1/23. 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.- - - - - - - - - - ranking = 1keywords = gland (Clic here for more details about this article) |
2/23. Excessive concern with physical appearance leading to a diagnosis of adrenal adenoma.We report an unusual case of Cushing's syndrome manifested by anxiety over body image in a 26-year-old nurse. Initial presentation was a 'dermatological non-disease'. Clinical signs became increasingly evident during the course of the disease, justifying an adrenal gland evaluation which showed a secreting adrenocortical adenoma. Surgical treatment led to recovery with return to normal appearance and cessation of her aesthetic complaints. Our observation shows that persistent aesthetic complaints may sometimes reveal early hypercorticism.- - - - - - - - - - ranking = 0.5keywords = gland (Clic here for more details about this article) |
3/23. Parotid oncocytoma in the birt-hogg-dube syndrome.birt-hogg-dube syndrome consists of fibrofolliculomas, trichodiscomas, and acrochordons and has been associated with several noncutaneous tumors. We report the first case of birt-hogg-dube syndrome in association with a parotid oncocytoma, a rare salivary gland tumor.- - - - - - - - - - ranking = 0.5keywords = gland (Clic here for more details about this article) |
4/23. Dermopathic changes in hypozincemia.In seven patients with chronic debilitating gastrointestinal disease who were being given total parenteral nutrition using L-amino acids and concentrated dextrose solution, severe hypozincemia (less than 60 micrograms/dL) developed with characteristic skin lesions and alopecia. The lesions were similar to seborrheic dermatitis, and they occurred in areas of high sebaceous gland concentration. Hyperkeratotic lesions were also seen on the extensor surfaces of large joints. The skin lesions and alopecia disappeared when serum zinc values were raised to levels above 60 micrograms/dL, with appropriate zinc sulfate replacement. A hypothesis on the etiology and clinical manifestations is presented.- - - - - - - - - - ranking = 0.5keywords = gland (Clic here for more details about this article) |
5/23. Cowden disease. Report of two additional cases.Two patients with Cowden disease, a syndrome consisting of cutaneous papules and nodules on the face, oral mucous membranes and dorsal aspects of the forearms and hands in association with tumours of the thyroid gland, breasts, gastro-intestinal tract, and female reproductive tract, are reported. This disease is important to the physician because recognition of these cutaneous lesions may allow early diagnosis of internal neoplasia.- - - - - - - - - - ranking = 0.5keywords = gland (Clic here for more details about this article) |
6/23. En coup de sabre.A man born in 1961 was apparently well until his marriage in May 1986. In August of that year, his attention was drawn to the presence of a reddish patch over the right forehead. It was initially asymptomatic. It continued to progress to form an apparent linear furrow extending from the receding hairline to the vertex and temporal part of the scalp. At this juncture, the patient experienced moderate pain when laughing. Five years later the patient experienced a constant boring pain affecting the right eyeball. Consultation with an ophthalmologist led to sacrificial enucleation of the right eye despite the lack of a precise diagnosis. Nevertheless, the initial condition continued to progress causing disfigurement of the right side of the face. Examination of the face was marked by perceptible asymmetry. A linear atrophic plaque in the form of a furrow was identified on the right side of the forehead extending from the eyebrow to the vertex and temporal part of the scalp. The skin over the furrow was taut and bound down (Fig. 1). A hematoxylin and eosin-stained section prepared from the lesion revealed marked thickening of the dermis. The collagen bundles were hypertrophied and closely packed together. The staining was homogeneously eosinophilic. It was largely devoid of inflammatory infiltrate. The changes in the blood vessels were conspicuous by narrowing of the lumina, thickening of the walls, and a sparse perivascular lymphocytic infiltrate. Pilosebaceous units were completely absent, whereas a few atrophic pulled-up sweat glands were located in the mid-dermis. Similar changes were observed in the subcutaneous tissue. The epidermis was largely atrophied, with flattening of the rete ridges.- - - - - - - - - - ranking = 0.5keywords = gland (Clic here for more details about this article) |
