Scleroderma (systemic sclerosis [SSc]) is a chronic disease of unknown cause involving connective tissue, characterized by diffuse fibrosis of skin and visceral organs and vascular a...
Raynaud phenomenon is generally the presenting complaint (differentiated from Raynaud disease, generally affecting younger individuals and without digital ulcers).
Skin thickening, “pu...
Treatment is symptomatic and supportive.
Esophageal dilation may be used for strictures.
Avoid cold; dress appropriately in layers for the weather; be wary of air conditioning.
...
Monitor every 3 to 6 months for end organ and skin involvement and medications. Provide encouragement.
Echocardiology and PFTs yearly
Drink pl...
Fernández-Codina A, Walker KM, Pope JE, et al.; Scleroderma Algorithm Group. Treatment Algorithms for Systemic Sclerosis According to Experts. Arthritis Rheuma...
M34.2 Systemic sclerosis induced by drug and chemical
M34.9 Systemic sclerosis, unspecified
M34.0 Progressive systemic sclerosis
L94.0 Localized scleroderma [morphea]
M34 Systemic sclerosis [sc...
Raynaud phenomenon is frequently the initial complaint.
Skin thickening, “puffy hands,” and GERD are often noted early in disease.
Patients must be followed proactively for development o...
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Scleroderma. The dermis is characterized by large reticular dermal collagen bundles that are oriented parallel to the epidermis. The large size and loss of basket-weave pattern of these collagen bundles are abnormal. No appendages are apparent, as these are destroyed in the disease process.
Scleroderma. The dermis is characterized by large reticular dermal collagen bundles that are oriented parallel to the epidermis. The large...
scleroderma: early stage
Scleroderma: hand, amputated distal digits
<bold>FIG. 77.6.</bold> Close-up hand roentgenogram of a 46-year-old woman with limited cutaneous systemic sclerosis. Note extensive subcutaneous calcinosis.
<bold>FIG. 77.6.</bold> Close-up hand roentgenogram of a 46-year-old woman with limited cutaneous systemic sclerosis. Note ext...
<bold>FIG. 77.11.</bold> Photomicrograph of the lung of a 52-year-old woman with diffuse cutaneous systemic sclerosis who died as the result of respiratory insufficiency. Note the dramatic interstitial fi osis and dilatation of air sacs (honeycomb lung).
<bold>FIG. 77.11.</bold> Photomicrograph of the lung of a 52-year-old woman with diffuse cutaneous systemic sclerosis who died...
<bold>FIG. 77.20.</bold> A child with linear scleroderma involving the left lower extremity with evidence of a left knee flexion contracture. There is a plaque of morphea on the right thigh.
<bold>FIG. 77.20.</bold> A child with linear scleroderma involving the left lower extremity with evidence of a left knee flexi...
FIG. 2.17. Juvenile dermatomyositis and scleroderma. A: Atrophic, hypopigmented lesions overlying extensor surfaces of interphalangeal joints, with periungual erythema typical of juvenile dermatomyositis. B: Linear scleroderma involving left lower extremity in a 12-year-old girl.
FIG. 2.17. Juvenile dermatomyositis and scleroderma. A: Atrophic, hypopigmented lesions overlying extensor surfaces of interphalangeal joi...
Figure 10-195 <bold>SCLERODERMA: ACROOSTEOLYSIS. A. PA Hand.</bold> Note the early resorption of the distal tufts (<italic>arrows</bold>). Incidentally noted is a bandage (<italic>arrowhead</bold>). <bold>B. PA Hand.</bold> Observe the advanced resorption of the distal tufts.
Figure 10-195 <bold>SCLERODERMA: ACROOSTEOLYSIS. A. PA Hand.</bold> Note the early resorption of the distal tufts (<italic...
Linear scleroderma: leg
<bold>FIG. 77.2.</bold> Face of a 19-year-old woman with diffuse cutaneous systemic sclerosis. Note loss of normal skin folds and retraction of lips.
<bold>FIG. 77.2.</bold> Face of a 19-year-old woman with diffuse cutaneous systemic sclerosis. Note loss of normal skin folds ...
Morphea: abdomen