HistoryThis section has been translated automatically.
Montgomery and Underwood, 1953; Rongioletti et al. 1986.
DefinitionThis section has been translated automatically.
Rare (<50 cases reported in international literature), sporadic, papular mucinosis that is considered a topographically distinct, vatiante of localized lichen myxoedematosus. The disease is characterized by largely asymptomatic (only occasionally mild itching is reported), ivory to skin-colored, soft, 0.2-0.5 cm large, smooth-surfaced papules, especially on the backs of the hands and the extensor forearms.
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EtiopathogenesisThis section has been translated automatically.
Thyroid disorders, gammopathy, and lupus erythematosus are not expected in acral persistent papular mucinosis, as in all localized forms of lichen myxedematosus.
The coincidence with monoclonal gammopathy has been reported in a single case (Thatte SS et al.2015).
ManifestationThis section has been translated automatically.
w:m=3:1; in a Japanese review study, the male-to-female ratio was reported to be 5:7 (Mori A et al. 2021). The mean age of affected individuals is approximately 40 years (14-77 years).
LocalizationThis section has been translated automatically.
Distal forearms, backs of the hands, edges of the hands, extensor wrists
Clinical featuresThis section has been translated automatically.
Single or multiple, usually disseminated, often symmetrically distributed, 0.2-0.4 cm in size, transparent to skin-colored or also white-yellowish or also brown-yellowish, little symptomatic papules, with a smooth surface. Occasional formation of smaller plaques due to confluence. See below Lichen myxoedematosus.
In larger reviews a malignancy frequency is stated (uterine carcinoma, mammary carcinoma, thyroid carcinoma). However, this statement needs further verification as it is not confirmed in other reviews (Luo DQ et al. 2011).
HistologyThis section has been translated automatically.
Unchanged epidermis; underneath broad, band-shaped, usually clearly delimited laterally and to the depth against the normal dermis, impressing in the HE preparation as a loosened collagen structure. There are no inflammatory infiltrates. Distally, there is again a normal collagen structure. Fibroblastic proliferation is variable but normally absent. In the Alcian blue staining the mucinous zone impresses by a distinct reactivity (see Fig.).
Differential diagnosisThis section has been translated automatically.
The differential diagnosis includes:
- Granuloma anulare: annular firm reddish surface smooth papules.
- Molluscum contagiosum: skin-colored bifurcated papules, eruptive appearance.
- Keratoelastoidosis marginalis of the hands: affects the lateral edges of the hands and fingers, small 0.1cm skin color, firm nodules
- Lichen amyloidosus: rarely on the forearms or hands. Concerns mainly the lower legs. Itching!
- Malignant atrophic papulosis (Köhlmeier-Degos disease): usually life-threatening systemic disease. Central spatter-like necrotic red papules. Often acute abdomen
Remark: Based on the localization of the lesions and the findings in histopathology (alcian blue staining), the diagnosis can be easily made.
TherapyThis section has been translated automatically.
The lesions of "acral persistent papular mucinosis" are limited to the skin; it is ultimately only a "cosmetic problem" with a good prognosis and without systemic involvement. Therefore, a wait-and-see approach may also be recommended (Rongioletti F 2006). S.a. Lichen myxoedematosus.
Topical and intralesional glucocorticoids have been used with variable success. However, this therapy is not recommended.
Individual papules can be removed electrocaustically or by ablative laser. Healing is generally without complications. Recurrences are possible.
Progression/forecastThis section has been translated automatically.
Case report(s)This section has been translated automatically.
A 31-year-old woman presented with a 10-year history of multiple, asymptomatic, symmetric, discrete papules on dorsum of hands and extensor forearms. The lesions started on the hands, slowly increased in size, and were persistent. They were not related to sun exposures. Apart from removal of a benign thyroid tumor six years earlier, there was no known history of other medical problems. The results of laboratory tests, including routine blood tests such as those of liver enzymes, renal function, thyroid profile, serum protein analysis, immunoglobulin levels, and autoantibody profile were o.b.. The patient denied previous trauma to the affected areas and recent contact with topical medications. No other family member was similarly affected (Luo DQ et al. 2011).
LiteratureThis section has been translated automatically.
- Alvarez-Garrido H et al (2014) Acral persistent papular mucinosis: is it an under-diagnosed disease? Dermatol Online J PubMed PMID: 24656271.
- Coulson IH et al (1992) Acral persistent papular mucinosis. Br J Dermatol 126: 283-285.
- Di-Qing Luo et al (2011) Acral persistent papular mucinosis: case report and review of the literature. JDDG 9: 354-359
- Fernández DG et al.(2014) Adult variant of self-healing papular mucinosis in a patient treated with interferon α2a. Indian J Dermatol Venereol Leprol 80:184-186.
- Gartner S et al (1998) Acral papular lichen myxedematosus. Dermatologist 49: 855-858
- Gómez Sánchez ME et al (2016) Acral papular mucinosis: a new case of this rare entity. An Bras Dermatol 91(5 suppl 1):111-113.
- Jackson EM et al (2002) Diffuse cutaneous mucinoses. Dermatol Clin 20: 493-501.
- Jun JY et al (2016) Acral persistent papular mucin.
- Luo DQ et al (2011) Acral persistent papular mucinosis: a case report and literature review. J Dtsch Dermatol Ges 9:354-359.
- Montgomery H et al (1953) Lichen myxedematosus. J Invest Dermatol 20: 213-236.
- Mori A et al (2021) Acral persistent papular mucinosis: A case report and summary of 24 Japanese cases. J Dermatol 48:1574-1578.
- Rongioletti F et al (2001) Updated classification of papular mucinosis, lichen myxedematosus, and scleromyxedema. J Am Acad Dermatol 44: 273-281.
- Rongioletti F et al (1986) Acral persistent papular mucinosis: a new entity. Arch Dermatol 122: 1237-1239
- Rongioletti F (2006) Lichen myxedematosus (papular mucinosis): new concepts and perspectives for an old disease. Semin Cutan Med Surg 25: 100-104.
- Rongioletti F et al (2021) Acral persistent papular mucinosis. Clin Dermatol 39:211-214.
- Thatte SS et al.(2015) Papular mucinosis associated with monoclonal gammopathy of unknown significance. Indian J Dermatol Venereol Leprol 81:213-214.
- Zeng YP et al (2012) Atypical case of discrete papular mucinosis involving acral and nuchal regions in an adult. JDDG 10: 266-267
Incoming links (4)Angiomyxoma cutaneous; Lichen myxedematosus and HIV-Infection ; Nodular lichen myxedematosus; Self-healing infantile cutaneous mucinosis;
Outgoing links (6)Contagious mollusc; Granuloma anulare (overview); Keratoelastoidosis marginalis; Kohlmeier Degos disease ; Lichen amyloidosis; Lichen myxoedematosus discrete type ;
Please ask your physician for a reliable diagnosis. This website is only meant as a reference.