Self-healing juvenile cutaneous mucinosis explained

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]

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

• Lichen myxedematosus
• Skin lesion

Further reading

• Carder KR, Fitzpatrick JE, Weston WL, Morelli JG . Self-healing juvenile cutaneous mucinosis . Pediatric Dermatology . 20 . 1 . 35–39 . 2003 . 12558844 . 10.1046/j.1525-1470.2003.03008.x . none . Wiley . 23338330 .
• Kofler H, Lipsker D, Maurer H, Burgdorf W, Requena L, Torrelo A, Zelger B, Kutzner H, Müller H . Self-healing juvenile cutaneous mucinosis: challenging diagnosis and management . Journal der Deutschen Dermatologischen Gesellschaft = Journal of the German Society of Dermatology . 12 . 9 . 815–817 . September 2014 . 25176461 . 10.1111/ddg.12379 . none . Wiley . 35875935 .

Notes and References

1. Book: James WD, Berger TG, Elston DM, Neuhaus IM, Micheletti RG . Chapter 9: Mucinoses: Lichen Myxedematosus . 179–182 . Andrews' Diseases of the Skin: Clinical Dermatology . Philadelphia . Saunders Elsevier . 2016 . 978-0-323-31967-6 . 12th .
2. Bishnoi A, Jindal AK, Anjani G, Patra PK, Chatterjee D, Vinay K, Kanwar AJ, Dogra S, Singh S . Self-healing juvenile cutaneous mucinosis, a sclerodermoid disorder simulating juvenile dermatomyositis: a case-based review . Rheumatology International . 40 . 11 . 1911–1920 . November 2020 . 32462254 . 10.1007/s00296-020-04578-5 . Springer Science and Business Media LLC .
3. Geagea C, Youssef N, Wakim G . Self-Healing Juvenile Cutaneous Mucinosis: A Case Report in the Middle East . The American Journal of Case Reports . 20 . 65–69 . January 2019 . 30647403 . 6345112 . 10.12659/ajcr.910047 . International Scientific Information, Inc. .
4. Wadee S, Roode H, Schulz EJ . Self-healing juvenile cutaneous mucinosis in a patient with nephroblastoma . Clinical and Experimental Dermatology . 19 . 1 . 90–93 . January 1994 . 8313651 . 10.1111/j.1365-2230.1994.tb01129.x . Oxford University Press (OUP) . 34931688 .
5. Nagaraj LV, Fangman W, White WL, Woosley JT, Prose N, Selim MA, Morrell DS . Self-healing juvenile cutaneous mucinosis: cases highlighting subcutaneous/fascial involvement . Journal of the American Academy of Dermatology . 55 . 6 . 1036–1043 . December 2006 . 17110219 . 10.1016/j.jaad.2006.06.024 . Elsevier BV .
6. Pucevich MV, Latour DL, Bale GF, King LE . Self-healing juvenile cutaneous mucinosis . Journal of the American Academy of Dermatology . 11 . 2 Pt 2 . 327–332 . August 1984 . 6480937 . 10.1016/s0190-9622(84)70167-8 . Elsevier BV .
7. Rongioletti F, Rebora A . Updated classification of papular mucinosis, lichen myxedematosus, and scleromyxedema . Journal of the American Academy of Dermatology . 44 . 2 . 273–281 . February 2001 . 11174386 . 10.1067/mjd.2001.111630 . Elsevier BV .
8. Kołodziejczyk B, Gazda A, Hernik E, Szczygielska I, Rutkowska-Sak L, Koprowska ML . Self-healing juvenile cutaneous mucinosis . Reumatologia . 55 . 1 . 53–56 . February 28, 2017 . 28386144 . 10.5114/reum.2017.66689 . Termedia Sp. z.o.o. .
9. Cowen EW, Scott GA, Mercurio MG . Self-healing juvenile cutaneous mucinosis . Journal of the American Academy of Dermatology . 50 . 5 Suppl . S97-100 . May 2004 . 15097940 . 10.1016/j.jaad.2003.07.019 . Elsevier BV .