Lichen myxoedematosus (classic type) L98.5

Author: Prof. Dr. med. Peter Altmeyer

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Last updated on: 21.04.2021

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Synonym(s)

Discrete lichen myxedematosus; lichen fibromucinoid; lichen myxedematosus; Mucinosis papulosa seu lichenoides; myxodermia papulosa; papular mucinosis; Papular mucinosis

History
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Dubreuilh, 1906, Montgomery and Underwood, 1953

Definition
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Very rare, papular, small-hearth-disseminated mucinosis in euthyroidism. The disease, also known as "discrete lichen myxedematosus" or "classical lichen myxoedematosus", is considered an independent, non-systemic minus variant of generalized (systemic) scleromyxedema; see below. Overview - Lichen myxoedematosus group).

The affected skin is not indurated, the face remains unaffected, paraproteinemia is not proven. Transitions to scleromyxoedema have not been described for the "discrete lichen myxoedematosus".

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Etiopathogenesis
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Unknown, fibroblast proliferation with accumulation of acidic mucopolysaccharides.

Manifestation
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Disease of adulthood, manifestation age between 30 and 40 years.

Efflorescence(s)
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Whitish, firm papules and plaques

Localization
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Especially the extensor sides of the arms, the back of the hands, trunk and thighs are affected.

Clinical features
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Integument: Frequently symmetrically distributed, non-pruritic, non-follicular, disseminated, 0.3- 0.4 cm large, lichenoid, soft or firm, skin-coloured, yellowish-white or yellowish-reddish papules, regionally also aggregated to plaques or confluent. It is not uncommon to see a linear arrangement of the nodules (see Fig.).

Optional associated symptoms: liver dysfunction, paraproteinemia, plasma cell infiltration of the bone marrow, plasmocytic lymphoma.

Histology
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Mucinous papules in circumscribed areas in the upper dermis. In some cases the epidermis is flattened, the retelepses have spread. Adnexae may be missing. Noticeable is the marked mucin proliferation with proliferation of fibroblasts (often with large star-shaped nuclei) in the upper and middle dermis. Vertebrally arranged collagen fibre bundles. Discrete lymphocytic infiltrates are localized perivascularly.

Differential diagnosis
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General therapy
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No causal therapy known. Tumor search is required. Treatment of a possible underlying systemic disease (e.g. plasmocytoma). Spontaneous healing is possible. Symptomatic therapy approaches should otherwise be provided with the necessary restraint (the disease is not life-threatening). S.a.u. scleromyxedema.

External therapy
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In circumscribed forms glucocorticoid crystal suspension intralesional (e.g. Volon A diluted 1:1 with LA, e.g. scandicain). Excision if necessary. Successes with dermabrasion andCO2 laser have been described.

Internal therapy
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  • Immunosuppressive therapy: if alkylating cytostatic drugs are used, consider the risk of secondary tumours. Successful treatments are described with melphalan (alkerane) and with cyclophosphamide (endoxane) 100-150 mg/day p.o. However, these are often accompanied by considerable NW (pancyto-, leuko-, thrombopenia).
  • Chlorambucil (Leukeran) 4-6 mg/day has proven to be better tolerated, especially with regard to long-term therapy.
  • On a trial basis, isotretinoin (e.g. isotretinoin-ratiopharm; acne normin) can also be used initially 0.5-1 mg/kg bw/day.

Progression/forecast
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Chronic course. Spontaneous healing is possible.

Literature
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  1. Adachi Y, Iba S, Horio T (2000) Successful treatment of lichen myxoedematosus with PUVA photochemotherapy. Photodermatol Photoimmunol Photomed 16: 229-231
  2. Bolton JG et al (2012) An interstitial granulomatous pattern in localized lichen myxedematosus with associated monoclonal gammopathy. J Cutan catheter 39:395-398
  3. de Cambourg G et al (2012) Atypical papular mucinosis with initial histological findings evocative of granuloma annulare. Ann Dermatol Venereol 139:58-62
  4. Dubreuilh W (1906) Fibromes miliares follicularies; scleroderma consecutive. Arch Dermatoll Syph 7: 569
  5. Montgomery H, Underwood LJ (1953) Lichen myxedematosus: Differentiation from cutaneous myxedemas or mucoid states. J Invest Dermatol 20: 213-233
  6. Podda M et al (2001) Cutaneous mucinosis of infancy: is it a real entity or the paediatric form of lichen myxoedematosus (papular mucinosis)? Br J Dermatol 144: 590-593
  7. Reserva J et al (2015) Discrete ('acral nonpapular') localised myxedematosus. BMJ Case Rep doi:10.1136/bcr-2015-209462
  8. Saez-Rodriguez M et al (2003) Localized lichen myxoedematosus (papular mucinosis) associated with morbid obesity: report of two cases. Br J Dermatol 148: 165-168
  9. Tam CC et al (2014) Discrete papular lichen myxedematosus with an unusualsegmental presentation. Acta Dermatovenerol Croat 22: 224-226

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Last updated on: 21.04.2021