Physical examination and imaging studies revealed Raynaud's phenomenon, stellate scars, sclerodactyly
, pulmonary fibrosis, pulmonary hypertension, and cardiac arrhythmias in a majority of patients (Table 1).
Over time, repeated bouts of Raynaud's phenomenon, in addition to the aforementioned pathologic features of CREST, lead to tissue fibrosis causing characteristic sclerodactyly
(loss of the digital pad) or may lead to digital ulceration descriptively termed "rat bite necrosis.
means basically hardening of the skin.
Similarly, our patient suffered from skin flattening, slimness, color change with sclerodactyly
and difficulty in opening his mouth, and tight lip skin.
Presence of Raynaud phenomenon, sclerodactyly
, and esophageal, joint and/or lung involvement was noted.
Other risk factors include severe Raynaud's phenomenon, low pulmonary diffusing capacity, and the calcinosis, Raynaud's disease, esophageal dysmotility, sclerodactyly
, and telangiectasia (CREST) syndrome, he said.
Examination was remarkable for normal optic fundi, multiple small telangiectasias on her face, diminished breath sounds at both lung bases with coarse crackles; her extremities were remarkable for swelling of both hands with sclerodactyly
Hence, systemic lupus erythematosus, discoid lupus erythematosus, scleroderma/CREST (calcinosis, Raynaud phenomenon, esophageal motility abnormalities, sclerodactyly
, and telangiectasia), Raynaud syndrome, SS, MCTD, overlap CTD syndromes, polymyositis, and dermatomyositis were considered disease-positive  and all other diagnoses were considered disease-negative.
1) Raynaud's phenomenon, joint involvement, sclerodactyly
, and constitutional symptoms found in scleroderma are not present in morphea.
ANA subtypes (a) Patterns Disease association Anti-double-stranded DNA Highly specific, but not sensitive for SLE (peripheral) Anti-Smith (speckled) Highly specific, but not sensitive for SLE Anti-Ro (speckled) SLE, SS, neonatal SLE Anti-La (speckled) SLE, SS, neonatal SLE Antihistone (diffuse) SLE, RA, aging, DILE Anti-RNP (speckled) SLE, MCTD Anticentromere Limited scleroderma (CREST syndrome) (a) ANA, antinuclear antibody; SLE, systemic lupus erythematosus; SS, Sjogrens syndrome; RA, rheumatoid arthritis; DILE, drug-induced lupus erythematosus; RNP, ribo-nuclear protein; MCTD, mixed connective tissue disease; CREST, calcinosis, Raynauds, esophageal dysfunction, sclerodactyly
They are associated with limited cutaneous systemic sclerosis and the CREST (calcinosis, Raynaud's syndrome, esophageal dysmotility, sclerodactyly
, telangiectasia) variant.
A 53-year-old Caucasian male with a history of heavy alcohol use, first presented in September 2000 with a 1-year history of sclerodactyly
and Raynaud's phenomenon.