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J L Verret

Publications and source records attributed to J L Verret.

At least 19 recordsLinked to original sources

[Schnitzler syndrome and Waldenström disease. Fatal outcome of the original case].

The association between chronic urticaria, macroglobulinaemia and various other manifestations has been individualized as Schnitzler's syndrome. We report the terminal course of an original case followed up for 20 years, which ended as lymphoplasmocytic lymphoma with multiple sites, whereas no lymphomatous proliferation, meticulously looked for, had never been found hitherto. The lymphoma, associated with a macroglobulinaemia level above 5 milligrams, was diagnosed as Waldenström disease. Although most cases of Schnitzler's syndrome seem to follow a benign course (but the follow-up is not always long) a few cases have been reported showing evolution towards, or association with, lymphoma. This indicates that the follow-up of patients with Schnitzler's syndrome should be prolonged.

Aged

["Tufted angioma". A benign vascular tumor to differentiate with Kaposi sarcoma].

Among recently characterized vascular tumors, tufted angioma or angioblastoma is a benign acquired slowly progressive cutaneous tumor, which most commonly arises in the neck and upper trunk in children and young adults. This case report emphasizes the clinical and histological features of tufted angioma. Light microscopic examination reveals numerous lobules of closely packed capillaries scattered throughout the dermis. Vascular lumina are difficult to define. There are no atypical cells. Familiarity with tufted angioma should prevent this lesion from being misdiagnosed as malignant vascular tumor arising in young persons, especially Kaposi's sarcoma.

Adolescent

[Anti-centromere antibodies. Study of 67 positive sera].

From a series of 67 sera containing anticentromere antibodies we endeavoured to determine the principal clinical or biological peculiarities of these antibodies. The titers of anticentromere antibodies were usually high, with few differences between patients. Humoral immunity was frequently perturbed, with antinuclear autoantibodies (without anti-Scl 70), anti-mitochondria antibodies, rheumatoid factors, circulating immune complexes, etc. The disease predominated in women (97%) whose age and duration of symptoms varied considerably. The most frequent clinical manifestation noted in the 47 reports analyzed was Raynaud's phenomenon (93%) which in most cases (90%) was part of a complete or incomplete CREST syndrome. Telangiectasias, calcinosis and acrosclerosis were the main witnesses to the duration of these sclerodermas. Our findings were concordant with those of previous studies. However, the frequency of sicca syndrome (76%) was unexpected, and must be related to 2 laboratory results: the quasi-absence of anti-SSA and anti-SSB antibodies in our patients and the presence of two monoclonal immunoglobulins (IgM kappa and IgG lambda). There may be some degree of independence between the sicca syndrome and the sclerodermal manifestations.

Adult

The treatment of 45 patients with cutaneous T-cell lymphoma with low doses of interferon-alpha 2a and etretinate.

Forty-five patients with cutaneous T-cell lymphomas (CTCL), 32 with mycosis fungoides (MF) and 13 with Sézary syndrome (SS), were treated with interferon-alpha 2a (IFN-alpha 2a) (6-9 x 10(6) IU daily) for 3 months. Those responding to treatment were then treated with interferon-alpha alone (6-9 x 10(6) IU three times weekly), and non-responders received a combination of etretinate (0.5 mg/kg/day) and IFN-alpha 2a in similar concentrations. After 12 months of treatment, 28/45 patients (62.2%) were in complete or partial (greater than 50%) remission. Of these, 17 (60.7%) were receiving IFN-alpha alone and 11 the combined interferon-retinoid therapy. Of the patients with MF stage I and II, 20/25 were responders (12 receiving IFN-alpha alone and eight on combined therapy), whereas only 8/20 with stage IV or SS responded to treatment (five receiving IFN-alpha 2a alone and three combined therapy). These results suggest that the association of etretinate with low-dose recombinant IFN-alpha 2a is an effective means of treating epidermotropic CTCL, particularly in the early stages.

Adolescent

Diagnosis of adult linear IgA dermatosis by immunoelectronmicroscopy in 16 patients with linear IgA deposits.

