11/61. Widespread scleredema accompanied with a monoclonal gammopathy in a patient with advanced ankylosing spondylitis. Scleredema is a rare cutaneous mucinosis characterized by chronic diffuse induration of the skin, and it is occasionally associated with a monoclonal gammopathy (MG). Ankylosing spondylitis (AS) is noted to be another, chronic systemic inflammatory disorder of the axial skeleton that may accompany the MG. However, patients with scleredema and AS accompanied with a MG have not been reported in the literature. We here report a 40-yr-old man with scleredema and advanced AS accompanied with a MG of IgA-kappa protein. Widespread, long-standing scleredema has been developed over 10 yrs after the initial manifestation of AS. It is uncertain whether the coexistence of scleredema and AS is more than coincidental. ( info) |
12/61. UVA-1 as a treatment for scleredema. Scleredema is a rare disease that is difficult to treat. Many therapies have been tried with varied and somewhat inconsistent results. Here we report two cases of scleredema successfully treated with low-dose UVA-1. ( info) |
13/61. Scleredema of Buschke: remission with factor xiii treatment. We describe the case of a 55-year-old man with scleredema of Buschke of the torso complicated by insulin-dependent diabetes mellitus. Due to (i) the patient's poor general health status, (ii) the similarity between scleroderma and scleredema of Buschke, and (iii) the well known efficacy of factor xiii infusions in scleroderma, we attempted an intravenous treatment with factor xiii. This therapy resulted in marked increase of movements and in softening of the skin, together with ultrasonographic and histopathological improvements. In conclusion, to the best of our knowledge, this is the first case in which factor xiii has been successfully used for the treatment of scleredema of Buschke. ( info) |
| scleredema adultorum is a rare skin disorder reported to occur usually following streptococcal infections. It has characteristic clinical and histopathologic findings; however, associated hyperkeratosis is not known. We report a 6-year-old girl with scleredema adultorum and hyperkeratosis. The patient's disease had a benign course. To our knowledge, this patient is the first reported with scleredema adultorum associated with hyperkeratosis. ( info) |
15/61. acanthosis nigricans in a plaque of scleredema on the back of a diabetic patient: a case report. acanthosis nigricans (AN) and scleredema are two skin conditions that have been reported in association with diabetes mellitus. Few cases associating scleredema and AN have been reported. A literature search did not reveal any reports of diabetic patients developing AN on top of a scleredema plaque. Here we report a patient with diabetes mellitus who developed AN within the same indurated scleredema plaque. ( info) |
16/61. Scleredema and smoldering myeloma. A 46-year-old white man had classic findings of scleredema; subsequently, a monoclonal immunoglobulin g lambda light chain was detected in his serum. Findings of a bone marrow biopsy specimen revealed that he had an increased percentage of plasma cells, some of which were atypical. A diagnosis of scleredema and smoldering myeloma was made. ( info) |
17/61. scleredema adultorum of Buschke presenting as periorbital edema: a diagnostic challenge. scleredema adultorum is a rare sclerotic disorder characterized by diffuse swelling and nonpitting induration of the skin. Its occurrence has been documented in association with infections, diabetes mellitus, paraproteinemia, multiple myeloma, and monoclonal gammopathy. We report an unusual case of a 48-year-old man with an asymptomatic bilateral eyelid edema of sudden onset. During a period of 6 months, the condition slowly progressed to extensive nonpitting edematous swelling restricted to the periorbital sites. The presumptive diagnosis of scleredema adultorum was confirmed by the presence of typical histologic findings. This case is unique in that the periorbital swelling remained as the sole clinical manifestation of scleredema during the 5-year follow-up and was complicated with partial vision blockage. ( info) |
| Scleredema (also called scleredema of Buschke) is a fibromucinous connective tissue disorder of unknown cause that belongs to a group of scleroderma-like disorders. We report the case of a 64-year-old lady with long-standing scleredema, associated with a paraprotein, and progressing to multiple myeloma and AL amyloidosis. The relationship of scleredema with paraprotein and multiple myeloma is well established, but only two cases of scleredema associated with amyloidosis have been reported to date. We suggest that amyloidosis may be underdiagnosed in patients with scleredema and paraproteinaemia. Features attributed to extracutaneous manifestations of scleredema could represent systemic amyloidosis. We review published reports of scleredema associated with paraprotein and discuss the difficulties in the differential diagnosis of scleroderma-like disorders. We discuss the diagnosis of plasma cell dyscrasias and amyloidosis and their relevance in rheumatology practice. ( info) |
| scleredema adultorum is a rare connective tissue disorder of unknown cause. Both bath-PUVA and cream-puva therapy were reported to be effective. We describe a patient with scleredema adultorum who showed a striking clinical improvement with a medium-dose UVA1 phototherapy (single dose, 50 J/cm(2); 35 treatments). ( info) |
20/61. Scleredema of Buschke in pediatric age group. Two cases of scleredema of Buschke are described, which occurred in pediatric age group--an uncommon occurrence after febrile illness. Both cases were self-limiting. Characteristic features are described. ( info) |