Mucinosis cutaneous (overview) L98.5

Author: Prof. Dr. med. Peter Altmeyer

All authors of this article

Last updated on: 31.10.2023

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dermal mucinosis; Dermal mucinosis; Mucinosis; Myxoderma

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Group of etiologically and clinically heterogeneous diseases with focal or diffuse accumulation of glycosaminoglycans(mucins), a mucus-like substance already physiologically present in the skin. The pathogenesis of mucinoses is usually unclear.

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A Primary cutaneous mucinoses

A1 Primary dysthyroid mucinoses

A2 Primary eutyhreote diffuse cutaneous mucinoses

A3 Primary euthyroid, neoplastic/naevoid/degenerative (localized) cutaneous mucinoses.

A4 Primary euthyroid follicular cutaneous mucinosis

B Secondary cutaneous mucinosis (increased cutaneous mucin deposition in various primary "non-mucinous" diseases, especially in collagenoses+various tumors (modified after Rongioletti and Rebora 2001a):

B1: Secondary cutaneous mucinosis (associated phenomenon with various tumors)

B2 Secondary cutaneous mucinosis (associated with autoimmune diseases + various other pathologies)

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The extracellular matrix of the dermis consists of collagenous and elastic fibers and an interfibrillar ground substance. This is composed of various glycoproteins, water and salt. The main component of this basic substance are the proteoglycans (also known as mucins), which are bound to glycosaminoglycans via their protein content with a filamentous, central core (core protein). The proteoglycan molecules consist mainly of hyaluronic acid, dermatan sulfate, chondroitin-4-sulfate and chondroitin-6-sulfate. The dermis obtains its particular consistency through hydrostatic interactions of these components. When glycosaminoglycan metabolism is disturbed, quantitative and/or qualitative shifts in the extracellular matrix of the dermis can occur, often noticeable by changes in the consistency of the dermis.

An overproduction of glycosaminoglycans can be triggered, for example, by thyroid diseases, by immunological disorders or by viral infections (HIV, Hepatis C).

It is remarkable that in cultured fibroblasts collagen and glycosaminoglycan production can be increased by recombinant interleukin-6 (Duncan MR et al. 1991).

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The complex classification of cutaneous mucinoses listed here, follows several principles at the same time. First, it is basically divided into

  • A) Primary (idiopathic) cutaneous mucinoses


  • B) Secondary cutaneous mucinoses

are subdivided. "Primary cutaneous mucinoses", are defined as those diseases in which pathological primary dermal mucin production is proven to be the causative, pathogenetic principle. Furthermore, primary cutaneous mucinoses are classified with respect to thyroid functions in:

  • Euthyroid primary cutaneous mucinoses


  • dysthyroid primary cutaneous mucinoses


Thegroup of dysthyroid primary cutaneous mucinoses is well manageable with 3 clinical representatives, essentially comprises the myxedema group, whereby dermatologically the mostly hyperthyroid, localized myxedema (mostly pretibially localized, therefore "pretibial myxedema", more rarely localized on the face, shoulders, abdomen) play the largest clinical role.

The group of euthyroid primary (distinct) cutaneous mucinoses , on the other hand, is extensive, clinically heterogeneous. The representatives of the extensive lichen myxedematosus group stand out as inflammatory diseases. The most prominent representative of this group is scleromyxoedema, which is listed in the new classifications as "generalized paraproteinemic lichen myxoedematosus". This renaming is intended to highlight the pathogenetic relationship to several other localized members of the localized lichen myxoedematosus group. "Scleromyxedema Arndt-Gottron of the historical nomenclature," is the rare, often fatal, systemic variant of lichen myxoedematosus with characteristic skin-colored lichenoid papules, areal thickening and induration of the skin with storage of mucins, and markedly increased fibrosis of the dermis. Facial involvement results in peculiar limitation of facial expression. Systemic considerations include cardiovascular involvement, as well as myopathies and neurologic symptoms. Monoclonal paraproteinemia (MGUS) is frequently present (>90%), usually of the IgG1 lambda type, less commonly of the IgG kappa type. The etiologic relationships between mucinosis and paraproteinemia have not yet been established.

The group of non-generalized, focal or attenuated (discrete lichen myxoedematosus) lichen myxoedematosus variants isarbitrarily composed, once according to localization (acral persistent papular mucinosis) according to clinical and morphological criteria (nodular lichen myxoedematosus, self-healing cutaneous mucinosis, discrete lichen myxoedematosus) or according to etiologies(HIV-associated (HCV-associated lichen myxoedematosus, "toxic oil syndrome", scleromyxedema in renal disease).In fact, the similarities are essentially found in the concordant histological findings. In other words, the individual variants cannot be differentiated on the basis of the fine tissue, so that the final diagnosis can only be made on the basis of histology, clinical findings and the course of the disease.

Other euthyroid primary diffuse cutaneous mucinoses: Another clinically important clinical picture, which also belongs to the group of euthyroid primary cutaneous mucinoses with diffusely interstitially distributed mucin deposits, but not to the lichen myxedematosus group, is scleroedema adultorum, which is often also diabetogenically triggered. Compared to the representatives of lichen myxoedematosus, clinically the papular component is completely absent. The skin is uniformly thickened, somewhat purple shimmering, with a "peau d`orange" surface. Histologically, there are distinct differences from lichen myxoedematosus in that fibroblast proliferation is absent. The thickened, window-like dispersed collagen fibers are more reminiscent of late-stage scleroderma. Scleroedema adultorum Buschke occurs in 3 variants:

  • Type I mainly after streptococcal infections (often self-limiting in younger women and children)
  • Type II in patients with monoclonal gammopathy (middle-aged men)
  • Type III in association with diabetes mellitus in middle-aged men.