Homogeneous linear IgA deposits at the dermo-epidermal junction (DEJ) shown by direct immunofluorescence are characteristic of what is termed linear IgA bullous dermatosis. However, it is not yet certain that this disease constitutes an entity distinct from other subepidermal blistering diseases, especially when IgG deposits are also present. Sixty-one cases of subepidermal blistering disease in adults were therefore investigated by immunoelectron microscopy (IEM), and the 16 patients observed to have homogeneous linear IgA deposits were compared with the 45 who had no IgA but had IgG and/or C3. In 11 of the 16 patients with IgA (four of whom also had IgG), the deposits were linear and formed a mirror image pattern on each side of the lamina densa from which they were separated by a clear space. In contrast to this monomorphic IEM pattern, clinical and other laboratory findings were very heterogeneous, making exact clinical diagnosis difficult. Of the remaining five patients in this group of sixteen, three (all with both IgA and IgG) had bullous pemphigoid, epidermolysis bullosa acquisita, and cicatricial pemphigoid, respectively, on IEM and clinical investigation. In the remaining two patients (one with both IgA and IgG, and one with IgA only) the deposits were located in the lamina lucida, making precise classification impossible. None of the 45 patients with isolated IgG and/or C3 deposition displayed the mirror image pattern. We conclude that this IEM pattern may constitute a specific diagnostic criterion of linear IgA dermatosis.

Adult

[Eruptive collagenoma. Apropos of a case with ultrastructural study].

A 29-year-old male patient was examined for numerous asymptomatic dermal nodules symmetrically distributed over the back and the shoulders. The nodules were firm, round to oval and varied in size from a few millimeters to 2 centimeters. They had appeared in normal skin without any known previous inflammation or injury, and they has been present without modification for nine years. At histological examination a dermal accumulation of abundant and large collagen bundles was observed. Elastic fibers seemed to be less numerous than normally. Alcian blue showed no increase in acid mucopolysaccharides. An ultrastructural study displayed normal, mature collagen fibers arranged in large bundles. Cutaneous collagenoma, initially described by Colomb as "eruptive collagenoma" is a rare disorder of unknown etiology occurring in young adults as symptomless dermal nodules without previous history of illness or injury and mostly localized to the back. No change is usually observed in these nodules for several years. Histologically, the nodules are characterized by a proliferation of collagen in the dermis; elastic fibers are generally reduced in number. Our ultrastructural study showed that proliferation of normal collagen fibers is the main morphological finding.

Adolescent

[Melanomatous meningitis. Apropos of 3 cases].

UNLABELLED: The meninges are frequently involved during the dissemination of malignant melanoma. This "meningeal melanoma" ranks fairly high on the list of metastatic meningites, side by side with meningeal carcinomatosis proper (i.e. related to a malignant epithelial tumour). Meningeal melanoma may be associated with a cerebral metastasis or isolated, as in the three cases reported here. Although its prognosis is sombre, its diagnosis is important since the possibilities of treatment are limited but exist. CASE-REPORTS: Case 1. This was a 68-year old woman who had initially presented with malignant melanoma in the maxillary region (SSM level IV, thickness 2.9 mm). Two years after the primary tumor was excised, secondary lesions developed in the lymph nodes and bones. A few weeks later, the patient fell into mental confusion progressing towards delusion of persecution. Neurological examination and CT scans were normal, but numerous melanoma cells were found in the CSF. This woman died one month after the first neurological signs had appeared. Case 2. This 63-year old man presented with bilateral axillary adenopathy and inflammatory thoracic plaques which at histology had proved to be metastases from a malignant melanoma of the shoulder surgically treated 10 years previously. After 14 months of almost complete remission under multiple chemotherapy (CPDD, ACTD, VDS), headaches and lumbosacral pain developed. Standard radiography and CT of the spine gave normal results, and it was only at the third lumbar puncture that the CSF was found to contain malignant cells. The patient died 2 months after the first neurological manifestations. Case 3. A 42-year old woman developed headaches and dysesthaesia in the arms and head. This was followed by oppositional semi-mutism. All exploratory examinations were normal, except for that of the CSF which showed 18 cells/mm3, 50 p. 100 of which were melanoma cells. The patient was transferred to the Dermatology department where he underwent excision of a left scapular melanoma difficult to classify (malignant blue naevus?). Combined treatment with Fotemustine* and DTIC produced an incomplete but relatively prolonged response. Death occurred after 8 months. DISCUSSION: A. Meningeal melanoma accounts for about 10 p. 100 of all metastatic meningites. The most frequent primary tumours in meningeal carcinomas are breast cancer and lung cancer. Malignant melanoma ranks third or fourth, but when the relative frequency of each of these three malignant diseases is taken into account, it appears that the risk of malignant melanoma is higher than that of any other solid tumour. B. In 410 cases extracted from the literature, the meninges were involved in 30 p. 100 of malignant metastatic melanomas found at autopsy. There is no publication that makes it possible to evaluate the relative frequency of melanomas affecting only the meninges or coexisting with cerebral metastases. This also applies to meningeal melanoma associated with non-neuro

Adult

Periungual and subungual arteriovenous tumours.

We describe the clinicopathological findings in five cases of an acquired vascular tumour involving periungual or subungual tissue. We believe that these represent acral arteriovenous tumours (cirsoid aneurysms). There are no previous reports of subungual or periungual acral arteriovenous tumours.

Female