1/223. A case of eosinophilic myocarditis complicated by Kimura's disease (eosinophilic hyperplastic lymphogranuloma) and erythroderma. This report describes a patient with eosinophilic myocarditis complicated by Kimura's disease (eosinophilic hyperplastic lymphogranuloma) and erythroderma. A 50-year-old man presented with a complaint of precordial pain. However, the only abnormal finding on examinatioin was eosinophilia (1617 eosinophils/microl). Three years later, the patient developed chronic eczema, and was diagnosed with erythroderma posteczematosa. One year later, a tumor was detected in the right auricule, and a diagnosis of Kimura's disease was made, based on the biopsy findings. The patient developed progressive dyspnea 6 months later and was found to have cardiomegaly and a depressed left ventricular ejection fraction (17%). A diagnosis of eosinophilic myocarditis was made based on the results of a right ventricular endomyocardial biopsy. The eosinophilic myocarditis and erythrodrema were treated with steroids with improvement of both the eosinophilia and left ventricular function. ( info) |
2/223. latex allergy in an orthognathic patient and implications for clinical management. A 19-year-old girl with mild asthma had had 16 months of orthodontic treatment as part of the joint orthodontic/orthognathic approach to her 9.5 mm overjet. At the time of banding her second molars she developed latex protein allergy as a reaction to the operator's non-sterile powdered latex gloves. She also gave a history of allergy to other substances as well as of eczema. The patient was confirmed as allergic to latex protein by radioallergosorbent test (RAST) for IgE, requiring precautions be taken during further orthodontic procedures as well as during the subsequent orthognathic surgery for the underlying Class II skeletal pattern. ( info) |
3/223. A new syndrome of spondyloepimetaphyseal dysplasia, eczema and hypogammaglobulinaemia. We describe a female infant with a combination of very short stature, severe eczema and igg deficiency causing recurrent infections in infancy. The radiological features of this condition are presented in the neonatal period, at the age of 5 months and at 2 years and 6 months. We propose that this condition is a previously undescribed type of spondyloepimetaphyseal dysplasia. ( info) |
| Atopic dermatitis is a common skin disease characterized by severely pruritic eczematous patches, papular and lichenified plaques, excoriations, cracks, and erosions. photopheresis has been shown to ameliorate the signs and symptoms of atopic dermatitis in some patients. We describe successful results with photopheresis for refractory disease in a patient who chronicled his quality of life weekly for more than 15 years before and during extracorporeal photochemotherapy. ( info) |
5/223. Nephropathy caused by Chinese herbs in the UK. The use of Chinese herbal remedies is increasing in the UK. We report the presence of a nephrotoxic compound in herb samples, which led to end-stage renal failure in two patients. We suggest that use of these products is regulated more tightly. ( info) |
6/223. Unresponsive eczematous dermatitis: a case of pancreatic cancer masquerading as cutaneous T cell lymphoma. skin diseases may be the presenting sign of malignancy, but strict criteria are required to make the diagnosis of a paraneoplastic syndrome. Common dermatoses may also herald an underlying malignancy without meeting these criteria. We report the case of an elderly man with an unresponsive eczematous dermatitis whose evaluation eventually revealed a pancreatic carcinoma. This case is presented to alert the clinician that a common dermatosis may be the first sign of an occult malignancy, especially if it behaves in an atypical or aggressive manner or is recalcitrant to standard therapies. ( info) |
7/223. Pentasomy X and hyper IgE syndrome: co-existence of two distinct genetic disorders. We describe a 10-year-old girl with features of a penta-X syndrome. cytogenetic analysis revealed a 49,XXXXX karyotype and molecular analysis of X-linked polymorphic markers showed that this aneuploidy arose by successive maternal non disjunctions. Apart from these features the patient has a lifelong history of eczema, recurrent pneumonia, and staphylococcal abscesses. Together with consistently increased serum IgE levels, low antibody responses, and low levels of serum IgA and IgG2, these findings are characteristic for the hyper IgE syndrome. While pentasomy X may be due to sequential non disjunctions in meiosis I and meiosis II in the mother, the underlying pathomechanism in hyper IgE syndrome remains unclear. This case is the first with co-existence of pentasomy X and hyper IgE syndromes. ( info) |
| We report a 63-year-old Japanese man with basosquamous carcinoma developing after ultraviolet therapy with artificial sunlight. Because he had had an eczematous lesion on the right leg five years previously, he had received ultraviolet therapy with artificial sunlight from a mercury-quartz lamp for a total of 30 sessions over a 3-year period. physical examination revealed an irregular erythematous plaque measuring 5 x 6 cm, with slight eruptions and erosions, on the right leg. A brownish plaque, measuring 9 x 12 cm, surrounded the erythematous plaque. Histopathologically, in the nests of atypical cells in the dermis, nuclear palisading was present at the edge of each tumor cluster. Adjacent clusters exhibited transitions to squamous cells and contained horn pearls. Most of the cells of the epidermis and adnexa were atypical, but cells of the normal adnexal epithelium extended over the atypical cells of the epidermis in an umbrella-like fashion in other lesions. We believe that, in the present case, artificial sunlight therapy caused solar keratosis and that this developed into basosquamous carcinoma. To our knowledge, skin cancer developing after ultraviolet therapy with artificial sunlight without PUVA has not previously been reported in the English literature. ( info) |
9/223. Radiographic features of eosinophilic gastroenteritis (allergic gastroenteropathy) of childhood. The child with eosinophilic gastroenteritis has failure to thrive, a history of allergy or asthma, iron deficiency anemia, and peripheral eosinophilia. This type of eosinophilic gastroenteritis has earned the designation "allergic gastroenteropathy." Pathologic change in the gastric antrum of six children with this disease correlated with an abnormal air-contrast examination of the antrum. The affected children had an irregular, lacy, antral surface rather than the smooth, bald surface seen in normal children. The small bowel in allergic gastroenteropathy is usually abnormal but nonspecific as to etiology. Much more specific is the combination of small bowel and gastric antral abnormalities. Findings encourage air-contrast radiography of the antrum in children with suspected allergic gastroenteropathy. ( info) |
| A 71-year-old man developed an exfoliative dermatitis of the palms of the hands and soles of the feet, and a generalized itch, during treatment with intravesical instillations of mitomycin C for an undifferentiated carcinoma of the bladder. patch tests with mitomycin C 0.03%, 0.1% and 0.3% aq. were positive. Because of the serious consequences of this finding, the patient was retested with mitomycin C in pet. (same concentrations), a more stable preparation. This showed clear positive reactions. During this last series of patch tests, he developed palpable purpura on the legs. We postulated that this reaction was an immune-complex-mediated reaction, caused by the 2nd series of patch tests with mitomycin C. To prove this, we performed histopathological and immunofluorescence investigations, and these showed the reaction to be consistent with Henoch-Schonlein-type purpura. We therefore conclude that this patient developed systemic reactions to mitomycin C, characterized by an eczematous dermatitis as well as purpuric reactions. The intravesical installations with mitomycin C have been stopped. The patient's skin problems (the purpura as well as the eczema) have completely resolved and have not recurred. ( info) |