Section 20: Disorders of the Dermis (Infiltrates, Atrophies, and Nodules)

Morphea This condition is characterized by discrete areas of hardened and discolored skin. The areas vary in size and shape, and may be single or multiple. Morphea is also sometimes referred to as localized scleroderma, but this condition must not be confused with scleroderma, a generalized disease with organ involvement. Families must be cautioned of this important difference so that their internet searches do not lead to unnecessary worry.

The solitary or individual lesion is a circle or oval of firm skin that is whitish, slightly depressed, and sometimes surrounded by a different color—lilac or purple. A lesion with a prominent purple vascular border is illustrated in Fig. 20-2. There may be only one such lesion, or multiple smaller and larger lesions covering large parts of the skin surface. The course of the condition is variable. Spontaneous recovery in children is common, but a wide variety of topical and systemic therapies have been recommended for lesions that cause significant problems with respect to appearance and function.

Morphea (linear) Figure 20-3 shows a type of morphea that is linear in shape and situated on the lateral surface of the leg. Again the affected skin is hard, slightly depressed, and dyschromic. Extensive areas of linear morphea are the most difficult to treat. There is a tendency toward permanent deformity. In this case, linear morphea appears to follow the lines of Blaschko, and suggests the possibility of mosaicism.

When the lesions cross over a joint, the rapid development of atrophy or contractures may occur. In those cases, systemic therapy, usually with pulsed intravenous steroids and methotrexate, is required.

Morphea (linear) This is an example of linear morphea involving the lip, chin, and neck. The skin is hard to the touch, and shiny with prominent vasculature. Rarely, linear morphea occurring in this location may lead to involvement of the gums and tongue.

Morphea (linear, en coup de sabre) This term refers to linear morphea occurring on the forehead and scalp. Rarely, the central nervous system is affected. Neurologic manifestations include seizures, focal neurologic deficits, and movement disorders. MRI in affected children shows some areas of T2 hyperintensity and evidence of focal tissue atrophy in the brain.

Morphea Illustrated in Fig. 20-7 is a lesion of morphea that is resolving. ...