Dermatology Online Journal
- Author(s): Strober, Bruce E, MD PhD
- et al.
From the Ronald O. Perelman Department of Dermatology, New York University
Bruce E Strober MD PhD
Dermatology Online Journal 9(4): 24
A 69-year-old man presented with symmetric erythematous, hyperpigmented, and ivory-colored plaques on the trunk and extremities. His clinical history, laboratory analysis, and histopathologic examination were consistent with generalized morphea, a subtype of localized scleroderma.
History.—A 69-year-old man presented with a 10-year history of a dermatosis involving the extremities, torso, and buttocks. More than 10 years ago the patient developed nonpruritic plaques symmetrically on the trunk and extremities. His disease progressed steadily with restriction of joint mobility. The patient was treated periodically with systemic glucocorticoids combined with either doxycycline (initially) or azathioprine (more recently). Azathioprine (150 mg daily) combined with regular physical therapy has been most effective in stabilizing the cutaneous disease and improving the joint mobility. Additionally, topical mid- and high-potency glucocorticoids and calcipotriene have been used concomitantly with the oral medications. The patient denies systemic complaints and a family history of a similar illness.
The past medical history includes hypertension, benign prostatic hypertrophy, Parkinson disease, and glaucoma. Currently he takes prednisone (2.5 mg every other day), losartan, amlodipine, and tamsulosin. He has no known drug allergies.
Physical examination.—Hyperpigmented, erythematous, and ivory-colored, firm, indurated plaques were present on the upper and lower extremities, trunk, and buttocks.
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Laboratory data.—A complete blood count with differential analysis, liver function tests, chemistry panel, and urinalysis were normal. Antinuclear antibodies, anti-Scl-70 antibodies, and rheumatoid factor were negative. A chest roentogram performed in 1999 detected no evidence of cardiopulmonary disease.
Histopathology.—There is a sparse superficial and predominantly deep dermal and subcutaneous perivascular infiltrate of lymphocytes and plasma cells. In the reticular dermis, the collagen bundles appear thickened and closely packed with paucity of adnexal structures and diminished peri-eccrine adipose tissue. The subcutaneous septae are widened and replaced by thick hypocellular hyalinized collagen bundles.
Generalized morphea, which lacks systemic manifestations, is a subtype of localized scleroderma. Like all other forms of scleroderma, generalized morphea is a disease of unknown etiology that affects both the microvasculature and the loose connective tissue. Other subtypes of localized scleroderma include localized morphea, guttate morphea, nodular morphea, subcutaneous morphea, linear scleroderma, and atrophoderma of Pierini and Pasini .
Generalized morphea displays widespread, multiple, well-circumscribed, indurated plaques that resemble the lesions of localized morphea. The plaques usually are ivory-colored and may show violaceous borders, especially when the disease is active. Other plaques may be hyperpigmented. Patients with generalized morphea do not have features of systemic sclerosis, but the disease may result in severe scars and functional disability .
The cause of localized scleroderma is unknown but may involve an autoimmune etiology. Antinuclear antibodies, antihistone antibodies, and rheumatoid factor may be present. Furthermore, antibodies to single-stranded DNA (ssDNA) are seen in over 50 percent of cases of generalized morphea . Eosinophilia may occur in localized scleroderma and indicates active illness . Borrelia burgdorferi, a tick-borne spirochete, has been associated with both morphea and lichen sclerosis in Europe and Japan . Unlike localized morphea, lesions of generalized morphea less frequently involute spontaneously. The treatment of generalized morphea is challenging. High-potency topical glucocorticoids may be applied locally, with their effect augmented by intralesional injections of triamcinolone. Systemic glucocorticoids, antimalarials, colchicine, and azathioprine are usually ineffective. D-penicillamine (2.0-5.0 mg/kg) may halt the formation of new lesions and induce the softening of the older lesions . Oral calcitriol (0.50-0.75 mg daily) may improve joint mobility and skin extensibility in adult patients with generalized morphea . UVA1 (340-450 nm) phototherapy may also be helpful . Finally, oral methotrexate (15-25 mg per week) may provide some benefit to a subset of patients .
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