Self-healing papular mucinosis in an adult

Sperber, Brian R.; Allee, Julie E.; James, William D.

doi:10.1016/s0190-9622(03)00790-4

Cited by 29 publications

(19 citation statements)

References 15 publications

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“…It usually affects middle-aged people and equal numbers of women and men. Diagnostic criteria are as follows: [1][2][3][4][5][6] (1) generalized papular and sclerodermoid eruption, (2) mucin deposition, fibroblast proliferation and the presence of fibrosis histopathologically, (3) paraproteinemi, i.e. monoclonal gammopathy (typically IgG λ), and (4) the absence of thyroid function disorder.…”

Section: Discussionmentioning

confidence: 99%

“…[3] Although increased mucin production by fibroblasts seems to be apparent, the trigger factor for abnormal mucin production is unclear. Monoclonal gammopathy of IgG with λ light chain is common and this abnormality has been considered as a fibroblast stimulant factor.…”

Section: Discussionmentioning

confidence: 99%

“…It is considered among the lichen myxedematosus (LM) or the papular mucinosis group of cutaneous disorders and also referred to as "generalized lichen myxedematosus." [1][2][3] The classification of these group disorders has been more recently updated by Rangioletti. LM [1] has been considered to have three main clinicopathological subsets as generalized, local and atypical or intermediate varieties in this new classification.…”

Section: Introductionmentioning

confidence: 99%

“…Actually, both the skin and the other organs are prone to be involved extensively in this variety of LM, leading significant morbidity. [1][2][3][4][5] Generalized papular and sclerodermoid eruption, namely scleromyxedema, is defined by a triad of specific histopathological features that include mucin deposition, fibrosis and fibroblast proliferation, monoclonal gammopathy and the absence of thyroid disorder. [1,2,5] The patient presented herein had generalized papular and sclerodermoid lesions, monoclonal gammopathy, and characteristic histopathologic findings in the skin biopsies.…”

Section: Introductionmentioning

confidence: 99%

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Generalized papular and sclerodermoid eruption: Scleromyxedema

Serdar

Altunay

Yaşar

et al. 2010

Indian J Dermatol Venereol Leprol

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Scleromyxedema (SM) is a rare chronic progressive and highly intractable cutaneous disease with unknown etiology, affecting both genders equally between 30 and 50 years. The disease is characterized with mucin deposits in the skin and/or other organs. In fact it is a clinicopathological subset of lichen myxedematosus (LM) according to a new classification. Sclerodermiform plaques and lichenoid papules are characteristic cutaneous lesions. An elevation of IgG λ (lambda) chain exists in most cases and extracutaneous involvement occurring with variable systemic findings is also detected. Generalized form is quite difficult to treat and may even be fatal. Herein, we present a male patient with typical features of generalized papular and sclerodermoid LM variety and with benign outcome by isotretinoin.

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Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

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Generalized papular and sclerodermoid eruption: Scleromyxedema

Serdar

Altunay

Yaşar

et al. 2010

Indian J Dermatol Venereol Leprol

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“…DPM'un klinik önemi hastal›¤›n HCV veya HIV infeksiyonlar›na efllik edebilmesidir, tan›s› bu nedenle önem kazanmaktad›r 4 . Histopatolojik olarak üst ve orta dermiste diffüz veya lokal müsin birikimi, de¤iflik derecelerde fibroblast proliferasyonu vard›r, kollajen yap›m› veya skleroz yoktur 1,[14][15][16] . Ay›r›c› tan›s›nda yer alan liken planus, erüptif papüler ksantom , leiomyom ve likenoid ilaç erüpsiyonundan klinik ve histolojik olarak kolayca ayr›l›r 5 .…”

Section: Tart›flmaunclassified

"Diskret" Papüler Müsinoz: Liken Miksödematozun Nadir Bir Subtipi

Akış¹,

Eskioğlu²,

Öztürk³

2011

Turkderm

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ÖzetLiken miksödematoz (papüler müsinoz) tiroid hastal›¤› olmaks›z›n dermal müsin birikimi ve fibrozise ba¤l› olarak geliflen likenoid papül, nodül ve/veya plaklar ile karakterize s›k görülmeyen, kronik, idiyopatik bir hastal›kt›r.Liken miksödematoz klinikopatolojik olarak iki subgrup içerir: monoklonal gammopati ile beraber sistemik, hatta letal bulgular› olan generalize papüler ve sklerodermoid form (skleromiksödem de denir) ve daha selim prognozlu lokalize papüler form."Discrete" papüler müsinoz, Hepatit C virüs (HCV) ve insan immünyetmezlik virüs (HIV) infeksiyonu ile iliflkili olabilen lokalize formun nadir bir subtipidir.Bugüne kadar literatürde HCV veya HIV infeksiyonu ile iliflkisi olmayan sadece 12 olgu bildirimi vard›r. Burada vücu-dunda çok say›da, asemptomatik, deri renginde papülleri olan, histopatolojik incelemede dermal müsin birikimi tespit edilen, tiroid hastal›¤› ve monoklonal gammopati saptanmayan, viral belirleyicileri negatif olan 64 yafl›nda kad›n hastay› sunuyoruz. SummaryLichen myxoedematosus (synonym, papular mucinosis) is an uncommon, chronic, idiopathic disorder characterized by lichenoid papules, nodules and/or plaques due to dermal mucin deposition and a variable degree of fibrosis in the absence of thyroid dysfunction. Actually, lichen myxoedematosus includes two clinicopathologic subsets: a generalized papular and sclerodermoid form (also called scleromyxedema) with a monoclonal gammopathy and systemic, even lethal, manifestations and a localized papular form with non-disabling course. Discrete papular mucinosis is a rare subtype of the localized form and can be associated with hepatitis C virus and human immunodeficiency virus (HIV) infection. Only 12 cases unrelated to HCV or HIV infection have been described in the literature to date. Herein, we report a 64-year-old woman who presented with asymptomatic, flat, flesh-coloured papules on her neck, upper trunk and proximal extremities. A skin biopsy from a papule on her neck demonstrated dermal mucin deposition after alcian blue staining. The number of fibroblasts was increased. Laboratory studies revealed normal thyroid function tests. Serum protein electrophoresis did not show any evidence of a monoclonal gammopathy. Serology tests for HCV and HIV were negative. (Turkderm 2011; 45: 104-6)

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