Congenital Localized Scleroderma (CLS): A Rare Presentation in a Newborn Baby


Congenital Localized Scleroderma (CLS), is an exceedingly rare form of morphea. Little is known about CLS clinical presentation and progression. Here we describe a case with a unique presentation of CLS noticed since birth. A 2- month-old baby girl presenting with an asymptomatic slightly hyperpigmented oval patch with hypertrophic center and mildly indurated borders involving the left forehead. She had no history of extra-cutaneous involvement. A skin biopsy was obtained and a diagnosis of CLS, circumscribed subtype, was made. Basic laboratory tests, radiological imaging, and ophthalmic examination were all unremarkable, excluding extracutaneous involvement. The patient was started on Topical Tacrolimus 0.1% ointment twice daily. After 12 months of follow-up, the lesion was stable with no signs of activity. This case represented a diagnostic challenge suggesting that CLS should be considered in the differential diagnosis of neonates or infants with fibrotic lesions to avoid delay in the diagnosis, to rule out any underlying systemic involvement, and allow prompt therapy.

Scleroderma is a spectrum of disorders characterized by thickening of the skin and subcutaneous tissue with increased collagen deposition. The two clinical patterns are systemic sclerosis and localized scleroderma (Morphea). The latter is ten times more common in children than systemic sclerosis. The incidence of morphea is generally estimated to be 0.4 to 2.7 per 100,000 people with a female: male ratio of (2 to 3:1). A study in the UK and Ireland estimated an incidence rate of 3.4 cases per million children per year aged ≤ 16 years. Morphea can be divided into several clinical subtypes, including circumscribed (with superficial and deep variant), Linear (with trunk/limb variant and head variant), generalized, pansclerotic and mixed type. Linear morphea is the predominant form of localized scleroderma in childhood. Extra-cutaneous manifestations occur in over 20 percent of children with Localized scleroderma.

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Kathy Andrews
Journal Manager
Journal of Clinical & Experimental Dermatology Research