7/23. Hypohydrotic ectodermal dysplasia: an unusual presentation and management in an 11-year-old Xhosa boy.ectodermal dysplasia (ED) is an inherited disorder in which two or more ectodermally derived structures fail to develop, or are abnormal in development. Hypohydrotic ectodermal dysplasia (HED) or Christ-Siemens-Touraine syndrome, is an X-linked recessive syndrome with an incidence of 1/10,000 to 1/100,000 births. Because of its X-linked inheritance pattern, it is more common in males. HED is characterised by hypohydrosis (diminished perspiration), hypotrichosis (decreased amount of hair) and microdontia (small teeth), hypodontia (lack of development of one or more teeth) or adontia (total lack of tooth development). These patients present diagnostic and treatment challenges because of variable oral manifestations. This report describes an 11-year-old Xhosa boy, who was referred to the University Dental faculty by his general medical practitioner because of hypodontia. General facial features included: frontal bossing, a depressed nasal bridge, 'butterfly' pattern of eczema over the nasal bridge to the malar process of each cheek, thinned out hair, loss of vertical dimension of face and dry skin. Intra-oral examination revealed hypodontia with peg-shaped anterior teeth and diastemas. Radiological examination revealed no developing permanent teeth or tooth buds. Diagnosis was confirmed by doing a sweat gland count. Management included oral hygiene instruction, fluoride treatments, construction of a partial lower denture and counselling about his condition with particular reference to the danger of hyperthermia and control of allergies.- - - - - - - - - - ranking = 0.5keywords = gland (Clic here for more details about this article) |
8/23. Facial lichen striatus: successful treatment with tacrolimus ointment.We report a 22-year-old Japanese woman with facial lichen striatus (LS). The distribution of the lesions corresponded to that of Blaschko's lines. histology of the lesional skin showed an inflammatory cell infiltrate around hair follicles and eccrine glands. Treatment of the linear lesions with tacrolimus ointment once or twice daily resulted in a dramatical improvement in a short time. LS is a T-cell-mediated inflammatory disease and tacrolimus ointment may be an effective alternative treatment for this disease especially when the lesions are located on the face.- - - - - - - - - - ranking = 0.5keywords = gland (Clic here for more details about this article) |
9/23. The concomitant occurrence of multiple epidermal cysts, osteomas and thyroid gland nodules is not diagnostic for gardner syndrome in the absence of intestinal polyposis: a clinical and genetic report.gardner syndrome, a phenotypic variant of familial adenomatous polyposis, is characterized by the classical clinical triad of skin and soft tissue tumours, osteomas and intestinal polyposis, but disease patterns with pairs of these findings have also been reported. Different mutations in the adenomatous polyposis coli (APC) gene have been shown to be associated with gardner syndrome disease phenotypes. A 36-year-old patient presented with multiple epidermal cysts on the face, left ear lobe and neck, and the possible diagnosis of gardner syndrome was based on the additional findings of two classical osteomas in the left radius and ulna and a cold non-malignant nodule of the thyroid gland. intestinal polyposis was lacking at the time of examination. Major deletions but not microdeletions were excluded by a cytogenetic analysis with 650 chromosomal bands per haploid set. Systematic sequencing of the entire coding region of the APC gene (> 8500 bp) of the patient and five healthy controls was also performed. As a results, new APC gene polymorphisms were identified in exons 13 [A545A (A/G)] and 15 [G1678G (A/G), S1756S (G/T), P1960P (A/G)]. We also detected D1822V (A/T) which has recently been reported to be potentially related to colorectal carcinoma, and genotyped 194 randomly chosen healthy individuals from the Glasgow area for this as well as for the above variants in exons 13 and 15. Interestingly, of the 194 controls, 112 carried the DD (57.7%), 71 the DV (36.6%), and the remaining 11 (5.7%), including our patient, the VV genotype. It is therefore unlikely that APC D1822V serves as an important marker for colorectal carcinoma. In conclusion, we failed to identify obvious germline candidate mutations in > 8500 bp of the coding region of the APC gene in a patient with multiple epidermal cysts, osteomas and a thyroid gland nodule; major chromosomal deletions were excluded. Therefore, we assume that only the presence of intestinal polyposis is a marker for gardner syndrome.- - - - - - - - - - ranking = 3keywords = gland (Clic here for more details about this article) |
10/23. Autoimmune thyroid disease in anti-Ro/SS-A-positive children with annular erythema: report of two cases.Anti-Ro/SS-A-associated recurrent annular erythema is a rare disorder, and represents a cutaneous manifestation of primary sjogren's syndrome (SS). We report two childhood cases complicated with autoimmune thyroid disease, one with Graves' disease and the other with autoimmune thyroiditis. Both children were positive for anti-Ro/SS-A and anti-La/SS-B antibodies. One patient was lacking clinical SS with objective evidence of salivary gland involvement, while the other was diagnosed with primary SS. Our observation suggests that autoimmune thyroid disease in the subset of anti-Ro/SS-A-positive children with annular erythema might occur with similar frequency to that in adult primary SS.- - - - - - - - - - ranking = 0.5keywords = gland (Clic here for more details about this article) |
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