Key Points
-
The cutaneous mucinoses are a heterogeneous group of disorders in which an abnormal amount of mucin accumulates in the skin.
-
The etiopathogenesis of cutaneous mucinoses is unknown, although cytokines such as tumor necrosis factor α and β, interleukin-1 and transforming growth factor β and/or polyclonal and monoclonal immunoglobulins and other unidentified factors in the serum of affected patients may induce the synthesis of glycoaminoglycans.
-
The cutaneous mucinoses are divided into two groups: primary cutaneous mucinoses in which the mucin deposit is the main histologic feature resulting in clinically distinctive lesions, and secondary mucinoses in which the mucin deposition is only an additional histologic epiphenomenon.
-
Primary mucinoses can be divided into dermal and follicular mucinoses. The former includes lichen myxedematosus, reticular erythematous mucinosis, scleredema, dysthyroidotic mucinoses (localized (pretibial) and generalized myxedema), papular and nodular mucinosis in connective tissue diseases, self-healing juvenile cutaneous mucinosis, cutaneous focal mucinosis, digital myxoid cyst, while the latter include Pinkus’ follicular mucinosis and urticaria-like follicular mucinosis.
-
Associated disorders include paraproteinemia (scleromyxedema, scleredema), diabetes mellitus (scleredema), hyperthyroidism (pretibial myxedema), hypothyroidism (generalized myxedema) and lupus erythematosus, and dermatomyositis or scleroderma (papular and nodular mucinosis in connective tissue diseases).
Keywords
Access this chapter
Tax calculation will be finalised at checkout
Purchases are for personal use only
References
Rongioletti F, Rebora A (2008) Mucinoses. In Bolognia J, Jorizzo JL, Rapini R. (eds). Dermatology, 2nd edn. Elsevier, St Louis, MO, pp 611–621
Rongioletti F (2006) Lichen myxedematosus (papular mucinosis): new concepts and perspectives for an old disease. Semin Cutan Med Surg 25:100–104
Scheidegger EP, Itin P, Kempf W (2005) Familial occurrence of axillary papular mucinosis. Eur J Dermatol 15:70–72
Rongioletti F, Rebora A (2001) Cutaneous mucinoses: microscopic criteria for diagnosis. Am J Dermatopathol 23:257–267
Caputo R, Marzano AV, Tourlaki A, Marchini M (2006) Reticular erythematous mucinosis occurring in a brother and sister. Dermatology 212:385–387
Gasior-Chrzan B, Husebekk A (2004) Reticular erythematous mucinosis syndrome: report of a case with positive immunofluorescence. J Eur Acad Dermatol Venereol 18:375–378
Lewerenz V, Ruzicka T (2007) Scleredema adultorum associated with type 2 diabetes mellitus: a report of three cases. J Eur Acad Dermatol Venereol 21:560–561
Ioannidou DI, Krasagakis K, Stefanidou MP et al (2005) Scleredema adultorum of Buschke presenting as periorbital edema: a diagnostic challenge. J Am Acad Dermatol 52(Suppl):41–44
Doshi DN, Blyumin ML, Kimball AB (2008) Cutaneous manifestations of thyroid disease. Clin Dermatol 26:283–287
Fatourechi V (2005) Pretibial myxedema: pathophysiology and treatment options. Am J Clin Dermatol 6:295–309
Ortiz VG, Krishnan RS, Chen LL, Hsu S (2004) Papulonodular mucinosis in systemic lupus erythematosus. Dermatol Online J 10:16
Rongioletti F, Rampini P, Parodi A, Rebora A (1999) Papular mucinosis associated with generalized morphoea. Br J Dermatol 141:905–908
Tan E, Tan S, Ng SH (2003) Cutaneous mucinosis in dermatomyositis associated with a malignant tumor. J Am Acad Dermatol 48:S41–S42
Bachmeyer C, Chesneau AM, Loirat C, Asensi D, Babinet JM, Blum L (2007) Papulonodular mucinosis in a child with systemic lupus erythematosus. Pediatr Dermatol 24:585–586
Nagaraj LV, Fangman W, White WL, Woosley JT, Prose N, Selim MA, Morrell DS (2006) Self-healing juvenile cutaneous mucinosis: cases highlighting subcutaneous/fascial involvement. J Am Acad Dermatol 55:1036–1043
Yokoyama E, Muto M (2006) Adult variant of self-healing papular mucinosis in a patient with rheumatoid arthritis: predominant proliferation of dermal dendritic cells expressing CD34 or factor XIIIa in association with dermal deposition of mucin. J Dermatol 33:30–35
Takemura N, Fujii N, Tanaka T (2005) Cutaneous focal mucinosis: a case report. J Dermatol 32:1051–1054
Lin YC, Wu YH, Scher RK (2008) Nail changes and association of osteoarthritis in digital myxoid cyst. Dermatol Surg 34:364–369
Connolly M, de Berker DA (2006) Multiple myxoid cysts secondary to occupation. Clin Exp Dermatol 31:404–406
Arca E, Köse O, Taştan HB, Gür AR, Safali M (2004) Follicular mucinosis responding to isotretinoin treatment. J Dermatolog Treat 15:391–395
Harthi FA, Kudwah A, Ailan A et al (2003) Urticaria-like follicular mucinosis responding to dapsone. Acta Derm Venereol 83:389–390
Passaro EM, Silveira MT, Valente NY (2004) Acneiform follicular mucinosis. Clin Exp Dermatol 29:396–398
Rongioletti F, De Lucchi S, Meyes D et al (2010) Follicular mucinosis: a clinicopathologic, histochemical, immunohistochemical and molecular study comparing the primary benign form and the mycosis fungoides-associated follicular mucinosis. J Cutan Pathol 37:15–19
Blum M, Wigley FM, Hummers LK (2008) Scleromyxedema: a case series highlighting long-term outcomes of treatment with intravenous immunoglobulin (IVIG). Medicine (Baltimore) 87:10–20
Rongioletti F, Zaccaria E, Cozzani E, Parodi A (2008) Treatment of localized lichen myxedematosus of discrete type with tacrolimus ointment. J Am Acad Dermatol 58:530–532
Rubegni P, Sbano P, Risulo M, Poggiali S, Fimiani M (2004) A case of reticular erythematous mucinosis treated with topical tacrolimus. Br J Dermatol 150:173–174
Kroft EB, de Jong EM (2008) Scleredema diabeticorum case series: successful treatment with UV-A1. Arch Dermatol 144:947–948
Felton J, Derrick EK, Prince ML (2003) Successful combined surgical and octreotide treatment of severe pretibial myxoedema reviewed after 9 years. Br J Dermatol 148:825–826
Author information
Authors and Affiliations
Corresponding author
Editor information
Editors and Affiliations
Rights and permissions
Copyright information
© 2010 Springer New York
About this chapter
Cite this chapter
Rongioletti, F. (2010). Mucinoses. In: Smoller, B., Rongioletti, F. (eds) Clinical and Pathological Aspects of Skin Diseases in Endocrine, Metabolic, Nutritional and Deposition Disease. Springer, New York, NY. https://doi.org/10.1007/978-1-60761-181-3_18
Download citation
DOI: https://doi.org/10.1007/978-1-60761-181-3_18
Published:
Publisher Name: Springer, New York, NY
Print ISBN: 978-1-60761-180-6
Online ISBN: 978-1-60761-181-3
eBook Packages: MedicineMedicine (R0)