The classical type of Ehlers-Danlos syndrome (EDS) is an inherited disorder characterized by fragile connective tissue shown to be caused by a type V collagen gene abnormality in cases in Europe and United States. Here in we report three Japanese cases of EDS classical type: Case 1, a 45-year-old woman; Case 2, a 30-year-old woman; and Case 3, a 27-year-old man, who is a brother of the Case 2. All three cases showed the typical clinical features of EDS classical type, including skin hyperextensibility, joint hypermobility, and tissue fragility. All three cases suffer from dislocation and subluxation of joints, and severe muscle weakness, resulting in many problems in their daily lives. Rehabilitation and daily muscle training in cooperation with orthopedists may avoid these complications. Therefore, earlier clinical diagnosis by dermatologists is important for EDS patients to obtain information to improve their quality of life.
We reviewed 11 cases of necrotizing fasciitis (NF) that were treated in our dermatology clinic in Osaka City General Hospital. We examined the recovered isolates, clinical laboratory data, and associated diseases. We also investigated the relationship between these findings and patient prognosis. Six strains of Streptococcus pyogenes, three strains of Staphylococcus aureus, and two strains each of Streptococcus agalactiae and Streptococcus equisimilis were isolated from these 11 cases of NF. Seven cases of NF were associated with diabetes mellitus. Marked elevation of CRP (an average of 19.9mg/dl), marked leukocytosis (an average of 21,155/μl), and renal dysfunction were detected in three patients. All the cases showed a good prognosis following treatment with strong systemic antibiotics, along with extensive debridement. The legs were amputated in four of these NF cases. Prompt extensive debridement and powerful antimicrobial treatment are beneficial in the treatment of NF, but amputations may be required in patients with life-threatening disease.
Detection of lupus anticoagulant (LA) is essential in the diagnosis of antiphospholipid syndrome (APS). However, this test may often be falsely negative, due to technical errors. We carefully prepared platelet free plasma and measured LA by use of three different methods: diluted Russell’s viper venom time, kaolin clotting time, and platelet neutralization procedure. We detected LA in 22 of 30 (73%) patients who presented with skin lesions such as livedo reticularis and leg ulcers. Seventeen of these 22 LA-positive patients had underlying collagen diseases or vascular diseases, including cutaneous polyarteritis nodosa (6), systemic lupus erythematosus (4), livedo vasculopathy (3), systemic sclerosis (2), overlap syndrome (1), and rheumatoid arthritis (1). The remaining five patients were diagnosed as primary APS. Anticardiolipin antibody (aCL) was positive in only four (18.2%) of the 22 LA-positive patients. We also investigated phosphatidylserine-dependent anti-prothrombin antibody (aPS/PT), which was detected in 10 (50%) of 20 the LA-positive cases examined. Radiological examinations revealed arterial or venous thromboses in other internal organs in 50% of the cases. These results strongly suggest the importance of appropriate, accurate LA detection tests in patients with livedo reticularis and leg ulcers.
During a one-year period from October 2004 to October 2005, we experienced five cases of erythema multiforme type drug eruption that appeared during or after Helicobacter pylori eradication therapy for peptic ulcer. A combination pharmaceutical preparation (brand name: Ransappu) that included lansoprazole, amoxicillin, and clarithromycin was used for H. pylori eradication therapy in four of the five cases. Results of drug lymphocyte stimulation tests were positive in three of the five cases (positive for amoxicillin in two cases and for clarithromycin in one case). One case was positive in a patch test (amoxicillin). One case was negative in both tests but was diagnosed as drug eruption due to H. pylori eradication therapy on the basis of the clinical course.