Primary cutaneous anaplastic large cell lymphoma (ALCL) may be associated with keratoacanthoma (KA)-like epithelial hyperplasia and dense eosinophilic and neutrophilic infiltrates. Diagnosis in such cases is challenging both clinically and histologically, because the large atypical lymphoid cells may be obscured by the massive infiltrate of eosinophils and neutrophils, or confused with invasive squamous cell carcinoma or KA. We recently encountered two cases of CD30+ ALCL presenting with a KA-like tumour on the eyelid and nose, respectively. One showed features of KA histologically, with marked tissue eosinophilia and neutrophilia.

Adult female rats were trained for one week to consume a subnormal amount of food (12 gm.) in two one-hour feeding sessions per day: 4 gm. between 8 and 9 a.m. and 8 gm. between 6 and 7 p.m. At the end of the training period the animals were subjected to either bilateral electrolytic destruction of the ventromedial hypothalamus (VMH) or to a sham operation. The restricted feeding regime was continued for forty-eight hours postoperatively, when comparative studies were made. Plasma insulin response to intravenous Glibenclamide was greater in the rats with VMH lesions than in the pair-fed, sham-operated controls. The former rats also exhibited decreased initial plasma glucose levels, degranulation of the beta cells and reduced pancreatic insulin content. In addition to marked islet enlargement, similar though more severe signs of islet hyperfunction were observed in rats with VMH lesions fed ad libitum, which became hyperphagic immediately after the operation.VMH lesions did not significantly affect the plasma and pituitary levels of growth hormone (GH). The restricted feeding regime, however, caused an increase in plasma GH, responsiveness to hypoglycemic stimulation and a tendency to decreased pituitary GH content both in injured and sham-operated rats.These findings indicate that VMH lesions per se enhance the responsiveness of the pancreatic islets to insulin secretory stimulation independent of changes in food intake, meal size and meal frequency. The resulting hyperinsulinism might play an essential role in the occurrence of hypothalamic hyperphagia and obesity following VMH injury. In hyperphagic rats with VMH damage, the islet stimulation is magnified, possibly due to the combination of a direct hypothalamic effect plus the secondary effect of increased food intake. Defective secretion of GH does not appear to be primarily involved in the induction of the hypothalamic syndrome. DIABETES 25:203-08, March, 1974.Preliminary reports of this work have appeared in abstract form (see references 1 and 2).Bilateral destruction of the ventromedial nuclei of the hypothalamus (VMH) results in hyperphagia, obesity and increased levels of circulating insulin. 3 " 5 The hyperinsulinism has been interpreted as secondary to overeating. 3 This view has been questioned lately because hyperinsulinism also occurs in weanling rats which do not become hyperphagic after injury to the VMH. 6 Furthermore, when the VMH lesions were produced in hypophysectomized adult rats fed by gavage to avoid hyperphagia, increased levels of plasma insulin were demonstrated between five and twentyeight days after operation. 7 More recently, hyperinsulinism was observed as early as two days after VMH injury in adult rats on restricted food intake. 8 These findings suggest the existence of a direct hypothalamic influence on insulin secretion.Here we report the effects of VMH lesions on the insulin secretory response to Glibenclamide stimulation, the pancreatic insulin content, the pancreatic islet mass and the pituitary and plasma growth hormone cont...

Adalimumab, an anti-tumour necrosis factor agent, especially used in the treatment of rheumatoid arthritis, has a good safety profile. One of the most common side-effects of adalimumab is the development of autoantibodies. Despite the induction of autoantibodies, the clinical presentation of immune-mediated complications upon adalimumab therapy, including a lupus-like syndrome, is very rare. We have recently evaluated a new case of adalimumab-induced lupus erythematosus.

CD4+/CD56+ hematodermic neoplasm, formerly known as blastic NK cell lymphoma, is an aggressive and rare preculsor hematologic neoplasm recently recognized by the WHO-EORTC classification consensus for cutaneous lymphomas. The neoplasm tends to affect elderly patients, who usually present with skin lesions but often have a disseminated disease, including bone marrow involvement. Although the lesions are composed of cells with a lymphoblast-like morphology and an NK-cell phenotype, exhibiting a CD4+, CD56+ positive immunophenotype, recent studies support a relationship to plasmacytoid dendritic cells. Because of the rarity of this disease, we describe two patients suffering a CD4+/CD56+ hematodermic neoplasm.

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Reflex sympathetic dystrophy (RSD) is a clinical syndrome defined in the English literature by pain, dystrophic tissue changes and local disturbance of autonomic function in a limb or part of a limb. Algodystrophy is the common name used for the condition in the French literature, in which the concept also includes the "transient regional osteoporosis" and the "regional migratory osteolysis". We want to discuss three points: 1) Are the RSD, transient regional osteoporosis and migratory osteolysis different diseases or different manifestations of a single condition? We believe that an objective differentiation is not possible between them. Our report about 28 cases of polytopic RSD shows the frequent association in the same patient of these manifestations and we believe that this represents the broad spectrum of a single disease. 2) Is the accepted classic pathophysiologic mechanism of RSD accurate? The conception of a disturbance of autonomic function is not easily linked with its association with conditions such as diabetes, hyperthyroidism, hyperlipidaemia and others. Even more difficult to explain is the association with malignancy and osteomalacia. The deposit of immunoglobulins that we have demonstrated in two cases in the palmar fascia of RSD associated with malignancy suggests a possible immunological mechanism. 3) What are the limits of RSD? The association between RSD and aseptic necrosis of the hip has been reported. Are they two different conditions or is the aseptic necrosis only a more developed form of RSD? Finally, we report the first single case of Munchausen syndrome mimicking a RSD of the hand with the same clinical, radiological and scintigraphic appearance.

106PBdiatrie, S bis, Hapita1 Edouard Herrlot and Unit6 INSERM U 34, Lyon, France. Abnormal adrenal dehydroepiandrosterone (DHA) production : a frequent, curable cause of hirsutism In ch~ldhood and adolescence ?The case of a 13 years old pubere g i r l wlth severe hirsutism and lrre,g~lar menses is presented. The hirsutism was noted since infancy ; puberty had s t a r t e d a t the age of 9. She had no sign of virilism. Important hirsutism was a l s o present i n the mother. Markedly elevated plasma DHA level was found (934 ng/lOOml) ; plasma testosterone (T) level was 34 ng/100ml. Urinary corticosterolds and plasma c o r t i s o l , 17 OH progesterone, ACTH, L H and FSH levels pearance of the hirsutlsm and a normalisation of menstruations m t h plasma DHA level of 100 ng/lOOml and plasma T level of 10,4 ng/lOOml. Follovnng t h i s observation we have studled 4 similar cases of DI W dependant hirsutism rnthout evidence of enzymatic defect : 3 adolescents 16 t o 17 years old i n whom hirsutism started a t puberty and a e years old g i r l rnth a hirsutlsm present slnce infancy.M. ZACHMANN, R.P. G1TZELMANN;:ond A. PRADER.107 Dept. of Pediatrics, University of Zurich, Switzerland.Effect of aminoglutethimide (AG) on urinary cortisol and metabolites in patients with Cushing's syndrome (CS).The effect of AG (4x 250 mg, 670 mg/m2 daily) on the excretion of cortisol (RIA) and its metabolites (gos chromatography on capillary column) was studied monthly during 2to5 months in 4 patients with CS not due to on adenoma. Basal cortisol metabolites were moderately (totol 10.9 f 1.3 (SEM), THE 5.2 f 0.8, THF-alloTHF 3.5 0.6, cortolone 1.5 2

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