Fig. 38.1
Upper panel shows an elderly woman with lesions that had the clinical and histological findings of both morphea and lichen sclerosis. Lower panel shows an elderly woman who presented with severe limitation to breathing and shrinkage of her breast tissue due to encasement of her chest by tight firm skin of morphea profunda
Case
A person presents with discrete sclerotic smooth plaques.
Clinical differential diagnosis includes
morphea (the most common diagnosis)
lichen sclerosis (LS)
some cases of early atrophoderma
chronic radiation dermatitis
scleromyxedema , and
nephrogenic systemic fibrosis (NSF) .
Scleroderma , whether diffuse or limited, is excluded due to the discrete nature of the eruption. Scleredema, although not a truly sclerotic disorder but dermal thickening may appear grossly sclerotic; however, patients are diffusely affected.
Clinical Clues
Common Disorders
Clinical clues here are extremely helpful. The clinical characteristics of plaque lesions of morphea whether localized or generalized are well known to the dermatology practitioners. A whitish surface that is rough to the touch due to hyperkeratosis is characteristic of lichen sclerosis, and a brownish hue with cliff border is characteristic of atrophoderma.
The end-stage of morphea and the (older) center of old lesions may acquire an ivory color reminiscent of the white color of lichen sclerosis so that the two disorders may be occasionally confused. This is especially true when the two disorders coexist in the same patient or the same lesion. In general, the induration in morphea (due to sclerosis in the whole reticular dermis) is much thicker and deeper than that in LS (in which sclerosis is limited to the papillary dermis).
The relationship between atrophoderma and morphea has been debated since the initial descriptions of atrophoderma, decades ago. Some authors propose that atrophoderma is a type of morphea, while others consider it a unique disorder.
Those in favor of atrophoderma being a type of morphea refer to the observation that an involuted or healed lesion of morphea may appear similar to lesions of atrophoderma, and that an early or active lesion of atrophoderma reveals similar, although milder, histological findings to morphea, namely, sclerosis and lymphocytic infiltrate. In addition, there are several reports of patients having a combination of lesions, some characteristic of morphea and others characteristic of atrophoderma, clinically and histologically. I have seen few such patients. I have also seen patients who have classic lesions of atrophoderma and who, years later, develop lesions clinically and histologically characteristic of plaque morphea.