Clinical examination revealed: erythema of the face, thin lips, erased expression lines, mild hardening of the skin, telangiectasias (Figure 1) of the lips, face and palms, sclerodactyly
(Figure 2), non-pitting edema of the lower limbs, subcutaneous calcinosis (Figure 3) in the extensor surface of the joints (elbow and knee), bilaterally and mildly impaired strength prehension.
Bununla birlikte KKB'u ilaclarin kronik anal fissur, keloidler, yanik izleri, idiyopatik veya CREST (Calsinosis, Raynaud fenomeni, Esophageal dysmotility, Sclerodactyly
, Telangiectasia) ile iliskili kalsinozis kutis tedavisinde kullanimi konusunda klinik arastirmalar ve denemeler de surmektedir.
Patients presenting with typical skin manifestations (tightness, thickening, and/or non-pitting induration of the skin affecting the face, neck, torso, and proximal portions of the upper and lower extremities) or with the presence of at least 2 of the following: sclerodactyly
, stellate pulp scars or ulceration of the fingertips, or bilateral pulmonary fibrosis, mainly at the lung bases.
Past medical history revealed presence of diffuse systemic sclerosis (SSc) which was diagnosed 2 years ago when she presented with Raynaud's phenomenon, arthralgias, sclerodactyly
, and malaise; hypertension for 4 years.
Calcinosis cutis is a well-known phenomenon in a variety of rheumatic conditions, particularly dermatomyositis, scleroderma and CREST (calcinosis, Raynaud's phenomenon, oesophageal dysfunction, sclerodactyly
and telangiectasia) but is rarely described in systemic lupus erythematosus (SLE).
Pope: Most of these conditions lack Raynaud's phenomenon, sclerodactyly
, and nailfold capillary changes.
Abnormalities disclosed on history and physical exam may include Raynaud's phenomenon, sclerodactyly
, mask-like face, telangiectasia, and esophageal dysfunction.
4) Two patients with traumatic bursitis had good results; however, one patient with an inflammatory bursitis, secondary to CREST (calcinosis, Raynaud's phenomenon, esophageal dysmotility, sclerodactyly
, telangiectasia) syndrome, obtained an unsatisfactory result.
There are two main groups of the disease: the limited cutaneous systemic sclerosis (lSSc) which is expressed as limited skin disease (CREST-Calcinosis, Raynaud's phenomenon, oesophageal dysmotility, sclerodactyly
, and telangectasia) with high prevalence of anti centromere antibody (ACA), and the diffuse systemic sclerosis (dSSc) which has high prevalence of anti topoisomerase antibodies I (anti-topo I), and worse prognosis (1).
Physical examination showed scleral icterus, jaundice, and sclerodactyly
, as well as a palpable liver 2 cm below the costal margin and multiple firm, whitish dermal papules on her left forearm.
Summary of idiopathic interstitial lung diseases (ILD) UIP NSIP DIP/RB-ILD Demographics 60s-80s; 40s-50s; 40s-50s; M = F Increased F > M Associated with risk with Associated smoking smoking with collagen vascular disease or with extrinsic allergic alveolitis History/course Long Subacute onset Subacute onset of asymptomatic of months to months to years period years (radiographic evidence); Several years progressive dyspnea Physical exam Velcro crackles Raynaud Crackles Clubbing sclerodactyly
Clubbing Lab RF ANA, SCL-70 Jo-1 CK; aldolase Serum Ab to birds, organisms, etc Biopsy (3) Variable from Uniform pattern DIP: Uniform area to area of involvement intra-alveolar ("temporal of the lung in macrophages, heterogeneity" biopsies.
Scleroderma with calcinosis, Raynaud's phenomenon, esophageal dysfunction, sclerodactyly
, and telangiectasia (the CREST variant of limited scleroderma).