Jessner lymphocytic infiltration - rare in childhood.

We present a 13-year-old girl with Jessner lymphocytic infiltrate of the skin, who has suffered from the disease since the age of 9 years. It is a rare disease in childhood, and we highlight the clinical features and therapeutic response of tacrolimus.

[Dermatomyositis as a paraneoplastic first symptom of ovarian cancer]. / Dermatomyositis som paraneoplastisk debutfænomen ved ovariecancer.

Dermatomyositis (DM) is a rare disease, which is associated with malignancy in around 20% of cases. DM can precede the diagnosis of ovarian cancer by years. In this case, a 63-year-old woman was diagnosed with DM by biopsy due to diffuse symptoms of oedema in both arms and a spreading violet/dark red rash of both arms and thighs. A large inoperable ovarian tumour was found subsequently. In order to reach an early diagnosis and curatively treat people with cancer, extra focus must be paid on paraneoplastic phenomenons including DM.

[Staphylococcal scalded skin syndrome in adults]. / Staphylococcal scalded skin syndrome hos voksne.

We describe a case of adult staphylococcal scalded skin syndrome (SSSS) in a 91-year-old woman. The patient had a Staphylococcus aureus infection in her right ear with redness and yellow secretion. A few days later she developed Nikolsky sign-positive tender enlarged flaccid bullae involving most parts of her body. The bullae ruptured easily and left a erythematous base. The histopathological changes were characteristic for adult SSSS. The patient was well-treated with intravenous dicloxacillin, topical antibiotic and antiseptic treatment. The patient had marked thrombocytosis, but no interest in further examination.

[Glucagonoma syndrome without diabetes mellitus]. / Glukagonomsyndrom uden diabetes mellitus.

A patient developed depression, weight loss, ulcers and a migrating, denuded erythematous skin area. Punch biopsy revealed necrolytic migrating erythema. Computerised tomography and endoscopic ultrasound showed a solid tumour of the pancreas. A blood sample showed an increased level of glucagon without diabetes. Glucagonoma syndrome is characterized by glucagon overproduction, diabetes, depression, deep venous thrombosis and necrolytic migrating erythema. Glucagonoma is frequently diagnosed late which increases the risk of metastases. It is important not to rule out glucagonoma in patients with a relevant clinical picture but without diabetes.

Sudden unexpected death associated with lymphocytic thyroiditis.

A forensic autopsy study comprising 125 cases was carried out retrospectively in order to evaluate pathological changes in the thyroid gland in different groups of death. The five groups selected consecutively were: (i) opiate addicts who died from an overdose, (ii) alcoholics who died as a result of their alcohol abuse, (iii) cases of fatal poisoning other than opiate addicts, (iv) unknown cause of death and (v) controls without prior disease. Tissue samples from the thyroid gland were cut and stained with haematoxylin and eosin and van Gieson. Histology examinations were subsequently performed blind with semiquantitative assessment of the following six parameters: (a) height of the follicular epithelium, (b) the amount of lymphocytes, (c) the presence of plasma cells, (d) hyperplastic follicular changes, (e) oxyphilic changes, and (f) fibrosis. The most striking result was the finding of extensive lymphocytic infiltration of the thyroid parenchyma in five of the 124 cases, of which four belonged in the group of 'unknown cause of death'. This discovery leads to reflections regarding lymphocytic thyroiditis as a cause of death, either by itself or in combination with other disorders. Silent (painless) thyroiditis, especially, is easily overlooked at autopsy as there are no macroscopic changes and often no prior symptoms or history of thyroid disease pointing towards this condition. Analyses of thyroid hormones are unreliable in predicting endocrine status in life. Routine microscopy of the thyroid gland is therefore advocated in cases of sudden unexpected death in order to diagnose thyroid disease, in particular silent (painless) thyroiditis.

Ovarian Sertoli-Leydig cell tumor of intermediate grade with heterologous elements of rhabdomyosarcoma. A case report and a review of the literature.

Ovarian Sertoli-Leydig cell tumors (SLCTs) are rare sex cord-stromal tumors, and among them, tumors with heterologous mesenchymal elements are exceptional and mainly associated with poorly differentiated tumors and are often fatal. We present the fourth case of an ovarian SLCT of intermediate differentiation with rhabdomyosarcoma and a review of the literature. Surgical treatment was conservative with preservation of the contralateral adnexa and uterus. No adjuvant treatment was given. At 4 years control post surgery, the patient was without evidence of disease. Extensive sampling of SLCTs is important because heterologous elements may be sparse. Immature skeletal muscle cells in SLCTs often reveal only moderate pleomorphism, and as they are closely admixed with the Sertoli cells or immature gonadal stroma, they can be rather difficult to differentiate from the latter ones. Immunohistochemical analysis with a panel of antibodies including antibodies against myogenin and alpha-inhibin is very important to diagnose the rhabdomyosarcoma and to grade the SLCT accurately.

Budesonide treatment and expression of inducible nitric oxide synthase mRNA in colonic mucosa in collagenous colitis.

OBJECTIVE: In collagenous colitis, the production of nitric oxide in the colon is found to be 50 to 100-fold higher than in healthy controls. The role of nitric oxide in collagenous colitis is debated and it has been suggested that nitric oxide has a causative role in diarrhoea. The aim of this study was to examine the possible effect of budesonide treatment on the level of inducible nitric oxide synthase mRNA. METHODS: In 20 patients with collagenous colitis, clinical activity was assessed by registration of the daily stool frequency and stool weight. Sigmoidoscopy was performed and biopsies for histological examination and one biopsy for determination of inducible nitric oxide synthase mRNA was obtained in 16 patients. RESULTS: Budesonide treatment was followed by a significant reduction of inducible nitric oxide synthase mRNA (P<0.01) whereas no change was observed after placebo treatment. Significant correlations between inducible nitric oxide synthase mRNA and the grade of inflammation (rho=0.47; P<0.01), the daily stool weight (rho=0.51; P<0.005) and the daily stool frequency (rho=0.49; P<0.005) were observed. No significant association was observed between inducible nitric oxide synthase mRNA and the thickness of the collagen layer. CONCLUSIONS: In patients with collagenous colitis, treatment with budesonide results in a reduction of inducible nitric oxide synthase mRNA. The level of inducible nitric oxide synthase mRNA in colonic mucosa correlates with the inflammatory and clinical activity. The results support that nitric oxide is a central factor in the pathogenesis of collagenous colitis.