3. Rarely, generalized hyperpigmentation similar to that seen in Addison's disease occurs.
f. Matlike telangiectases may occur on the face, lips, hands, oral mucosa, and upper trunk. There is a high correlation between the degree of nail fold capillary abnormality, as viewed with a wide-field microscope, and the extent of internal organ involvement in scleroderma.
g. Bullae may rarely occur in areas of sclerosis.
h. Calcinosis cutis generally occurs late in the course of scleroderma and is usually limited to the skin over joints. The CREST syndrome consists of calcinosis, Raynaud's phenomenon, esophageal dysfunction, sclerodactyly, and telangiectasia. It identifies a group of PSS patients with a more favorable prognosis.
2. Diffuse systemic sclerosis often begins on the trunk and rapidly spreads to involve the extremities and face.
3. The differential diagnosis of fibrotic skin includes the following:
a. Eosinophilic fasciitis.
b. Tryptophan-induced eosinophilia myalgia syndrome.
d. Graft-versus-host disease.
e. Porphyria cutanea tarda.
g. Carcinoid syndrome.
i. Lichen sclerosis et atrophicus. j. Bleomycin-induced sclerosis.
k. Chlorinated hydrocarbon-induced sclerosis (polyvinyl chloride). l. Occupational trauma. m. Primary amyloidosis. n. Melorheostosis with linear scleroderma. o. Progeria. p. Werner syndrome.
r. Toxic oil syndrome (caused by adulterated rapeseed oil).
C. Eosinophilic fasciitis can usually be distinguished from systemic scleroderma by examination of the skin. On the trunk and proximal extremities, localized areas of induration develop that are firmly bound to underlying tissues. A cobblestone or puckered surface is formed secondary to involvement of the subcutaneous fibrous tissue and is most obvious during overhead extension of the upper extremities. Raynaud's phenomenon and internal organ involvement do not occur. A biopsy specimen that includes skin, fascia, and muscle is necessary for diagnosis. Fibrotic thickening and a cellular infiltrate, often including eosinophils, are found in the deep fascia and may also be present in the lower dermis, fat, and muscle. Laboratory abnormalities include blood eosinophilia in 30% of patients, an increased erythrocyte sedimentation rate, and hypergammaglobulinemia. A history of tryptophan ingestion should be elicited.
D. Tryptophan-induced eosinophilia myalgia syndrome. In 1989, a disease characterized by peripheral eosinophilia and myalgia with features of both eosinophilic fasciitis and systemic scleroderma was seen following tryptophan ingestion. Clinical features include fever, fatigue, weakness, muscle cramps, arthralgia, cutaneous edema, rashes, pruritus, and thickening of the skin. The degree of both eosinophilia and myalgia is variable. Scleroderma-like skin changes can involve the extremities as in eosinophilic fasciitis, or become more generalized as in PSS.
E. Undifferentiated connective tissue syndrome, also known as mixed connective tissue disease, denotes combined clinical features of SLE, scleroderma, and polymyositis. High titers of antibodies to U1RNP occur in many of these patients. Some authorities feel that undifferentiated connective tissue syndrome represents a prodrome of SLE or scleroderma in most patients (see Chapters!). Raynaud's phenomenon and swollen hands are the most frequent skin manifestations.
Direct immunofluorescent studies of clinically normal skin in patients with undifferentiated connective tissue syndrome reveal subepidermal immunoglobulin deposits in approximately one-third of cases.
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