosporine A didn’t show significant therapeutic effect. In 2008 adalimumab therapy was started. After 6 months without achieving satisfactory effect, the patient was switched to etanercept that led to almost complete remission. However, after a year, optic neuritis of the right eye developed. Magnetic resonance, visual evoked potentials and analysis of cerebrospinal fluid led to the diagnosis of neuroborreliosis due to detection of intrathecal production of anti-borrelia antibodies. Patient was treated with ceftriaxone and later he was switched to ustekinumab. After 2 years of very efficacious treatment, patient developed optic neuritis again, this time of the left eye. Ustekinumab was withdrawn and neuritis was treated by high-dose bolus of corticosteroids. Magnetic resonance revealed several demyelinating lesions. Analysis of cerebrospinal fluid was negative for anti-borrelia antibodies and showed oligoclonal bands typical for multiple sclerosis (MS). Attending neurologist decided to treat MS with low-dose (15 mg) intravenous immunoglobulin (IVIG) applied every month. After 2 weeks from the first administration of IVIG patient referred to our department with erythrodermic flare of psoriasis, swollen joints, chills, inguinal lymphadenopathy and general malaise. (Fig. 1) C-reactive protein was elevated, other laboratory findings were normal. The exacerbation was thought to be caused by rebound phenomenon after discontinuation of ustekinumab or after bolus of corticosteroids for optic neuritis 3 months before first application of IVIG. Patient received low dose systemic corticosteroids and methotrexate with gradual improvement of condition in the next 2 weeks that preceded second application of IVIG that again led to fast relapse of psoriatic erythroderma. During that time patient was still on corticosteroids and methotrexate and there weren’t other apparent precipitating factors. After consultation with neurologists ustekinumab therapy was restarted because of his severe psoriasis and he was kept on low-dose

Figure. 1 Psoriasis vulgaris. Erythrodermic flare of psoriasis after first dose of IVIG.

© 2014 European Academy of Dermatology and Venereology

POEMS syndrome (polyneuropathy organomegaly endocrinopathy M-protein skin changes) with xanthoma--a case report.

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