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We present three new cases of POEMS syndrome with the most common cutaneous signs of this entity. The syndrome is a multivisceral complex, mostly described in Japanese patients, which includes polyneuropathy, organomegaly, endocrine disorders, monoclonal gammopathy and skin changes. The latter are constant but sometimes reduced to one type of lesion. The most frequent are hyperpigmentation sparing the mucosae, hypertrichosis, scleroderma-like skin thickening and capillary angiomas. The other signs are much less common, as shown in table I. Histological findings are seldom reported and usually have little specificity. None of these abnormalities is pathognomonic, but their predominance at the extremities may be suggestive and leads to a search for other elements of the syndrome, notably gammopathy. The main differential diagnostic problem is with scleroderma, sometimes circumscribed and often systemic. Some cases are very ambiguous. The relationship between the two diseases would suggest the existence of one or several common pathogenic factors. The overall physiopathology of POEMS syndrome is obscure. The role of endocrine disorders in the genesis of the cutaneous signs is subject to discussion: hyperoestrogenism may explain some of these signs, but hypertrichosis is incompatible with the frequent finding of low androgen secretion. The role of one or several "toxic" substances secreted by plasmocytes is highly hypothetical. In some cases, the skin abnormalities have regressed after treatment of gammopathy, and in rare patients the recurrence of gammopathy was followed by that of skin lesions. In a few cases, a "paraneoplastic" character may be suspected.

The three cases reported, two mesenteric venous infarctions and one asymptomatic carrier, prove the responsibility of the anti-thrombin III deficiency in the development of apparently primary entero-mesenteric venous infarctions. Thus such a deficiency should be sought routibs. Furthermore, these 3 cases confirm the usual characteristics of the 10 familial cases collected since the princeps description of Egeberg: recurrent thromboembolic disease in the young subject involving essentially the lower limbs, relative resistance to heparin, family history of thromboembolic disease confirming the hereditary nature of the disease with dominant transmission, laboratory confirmation of the quantitative deficiency in antithrombin III, the levels and activity of which are reduced by half, and decrease in laboratory sensitivity to heparin contrasting with normal clotting studies. The family history reveals associated conditions within the syndrome: asthma and Biermer's anemia as well as similarities in leucocyte HLA groups.

This is the case report of a 13 years old white young girl in whom numerous moving abdominal calcifications are discovered during investigation for chronic anemia. Because of the existence of a severe vascular malformation of the face, a digestive arteriography is performed with no evidence of a calcified angiomatosis of the intestine tract. Preoperative diagnosis was intestinal duplication with enterolithiasis. Exploratory laparotomy confirmed that it was a huge communicating ileal duplication with numerous intestinal calculi and alimentary fragments. Surgical excision was easy and post-operative course, uneventful. This is a standard association but never reported in the pediatric literature. The authors stress on the different diagnosis of calcifications encountered in abdominal X rays in children and on chronic intestinal stasis which is the main factor in enteroliths formation.

Necrotizing angiodermatitis is of characteristic clinical expression and is often difficult to treat. It is associated with microcirculatory disorders of hypertensive or diabetic origin. A particularly long course (m = 8 months) in 8 patients with necrotic angiodermatitis (of hypertensive origin in 6 cases and diabetes in 2) resulted in treatment by plasma exchange, two other patients with immunological angiodermatitis being treated similarly because of very similar symptomatology. The 10 patients (3 men, 7 women age range 55 to 80 years) received between 4 and 18 small volume plasma exchanges over 2 to 12 weeks. Results were evaluated from pain and duration of healing, absence of pain being reported by 7 patients and a beneficial effect on healing by 6 patients. No correlation existed between ESR and hematocrit values during treatment and the clinical course. The question arises as to whether the mode of action of plasma exchange in angiodermatitis is related to the purifying of serum factors or a rheological role.