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Unusual Association (unusual + association)
Selected AbstractsAn unusual association with carcinoma pancreas: a case reportCYTOPATHOLOGY, Issue 4 2005S. Mohan No abstract is available for this article. [source] An unusual association of pemphigus vulgaris with hyperprolactinemiaINTERNATIONAL JOURNAL OF DERMATOLOGY, Issue 10 2002MNAMS, Sujay Khandpur MD A 21-year-old unmarried woman presented with oral ulcerations and generalized, itchy, fluid-filled, skin lesions of 10 days' duration. The lesions ruptured spontaneously, resulting in extensive denuded areas covered by crusts. One month prior to this, she experienced pain and enlargement of both breasts with galactorrhea. Her menstrual cycles were normal initially, but later she developed menstrual irregularities. No past history suggestive of any other systemic or skin disease, including atopy or drug allergies, could be obtained. Her family history was not contributory. Dermatologic examination revealed multiple, flaccid bullae and extensive denuded areas of skin covered with crusts over the scalp, face, trunk, and upper and lower limbs (Fig. 1). Bulla spread sign and Nikolsky's sign were positive. The oral mucosa, including the lips, buccal surface, tongue, and palate, showed multiple erosions covered with necrotic slough. The rest of the mucocutaneous and systemic examination was within normal limits. Figure 1. Extensive erosions and flaccid bullae over the trunk with breast enlargement The patient's diagnostic work-up revealed: hemoglobin, 11.2 g%; total leukocyte count, 7400/mm3; differential leukocyte count, P62L34E2M2; erythrocyte sedimentation rate, 34 mm/h. A peripheral blood smear examination, urinalysis, blood sugar, and renal and liver function tests were normal. Venereal Disease Research Laboratory (VDRL) test and enzyme-linked immunoabsorbent assay (ELISA) for human immunodeficiency virus (HIV) were nonreactive. Antinuclear antibody, lupus erythematosus (LE) cell, rheumatoid factor, and anti-dsDNA levels were normal. Serum protein electrophoresis demonstrated increased levels of immunoglobulin G (IgG) antibody. The serum prolactin level was significantly raised to 139.49 ng/mL (normal, 3.6,18.9 ng/mL). The sex hormone levels, however, including follicle-stimulating hormone (FSH), luteinizing hormone (LH), estradiol, and progesterone, were within normal limits. The thyroid hormone profile was also unaltered. Chest X-ray was normal. Ultrasound of the abdomen and pelvis revealed no visceral abnormality and computerized tomography (CT) scan of the pituitary sella showed no adenoma. Mammography was negative for breast malignancy. A Tzanck smear prepared from the base of the erosion showed multiple acantholytic cells and lymphocytes. Histologic examination from an intact vesicle was suggestive of pemphigus vulgaris (PV), showing a suprabasal cleft with acantholytic cells and the basal layer demonstrating a "row of tombstones" appearance (Fig. 2). Direct immunofluorescence (DIF) revealed the intercellular deposition of IgG and C3 throughout the epidermis in a "fishnet pattern." Indirect immunofluorescence (IIF) test performed on rat esophagus for circulating IgG antibody was positive in a titer of 1 : 120. Figure 2. Photomicrograph showing suprabasal cleft with "row of tombstones" appearance, suggestive of pemphigus vulgaris (hematoxylin and eosin, × 40) Based on the clinical and immunohistological features, a diagnosis of PV with idiopathic hyperprolactinemia was made. The patient was treated with bromocriptine mesylate (Tablet Proctinal, Glaxo Wellcome Ltd, India) at a dose of 2.5 mg twice a day. After 2 months of therapy, significant improvement in the skin lesions was observed. The existing lesions re-epithelialized with a drastic reduction in the number and distribution of new vesicles. However, no change in the mucosal erosions was noticed. IIF test demonstrated a lower antibody titer (1 : 40). The breast complaints also improved with a reduction in serum prolactin level to 6.5 ng/mL. The patient refused further treatment as she experienced nausea and dizziness with bromocriptine. After 2 weeks, the disease relapsed with the appearance of new vesicles over the forearms, abdomen, back, and thighs. She again complained of breast tenderness and galactorrhea, and the serum prolactin level was 95 ng/mL. The IgG titer increased to 1 : 120. Hence, treatment with oral prednisolone (2 mg/kg/day) and bromocriptine (2.5 mg twice a day) with an antiemetic was initiated. After 6 weeks, the skin lesions had cleared completely, the breast symptoms had improved, menses had become regular, and the prolactin level had decreased to 4 ng/mL. IIF test was negative for circulating antibody. Steroids were tapered off and maintenance therapy with bromocriptine at a dose of 2.5 mg/day was continued. [source] Multiple perforating and non perforating pilomatricomas in a patient with Churg,Strauss syndrome and Rubinstein,Taybi syndromeJOURNAL OF THE EUROPEAN ACADEMY OF DERMATOLOGY & VENEREOLOGY, Issue 5 2004P Bayle SUMMARY We report an unusual association of multiple perforating and non-perforating pilomatricomas with Churg,Strauss syndrome, and a dysmorphic syndrome evocative of Rubinstein-Taybi syndrome. These syndromes may be independent, but these rare diseases and genetic abnormalities may be linked together. [source] Squamous cell carcinoma arising in long-standing necrobiosis lipoidicaJOURNAL OF THE EUROPEAN ACADEMY OF DERMATOLOGY & VENEREOLOGY, Issue 2 2004J Santos-Juanes ABSTRACT Necrobiosis lipoidica (NL) is a disease of collagen. Squamous cell carcinomas developing in areas of chronic ulceration and scarring have been well documented in a variety of skin diseases but rarely in areas of necrobiosis lipoidica. The case history of a 76-year-old female is presented, whose squamous cell carcinoma appeared 30 years after the diagnosis of necrobiosis lipoidica. The clinical and histopathological picture is described, stressing the importance of