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Skin Disease
Published in John S. Axford, Chris A. O'Callaghan, Medicine for Finals and Beyond, 2023
Christopher Bunker, Richard Watchorn
Sclerodactyly: tight shiny skin or waxy swollen sausage fingers (CLINICAL EXAMINATION AT A GLANCE, Figure P), dorsal erythematous patches, digital ulceration, calcinosis, flexion deformity, muscle wasting, nail dystrophy, periungual erythema and telangiectasia, and ragged cuticles (CLINICAL EXAMINATION AT A GLANCE, Figure C)
Other Complications of Diabetes
Published in Jahangir Moini, Matthew Adams, Anthony LoGalbo, Complications of Diabetes Mellitus, 2022
Jahangir Moini, Matthew Adams, Anthony LoGalbo
Musculoskeletal complications of diabetes include carpal tunnel syndrome, Dupuytren contracture, adhesive capsulitis, and sclerodactyly. Carpal tunnel syndrome is compression of the median nerve along its course through the carpal tunnel in the wrist. Dupuytren contracture is progressive contracture of the palmar fascial bands, resulting in finger flexion deformities. Adhesive capsulitis is commonly known as frozen shoulder, associated with shoulder pain and stiffness. Sclerodactyly is a localized thickening and tightness of the skin on the fingers or toes.
Spot diagnosis and pattern recognition
Published in Caroline J Rodgers, Richard Harrington, Helping Hands: An Introduction to Diagnostic Strategy and Clinical Reasoning, 2019
Caroline J Rodgers, Richard Harrington
The history is consistent with Raynaud’s phenomenon. In the photos, the prominent features are of tight, thickened, shiny, hairless skin with loss of skin creases (sclerodactyly) and joint deformity. Remember that the thickening and tightness of the skin is detected on palpation. This pattern of features coupled with the history of Raynaud’s phenomenon would fit with scleroderma (note that at this stage, without examining the rest of the patient, we do not know if this represents limited or diffuse systemic sclerosis). Approximately 90% of patients with systemic sclerosis have Raynaud’s phenomenon, so it is important to take a careful history in this respect.10 In addition, approximately 95% of individuals with the diagnosis show signs of sclerodactyly.10
Impact of double positive for anti-centromere and anti-SS-a/Ro antibodies on clinicopathological characteristics of primary Sjögren’s syndrome: a retrospective cohort study
Published in Modern Rheumatology, 2018
Yasunori Suzuki, Hiroshi Fujii, Hideki Nomura, Ichiro Mizushima, Kazunori Yamada, Masakazu Yamagishi, Mitsuhiro Kawano
In conclusion, we identified several distinct clinical characteristics of ACA/SS-A double positive SS. Affected patients were older than those with SS-A single positive SS, and had higher disease activity of SS according to ESSDAI and higher serum IgG than those with ACA single positive SS. Anti SS-A antibody positivity was related to the features of B cell activation such as elevated serum IgG, and ACA positivity with features of scleroderma such as Raynaud’s phenomenon. Nevertheless, a low frequency of capillary abnormalities and absence of sclerodactyly in ACA positive SS were salient characteristics which clearly distinguish the group from scleroderma. The combination of SS-A and ACA define the clinical phenotype of SS, and so the combination of these antibodies should attract attention in clinical practice.
Removal of breast implants as primary treatment for autoimmune/inflammatory syndrome induced by adjuvants
Published in Scandinavian Journal of Rheumatology, 2023
S Ramakrishnan, A Abbas, N Jordan
A 59-year-old woman with early diffuse systemic sclerosis (SSc) and positive anti-RNA-polymerase III antibodies showed a marked improvement in symptoms following removal of bilateral breast implants. She presented to the clinic with a 3 month history of swelling, stiffness, and pain in her hands and feet. She also reported symptoms of new-onset, progressively worsening Raynaud’s phenomenon. She had been previously fit and well, had no family history of autoimmune disease, and had undergone bilateral breast implantation 22 years previously for cosmetic reasons. On examination, there was evidence of sclerodactyly, with puffiness and swelling of all fingers, but no obvious synovitis. Serology was positive for anti-nuclear antibodies on enzyme-linked immunosorbent assay and HEp-2 (speckled pattern), and also for anti-RNA polymerase III. Nailfold capillaroscopy demonstrated an abnormal pattern with multiple dilated and tortuous loops, suggestive of scleroderma spectrum connective tissue disease. She was initially treated with hydroxychloroquine, but her disease progressed rapidly, with skin texture thickening to the distal forearms. She was then commenced on mycophenolate mofetil, but her symptoms worsened following this. She reported pruritus, increased ankle and elbow stiffness, and worsening of her Raynaud’s phenomenon. Following potential implant dislodgement, she underwent bilateral removal of her breast implants. This led to a marked improvement in her symptoms of joint pain, stiffness, and skin thickening, such that she was able to discontinue mycophenolate mofetil, and remains stable on hydroxychloroquine monotherapy as of her most recent follow-up.
Advances in understanding and treatment of scleromyxedema
Published in Expert Opinion on Orphan Drugs, 2018
Laura Atzori, Caterina Ferreli, Franco Rongioletti
Scleromyxedema consists of two different findings: the papular component and the indurated modification (sclerodermoid) of the underlying skin, involving the hands, forearms, head, neck, upper trunk, and thighs. The widespread papular eruption is characterized by small lesion, 2–3 mm, firm, waxy, closely spaced, dome shaped, or flat topped, often arranged in a linear array (Figure 1). A typical finding on the proximal interphalangeal joints is the ‘doughnut sign’, consisting of a central depression surrounded by an elevated rim of thickened skin. Non-tender subcutaneous nodules are sometimes present. At palpation, consistency of the involved skin is harden fibromatous. Progression to stiffening and decreased joint motility can lead to sclerodactyly. In respect to scleroderma, the Raynaud phenomenon is rare, and telangiectasias or calcinosis is absent [2,14]. The other pathognomonic finding is the erythematous and edematous induration of large areas of the skin, progressing to plaques, with a shiny appearance or sometimes with a brownish discoloration. On the trunk and radix of the limbs, induration and loss of elasticity are responsible for the so-called ‘Shar-pei sign’ (Figure 2), which consists of deep longitudinal furrowing similar to the dog’s skin appearance. The same induration and furrowing on the glabella cause the ‘leonine face’ (Figure 3). Another peculiar site of involvement is the auricle (Figure 4). A decrease in eyebrows, and axillary and pubic hair is common. Lesion’s itching is common and may induce the occurrence of a Koebner phenomenon [1]. Mucous membranes are spared, but skin infiltration around the mouth is frequent, and the reduced motility is a very disturbing symptom for the patient, characteristic of active disease (Figure 5).