Is scleromyxedema a skin problem or systemic pathological process?

Close metadata

Recommended articles:

Alveolar adenoma of the lung. Russian Journal of Archive of Pathology. 2024;(4):38-41

Scleromyxedema is regarded as a rare cutaneous mucinosis from a group of lichen myxedematosus characterized by diffuse mucin deposition, sclerosis, and lichenoid eruptions in the absence of thyroid disease. The paper discusses the pathogenesis of the disease and histological changes in tissues. It underlines the need for using histochemical tests to identify acidic and neutral glycosaminoglycans and gives a differential diagnosis of this disease.

Keywords:

mucinoses

lichen myxedematosus

scleromyxedema

morphological diagnosis

Authors:

Radenska-Lopovok S.G.

FGBU "Nauchno-issledovatel'skiĭ institut revmatologii im. V.A. Nasonovoĭ" RAMN, Moskva

Volkova P.

N.I. Pirogov Russian National Research Medical University, Moscow, Russia

List of references:

Rongioletti F. Mucinoses. In: Rongioletti F, Smoller BR, eds. Clinical and pathological aspects of skin diseases in endocrine, metabolic, nutritional and deposition disease. New York (NY): Springer; 2010:139-152. https://doi.org/10.1007/978-1-60761-181-3
Alligood TR, Burnett JW, Raines BL Jr. Scleromyxedema associated with esophageal aperistalsis and dermal eosinophilia. Cutis. 1981;28:60-63. PMID: 7261674
Rongioletti F, Cozzani E, Parodi A. Scleromyxedema with an interstitial granulomatous-like pattern: a rare histologic variant mimicking granuloma annulare. J Cutan Pathol. 2010;37(10):1084-1087. https://doi.org/10.1111/j.1600-0560.2009.01386.x
Dubreuilh W. Fibromes miliaires folliculaires: sclerodemie consecutive. Ann Dermatol Syph. 1906;37:569-572. https://doi.org/10.2147/IMCRJ.S115315
Reitmann K. Uber eineeigenartige, der Sklerodermie nahestehende Affektion. Arch Dermatol Syph. 1908;92:417-424. https://doi.org/10.1007/BF01949029
Montgomery H, Underwood LJ. Lichen myxedematosus; differentiation from cutaneous myxedemas or mucoid states. J Invest Dermatol. 1953;20(3):213-236. https://doi.org/10.1038/jid.1953.25
Gottron HA. Scleromyxedema; a strange manifestation of myxothesaurodermy. Arch Klin Exp Dermatol. 1954;199(1):71-91.
Rongioletti F, Rebora A. Updated classification of papular mucinosis, lichen myxedematosus, and scleromyxedema. J Am Acad Dermatol. 2001;44(2):273-281. https://doi.org/10.1067/mjd.2001.111630
Rongioletti F, Merlo G, Carli C, Cribier B, Metze D, Calonje E, Kempf W, Stefanato CM, Marinho E, Kanitakis J. Histopathologic characteristics of scleromyxedema: a study of a series of 34 cases. J Am Acad Dermatol. 2016;74(6):1194-1200. https://doi.org/10.1016/j.jaad.2015.12.021
L’vov AN, Sheklakova MN, Matushevskaya YuI, Znamenskaya LF, Svishchenko SI, Katunina OR, Chikin VV, Yakovleva AO. Arndt—Gottron scleromyxedema. Vestnik dermatologii i venerologii. 2012;(5):71-77. (In Russ.)
Lacy MQ, Hogan WJ, Gertz MA, Dispenzieri A, Rajkumar SV, Hayman S, Kumar S, Litzow MR, Schroeter AL. Successful treatment of scleromyxedema with autologous peripheral blood stem cell transplantation. Arch Dermatol. 2005;141(10):1277-1282. https://doi.org/10.1001/archderm.141.10.1277
Dinneen AM, Dicken CH. Scleromyxedema. J Am Acad Dermatol. 1995;33(1):37-43. PMID: 7601944
Donato ML, Feasel AM, Weber DM, Prieto VG, Giralt SA, Champlin RE, Duvic M. Scleromyxedema: role of high-dose melphalan with autologous stem cell transplantation. Blood. 2006;107(2):463-466. https://doi.org/10.1182/blood-2004-12-4870
Harper RA, Rispler J. Lichen myxedematosus serum stimulates human skin fibroblast proliferation. Science. 1978;199(4328):545-547. https://doi.org/10.1126/science.622555
Braun-Falco O, Weidner F. Arndt-Gottron scleromyxedema with medullary plasmacytosis and myositis. Arch Belg Dermatol Syphiligr. 1970;26(2):193-217. https://doi.org/10.1007/978-3-642-97931-6_43
