Bilharziasis cutanea tarda: a rare presentation of schistosomiasis.
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Biomedical subjects
Publications and source records attributed to Sarah Brenner.
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In this article, we describe a case of seemingly stress-induced SAPHO (synovitis, acne, pustulosis, hyperostosis, osteitis) syndrome in a man who exhibited the central features of palmoplantar psoriasis and anterior chest involvement. We also review the etiology, pathogenesis, and treatment of SAPHO syndrome and emphasize the important differences between this syndrome and psoriatic arthritis.
BACKGROUND: Despite the high incidence of pemphigus in the Jewish population, data on the epidemiology and etiology of the disease in Israel are sparse. OBJECTIVE: This study was conducted to identify clinical and epidemiologic features of pemphigus patients in Israel, while searching for risk factors that induce or exacerbate the disease. METHODS: Demographic and clinical information was recorded from the charts of 55 pemphigus patients treated over a 5 year period. A sample of 22 patients was compared to 22 age and gender-matched controls by means of a questionnaire querying details on lifestyle, including occupation, diet, sun exposure, and smoking. RESULTS: The findings show that the typical Israeli pemphigus patient is middle-aged, married, and of East European or Middle Eastern origin. The most common diagnosed clinical variant was pemphigus vulgaris, followed by pemphigus erythematosus. Some 70% of patients were treated with two or more immunosuppressive drugs and 62% entered long-lasting remission. Twenty-three percent of patients were exposed through their work to chemical substances, mainly pesticides, at the beginning of the disease and 18% of patients were continually exposed to ultraviolet radiation 5 years prior to onset of the disease. CONCLUSIONS: There is a possible correlation between occupational exposure to pesticides and UV radiation, and pemphigus induction.
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Autoimmune vesiculobullous skin diseases are a common occurrence in dermatology practice and do have gender differences. While most dermal antigens and autoantibodies in these diseases have been identified, the etiology and clinical characteristics of the autoimmunity remain obscure, such as why more than 70% of autoimmune diseases are more frequent in women. Researching gender differences and the mechanisms of autoimmune diseases will shed light on the answers to such questions.
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The acronym PEMPHIGUS is suggested to encompass the numerous factors involved in the pathogenesis and course of the disease. In the following review the authors present studies documenting these factors. The acronym can serve as a handy tool to direct the physician's investigation of a case of pemphigus, aiding in its diagnosis and in the prevention of future flare-ups.
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Male gender is interpreted as a risk factor for developing mycosis fungoides and perhaps other cutaneous and extracutaneous lymphomas. In this review the authors examine epidemiologic, clinical, pathologic, and molecular data in an effort to understand whether and to what extent gender or sex hormones are involved in the pathogenesis of lymphoproliferative disorders.
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A 54-year-old man of Persian origin presented to our department with a 1-year history of ulcers on the right leg that had been unresponsive to numerous topical treatments, accompanied by lymphedema of the right leg. Medical history included hypergonadotropic hypogonadism, which had not been further investigated. He was treated for 20 years with testosterone IM once monthly, which he stopped a year before the current hospitalization for unclear reasons. The patient reported no congenital lymphedema. Physical examination revealed two deep skin ulcers (Figure 1) on the right leg measuring 10 cm in diameter with raised irregular inflammatory borders and a boggy, necrotic base discharging a purulent hemorrhagic exudate. Bilateral leg pitting edema and right lymphangitis with lymphadenitis were noted. He had low head hair implantment, sparse hair on the body and head, hyperpigmentation on both legs, onychodystrophia of the toenails (mainly the