1/13. Bazex syndrome mimicking a primary autoimmune bullous disorder.Bazex syndrome is a paraneoplastic condition that is most frequently associated with squamous cell carcinoma of the upper aerodigestive tract. The lesions affect acral areas of the skin, including hands, feet, ears, nose, and, to a lesser extent, elbows and knees. Lesions mimic psoriasis and dermatitis. paronychia and nail dystrophy are frequent. Bullous lesions have been reported only rarely. We report a patient with Bazex syndrome with predominantly bullous lesions that mimicked a primary autoimmune bullous disorder.- - - - - - - - - - ranking = 1keywords = upper (Clic here for more details about this article) |
2/13. sarcoidosis presenting as nail dystrophy.A 45-year-old woman was referred to the dermatology clinic for assessment of "refractory onychogryphosis." She had a 3-year history of lesions involving distal phalanges of the first and third of her left foot. Initially she described periungual erythema and swelling. Three weeks later she noted a whitish growth and thickening of her third toenail. X-ray films of the digit were reported as normal. Several months later the same changes occurred in her great toe. These lesions were asymptomatic. There was no history of trauma. Numerous fungal cultures were negative. No light microscopic examinations were undertaken. She had a trial of both topical and systemic terbinafine of 3-months duration with no clinical improvement. Several clinical opinions were obtained from two dermatologists, a surgeon, and a chiropodist. Past medical history of note was significant for tubal ligation, cervical cancer, and chronic sinusitis. The latter condition in retrospect was thought to be secondary to sarcoidosis. physical examination revealed periungual violaceous discolouration of the first and third toes of the left foot. There was evidence of significant nail changes including dystrophy, onycholysis, and hyperkeratosis (Fig. 1). The fingernails were normal. There were no other skin abnormalities. A punch biopsy of the tip of the third toe showed granulomatous inflammation. There was evidence of hyperkeratosis, exocytosis, and a dense infiltrate composed of collections of histiocytes and a few giant cells forming granulomas (Fig. 2). Repeat x-ray films of the foot showed soft tissue swelling of the first and third digits. There was bony resorption in the distal phalanges with a lacey trabecular pattern compatible with sarcoidosis (Fig. 3). Chest x-ray films revealed marked hilar adenopathy. The patient was sent to a respirologist who concurred with the diagnosis of sarcoidosis. Further investigations included a low serum calcium of 2.07 mmol/L, serum ACE of 70 U/L (upper limit of normal is 75), Wintrobe erythrocyte sedimentation rate (ESR) of 10 mm per hour, thyroid stimulating hormone concentration of 0.65 mU/L, and a urinary calcium excretion rate that was elevated at 7.3 mmol/day. Pulmonary function tests were unremarkable. The patient was initially treated with clobetasol under occlusion and intralesional triamcinolone with minimal improvement. She was subsequently started on prednisone, 15 mg per os daily because of the lung and bone involvement with significant improvement noted in the toe lesions with diminution of both the swelling and violaceous discolouration.- - - - - - - - - - ranking = 1keywords = upper (Clic here for more details about this article) |
3/13. Docetaxel-induced nail changes--a neurogenic mechanism: a case report.Docetaxel is a new taxoid widely used in chemotherapy for advanced breast cancer and other solid malignancies. Painful nail changes with onycholysis occur in about 40% of docetaxel-treated patients as a prominent adverse effect. We report a patient with a complete peripheral palsy of the right arm due to advanced breast cancer with diffuse tumor infiltration of the brachial plexus. Treatment with docetaxel led to onycholysis at all extremities except the paretic hand. Sensory and motoric innervation measured by nerve conduction studies showed a complete loss of large nerve fiber function of the right arm. Function of deep mechanosensitive A beta-fibers (quantitative vibrametry) was severely decreased, but not absent. Sympathetic reflexes (induced by deep inspiration and measured with laser Doppler flowmetry) were absent on the right side and skin temperature was decreased consistent with a complete sympathetic denervation. Small afferent fibers investigated by quantitative thermotesting revealed a total loss of thermal and pain sensation. Furthermore, iontophoresis of histamine failed to induce any axon reflex-vasodilatation indicating a complete peripheral degeneration of small fiber afferents. In summary, a severe denervation of small and large fibers of the right upper limb was revealed. These results indicate that integrity of peripheral nerves seems to be a substantial factor for docetaxel-mediated nail changes. The role of an inflammatory process in onycholysis maintained by postganglionic sympathetic terminals and nociceptive C-fiber afferents is discussed. In accordance with this hypothesis, a cyclooxygenase-2 inhibitor improved nail alterations of the non-paretic limbs.- - - - - - - - - - ranking = 1keywords = upper (Clic here for more details about this article) |
4/13. Acrokeratosis paraneoplastica of Bazex.Bazex disease is one of the rarer cutaneous paraneoplastic syndromes. It is characterized by psoriasiform changes on the digits, and in some patients spread to the ears, nose and in later stages to the limbs and trunk. The associated malignancy is typically a squamous cell carcinoma of the upper aerodigestive tract. We review the literature regarding acrokeratosis paraneoplastica of Bazex and report three cases which illustrate both the typical and some of the less common changes that are seen in the condition.- - - - - - - - - - ranking = 1keywords = upper (Clic here for more details about this article) |
