| A woman presented with a swollen red leg of 2 weeks' duration. culture of the skin biopsy specimen confirmed this to be an infection caused by sporothrix schenckii. Systemic evaluation led to the diagnosis of Cushing's disease and explained the unusual morphology. Treatment-related complications are also discussed. ( info) |
2/863. poems syndrome, steroid-dependent diabetes mellitus, erythema elevatum diutinum, and rheumatoid arthritis as extramedullary manifestations of plasma cell dyscrasia. poems syndrome is a rare synopsis of different multisystemic disorders (polyneuropathy, organomegaly, endocrinopathy, monoclonal gammapathy, and skin lesions) associated with plasma cell dyscrasia. We herein report the atypical case of a 44-year-old white man presenting with glomerulopathy, poems syndrome, and erythema elevatum diutinum with a few-year history of non-insulin-dependent diabetes mellitus (NIDDM) and seronegative rheumatoid arthritis (RA) as early manifestations of IgAlambda multiple myeloma. The prescription of 1 mg/kg/day prednisone improved the patient's features dramatically. skin lesions improved by the association of glucocorticoids and plasma exchange, recurred when plasmapheresis ceased, and remitted when plasma exchange was reintroduced. NIDDM requiring insulinotherapy recurred when corticoids were discontinued and remitted when prednisone was reintroduced. However, prednisone and plasmapheresis had no effect on polyneuropathy, M-paraprotein, and plasma cell dyscrasia in our patient, who developed indolent multiple myeloma a few years later. We thus concluded that poems syndrome, steroid-dependent diabetes mellitus, rheumatoid arthritis, RA, and skin vasculitis in our patient were triggered by plasma cell dyscrasia. ( info) |
| dyskeratosis congenita is recognized by its dermal lesions and constitutional aplastic anemia in some cases. We report successful allogeneic bone marrow transplantation in two siblings with this disease from their sister, and their long term follow-up. We used reduced doses of cyclophosphamide and busulfan for conditioning instead of total body irradiation. Also, we report late adverse effects of transplantation which are not distinguishable from the natural course of disease. ( info) |
4/863. Adverse skin reaction from vitamin K1. Two patients with erythematous, tender, indurated plaques at the site of intramuscular injections of vitamin K1 are described. In one of these, condition was reproduced by the injection of a test dose. In both cases, there was associated liver disease. The red, indurated plaques cleared in 4-14 days. There have been two previous reports in the literature of similar lesions. Altough some of the patients showed sclerodermatous changes at the same site within 2 years of the injections. These unusual reactions have only been seen in patients with liver disease and when a variety of drugs have been administered. It may be that these factors are important in the initiation of the cutaneous response. ( info) |
5/863. "Centripetal flagellate erythema": a cutaneous manifestation associated with dermatomyositis. We describe 3 patients with dermatomyositis who presented with flagellate erythema. This cutaneous eruption is characterized by erythematous linear lesions on the trunk and proximal extremities. Histologic examination of this eruption in one of our cases revealed an interface dermatitis. review of the literature and records of 183 patients with connective tissue diseases from our institution has shown that this peculiar eruption has been reported only in dermatomyositis. Because of the location of this eruption, we encourage the use of the term "centripetal flagellate erythema" to distinguish this entity from other linear eruptions seen in patients with connective tissue diseases. ( info) |
6/863. Treatment of reticular erythematous mucinosis with a large dose of ultraviolet B radiation and steroid impregnated tape. To date, there are no successful treatments for reticular erythematous mucinosis (REM) other than antimalarial drugs such as chloroquine; these have many adverse side effects and are prohibited for use in japan. We report a case of REM improved by a large dose of ultraviolet B (UVB) radiation and a steroid impregnated tape. The large dose of UVB radiation improved the erythema after 5 weeks. Application of steroid impregnated tape to a lesion where a large dose of UVB had been given produced an additive clinical effect. UVB radiation and steroid impregnated tape are considered to be effective treatments for REM. ( info) |
7/863. Erythema dyschromicum perstans in early childhood. Erythema dyschromicum perstans (EDP) is a rare disorder characterized by asymptomatic, slowly progressive, ash-gray macular pigmentation of the skin which usually occurs from age 5 through adult life. We have experienced two cases of EDP in children aged 2 and 3, both exceptionally younger than the previously reported cases. We therefore suggest that EDP should be included in the differential diagnosis of pigmentary disorders occurring at an early age. ( info) |
| erythema chronicum migrans is characterized by an expanding erythematous band about an arthropod bite. While rare, it is an important disease to recognize because of its rapid response to even modest doses of antibiotics. This response and the association with arthropod bites suggest a microbial vector. Richettsial titers have been demonstrated in several cases but we were unable to show them in our patient, who we believe is the second reported case of erythema chronicum migrans incurred by an American without traveling abroad. ( info) |
9/863. The relationship between facial annular erythema and anti-SS-A/Ro antibodies in three East Asian women. A distinct annular erythema developed on the cheeks of three East Asian women who had anti-SS-A/Ro (SSA) antibodies. The erythema was characterized by a wide, elevated border and central pallor. Histologically, there was a coat-sleeve-like infiltration of lymphocytes around the blood vessels, appendages, and secretory gland cells in the dermis. Immunohistological analysis clarified that the majority of infiltrating lymphocytes were CD4-positive T cells. Abnormal expression of hla-dr antigens in the perivascular, appendage, and secretory gland cells in the dermis was also observed. The differential diagnosis of the three patients lay between Sjogren syndrome (SjS), Sjogren/systemic lupus erythematosus overlap syndrome and an asymptomatic clinical state. These results are consistent with recent findings of major histocompatibility complex class II expression on target organs in various autoimmune diseases. Based on these findings, erythema appears to represent a broad cutaneous manifestation of these diseases. Furthermore, the presence of SSA antibodies, aberrant HLA-DR expression, and sun exposure may be responsible for the development of erythema. ( info) |
10/863. Chemotherapy-induced acral erythema (CIAE) with bullous reaction. Chemotherapy-induced acral erythema (CIAE) is a cutaneous response to a number of different chemotherapeutic agents. It causes a symmetrical, painful erythema of both the palms and soles which is self-limiting. CIAE with bullous reaction has been reported in relation to methotrexate, but it has been more commonly associated with cytosine arabinoside. We describe a case of CIAE with bullous reaction in a patient treated for Hodgkin's disease with a number of chemotherapeutic agents. We discuss the differential diagnosis of this condition which includes eccrine squamous syringometaplasia and acute graft vs. host disease ( info) |