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]
| Self-healing juvenile cutaneous mucinosis | |
|---|---|
| Specialty | Dermatology  |
Signs and symptoms edit
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 edit
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 edit
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 edit
References edit
- ^ James WD, Berger TG, Elston DM, Neuhaus IM, Micheletti RG (2016). "Chapter 9: Mucinoses: Lichen Myxedematosus". Andrews' Diseases of the Skin: Clinical Dermatology (12th ed.). Philadelphia: Saunders Elsevier. pp. 179–182. ISBN 978-0-323-31967-6.
- ^ Bishnoi A, Jindal AK, Anjani G, Patra PK, Chatterjee D, Vinay K, et al. (November 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. PMID 32462254.
- ^ Geagea C, Youssef N, Wakim G (January 2019). "Self-Healing Juvenile Cutaneous Mucinosis: A Case Report in the Middle East". The American Journal of Case Reports. 20. International Scientific Information, Inc.: 65–69. doi:10.12659/ajcr.910047. PMC 6345112. PMID 30647403.
- ^ Wadee S, Roode H, Schulz EJ (January 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. PMID 8313651. S2CID 34931688.
- ^ Nagaraj LV, Fangman W, White WL, Woosley JT, Prose N, Selim MA, et al. (December 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. PMID 17110219.
- ^ Pucevich MV, Latour DL, Bale GF, King LE (August 1984). "Self-healing juvenile cutaneous mucinosis". Journal of the American Academy of Dermatology. 11 (2 Pt 2). Elsevier BV: 327–332. doi:10.1016/s0190-9622(84)70167-8. PMID 6480937.
- ^ Rongioletti F, Rebora A (February 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. PMID 11174386.
- ^ Kołodziejczyk B, Gazda A, Hernik E, Szczygielska I, Rutkowska-Sak L, Koprowska ML (February 28, 2017). "Self-healing juvenile cutaneous mucinosis". Reumatologia. 55 (1). Termedia Sp. z.o.o.: 53–56. doi:10.5114/reum.2017.66689. PMID 28386144.
- ^ Cowen EW, Scott GA, Mercurio MG (May 2004). "Self-healing juvenile cutaneous mucinosis". Journal of the American Academy of Dermatology. 50 (5 Suppl). Elsevier BV: S97-100. doi:10.1016/j.jaad.2003.07.019. PMID 15097940.
Further reading edit
- Carder KR, Fitzpatrick JE, Weston WL, Morelli JG (2003). "Self-healing juvenile cutaneous mucinosis". Pediatric Dermatology. 20 (1). Wiley: 35–39. doi:10.1046/j.1525-1470.2003.03008.x. PMID 12558844. S2CID 23338330.
- Kofler H, Lipsker D, Maurer H, Burgdorf W, Requena L, Torrelo A, et al. (September 2014). "Self-healing juvenile cutaneous mucinosis: challenging diagnosis and management". Journal der Deutschen Dermatologischen Gesellschaft = Journal of the German Society of Dermatology. 12 (9). Wiley: 815–817. doi:10.1111/ddg.12379. PMID 25176461. S2CID 35875935.