Page 133 - The Netter Collection of Medical Illustrations - Integumentary System_ Volume 4 ( PDFDrive )

Plate 4-48 Rashes

MORPHEA En coup de sabre. Rare form of localized
morphea on the forehead and face. May
be associated with the Parry-Romberg
Morphea is a skin dermatosis that is idiopathic in nature. syndrome
The most common form is solitary, but many clinical
variants have been described, including linear, guttate,
and generalized forms. A small subset of patients (<1%)
progress to progressive systemic sclerosis. It is likely
that many patients do not seek medical advice because
the onset is insidious or the area of involvement is so
small that it is hardly noticeable or bothersome.
Clinical Findings: Morphea is typically seen in
young Caucasian females. The ratio of females to males
has been estimated at 2 : 1. Morphea begins as a small
erythematous macule. The lesion expands outward with
a violaceous to red border. As it expands, the central
portion becomes slightly hypopigmented and indurated
in nature. The trunk is the most commonly involved
region of the body. Most areas of involvement are
asymptomatic to slightly pruritic. If the involved area
crosses over a joint, there may be some loss of motion
of the affected joint and pain with flexion and extension.
The main differential diagnosis is between morphea
and lichen sclerosis et atrophicus. Lichen sclerosis et
atrophicus is typically more strikingly white in color-
ation and is less indurated.
Many variants of morphea have been described.
Guttate morphea manifests with tiny, teardrop-shaped
areas of hypopigmented macules with slight induration
scattered about the trunk or extremities. The induration
of guttate morphea is not nearly as prominent as that of
localized morphea and may not be appreciable. These
guttate lesions may be impossible to distinguish clini-
cally from lichen sclerosis et atrophicus, and a biopsy is
the only way to differentiate the two. Biopsies are not Localized morphea. Atrophic plaques
always conclusive, and the term morphea–lichen sclerosis that are firm and nonflexible on
overlap has been used to describe these lesions with fea- palpation. Often surronded by a
tures of both conditions. Generalized morphea is a rare violaceous or erythematous rim
variant with extensive involvement of the cutaneous
surface. By definition, generalized morphea does not
have systemic involvement, differentiating it from pro-
gressive systemic sclerosis. However, patients with gen-
eralized morphea may develop atrophy of the adipose
and muscle tissues underlying the areas of involvement.
Linear morphea, also called linear scleroderma, is a
unique cutaneous variant that is well described and has
a distinctive appearance and potential underlying com-
plications. It is commonly found along the length of the
affected extremity. This form occurs most commonly Progressive systemic sclerosis
in childhood. The affected skin may become bound (scleroderma). Typical skin
down and cause limb length discrepancies as the child changes in scleroderma: extensive
grows. Joint mobility is also a potential complication. collagen deposition and some
Cortical hyperostosis of the long bones underneath the epidermal atrophy
area of linear morphea has been well reported and is
termed melorheostosis. There are subtypes of linear
morphea that have been given the names en coup de sabre
and Parry-Romberg syndrome.
En coup de sabre is a specific type of morphea
that occurs along the forehead, as well as partially onto
the cheek and into the scalp. It appears as a depressed
linear furrow from the scalp vertically down the fore-
head. The appearance can be subtle or extremely notice- reaction in which an excessive amount of collagen is expanded with excessive amounts of collagen. A slight
able and can cause significant cosmetic problems. produced locally by fibroblasts. Potential factors that inflammatory infiltrate is often seen along the dermal-
Parry-Romberg syndrome is a name given to linear may initiate the reaction are endothelial damage, subcutaneous border. Plasma cells are common.
morphea that occurs vertically across the face, causing certain Borrelia burgdorferi infections, and fibroblast Treatment: Therapy for localized morphea is not
hemifacial atrophy. The underlying adipose tissue, abnormalities that lead to increased collagen produc- needed but can be attempted with topical corticoste-
muscle, and bone are involved, with significant disfig- tion. Borrelia-induced morphea has yet to be described roids, calcipotriene, and phototherapy. Linear morphea
urement. Patients may have neurological involvement in the United States; it has been reported in Europe should be treated, because it has significant functional
leading to seizures. and Asia. and cosmetic implications. Immunosuppressive agents
Pathogenesis: The pathogenesis of morphea is poorly Histology: A punch biopsy specimen of morphea such as methotrexate and prednisone have been the
understood. An unknown factor sets off this cutaneous appears as a nicely formed cylinder. The dermis is most thoroughly studied therapeutic agents.

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