large toe and less prominent on the other toes), which was atrophic lichen-planus-like in appearance and needed no trimming (Figure 2), normal hand nails, oral thrush, and angular cheilitis. Other physical findings were gynecomastia, pectus excavatum, small and firm testicles, long extremities, asymmetrical goiter, systolic murmur 2/6 in left sternal border, and slow and inappropriate behavior. The patient's temperature on admission was 39 degrees C. Blood cultures were negative for bacterial growth. Results of laboratory investigations included hemoglobin (11.2 g/dL), hematocrit (26.8%), normal mean corpuscular volume and mean corpuscular hemoglobin volume, and red blood cell distribution width (16%). Blood smear showed spherocytes, slight hypochromia, anisocytosis, macrocytosis, and microcytosis. Blood chemistry values were taken for iron (4 micro g/dL [normal range 40-150 micro g/dL]), transferrin (193 mg/dL [normal range 220-400 mg/dL]), ferritin (1128 ng/mL [normal range 14-160 ng/mL]), transferrin saturation (1.5% [normal range 20%-55%]), serum folate (within normal limits), and vitamin B12 (within normal limits). Direct Coombs' test equaled positive 2 + IgG. All these values indicated anemia of chronic diseases combined with hemolytic anemia. Further blood work-up tested antinuclear antibody (positive <1:80 homogeneous pattern), rheumatoid factors (143 IU/mL [positive >8.5 IU/mL]), C-reactive protein (286 mg/L [normal range 0-5 mg/L]), anticardiolipin IgM antibody (9.0 monophosphoryl lipid U/mL [normal range 0-7.00 MPL U/mL]) and antithrombin III activity (135% [normal range 74%-114%]). Results of other blood tests were within normal limits or negative, including lupus anticoagulant, beta2 glycoprotein, anticardiolipin IgG Ab, anti-ss DNA Ab, C3, C4, anti-RO, anti-LA, anti-SC-70, anti-SM Ab, P-ANCA, C-ANCA, TSH, FT4, anti-T microsomal, antithyroglobulin, protein C activity, protein S free, cryoglobulins, serum immunoelectrophoresis, VDRL, hepatitis C antibodies, hepatitis B antigen, and human immunodeficiency virus. Endocrinological work-up examined luteinizing hormone (22.9 mIU/mL [normal range for adult men 0.8-6 mIU/mL]), follicle stimulating hormone (49.7 mIU/mL [normal range for adult men 1-11 mIU/mL]), testosterone (0.24 ng/mL [normal range for adult men 2.5-8.0 ng/mL]), bioavailable testosterone (0.02 ng/mL [normal range for adult men >0.6 ng/mL]), and percent bioavailable test (8.1% [normal value >20%]). These results indicate hypergonadotropic hypogonadism. Plasminogen activator inhibitor 1 was 6 U (normal value 5-20 U/mL). Karyotyping performed by G-banding technique revealed a 47 XXY karyotype, which is diagnostic of Klinefelter's syndrome. Doppler ultrasound of the leg ulcers disclosed partial thrombus in the distal right femoral vein. X-rays and bone scan displayed osteomyelitis along the right tibia. Histological examination of a 4-mm punch biopsy from the ulcer border revealed hyperkeratosis, acanthosis, hypergranulosis, and mixed inflammatory infiltrate containing eosinophils compatible with chronic ulcer. Multiple vessels were seen, compatible with a healing process. Direct immunofluorescence of the biopsy revealed granular IgM in the dermo-epidermal junction. Indirect immunofluorescence was negative. Thyroid function tests showed normal thyroid stimulating hormone and free throxine4. Multinodular goiter was seen on thyroid scan and ultrasound. Thyroid fine needle aspiration was compatible with multinodular goiter (normal follicular cells, free colloid, macrophages with pigment). IV treatment with amoxicillin-clavulanic acid 1 g t.i.d. was administered for 2 weeks, with a decrease in temperature and normalization of the leukocyte level. Oral antibiotic treatment with amoxicillin-clavulanic acid was continued for 10 more days, followed by 25 days of ciprofloxacin for the osteomyelitis. Local treatment included saline soakings followed by application of Promogran (Johnson & Johnson, New Brunswick, NJ) and Kaltostat (ConvaTec Ltd., a Bristol-Myers Squibb Company, New York, NY) with slight improvement. At the same time, the patient was treated with warfarin sodium due to deep vein thrombosis under international normalized ratio 2-3. The patient was treated with IM testosterone once monthly for 1 year, which resulted in a reduction in the diameter and depth of the leg ulcers (Figure 3). Blood tests were not performed for follow-up of the immune state.