Multiple myeloma (MM) is a rare malignancy, characterized by clonal proliferation of plasma cells. Monoclonal immunoglobulin (paraprotein), secreted by plasmocytes, can cause plasma hyperviscosity and renal damage. Proliferation of plasma cells can lead to bone marrow suppression and cause hypercalcaemia. These features of MM give rise to different symptoms, such as bone pain from direct skeletal involvement, fatigue from anemia, or headache from hyperviscosity. The incidence of MM is estimated at 4.5‑6/100 000 . It mostly (90%) affects people above 50
Teresa Iwaniec, Joanna Zdziarska and Artur Jurczyszyn
Danijela Popović, Mirjana Paravina, Dragan Jovanović, Vesna Karanikolić and Dragana Ljubisavljević
Lichen myxedematosus, also known as papular mucinosis, is a primary diffuse cutaneous mucinosis. It is a rare cutaneous myxedematous condition characterized by formation of numerous lichenoid papules. Scleromyxedema, also known as Arndt–Gottron syndrome, is a rare, confluent, papular and sclerotic variant of lichen myxedematosus, characterized by diffuse thickening of the skin underlying the papules. The condition is associated with systemic, even lethal manifestations, fibroblast proliferation and accumulation of acid mucopolysaccharides of the hyaluronic acid. Serum IgG class paraproteinemia is always present and it can be detected in all patients if appropriate or even repeat testing is used.
Herein, we present a 67-year-old patient with a 2-year history of skin problems. He had no health problems other than hypertension and diabetes, wich were both diagnosed 15 years before. On examination, the patient exhibited sclerodermoid lesions with diffuse pseudo-sclerodermatous thickening of the exposed skin, microstomia and sclerodactyly-like changes; on the face, there were numerous solid, shiny 2 - 4 mm in diameter skin-coloured lichenoid papules, scattered across the forehead, glabellar area, nasolabial folds, perioral region, ear lobes and the neck. Histopathological examination revealed: highly distinctive collagenosis and fibrosis in the middle dermis, increased fibroblasts; collagen bundles with irregular arrangement and fragmentation; alcian blue-positive deposits with appearance consistent with acid mucins. Serum protein electrophoresis detected IgG lambda paraproteinemia. The patient was treated with systematic corticosteroids during 9 months with subsequent introduction of methotrexate and showed satisfactory results.
The etiology of scleromyxedema remains unknown, since the purified IgG paraprotein itself has no direct effects on fibroblast proliferation. In scleromyxedema, numerous therapeutic modalities are proposed, unfortunatelly with limited effects.
In colclusion, we report a case of an adult male with lichenoid papules; after a two-year progression, they evolved into scleromyxedema and exhibited well response to conventional therapy.
Predrag Rodić, Milan Lakočević, Sonja Pavlović, Teodora Karan Đurašević, Tatjana Kostić, Nada Suvajdžić Vuković, Zorica Šumarac, Milan Petakov and Dragana Janić
. Performance of the Sebia CAPILLARYS 2 for detection and immunotyping of serum monoclonal paraproteins. Am J Clin Pathol 2007; 128(2): 293-9. 12. Bossuyt X, Bogaerts A, Schiettekatte G, Blanckaert N. Serum protein electrophoresis and immunofixation by a semiautomated electrophoresis system. Clin Chem 1998; 44(5): 944-9. 13. Green E, McConville CM, Powell JE, Mann JR, Darbyshire PJ, Taylor AM, et al. Clonal diversity of Ig and T-cellreceptor gene rearrangements identifies a subset of childhood B-precursor acute lymphoblastic leukemia with
Maciej Korpysz, Marta Morawska, Agata Burska and Helena Donica
, 2009. 30. Van Hoeven K.H. et al.: Serum free light chain assays are more sensitive than urinary tests for free light chain monoclonal paraproteins. Clin. Chem., 55, C30a, 2009. 31. Viedma J.A., Garrigos N., Morales S.: Comparison of the sensitivity of 2 automated immunoassays with immunofixation electrophoresis for detecting urine Bence Jones proteins. Clin. Chem., 51, 1505, 2005.
Kamila Skwierawska, Anna Waszczuk-Gajda, Agnieszka Perkowska-Ptasińska, Grzegorz Basak and Wiesław W. Jędrzejczak
institution. Clin J Am Soc Nephrol. 2011; 6(4): 775–784. 10.2215/CJN.08300910 Nasr SH Valeri AM Cornell LD Fibrillary glomerulonephritis: a report of 66 cases from a single institution Clin J Am Soc Nephrol. 2011 6 4 775 784  Motwani SS, Herlitz l, Monga D, et al. Paraprotein-Related Kidney Disease : Glomerular Diseases Associated with Paraproteinemias. Clin J Am Soc Nephrol. 2016;11(12):2260-2272. 10.2215/CJN.02980316 Motwani SS Herlitz l Monga D Paraprotein-Related Kidney Disease : Glomerular Diseases Associated with Paraproteinemias Clin J Am Soc Nephrol. 2016 11