Of clinical note, the "self-healing juvenile cutaneous mucinosis" described by pediatricians, which is listed in the lichen myxoedematosus group, takes a clinical course quite similar to that of type I Buschke's scleroedema. This disease also begins febrile, accompanied by arthralgias and arthritides, muscle pain, and generalized weakness. Conspicuous are areal periorbital edema reminiscent of juvenile dermatomyositis.

The REM syndrome (reticular erythematousmucinosis) described by Gerd-Klaus Steigleder in 1974, and its 14 years older sister, the "plaque-like form of cutaneous mucinosis" described by the American dermatologist Harald Otto Perry, also belong to the group of euthyroid primary, diffuse, cutaneous mucinoses (note: probably they are one and the same entity). Both clinical pictures are a "black box" regarding their etiopathogenesis. An increased photosensitivity, a viral induction but also immunological triggers are discussed. An associated lupus erythematosus has to be clarified as well as an HIV/HCV infection. Chloroquine and hydroxychloroquine are used therapeutically for idiopathic forms.

Primary euthyroid follicular cutaneous mucinoses: Primary euthyroid follicular cutaneous mucinoses are a category all their own. Here, the follicular epithelia and not the fibroblasts are causative for mucin formation. Again, the statement that the clinical diagnosis is based solely on a histologic phenomenon that can occur as an epiphenomenon in a number of inflammatory diseases is valid. On the one hand, in children and young adults, this histopathologic feature may conceal harmless, usually localized, follicular inflammatory disease patterns. In combination with clinically conspicuous hair loss, the clinical picture is called alopecia mucinosa. A different clinical emphasis is given to disseminated mucinosis follicularis, which may occur as a manifestation of folliculotropic cutaneous T-cell lymphoma (see folliculotropic mycosis fungoides below).

Secondary cutaneous mucinoses. In the so-called secondary cutaneous mucinoses, one cannot speak of autochthonous "mucinous disease patterns". It is a heterogeneous collection of skin diseases in which secondary mucinous deposits occur. The commonality of this group thus refers to a histological phenomenon without specific clinical relevance.

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Detection of mucins by Hale reaction or Alzian blue staining. If mucinosis is suspected, the specimen should be fixed in absolute alcohol with 1% formalin.

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Clinical picture; histological evidence of mucin deposits in the skin.

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See below the clinical pictures listed in the tables.

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Classification of mucinoses

Classification n. Etiology

Disease pattern

Mucinosis in hypothyroidism

Diffuse myxoedema

Circumscribed myxoedema

Mucinosis in hyperthyroidism

Myxoedema circumscriptum symmetricum praetibiale

Mucinosis in euthyroidism

Lichen myxoedematosus (further classification see there)

Acral persistent papular mucinosis (special form of lichen myxoedematosus)

Scleromyxoedema (Arndt-Gottron)

REM syndrome

Plaque-like form of cutaneous mucinosis (Perry)

Myxoma (cutaneous focal mucinosis)

Scleroedema adultorum Buschke

Mucinosis follicularis

Primary mucinosis

Idiopathic mucinosis (see above, in euthyroidism)

Secondary mucinosis

After inflammatory diseases, e.g. eczema, psoriasis, also in tumors (fibroma, lipoma, liposarcoma, myxosarcoma, basalioma)

Mucinoses in the narrower sense

Diffuse myxoedema

Circumscript myxoedema

Myxoedema circumscriptum symmetricum praetibiale

Lichen myxoedematosus

Scleromyxoedema (Arndt-Gottron)

REM syndrome

Plaque-like form of cutaneous mucinosis (Perry)

Myxoma, cutaneous (cutaneous focal mucinosis)

Mucinosis, cutaneous, infantile

Mucinosis in a broader sense

Scleroedema adultorum Buschke

Mucinosis follicularis

Epithelial mucinosis

Mucinosis follicularis (Mißnomen)

Spongiotic dermatitis (!)

"Dermal reactive mucinosis" (mucin deposits as epiphenomenon)

In: lupus erythematosus, dermatomyositis, scleroderma, granuloma anulare, mycosis fungoides, pseudolymphoma, papulosis maligna atrophicans, mucinosis in basal cell carcinoma, neural tumors.

Localized mucinosis

Circumscript myxoedema

Myxoedema circumscriptum symmetricum praetibiale

Myxoma, cutaneous (cutaneous focal mucinosis)

Nevus mucinosus

Dorsal cyst, mucoid

Mucinosis, cutaneous, infantile

Mucous granuloma (mucocele)

Diffuse mucinosis

Lichen myxoedematosus

Scleromyxoedema (Arndt-Gottron)

REM syndrome

Plaque-like form of cutaneous mucinosis (Perry)

Diffuse myxoedema

Scleroedema adultorum Buschke

Mucinosis follicularis

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Mucin consists of a mixture of glycosaminoglycans (mainly hyaluronic acid and dermatan sulphate), which are either bound to proteins (proteoglycans) or occur freely (hyaluronic acid). Glycosaminoglycans are formed either in fibroblasts or in keratinocytes. In case of disturbances of the glycosaminoglycan metabolism (increased production/reduced degradation), a qualitative or quantitative change of the balance occurs.

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Last updated on: 31.10.2023