Journal of the American Academy of Dermatology
Clinical ReviewUpdated classification of papular mucinosis, lichen myxedematosus, and scleromyxedema☆,☆☆
Section snippets
Scleromyxedema
Scleromyxedema is an uncommon disease; approximately 114 cases have been reported in the Englishlanguage literature. Our experience deals with 3 cases (two men and one woman). It typically affects middle-aged adults without sex predilection. A widespread symmetric eruption of 2 to 3 mm, firm, waxy, closely spaced papules is most commonly located on the hands, forearms, face, neck, upper trunk, and thighs (Fig 1).
Localized LM or papular mucinosis
Clinically the patients exhibit small, firm, waxy papules (or nodules and plaques produced by the confluence of papules) confined to few sites only (usually upper and lower limbs and trunk). Histopathologic examination reveals mucin deposition with variable fibroblast proliferation without sclerotic features, paraproteinemia, systemic involvement, and thyroid disease.
We identify 5 subtypes: a discrete form, acral persistent papular mucinosis, self-healing papular mucinosis, papular mucinosis of
Atypical (intermediate) forms of LM (papular mucinosis)
Some cases of LM share intermediate or atypical features. There are cases of localized LM, for example, showing scleromyxedema-like systemic involvement, such as dysphagia, hoarseness, pulmonary disturbances, and carpal tunnel syndrome60 or myositis61 without skin sclerosis or paraproteinemia. One case of localized nodular mucinosis was associated with myalgias, carpal tunnel syndrome, and deep venous thrombosis was diagnosed as sclero-myxedema.62 Rare cases of localized LM are associated with
Discussion
Although the classification of Montgomery and Underwood1 deserves credit for distinguishing LM from myxedema and scleroderma, it should be updated, not least because these authors detected 4 variants on the basis of only 3 cases. Their first and second variants correspond to scleromyxedema and to the discrete papular form, respectively. The third variant, which is described as localized to generalized lichenoid plaques, is probably a localized cutaneous form in which the papules coalesced into
References (75)
- et al.
Lichen myxedematosus (differentiation from cutaneous myxedemas or mucoid states)
J Invest Dermatol
(1953) - et al.
Scleromyxedema
J Am Acad Dermatol
(1995) - et al.
Fatal scleromyxedema: report of a case and review of the literature
J Am Acad Dermatol
(1998) - et al.
The new cutaneous mucinoses:a review with an up-to-date classification of the cutaneous mucinoses
J Am Acad Dermatol
(1991) - et al.
The association of potentially lethal neurologic syndromes with scleromyxedema (papular mucinosis)
J Am Acad Dermatol
(1993) - et al.
Scleromyxedema associated with scleroderma, renal disease, and acute psychosis
J Am Acad Dermatol
(1986) - et al.
Scleromyxedema
J Am Acad Dermatol
(1999) - et al.
Ophthalmic findings in scleromyxedema
Ophthalmology
(1994) - et al.
Scleromyxedema: treatment with interferon alfa
J Am Acad Dermatol
(1999) - et al.
Worsening of lichen myxedematosus during interferon alfa-2a therapy for chronic active hepatitis C
J Am Acad Dermatol
(1998)
Acral persistent papular mucinosis
J Am Acad Dermatol
Multiple periorbital cutaneous myxomas progressing to scleromyxedema
J Am Acad Dermatol
Skleromyxedema
Arch Dermatol Syphilol
Papular mucinosis
Int J Dermatol
Acral persistent papular mucinosis: a new entity
Arch Dermatol
Papular mucinosis: a clinicopathologic study of four patients
Arch Dermatol
The dermal mucinoses
Adv Dermatol
Scleromyxedema: a scleroderma-like disorder with systemic manifestations
Medicine
Normalization of skin appearence in a patient with scleromyxedema after intensive chemotherapy for Hodgkin's disease
Dermatologica
Scleromyxedema and severe myositis
Int J Dermatol
Papular mucinosis, destructive arthropathy, median neuropathy and sicca complex
Clin Rheumatol
Atteinte articulaire spécifique au cours d'une mucinose papuleuse
Ann Dermatol Venereol
Scleromyxedema with systemic involvement mimics rheumatic diseases
Arthritis Rheum
Autopsy findings in lichen myxedematosus
Arch Dermatol
Scleromyxédème avec modifications laryngées
Ann Dermatol Venereol
Further observations on lichen myxedematosus
Ann Intern Med
Scleromyxedema
Cutis
Lichen myxedematosus serum stimulates human skin fibroblast proliferation
Science
Lichen myxedematosus (scleromyxedema) serum stimulates hyaluronic acid and prostaglandin E production by human fibroblasts
J Rheumatol
Scleromyxedema: systemic manifestations and cosmetic improvement from dermabrasion
Cutis
Regression of scleromyxedema with topical betamethasone and dimethyl sulfoxide: a 30-month follow-up
Arch Dermatol
Beneficial effect of granulocyte-colony stimulating factor in scleromyxedema associated with severe idiopathic neutropenia
Br J Dermatol
Spontaneous improvement of scleromyxedema
Clin Exp Dermatol
Fibromes miliaries folliculaires: sclérodermie consécutive
Arch Dermatol Syphilol
Collagenome eruptif: a separate entity?
Br J Dermatol
Papular mucinosis in chronic psoriatic erythroderma
Arch Dermatol
Cited by (373)
Case for diagnosis. Multiple infiltrated plaques in a patient with human immunodeficiency virus and hepatitis C co-infection: lichen myxedematosus
2023, Anais Brasileiros de DermatologiaA Pseudovesicular Plaque on the Shoulder
2023, Actas Dermo-SifiliograficasAn unusual case of granulomatous scleromyxedema
2022, JAAD Case ReportsMonoclonal gammopathy of clinical significance: what the rheumatologist needs to know
2022, The Lancet RheumatologyPlaque-like cutaneous mucinosis in a patient with KRAS-mutated giant cell granuloma of the jaw
2024, International Journal of DermatologyUnusual variant of scleromyxedema presenting with severe systemic involvement and atypical adnexal proliferations – A histological pitfall with the risk of unnecessary surgeries
2024, JDDG - Journal of the German Society of Dermatology