By Gary M. White, MD
Morphea with central lichen sclerosus on the anterior thigh of a young girl.
Morphea is condition in which localized areas of skin become thickened and sclerotic. It may occur in a linear fashion and this variant is more common in children. Unilateral involvement of the face is called Parry-Romberg syndrome. Rarely, morphea may occur postvaccination.
An indurated, brown plaque or plaques is seen. The central area may take an ivory color, indicating changes of lichen sclerosus. Lesions over the joint may cause restriction of movement and extremity lesions may cause limb-length discrepancy. Atrophy may underly the lesion, especially on the face. Deep morphea can sometimes cause muscle atrophy.
Rarely, early morphea can be very inflammatory. Some cases have resembled an acquired port wine stain [PD 2014 Sept;31;591-4]
Blood work can include ANA, anti-ssDNA and dsDNA and rheumatoid factor. If there is any question of bone involvement, X-rays may be obtained. For scalp or facial lesions, referral to a neurologist or ophthalmologist is at times indicated.
For mild disease, where there is no underlying atrophy, joint contracture, or growth retardation, etc., topical therapy with a potent topical steroid, calcipotriene, a combination of the two, or topical tacrolimus may be given.
In a RDBPC study, oral MTX (15 mg/m²/week, maximum 20 mg) for 12 months plus oral prednisone (1 mg/kg/day, maximum 50 mg) for the first 3 months was better than prednisone alone in reducing lesion size and preventing new lesions [Arthritis Rheum. 2011; 63(7):1998-2006].
Mycophenolate mofetil is a second-line possibility.
UVA1 can be very effective.
Daily topical imiquimod 5% cream 3-5 times per week has been shown to help reduce dyspigmentation, induration, erythema, and telangiectasias [J Cutan Med Surg. 2015 Mar-Apr;19(2):132-9]. This treatment appears safe in children [Dermatology. 2011;223(4):363-9].
Physical therapy and surgical intervention, e.g., plastic surgery, in the case of facial involvement may be needed.
Purple marks indicate the extent of the lesion. Photographic monitoring helps to assess spread and response (or lack thereof) to treatment.
Morphea may initially be misdiagnosed as an acquired port-wine stain. PD Sept 2014
Early inflammatory morphea, initially and followup. JAAD April 2011 Volume 64, Issue 4, Pages 779–782
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