Self-healing juvenile cutaneous mucinosis

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Self-healing juvenile cutaneous mucinosis
SpecialtyDermatology Edit this on Wikidata

Self-healing juvenile cutaneous mucinosis is a skin condition caused by fibroblasts producing abnormally large amounts of mucopolysaccharides, and is characterized by the sudden onset of skin lesions and polyarthritis.[1]: 185 

Signs and symptoms

Combinations of nodules affecting the head, neck, and periarticular (particularly interphalangeal) joints, as well as linearly arranged ivory white papules over erythematous indurated skin, are examples of cutaneous manifestations.[2]

Myalgia, pyrexia, and muscle exhaustion are examples of mild inflammatory symptoms that typically appear at the same time as cutaneous lesions. More significantly, although they are uncommon, inflammatory symptoms can be connected to cutaneous lesions. These consist of transient hypertension, arthritic and joint pain, and severe hand and joint edema. Lesions lack extracutaneous involvement and are linked to absent to mild inflammatory symptoms like arthralgia, fever, weakness, and muscle tenderness. Additional symptoms include swelling in the hands, elbows, and knees, as well as excruciating polyarthritis or momentary hypertension.[3]

Causes

It is still unclear what initially cause the overproduction of mucin and the rise in fibroblast production.[4][5] Some authors speculated that it might be because of an infection or inflammation that stimulates the antigenic response continuously at the level of initial fibroblast and mucin production.[6][7]

Diagnosis

MRI findings of myositis and subcutaneous tissue edema have been identified along with other abnormalities such as elevated aldolase levels, lymphocytosis, accelerated erythrocyte sedimentation rate, and antibodies against Bartonella.[8][9]

See also

References

  1. ^ James, William D.; Berger, Timothy G.; et al. (2006). Andrews' Diseases of the Skin: clinical Dermatology. Saunders Elsevier. ISBN 978-0-7216-2921-6.
  2. ^ Bishnoi, Anuradha; Jindal, Ankur Kumar; Anjani, Gummadi; Patra, Pratap Kumar; Chatterjee, Debajyoti; Vinay, Keshavamurthy; Kanwar, Amrinder J.; Dogra, Sunil; Singh, Surjit (April 22, 2020). "Self-healing juvenile cutaneous mucinosis, a sclerodermoid disorder simulating juvenile dermatomyositis: a case-based review". Rheumatology International. 40 (11). Springer Science and Business Media LLC: 1911–1920. doi:10.1007/s00296-020-04578-5. ISSN 0172-8172. PMID 32462254.
  3. ^ Geagea, Caroline; Youssef, Nour; Wakim, Gerard (January 16, 2019). "Self-Healing Juvenile Cutaneous Mucinosis: A Case Report in the Middle East". American Journal of Case Reports. 20. International Scientific Information, Inc.: 65–69. doi:10.12659/ajcr.910047. ISSN 1941-5923. PMC 6345112. PMID 30647403.
  4. ^ WADEE, S.; ROODE, H.; SCHULZ, E.J. (1994). "Self-healing juvenile cutaneous mucinosis in a patient with nephroblastoma". Clinical and Experimental Dermatology. 19 (1). Oxford University Press (OUP): 90–93. doi:10.1111/j.1365-2230.1994.tb01129.x. ISSN 0307-6938. PMID 8313651. S2CID 34931688.
  5. ^ Nagaraj, Lavanya V.; Fangman, William; White, Wain L.; Woosley, John T.; Prose, Neil; Selim, M. Angelica; Morrell, Dean S. (2006). "Self-healing juvenile cutaneous mucinosis: Cases highlighting subcutaneous/fascial involvement". Journal of the American Academy of Dermatology. 55 (6). Elsevier BV: 1036–1043. doi:10.1016/j.jaad.2006.06.024. ISSN 0190-9622. PMID 17110219.
  6. ^ Pucevich, Maria V.; Latour, Dana L.; Bale, George F.; King, Lloyd E. (1984). "Self-healing juvenile cutaneous mucinosis". Journal of the American Academy of Dermatology. 11 (2). Elsevier BV: 327–332. doi:10.1016/s0190-9622(84)70167-8. ISSN 0190-9622. PMID 6480937.
  7. ^ Rongioletti, Franco; Rebora, Alfredo (2001). "Updated classification of papular mucinosis, lichen myxedematosus, and scleromyxedema". Journal of the American Academy of Dermatology. 44 (2). Elsevier BV: 273–281. doi:10.1067/mjd.2001.111630. ISSN 0190-9622. PMID 11174386.
  8. ^ Kołodziejczyk, Beata; Gazda, Agnieszka; Hernik, Elżbieta; Szczygielska, Izabela; Rutkowska-Sak, Lidia; Koprowska, Marta Legatowicz (February 28, 2017). "Self-healing juvenile cutaneous mucinosis". Rheumatology. 55 (1). Termedia Sp. z.o.o.: 61–64. doi:10.5114/reum.2017.66689. ISSN 0034-6233. PMID 28386144.
  9. ^ Cowen, Edward W; Scott, Glynis A; Mercurio, Mary Gail (2004). "Self-healing juvenile cutaneous mucinosis". Journal of the American Academy of Dermatology. 50 (5). Elsevier BV: 97–100. doi:10.1016/j.jaad.2003.07.019. ISSN 0190-9622. PMID 15097940.

Further reading

  • Carder, K. Robin; Fitzpatrick, James E.; Weston, William L.; Morelli, Joseph G. (2003). "Self-Healing Juvenile Cutaneous Mucinosis". Pediatric Dermatology. 20 (1). Wiley: 35–39. doi:10.1046/j.1525-1470.2003.03008.x. ISSN 0736-8046. PMID 12558844. S2CID 23338330.
  • Kofler, Heinz; Lipsker, Dan; Maurer, Heiner; Burgdorf, Walter; Requena, Luis; Torrelo, Antonio; Zelger, Bernhard; Kutzner, Heinz; Müller, Hansgeorg (August 29, 2014). "Self-healing juvenile cutaneous mucinosis: challenging diagnosis and management". JDDG: Journal der Deutschen Dermatologischen Gesellschaft. 12 (9). Wiley: 815–817. doi:10.1111/ddg.12379. ISSN 1610-0379. PMID 25176461. S2CID 35875935.

External links