A healthy 16-year-old presents with a raised, two-year-old scar-like lesion on his scalp which has caused hair loss.
• What's the diagnosis?
• How would you treat him?
The young man's lesion is consistent with linear morphea, or localized cutaneous scleroderma. This disorder, which occurs most commonly in children and young adults, should not be confused with progressive systemic sclerosis or scleroderma (it literally means "hard skin"), which is characterized by Raynaud phenomenon and systemic organ involvement (that's why we prefer the term morphea).
The clinical variants include linear, plaque, guttate, nodular, and subcutaneous morphea. Linear morphea is the most common type, accounting for 65% of cases. There is a 2:1 female to male predominance in linear morphea, and the mean age of onset is 6 years. There is no known racial or familial predilection. Although the cause is unknown, several etiologies including vasculopathy, inflammation, trauma, and infection have been postulated. Interestingly, 23% to 67% of patients are antinuclear antibody–positive, 25% to 40% are rheumatoid factor–positive, and many have elevated immunoglobulins or eosinophilia.1
Children with linear morphea typically develop lesions that initially appear somewhat erythematous, then progress to hyperpigmented plaques that coalesce longitudinally into continuous bands. Finally, the skin becomes sclerotic and atrophic with loss of hair and other adnexal structures. The lesions are mostly unilateral and tend to involve the lower extremities more frequently than the upper extremities. When bands cross joints, contractures with resultant decreased range of motion of the joints may result.1
Our patient has a rare subtype of linear morphea called "en coup de sabre," or "stroke of the sword," so-called because of the characteristic linear lesions that occur on the frontoparietal scalp and forehead. En coup de sabre has been associated with neurological complications, including facial palsy, facial hemiatrophy, and seizures. Less common systemic manifestations include ophthalmologic involvement (ptosis, exophthalmos, and uveitits) and oral involvement (tongue atrophy).2
Although evidence-based literature about therapy is limited, various treatment options-including high-potency topical corticosteroids, topical calcipotriene, oral steroids, systemic methotrexate, and photochemotherapy-have been advocated. Physical therapy is important when skin lesions cross joints.
Weibel et al performed a retrospective analysis of 34 pediatric patients with morphea who were treated with pulsed intravenous methylprednisolone/methotrexate, followed by an oral steroid taper and long-term (one to three years) maintenance therapy with methotrexate. The results were promising, with 94% of the patients demonstrating a halt in disease progression and 74% maintaining clinically inactive disease at two-year follow-up. However, of those patients whose methotrexate was stopped, 44% developed a relapse between five and 32 months after discontinuation of the treatment, thus requiring additional treatment. Interestingly, six of the patients were treated with only oral steroids and methotrexate (and not pulse IV steroids). These patients showed no differences with regard to time of clinical improvement or prevalence of relapse.3
Our patient's lesion has been stable for over a year. He was concerned about the hyperpigmentation, and we suggested topical 4% hydroxychloroquine cream twice a day, sunscreen, and continued close monitoring for evidence of progression. Involvement of the upper eyelid and concern about possible involvement of the soft tissues of the orbit prompted a visit to ophthalmology, and a normal examination.
DR. ZIMMERMAN is a senior resident in pediatrics at Johns Hopkins Children's Center.
DR. COHEN, who serves as section editor for Dermatology: What's your Dx?, is director, Pediatric Dermatology and Cutaneous Laser Center, and professor of pediatrics and dermatology, Johns Hopkins University School of Medicine, Baltimore. He is a contributing editor for Contemporary Pediatrics.
The author and the section editor have nothing to disclose in regard to affiliations with, or financial interests in, any organization that may have an interest in any part of this article.
Vignettes are based on real cases which have been modified to allow the authors and editor to focus on key teaching points. Images may also be edited or substituted for teaching purposes.
1. Liou JS, Morrell DS: Firm and dyspigmented linear plaques: childhood linear morphea. Pediatr Ann 2007;36;792
2. Holland KE, Steffes B, Nocton JJ, et al: Linear scleroderma en coup de sabre with associated neurologic abnormalities.Pediatrics 2006;117;132
3. Weibel L, Sampai MC, Visentin MT, et al: Evaluation of methotrexate and corticosteroids for the treatment of localized scleroderma (morphoea) in children. Brit J Dermatol 2006;155;1013