Derm Dx: A linear lesion on the anterior frontal scalp

Scleroderma describes a group condition in which the skin becomes fibrotic, hardened and immobile. When only skin is involved, the term morphea is favored. When there is systemic involvement (such as pulmonary and renal involvement), the terms scleroderma or systemic...

Submit your diagnosis to see full explanation.

Scleroderma describes a group condition in which the skin becomes fibrotic, hardened and immobile. When only skin is involved, the term morphea is favored. When there is systemic involvement (such as pulmonary and renal involvement), the terms scleroderma or systemic sclerosis are favored.

In the above answer choices CREST syndrome and generalized systemic sclerosis are types of systemic sclerosis, whereas linear morphea and atrophoderma of Pasini and Pierini are types of morphea.

There are multiple subtypes of morphea including localized, linear, pansclerotic, profunda and atrophic. Localized morphea usually appears on the trunk as a discrete hardened yellowish, white or lilac plaques. Histologically, the dermis and subcutaneous tissue is replaced by thickened and sclerotic collagen. 

In pansclerotic morphea, the lesions may extend deep to muscle and even bone.  Generalized morphea is the term used when there are widespread plaques, but without internal involvement. Atrophic morphea, also called atrophoderma of Pasini and Pierini, consists of a well-demarcated atrophic plaque on the trunk. 

The patient in this case has linear mophea, which is characterized by the spontaneous development of a linear scar-like plaque. The plaque may extend down an entire extremity such as an arm or leg. Another common presentation is the “en coup de sabre” presentation in which the lesion occurs on the frontal scalp and progresses to the forehead.  When the lesions are deep or overlie joints, significant deformity and disability may occur. Anti-nuclear antibody (ANA) may be positive in up to 40% to 80% of linear scleroderma cases. ANA is less common in localized morphea. 

CREST syndrome – also known as limited systemic sclerosis – is a variant of systemic scleroderma. CREST is an acronym for Calcinosis, Raynaud phenomenon, Esophageal dysmotility, Sclerodactyly and Telangiectasia. 

Sclerodactyly describes the claw-like fibrosis of the fingers. As fibrosis occurs around the mouth, the oral aperture shrinks. The nose becomes beak like as the facial skin tightens.  There may be lung and pulmonary involvement.  Anti-centromere antibodies are present in 50% to 90% of patients. By definition, in limited systemic sclerosis, the cutaneous sclerosis is limited to the face and the skin distal to the metacarpalphalangeal and metatarsophalangeal joints.

In generalized systemic sclerosis, the sclerotic skin changes include the fingers, toes and face, as well as the skin proximal to the metacarpalphalangeal and metatarsophalangeal joints. 

Many CREST syndrome features may be present in generalized systemic sclerosis, and many experts now consider CREST syndrome and generalized systemic sclerosis to be different spectrums of the same disease.  Anti-Scl-70 antibody is present in 20% to 60% of patients with generalized systemic sclerosis. Lung and pulmonary involvement may be present leading to death. ANA is positive in over 90% of patients with systemic sclerosis, regardless of whether it is generalized or limited.

Morphea treatment includes topical and intralesional corticosteroids, topical vitamin-D derivatives, phototherapy, photochemotherapy and methotrexate. In cases of linear morphea in which the joint is affected, physical therapy is critical to maintain range of motion and avoid contractures. 

Some cases of morphea spontaneously resolve within three to five years. Treatment for systemic sclerosis is outside of the scope of this article. 

Adam Rees, MD, is a graduate of the University of California Los Angeles School of Medicine and a resident in the Department of Dermatology at Baylor College of Medicine in Houston.