JAMA DERMATOLOGY PATIENT PAGE Morphea (Localized Scleroderma)

Morphea is an autoimmune disease that causes sclerosis, or scarlike, changes to the skin.

Autoimmune diseases occur when the immune system, which nor- morpheaorlinearmorpheathatinvolvesjusttheskin.Generalizedmor- mally protects us from bacteria, viruses, and fungi, mistakenly at- phea,ormorpheathatinvolvesthemuscleandbone,isusuallytreated tacks a person’s own body. Morphea lesions have 5 main presenta- with phototherapy (regular exposure to UV light in a special machine) tions: (1) circumscribed (few circles on the trunk or limbs); or a combination of oral steroids and methotrexate (medications that (2) generalized (many circles on the trunk and limbs); (3) linear (lines suppress the immune system). of involvement on the limbs or head); (4) mixed (combination of cir- cumscribed and linear or generalized and linear); and (5) panscle- rotic (involvement of all of the skin). Morphea is usually limited to Morphea (localized scleroderma) the skin, but it may extend deeper to involve muscle or bone. Mor- Typical progression of skin lesions Examples of morphea phea may also involve the inside of the mouth, the genitals, and the Affected skin begins as a Circumscribed eyes. Morphea often first occurs in childhood or middle adulthood. discolored area (red or purple) (few patches) or that hardens and thickens. generalized (many patches) on Treatment decreases the risk of forming new lesions and the expan- trunk, arms or legs sion of lesions. There is currently no cure for morphea.

Symptoms Morphea is usually asymptomatic, with occasional itch and rarely pain. Morphea usually begins as a red or purple area of skin that then The area spreads into a thickened oval and develops a whitish center. becomes thickened and white. The thick white areas usually thin out over time and turn brown. Once a lesion has formed, it is unlikely to completely go away.In children, linear morphea on the arms and legs Linear (typically on arms and legs where can affect the underlying bone and interfere with bone growth. Lin- it may affect joint ear morphea on the head (also called en coup de sabre) may extend mobility, or on head) inwards to the brain and cause seizures. If morphea crosses a joint, Gradually the area thins and the thickening may limit joint movement. Morphea tends to have a tightens, leaving the skin darker. waxing and waning course.

Diagnosis Morphea is diagnosed based on findings of skin examination and skin biopsy. Patients with morphea do not have Raynaud phenomenon (fingers turning red, white, and blue with cold exposure), redness and swelling of both hands (red puffy hands), changes in the blood vessels along the nail edge (nailfold capillary changes), or tighten- ing of the skin of the fingers (sclerodactyly). Patients who have these FOR MORE INFORMATION findings are likely to have systemic sclerosis, also called sclero- • Medscape Reference derma. Systemic sclerosis and morphea are different diseases. Pa- emedicine.medscape.com/article/1065782-overview tients with morphea are not at increased risk of developing sys- • Scleroderma Care Foundation temic sclerosis. sclerodermatt.org/articles/better-health/129-morphea -scleroderma Treatment Treatments help to control itch and decrease the growth and forma- To find this and previous JAMA Dermatology Patient Pages, go to the Patient Page link on JAMA Dermatology’s website tionofnewlesions.Thetreatmentwilldependonmorpheatype.Gen- at jamadermatology.com. erally,creams and ointments are given to patients with circumscribed

Author: Nicole M. Fett, MD The JAMA Dermatology Patient Page is a public service of JAMA Dermatology. Conflict of Interest Disclosures: None reported. The information and recommendations appearing on this page are appropriate in most instances, but they are not a substitute for medical diagnosis. For Sources: Fett N. Clin Dermatol. 2013;31(4):432-437. specific information concerning your personal medical condition, JAMA Fett N, Werth VP. J Am Acad Dermatol. 2011;64(2):231-242. Dermatology suggests that you consult your physician. This page may be Fett N, Werth VP. J Am Acad Dermatol. 2011;64(2):217-228. photocopied noncommercially by physicians and other health care professionals to share with patients. To purchase bulk reprints, call 312/464-0776.

1124 JAMA Dermatology September 2013 Volume 149, Number 9 jamadermatology.com

Downloaded From: https://jamanetwork.com/ on 10/01/2021