Harris RB, Perry HO, Kyle RA, Winkelmann RK. Treatment of scleromyxedema with melphalan. Arch Dermatol. 1979;115(3):295-299. https://doi.org/10.1001/archderm.1979.04010030003002
James K, Fudenberg H, Epstein WL, Shuster J. Studies on a unique diagnostic serum globulin in papular mucinosis (lichen myxedematosus). Clin Exp Immunol. 1967;2(2):153-166. PMC 1578836
Shapiro CM, Fretzin D, Norris S. Papular mucinosis. JAMA. 1970;214(11):2052-2054. https://doi.org/10.1001/jama.1970.03180110060017
Piper W, Hardmeier T, Schafer E. Scleromyxedema Arndt-Gottron: a paraproteinemic disease. Schweiz Med Wochenschr. 1967;97(26):829-838. PMID: 4166238 PMCID: PMC1578836
McCarthy JT, Osserman E, Lombardo PC, Takatsuki K. An abnormal serum globulin in lichen myxedematosus. Arch Dermatol. 1964;89:446-450. https://doi.org/10.1001/archderm.1964.01590270132030
Rowell NR, Waite A, Scott DG. Multiple serum protein abnormalities in lichen myxoedematosus. Br J Dermatol. 1969;81(10):753-758. https://doi.org/10.1111/j.1365-2133.1969.tb15936.x
Sawada Y, Ohashi M. Scleromyxedema — report of a case with hyperpolygammopathy and immunoglobulin and complement deposition in cutaneous lesions. J Dermatol. 1980;7(3):207-212. https://doi.org/10.1111/j.1346-8138.1980.tb03534.x
Ferrarini M, Helfrich DJ, Walker ER, Medsger TA Jr, Whiteside TL. Scleromyxedema serum increases proliferation but not the glycosaminoglycan synthesis of dermal fibroblasts. J Rheumatol. 1989;16(6):837-841. PMID: 2778769
Yaron M, Yaron I, Yust I, Brenner S. Lichen myxedematosus (scleromyxedema) serum stimulates hyaluronic acid and prostaglandin E production by human fibroblasts. J Rheumatol. 1985;12(1):171-175. PMID: 3981505
White GM, Jeffes EW, 3-rd. Stimulation of fibroblasts by paraprotein in scleromyxedema. J Am Acad Dermatol. 1989;21(4,Pt1):816. https://doi.org/10.1016/S0190-9622(89)80294-4
Boin F, Hummers LK. Scleroderma-like fibrosing disorders. Rheum Dis Clin North Am. 2008;34(1):199-220. https://doi.org/10.1016/j.rdc.2007.11.001
Rongioletti F, Merlo G, Cinotti E, Fausti V, Cozzani E, Cribier B, Metze D, Calonje E, Kanitakis J, Kempf W, Stefanato CM, Marinho E, Parodi A. Scleromyxedema: a multicenter study of characteristics, comorbidities, course, and therapy in 30 patients. J Am Acad Dermatol. 2013;69(1):66-72. https://doi.org/10.1016/j.jaad.2013.01.007
Helfrich DJ, Walker ER, Martinez AJ, Medsger TA Jr. Scleromyxedema myopathy: case report and review of the literature. Arthritis Rheum. 1988;31(11):1437-1441. https://doi.org/10.1002/art.1780311115
McAdam LP, Pearson CM, Pitts WH, Sadoff L, Verity MA. Papular mucinosis with myopathy, arthritis, and eosinophilia. A histopathologic study. Arthritis Rheum. 1977;20(4):989-996. https://doi.org/10.1002/art.1780200412
Gabriel SE, Perry HO, Oleson GB, Bowles CA. Scleromyxedema: a scleroderma-like disorder with systemic manifestations. Medicine (Baltimore). 1988;67(1):58-65. https://doi.org/10.1097/00005792-198801000-00004
Stetsenko GY, Vary JC Jr, Olerud JE, Argenyi ZB. Unusual granulomatous variant of scleromyxedema. J Am Acad Dermatol. 2008;59(2):346-349. https://doi.org/10.1016/j.jaad.2008.04.017
Rongioletti F, Cerroni L, Massone C, Basso M, Ciambellotti A, Rebora A. Different histologic patterns of cutaneous granulomas in systemic lymphoma. J Am Acad Dermatol. 2004;51(4):600-605. https://doi.org/10.1016/j.jaad.2004.02.024
Perry HO, Montgomery H, Stickney JM. Further observations on lichen myxedematosus. Ann Intern Med. 1960;53:955-969. https://doi.org/10.7326/0003-4819-53-5-955
McCuistion CH, Schoch EP Jr. Autopsy findings in lichen myxedematosus. AMA Arch Derm. 1956;74(3):259-262. https://doi.org/10.1001/archderm.1956.01550090033007
Loggini B, Pingitore R, Avvenente A, Giuliano G, Barachini P. Lichen myxedematosus with systemic involvement: clinical and autopsy findings. J Am Acad Dermatol. 2001;45(4):606-608. https://doi.org/10.1067/mjd.2001.111903

Close metadata