Scleredema of Buschke associated with multiple myeloma

Salisbury, Jon; Shallcross, H.; Leigh, Irene M.

doi:10.1111/j.1365-2230.1988.tb00698.x

Cited by 35 publications

(15 citation statements)

References 6 publications

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“…There are also increasing number of reports of monoclonal gammopathy associated with SS and this association seems to be more than coincidental [9,11]. Reviewing the literature, we found 25 previously reported cases of SS with monoclonal gammopathy, in only 10 of these cases, SS was associated with MM [3,6,8,13,14,16,18,19,21]. In all reported patients, the paraprotein was detected years after the onset of SS and had never been found in affected skin.…”

Section: Discussionmentioning

confidence: 58%

FGF-R3 and OPG expression in patient with multiple myeloma following systemic sclerosis: case report and review of the literature

et al. 2011

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The presence of multiple myeloma (MM) in a patient with systemic sclerosis is a rare and unusual occurrence with unclear significance. We report the case of a 55-year-old woman with a 20-year history of systemic sclerosis, who subsequently presented with clinical stage IIIA IgG-λ MM. The systemic sclerosis was diagnosed and treated in 1988 with D: -penicillamine and methotrexate. Twenty years later, in December 2008, she presented with symptoms of Raynaud's phenomenon and intense facial pruritus. Immunoelectrophoresis confirmed the presence of a IgG-λ paraprotein (71.90 g/l) and Bence Jones proteinuria of the lambda light chains. Bone marrow histology revealed infiltrates of plasmocytes and lymphoplasmocytes which were on immunohistochemistry CD38+, FGF-R3+ and OPG+. An extensive X-ray skeletal survey did not show any osteolytic lesions or fractures. The patient was treated according to the CTD protocol (cyclophosphamide, thalidomide, and dexamethasone) which was effective against the myeloma as well as the systemic sclerosis and patient achieved complete remission.

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Section: Discussionmentioning

confidence: 58%

FGF-R3 and OPG expression in patient with multiple myeloma following systemic sclerosis: case report and review of the literature

et al. 2011

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“…The association of monoclonal gammopathy with scleredema has been reported on several occasions 1 . However, multiple myeloma in association with scleredema has been reported only in 10 cases, four with Ig G‐ kappa, one with Ig G‐ lambda, and five with Ig A‐ kappa 3–8 . To the best of our best knowledge this is the first associated with Ig A‐ lambda.…”

Section: Discussionmentioning

confidence: 87%

Treatment with chemotherapy of scleredema associated with Ig A myeloma

et al. 2001

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A 70‐year‐old woman was referred to the dermatology outpatient clinic in January 1999. During the previous 12 months she had developed a progressive induration and stiffness of the skin of her face, neck, shoulders, and upper aspect of her arms. There was no history of a preceding infection or of diabetes mellitus. An examination revealed that the skin of her face, back, shoulders, arms and chest was shiny, erythematous, and diffusely indurated, with a wooden consistency and decreased range of motion. Consistent firm pressure demonstrated a brawny edema. The face lacked expression, with minimal wrinkling (Fig. 1). Lymphadenopathies were not found in any location. 1 Woody face with minimal wrinkling A skin biopsy specimen was taken from the left shoulder. A hematoxylin‐eosin stain revealed fenestrated collagen bundles separated by wide, clear spaces in the mid and reticular dermis (Fig. 2). Alcian blue staining (present at pH 2.5 but lost at 0.5) demonstrated nonsulfated acid mucopolysaccharides in bands and streaks between collagen bundles in the mid and reticular dermis. A diagnosis of scleredema was made. 2 Collagen bundles separated by wide, clear spaces in the mid and reticular dermis The hemoglobin value, RBC and WBC counts, and differential cell count, chemistry, urinalysis, glucose tolerance test, antistreptolysin titre, and thyroid function tests were all within normal limits. The erythrocyte sedimentation rate was raised to 70 mm/h. Serum protein electrophoresis showed an abnormal ‘‘M’' band in the lambda region composed of Beta chains. Serum protein was abnormal with a monoclonal spike in the Beta region, which on subsequent immunoelectrophoresis was identified as a monoclonal Ig A‐lambda protein. Beta 2 microglobulin level was 28.6 g/L (6.0–11.5). Inmunoglobulin levels in the serum samples were as follows: Ig A, 18.30 g/L (0.8–4.0); Ig G, 4.12 g/L (6.15–16); Ig M, 2.6 g/L (0.4–3.0). Urine contained Bence‐Jones protein (L light chain). Proteins in urine at 24 h: 0.3 gr/day. ANA negative, C3 y C4 normal. Chest X‐rays was normal and skeletal survey revealed diffuse osteoporosis. CT scan of the abdomen was normal. Bone marrow aspirate and biopsy specimens were taken from the sternum. Their examination showed 38% plasma cells. A diagnosis of scleredema associated with Ig A lambda multiple myeloma was made. The patient was commenced on treatment with six pulses of oral melphalan 17 mg, given over 4 days (10 mg/m2 ) accompanied by oral prednisona 100 mg (60 mg/m2 ), each pulse at an interval of one month. After the third treatment, her skin began to become mobile, the Ig A levels decreased to 6.0 (0.8–4.0) and Beta 2 microglobulin level was 11.4 g/L (6.0–11.5). No bone marrow aspirate after chemotherapy was made. After the third treatment laboratory studies revealed a mild trombopenia: 98 × 109/L (140–440 × 109/L). The fourth course was delayed 2 weeks. No more complications were noted. During a follow‐up period of 18 months, a clinical evidence of softening of the involved skin was observed. The curren...

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“…296 It has been suggested that all digital mucous cysts connect with this joint. [256][257][258][259][260][261][262][263][264][265][266] Extracorporeal photopheresis and chemotherapy have been used to treat such cases. 298,299 A superficial frequently than previously.…”

Section: Digital Mucous (Myxoid) Cystmentioning

confidence: 99%