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. The first sign of improvement is softening in the area of involvement. In this patient, the lesion is becoming softer, and turning brown. In the center there is still an area of sclerosis.

Figure 20-8 shows a lesion that is reddish-brown and is now level with the surrounding skin. It will eventually completely resolve or leave minor dyschromia. The cause of morphea is unknown.

Generalized morphea Sometimes morphea can be more extensive and involve large areas of the skin surface. The lesions begin as erythematous to violaceous plaques and become sclerotic with a central ivory color. Sometimes only hyperpigmentation can be discerned.

These lesions may enlarge peripherally, becoming quite extensive. When involvement occurs on the extremities, contractures and atrophy may result. Scarring may result in disability, but these patients do not have the internal organ involvement that is seen in scleroderma (systemic sclerosis).

Atrophoderma (Pasini-Pierini) This condition is most common in adolescent girls, and its etiology is completely unknown. Characteristically, there develop single or multiple well-circumscribed, slightly hyperpigmented areas of cutaneous atrophy, with a sharp “cliff-drop” border. The lesions tend to enlarge slowly and then persist. Some believe that this form of atrophoderma represents a variant of morphea, in which atrophy is more evident than is sclerosis.

Lichen sclerosus et atrophicus Figures 20-12 and 20-13 illustrate lichen sclerosus et atrophicus in its most typical location. This cutaneous disorder is more common in girls and is usually confined to the skin surrounding the anogenital region. The onset of the disease may be accompanied by pruritus, burning, constipation, or vaginal discharge, or it may be completely asymptomatic. Porcelain-colored or slightly erythematous macules gradually coalesce to form plaques.

The end result is an area of shiny atrophy in an hourglass shape around the rectum and vagina. Childhood lichen sclerosus et atrophicus tends to be a self-limited disease, with improvement occurring at the time of puberty. It may persist into adulthood. The use of topical corticosteroids provides symptomatic relief and may hasten the resolution of the process.

Lichen sclerosus et atrophicus Figure 20-14 shows the discrete and confluent ivory-colored macules that are typical of extragenital lichen sclerosus et atrophicus. Involvement as shown here, on the skin behind the ear is unusual. Extragenital lesions tend to be asymptomatic.

Figure 20-15 illustrates lichen sclerosus et atrophicus involving the ankle, which is not an unusual location. The classic lesion contains numerous white round and oval-shaped macules that, in some cases, form areas of confluence. Rarely, extragenital lichen sclerosus is widespread and disfiguring.

Lichen sclerosus et atrophicus (Balanitis xerotica obliterans) This is a form of lichen sclerosus et atrophicus affecting the penis. It usually presents as recurrent balanitis, associated with redness, tightening, and fissuring of the foreskin. Eventually, the disease process may result in phimosis and difficulty in urination. In many cases, circumcision is either very helpful or curative. However, involvement of the glans penis may persist after surgery. Although this disorder is generally rare, it is not an unusual finding among young boys who present with phimosis.

Anetoderma (macular atrophy) The term anetoderma, meaning slack skin, is used to describe a form of cutaneous atrophy. The classic lesion is a macule of atrophic and wrinkled skin. The lesions of anteoderma may be preceded by inflammation, or may occur de novo on normal skin. Anetoderma may arise without a known cause, or it may be a sequelae of syphilis, or lupus erythematosus.

Anetoderma Illustrated in Fig. 20-18 is a lesion of anetoderma on the arm of a teenage girl. Direct pressure with a wooden applicator causes an inpouching of the involved skin.

Striae distensae Stretch marks are parallel streaks of glossy and erythematous skin, that gradually become hypopigmented and scar-like in appearance. The stretch marks often have a different texture from normal skin and may be slightly depressed. Striae are commonly seen in healthy adolescents and may be found on the back, around the breasts, upper arms, hips, inner thighs, and around the popliteal fossae.

Other causes of striae include pregnancy and rapid weight gain. Striae may also be seen in children with Cushing syndrome. Athletes, particularly weight lifters, sometimes develop striae distensae. In Fig. 20-19, one sees striae on the back that may well be of a weight lifter. Figure 20-20 shows striae and telangiectasia as a side effect of topical corticosteroids, which is most frequent in older children and adolescents.

Striae distensae This is an example of striae resulting from the use of topical corticosteroids in the antecubetal fossa, a typical area of involvement of atopic dermatitis. Older children and teenagers must be made particularly aware of this potential side effect, and should be cautioned against using stronger topical steroids on the inner thighs and axillae.

Connective tissue nevus This group of hamartomas may be composed of various elements of the extracellular connective tissue: collagen, elastic fibers, and glycosaminoglycans. One example of the collagen variety is the shagreen patch, a cutaneous manifestation of tuberous sclerosis (Fig 14-69). These yellow or orange plaques of coalescent papules are often seen in the lumbosacral area. Other varieties of connective tissue nevus are not associated with tuberous sclerosis and may be seen elsewhere on the body.