Solitary cutaneous focal mucinosis L98.5

Last updated on: 23.09.2023

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History
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Cutaneous focal mucinosis was first described by Johnson and Helwig in 1966 (Johnson and Helwig, 1966). In 2004, Chen et al. referred to the disease as solitary soft fibroma-like polypoid mucinosis. In 2016, it was recommended to refer to these lesions as "solitary cutaneous focal mucinosis" to distinguish it from "multiple cutaneous focal mucinosis," which may be associated with systemic disease (Kuo KL et al. 2017).

Definition
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Cutaneous focal mucinosis is a rare, "pseudotumorous" papular or even nodular skin disease characterized by localized (inflammatory or degenerative) deposits of mucin, a hyaluronic acid complex in the dermis.

The lesion typically presents as an isolated, asymptomatic, unspectacular papule on the extremities or back and in most cases proves to be an incidental hsitologic finding. It is not associated with systemic disease.

Occurrence/Epidemiology
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Etiopathogenesis
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Unclear; unlike multiple cutaneous focal mucinosis, this is a localized skin disease not associated with "mucinosis-associated systemic disease." Traumatic cutaneous focal mucinosis is known (Kempf W et al. 2014; Verma G et al. 2018).

Manifestation
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Adults aged 29 to 60

Localization
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V.a. extremities, with arms more commonly affected than legs. Lesions on the head, neck, and trunk have been described.

Clinical features
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Characteristic is an asymptomatic flesh-colored, whitish but also red papule, usually smooth on the surface.

Laboratory
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Unlike multiple cutaneous focal mucinosis, solitary lesions are usually not associated with systemic disease; therefore, no further laboratory testing is required in these individuals (Cohen PR et al 2020).

Histology
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The predominant pathologic feature of cutaneous focal mucinosis is unencapsulated mucin, a hyaluronic acid complex, in the upper dermis. It can be visualized as mild basophilic staining in sections stained with hematoxylin and eosin (HE). Although the diagnosis can be made on sections stained with HE, stains that detect mucin (such as alcian blue, colloidal iron, and toluidine blue) can be helpful in confirming the diagnosis.

Therapy
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Excision; recurrences are not observed.

Note(s)
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Cutaneous focal mucinosis can occur as a solitary skin lesion (solitary cutaneous focal mucinosis). However, it also occurs as a multiple variant (multiple cutaneous focal mucinosis), but in these cases it is usually associated with systemic disease (Kuo KL et al. 2017).

The oral counterpart of cutaneous solitary focal mucinosis is called oral (solitary) focal mucinosis.

Literature
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  1. Biondo G et al (2019) Clinical, dermoscopic, and histologic aspects of two cases of cutaneous focal mucinosis. An Bras Dermatol 94:334-336.
  2. Chen HH et al (2004) A solitary soft fibroma-like polypoid mucinosis: report of an unusual case. Chen HH, Chung CJ, Kuo TT, Hong HS. Dermatol Surg 30:450-451.
  3. Erickson CP et al (2020) Case report and review of solitary cutaneous focal mucinosis: a unique primary cutaneous mucinosis unrelated to mucinosis-associated systemic diseases. Dermatol Online J 26
  4. Gutierrez N et al (2021) Solitary cutaneous focal mucinosis. Cureus 13:e18618.
  5. Jakubovic HR et al (1982) Multiple cutaneous focal mucinoses with hypothyroidism. Ann Intern Med 96:56-58.
  6. Johnson WC et al (1966) Cutaneous focal mucinosis. A clinicopathological and histochemical study. Johnson WC, Helwig EB. Arch Dermatol 93:13-20.
  7. Kempf W et al (20q14) Trauma-induced cutaneous focal mucinosis of the mammary areola: an unusual presentation. Dermatopathology (Karger) 1: 24-8.
  8. Kuo KL et al (2017) Solitary cutaneous focal mucinosis: a clinicopathological study of 11 cases of soft fibroma-like cutaneous mucinous lesions. J Dermatol 44:335-338.
  9. Lindor NM et al (2001) Birt-Hogg-Dube syndrome: an autosomal dominant disorder with predisposition to cancers of the kidney, fibrofolliculomas, and focal cutaneous mucinosis. Int J Dermatol 40:653-656.
  10. Rongioletti F et al (2001) Cutaneous mucinoses: microscopic criteria for diagnosis. Am J Dermatopathol. 23:257-267.
  11. Schneider BV et al (1991) Nodular cutaneous mucinosis--an unusual multiple type of euthyreotic focal mucinosis. Dermatologica 183:73-76.
  12. Truhan AP et al (1986) The cutaneous mucinoses. J Am Acad Dermatol14:1-8.
  13. Verma G et al (2018) Trauma-induced focal nodular mucinoses: a rare entity. Indian Dermatol Online J 9:50-52.

Disclaimer

Please ask your physician for a reliable diagnosis. This website is only meant as a reference.

Last updated on: 23.09.2023