the unusual association of the two pathologies in the prognostic. [source] Pachydermoperiostosis and psoriatic onychopathy: an unusual associationJOURNAL OF THE EUROPEAN ACADEMY OF DERMATOLOGY & VENEREOLOGY, Issue 1 2003P Fietta ABSTRACT A 33-year-old man, suffering from cutaneous psoriasis since the age of 16, in the last 6 years experienced slow and painless enlargement at his fingertips and later at his big toes, which resulted in digital clubbing. Since the age of 31, the patient also presented psoriatic nail changes involving all his fingernails, without joint pain or inflammation. The patient's family history was negative for psoriasis, however, his brother, a 29-year-old healthy man, also presented digital clubbing. The diagnosis of pachydermoperiostosis coexistent with ungual and cutaneous manifestations of psoriasis vulgaris was made. The differential diagnosis with psoriatic onycho-pachydermo-periostitis, as well as other clinical conditions that involve the distal interphalangeal joints is discussed. [source] The changing faces of Streptococcus antigen I/II polypeptide family adhesinsMOLECULAR MICROBIOLOGY, Issue 2 2010L. Jeannine Brady Summary Streptococcus mutans antigen I/II (AgI/II) protein was one of the first cell wall-anchored adhesins identified in Gram-positive bacteria. It mediates attachment of S. mutans to tooth surfaces and has been a focus for immunization studies against dental caries. The AgI/II family polypeptides recognize salivary glycoproteins, and are also involved in biofilm formation, platelet aggregation, tissue invasion and immune modulation. The genes encoding AgI/II family polypeptides are found among Streptococcus species indigenous to the human mouth, as well as in Streptococcus pyogenes, S. agalactiae and S. suis. Evidence of functionalities for different regions of the AgI/II proteins has emerged. A sequence motif within the C-terminal portion of Streptococcus gordonii SspB (AgI/II) is bound by Porphyromonas gingivalis, thus promoting oral colonization by this anaerobic pathogen. The significance of other epitopes is now clearer following resolution of regional crystal structures. A new picture emerges of the central V (variable) region, predicted to contain a carbohydrate-binding trench, being projected from the cell surface by a stalk formed by an unusual association between an N-terminal ,-helix and a C-terminal polyproline helix. This presentation mode might be important in determining functional conformations of other Gram-positive surface proteins that have adhesin domains flanked by ,-helical and proline-rich regions. [source] Restless legs syndrome: an early manifestation of Huntington's disease?ACTA NEUROLOGICA SCANDINAVICA, Issue 4 2009E. Savva Background,,, Far from being uniform, Huntington's disease (HD)'s phenotype encompasses a large variety of motor and non-motor symptoms which occur in various combinations in individual patients. Aim,,, To describe an unusual association between restless legs syndrome (RLS) and HD. Methods and results,,, We report a patient with an atypical presentation of genetically demonstrated HD, who developed typical RLS a few years prior to and during the course of HD. Common causes of RLS were excluded and the polysomnography confirmed frequent and severe periodic limb movements during sleep and while awake. Conclusions,,, We propose RLS as an uncommon but early feature of HD in some patients, and suggest adding HD to the already long list of RLS secondary to neurodegenerative conditions. [source] Choroidal neovascularization associated with cancer-associated retinopathyACTA OPHTHALMOLOGICA, Issue 5 2010Giuseppe Querques Abstract. Purpose:, To report an unusual association between cancer-associated retinopathy (CAR) associated with invasive thymoma and choriodal neovascularization (CNV), treated by photodynamic theraphy (PDT). Methods:, A 39-year-old man affected with thymoma and paraneoplastic syndrome (myasthenia gravis and diarrhoea) was observed between October 1997 and September 2007. The patient developed progressive visual dysfunction including bilateral visual acuity loss and concentric constriction of visual fields. Ophthalmological, immunological and systemic examinations were performed. Immunological evaluations included an assessment of antibody activity by indirect immunohistochemistry on sectioned rhesus monkey eye, and Western blot reactions upon an extract of pig retina. Results:, Fundus ophthalmoscopy and fluorescein angiography revealed retinal vessel attenuation and retinal pigment epithelium degeneration. Electroretinogram suggested both rod and cone dysfunction. Indirect immunohistochemistry identified antibody activity within the photoreceptor outer segments. Western blots on the retina revealed that most of the patient's antibody activity was focused upon a retinal protein antigen approximating 145 kD. These findings share the commonalities of size and retinal distribution of the interphotoreceptor retinoid-binding protein (IRBP), a recognized autoantigen. The surgically resected mediastinal tumour was diagnosed as invasive thymoma. No other malignancy has since been found throughout nearly 10 years of follow-up. In March 2006, the patient developed a subfoveal CNV in his left eye, which was treated by PDT. Conclusion:, We describe the third case of paraneoplastic retinopathy associated with invasive thymoma. This is the first example of CAR involving autoantibodies reactive with a retinal protein having the characteristics of the IRBP, and is also the first complicated by CNV treated by PDT. [source] An unusual association between scalp psoriasis and ophiasic alopecia areata: the Renbök phenomenonCLINICAL & EXPERIMENTAL DERMATOLOGY, Issue 3 2007P. R. Criado No abstract is available for this article. [source] An unusual association of pigmentary mosaicism (hypomelanosis of Ito) with generalized hypertrichosisCLINICAL & EXPERIMENTAL DERMATOLOGY, Issue 3 2006S. Khandpur No abstract is available for this article. [source] | ||||||||||