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Biomedical subjects

B Guillot

Publications and source records attributed to B Guillot.

At least 145 records · Page 8Linked to original sources

[Cutaneous lesions in POEMS syndrome. Apropos of 3 case reports and a review of the literature].

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.

Adult↗

[Diabetic cheiroarthropathy. Microcirculatory aspects].

Diabetic cheiroarthropathy (DCA) or pseudosclerodermatous hand of the diabetic is characterized by nonpainful limited extension of the proximal metacarpophalangeal and/or interphalangeal joints with spontaneous flexum of the fingers. The mechanism of lesion formation is poorly known but apparently associates neurogenic, vascular and cutaneous phenomena. Fifteen patients with DCA (9 men, 6 women; range 20-74 years) were studied by capillaroscopy, photoplethysmography and skin biopsy. Eleven had type 1 diabetes and 4 type 2 over periods ranging from 1 to 42 years (mean 19.9 years). Diabetic retinopathy was noted 10/15 times, nephropathy 5/15 times and neuropathy of the lower limbs 13/15 times. All patients had at least one of these abnormalities. In capillaroscopy, "Shoal of fish" features of diabetic microangiopathy were found only 4 times, but minor dystrophy was noted in 12 cases. In digital photoplethysmography, a drop in digital systolic pressure or an increase in pulse time was noted in 5 cases. The Hillestad test was less than or equal to 2 in 8 patients. Histological study showed constant dermal collagenous fibrosis in diseased skin, which was also found in normal skin in 6/13 patients. PAS staining showed a thickening of vascular basal membrane 14/15 times in diseased skin and 11/13 times in normal skin. The relation between DCA and microangiopathy is discussed in terms of collagen metabolism abnormalities observed during diabetes.

Adult↗

Studies of polymorphonuclear migration into psoriatic skin using a new in vivo method.

A new method, employing a skin-implanted cell trap already used to study chemotaxis in cancer patients, was applied to 35 healthy volunteers and 12 psoriatic patients. A dacron disk impregnated with 10 microliters of 4-6.10(6) live BCG suspension was implanted in the deep dermis. After 24 h the disk was removed, and five sections of each disk were counted for polymorphonuclear leukocytes (PMNs) and monocytes. Involved and uninvolved psoriatic skin showed a decrease of PMN migration into the disk as compared with controls. No difference could be demonstrated between involved and uninvolved skin. Mononuclear cell chemotaxis was the same in psoriasis as in controls. These results are in agreement with other in vivo data using mainly the skin chamber technique indicating a decrease of PMN chemotaxis in psoriatic skin at 24 h.

Adult↗

Pemphigus associated with Castelman's pseudolymphoma.

Several cases of association between pemphigus vulgaris and Castleman's pseudolymphoma have been reported. We describe a new case with mucocutaneous involvement and improvement after tumor removal. A review of the literature is presented.

Adolescent↗

[Diabetic cheiroarthropathy].

Cheiroarthropathy is quite frequent in diabetics, but is only really specific at stage III, which is the most characteristic form. It is all the more frequent as the diabetes is old, but remains unrelated to sex, age and type of diabetes. The stiffening of the joint readily extends to other joints. The patients are moderately alerted in the absence of other associated pathologies: trigger finger, Dupuytren's disease, carpal tunnel syndrome. All these manifestations form the "diabetic hand", of which cheiroarthropathy is only one component. The need for an accurate analysis with the purpose of appropriate treatments, should be emphasized. The angiologic and histopathological study of patients with stage III cheiroarthropathy, enables us to demonstrate moderate abnormalities of the microcirculation, which are quite different from those encountered in sclerodermia. The etiopathogenesis of cheiroarthropathy remains mysterious and is probably related to an alteration of the collagen metabolism. One of the most interesting component is the association between cheiroarthropathy and the micro-angiopathic complications of diabetes mellitus: cheiroarthropathy being the indicator of such diabetes.

Adult↗

[Probable association of HLA-DR5 with bullous pemphigoid].