5/13. Coexistent yellow nail syndrome and selective antibody deficiency.BACKGROUND: yellow nail syndrome (YNS) is a rare, often underdiagnosed condition of unknown origin. The clinical features of the syndrome include yellow nails, chronic sinusitis, bronchiectasis, pleural effusion, and lymphoedema. Despite the frequent occurrence of upper and lower respiratory tract infections in patients with YNS, comprehensive analysis of their humoral immunity has not been previously reported. OBJECTIVE: To present the case of a patient with YNS whose recurrent upper and lower respiratory tract infections may have been caused by an underlying selective antibody deficiency that manifests as impaired IgG antibody response to polysaccharide antigens. methods: The patient underwent cultures of purulent sputum for streptococcus pneumoniae and haemophilus influenzae, bronchial washings for H. influenzae, and nail scrapings for fungi. Her serum levels of IgG, IgA, IgM, IgG subclasses, and serum titers of IgG antitetanus toxoid, anti-H. influenzae, and anti-S. pneumoniae antibodies were measured. RESULTS: Cultures of purulent sputum were positive on multiple occasions for S. pneumoniae and H. influenzae and bronchial washings were positive for H. influenzae. Nail scrapings were consistently negative for fungi. She had no reductions in serum levels of IgG, IgA, IgM, or IgG subclasses and had normal serum titers of IgG antitetanus toxoid antibodies. However, she demonstrated impaired IgG antibody responses following immunization with Pneumovax and an H. influenza B vaccine. CONCLUSIONS: This case report describes the first comprehensive analysis of humoral immune function in a patient with YNS. The finding of a selective antibody deficiency in our patient provides a potential explanation for the occurrence of respiratory infections in YNS. Accordingly, we recommend that functional antibody determinations and quantitative serum immunoglobulins be evaluated in patients diagnosed as having this unusual, enigmatic syndrome.- - - - - - - - - - ranking = 2keywords = upper (Clic here for more details about this article) |
6/13. Transverse leukonychia in severe hypocalcemia.We report the case of a 26-year-old man with acral tetany and muscular cramps of the upper limbs associated with hypocalcemia caused by hypoparathyroidism and celiac disease. physical examination revealed a transverse leukonychia which disappeared after treatment with calcium lactogluconate, calcium carbonate and calcitriol. hypocalcemia may induce nail alterations with a double mechanism represented by angiospasm and disorganization of the hard keratin and of the integrin subunits.- - - - - - - - - - ranking = 1keywords = upper (Clic here for more details about this article) |
7/13. Subungual epidermoid inclusions.Subungual epidermoid inclusions are protrusions of the nail bed epithelium into the upper most bed stroma. They may be associated with hyperplasia of the bed epithelium resulting in subungual keratosis, onycholysis or even clubbing. An 11-year-old girl presented to our dermatology clinic with a one year history of progressive increase in the size of the distal part of her left thumb associated with mild pain on pressure. The diagnosis became evident perioperatively, which showed multiple subungual epidermoid inclusions.- - - - - - - - - - ranking = 1keywords = upper (Clic here for more details about this article) |
8/13. Dystrophic epidermolysis bullosa with toe nail deformities--report of a case in benin City, nigeria.We report the first case of Dystrophic epidermolysis bullosa with toe nail deformities in a Nigerian child in benin City. The patient had characteristic dermolytic bullae on the skin and upper gastrointestinal tract mucosa with conjunctivities, dystrophic lesions of the toe nails and dysplastic teeth.- - - - - - - - - - ranking = 1keywords = upper (Clic here for more details about this article) |
9/13. Bazex's syndrome (acrokeratosis paraneoplastica)--first case report of association with a bladder carcinoma.Bazex's acrokeratosis paraneoplastica is a clinically well-defined entity consisting of a distal erythemato-squamous eruption and nail dystrophy. It is always associated with neoplasia, mainly squamous carcinomas of the upper digestive and respiratory tracts; therefore, Bazek's acrokeratosis is considered to be a specific marker of internal malignancy. Two new cases of this syndrome are reported. The first was in a 41-year-old man with a squamous cell carcinoma of the oesophagus, the second in a 73-year-old man who presented with a transitional cell bladder carcinoma, an association not previously reported.- - - - - - - - - - ranking = 1keywords = upper (Clic here for more details about this article) |
10/13. dyskeratosis congenita or chronic graft-versus-host disease? A diagnostic dilemma in a child eight years after bone marrow transplantation for aplastic anemia.A 12-year-old boy had striking reticulate hyperpigmentation of the neck and upper chest, dystrophic nails, patchy alopecia, and a white streak on the buccal mucosa. He was diagnosed as having chronic graft-versus-host disease (GVHD) based on clinical findings, skin biopsy findings, and his history of a bone marrow transplantation for aplastic anemia eight years earlier. dyskeratosis congenita (DC) was not a diagnostic consideration, although the clinical findings and history of aplastic anemia made it a compelling possibility. This case highlights the clinical similarities between DC and chronic GVHD and the difficulty in arriving at an unequivocal diagnosis.- - - - - - - - - - ranking = 1keywords = upper (Clic here for more details about this article) |
| | Next -> |