MORPHEA IN A CHILD

By Gary M. White, MD


Morphea in a child 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.

Clinical

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]

Workup

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.

Treatment

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.

Methotrexate

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.

UVA

UVA1 can be very effective.

Imiquimod

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].

Other Treatments

Physical therapy and surgical intervention, e.g., plastic surgery, in the case of facial involvement may be needed.

Additional Pictures

Purple marks indicate the extent of the lesion. Photographic monitoring helps to assess spread and response (or lack thereof) to treatment.
Morphea in a child

Parry-Romberg syndrome
Morphea in a child as Parry-Romberg syndrome

References

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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