HLA typing was performed in 35 French Caucasoids with bullous pemphigoid and compared with 160 healthy controls. 47 HLA antigens were characterized by a lymphocytotoxicity micromethod. Analysis of the results only reveals one statistically significant difference: an increased incidence of HLA-DR5, which reaches 51.43% in patients versus 22.42% in controls, with P = 0.0007 and Pc = 0.0329. Several bullous dermatosis are associated with various HLA-DR antigens. These data suggest a direct role of HLA-DR molecules in the constitution of these autoimmune disease. An abnormal expression of DR products on some skin cells membrane would permit the presentation of a non self peptide, accumulated in skin cells, to helper T lymphocytes. An heteroimmunization against the non self peptide could lead to lesion of self cells. This peptide perhaps derives from food protein.

HLA-D Antigens↗

Kaposi's sarcoma of the bronchial tree in a renal transplant recipient.

An unusual case of disseminated Kaposi's sarcoma with endobronchial lesions is presented; these localizations are quite uncommon in the course of the disease, particularly in kidney transplant recipients. Some of the multifactorial etiologies of Kaposi's sarcoma are present: immunosuppression, ethnic origin and CMV infection. We emphasize that the diagnosis of bronchial involvement can be made during fiberoptic bronchoscopy and confirmed by biopsy without excessive complications.

Bronchial Neoplasms↗

[Acute febrile neutrophilic dermatosis and malignant hematologic diseases: report of a new bullous case and review of the literature].

A new case of Sweet's syndrome (acute febrile neutrophilic dermatosis) associated with a malignant hemopathy is presented. The blood disease was a chronic myelomonocytic dysmyelopoiesis which was discovered during the eruption and resulted in the patient's death within a few months, probably through acutization. The skin lesions were atypical, bullous and ulcerated. On this occasion, the international literature concerning all cases of Sweet's syndrome associated with malignant or premalignant hemopathies is reviewed. Several concepts emerge from this study: the association is frequent (about 20 p. 100 of all published cases of Sweet's syndrome); there is a strong predominance of granulocytic hemopathies over lymphoplasmocytic and monocytic hemopathies; the blood disease is revealed by the skin eruption in some 50 p. 100 of the patients; there are frequent chronological relations between Sweet's syndrome and the events that occur in the course of the hemopathy; finally, the association is usually of poor prognosis. A comparison with Sweet's syndrome unassociated with a blood disease showed only three significant points: the frequency of bullous lesions, of the initial anaemia (the most important element) and of extreme figures in leucocyte counts (leucopenia or major hyperleukocytosis). The atypical character of the skin lesions in the patient presented here incites to discuss the nosological relationship between Sweet's syndrome and bullous pyoderma, an entity closely associated with hemopathies. It has recently been suggested by several authors that this anatomico-clinical kinship should be turned into a wide spectrum of acute neutrophilic dermatoses, with typical Sweet's syndrome at one end and Pyoderma gangrenosum at the other end. The interface between this spectrum and haemopathies seems to be maximum at its intermediate stage: the bullous and superficially ulcerated lesions. The aetiology and pathogenesis of this new nosological entity are uncertain. The presence of chemoattractants or of polymorphonuclear cell abnormalities is still open to discussion. The relationship between the entity and leukocytoclastic vasculitis has recently been questioned.

Aged↗

Controlled study of plasma exchange in pemphigus.

To determine the potential steroid sparing effect of plasma exchange in pemphigus we enrolled 40 patients in a multicenter randomized study. Eighteen patients were treated by prednisolone alone, 22 by prednisolone plus ten large-volume plasma exchanges over four weeks. All patients received oral prednisolone in the same initial dosage (0.5 mg/kg/d), which was increased weekly if needed. The number of cases controlled at each therapeutic step did not differ between the two groups. In eight cases, four in each group, the disease was not controlled by the highest therapeutic step of the protocol, with four deaths from sepsis in the plasma exchange group. The controlled cases needed similar cumulative prednisolone doses (5237 +/- 5512 mg in the plasma exchange group vs 4246 +/- 1601 mg in the control group). The evolution of serum pemphigus antibody was not different in the two groups. These findings suggest that plasma exchange in association with low steroid doses alone are not effective in the treatment of pemphigus and may even promote sepsis.

Adult↗

[Does plasma exchange have a place in the treatment of necrotizing angiodermatitis?].

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.

Aged↗