Schnitzler's syndrome: diagnosis, treatment, and follow-up.

Schnitzler's syndrome is characterized by recurrent urticarial rash and monoclonal gammopathy, associated with clinical and biological signs of inflammation and a long-term risk of AA amyloidosis and overt lymphoproliferation. An extensive literature review was performed, and the following questions were addressed during an expert meeting: In whom should Schnitzler's syndrome be suspected? How should the diagnosis of Schnitzler's syndrome be established? How should a patient with Schnitzler's syndrome be treated? How should a patient with Schnitzler's syndrome be followed up?. A diagnosis of Schnitzler's syndrome is considered definite in any patient with two obligate criteria: a recurrent urticarial rash and a monoclonal IgM gammopathy, and two of the following minor criteria: recurrent fever, objective signs of abnormal bone remodeling, elevated CRP level or leukocytosis, and a neutrophilic infiltrate on skin biopsy. It is considered probable, if only 1 minor criterion is present. In patients with monoclonal IgG gammopathies, diagnosis is definite if three minor criteria are present and possible if two are present. First-line treatment in patients with significant alteration of quality of life or persistent elevation of markers of inflammation should be anakinra. Follow-up should include clinical evaluation, CBC and CRP every 3 months and MGUS as usually recommended.

[Schnitzler syndrome and Waldenström disease. Fatal outcome of the original case]. / Syndrome de Schnitzler et maladie de Waldenström. Evolution terminale du cas princeps.

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.

Anti-lamin-B autoantibodies in a patient with cold urticaria.

Anti-lamin-B autoantibodies at a significant level had been found on two occasions in the serum of a 56-year-old woman who was suffering from an apparently idiopathic chronic cold urticaria. Anti-lamin autoantibodies can be detected in various autoimmune disorders including hepatitis, vasculitis and peripheral blood cytopenia. In our patient, there was no other clinical or biological abnormality. A chance association cannot be ruled out.

[Cutaneous electric stimulation in aging. Electroacupuncture of wrinkles following the procedure of Ph. Simonin]. / Stimulation électrique cutanée dans le vieillissement. Electroridopuncture suivant le procédé de Ph. Simonin.

The electroacupuncture process developed by Ph. Simonin has been used in the treatment of slack skin and age wrinkles of the face and neck. At present, with data in hand on a large number cases and spanning several years, it appears that substantial improvements are obtained in some 70 percent of cases after 10 to 15 sessions, maintained by periodic booster sessions, without any side effects. Although the exact physiological process of tissue regeneration via electric stimulation is still imperfectly understood, the reappearance of collagen and elastic fibers in the treated areas seems probable. Comparable results, derived from the use of similar electrical currents, have been documented in a variety of treatments, e.g. consolidation of fractures or of non-union and congenital pseudarthrosis, beneficial effects in urinary incontinence or in neuropathology.

[The injectable collagens. Technique of implantation, indications, limits]. / Les collagènes injectables. Technique d'implant, indications, limites.

Collagen implants have extended our therapeutic possibilities: used either alone or as a complement to surgical methods, they are an answer to the demand of many patients (who are carefully selected on clinical and immunological criteria) for obvious short-term efficacy, the frequency of complementary injections to complete filling, depending on the location of the areas treated. The main reservation lies in the onset of phenomena of local delayed hypersensitivity, which appears to be reduced as far as possible by concomitant performance of the skin and serum tests for anti-collagen antibody. Human, placental collagen, which has not yet been marketed nor have the purified fluid silicones which are currently experiencing a revival of interest, may be the future hope for these often ignored allergies.

Reactions to a bovine collagen implant. Clinical and immunologic study in 705 patients.

A small percentage of patients treated with bovine collagen implants have adverse reactions involving both the cellular and humoral types of immune response. We report a clinical follow-up of 705 subjects treated with a new bovine collagen implant, Atelocollagen (Koken Co. Ltd., Tokyo, Japan). Sensitization to the implant was evaluated in all subjects by skin testing, and humoral response was monitored in 166 subjects by measuring the level of circulating antibodies directed against bovine collagen. Twenty-seven patients (3.8%) exhibited a positive response to a skin test, and of the remaining 656 patients, an adverse reaction to the implant developed in 2.3%. We found a strong correlation between the presence of antibodies to collagen and a positive response to skin testing (92%) or an adverse reaction (100%). In the case of a borderline clinical response to bovine collagen implantation, anticollagen serologic tests appeared to be a useful tool for the identification of clinically reactive patients.

[Pseudotumoral lupus anetoderma. Child chorea. Development over 28 years]. / Anétodermie lupique pseudo-tumorale. Chorée infantile. Evolution sur 28 ans.

The finding in a 40-year old and apparently healthy woman of a pseudotumoral lesion of the left arm which had gradually developed over a 9-year period led to a tentative clinical diagnosis of lupus anetoderma. This diagnosis was confirmed on the one hand by histological examination of the skin--which showed almost total disappearance of dermal elastic fibres, while the lympho-histiocytic infiltrate was so discreet that it excluded a deep lupus--and on the other hand by direct immunofluorescence, with lupus band on the lesion and a few abnormalities of the exposed and covered healthy skin. A history of chorea at the age of 13 years raised the problem of a possible relationship between this neurological disease and a systemic lupus erythematosus. This hypothesis was supported by resolutive episodes of arthralgia and abnormal laboratory findings, such as accelerated ESR, leucopenia, decrease of complement and presence of homogeneous antinuclear antibodies. Anticardiolipin antibodies, circulating anticoagulants and VDRL test were negative. Treatment with anti-malarials brought about some degree of cutaneous shrinkage and a significant decrease in ESR. This case is reported because anetoderma as sole manifestation of systemic lupus erythematosus and the occurrence, 17 years previously, of chorea are exceptional events.

[Multiple tumors of the follicular infundibulum with basocellular degeneration. Apropos of a case]. / Tumeurs multiples de l'infundibulum pilaire avec dégénérescence baso-cellulaire. A propos d'un cas.

A progressive papular eruption on the face, limbs and vulva had been present for seven years in a 38-year old female patient. Some papules were small and confluent while others were sparse, as in nodular prurigo. Histology showed massive proliferation of the follicular sheath epithelium, connected to the lower surface of the epidermis. The lesion formed a plate-like area very similar to that observed in superficial basal cell carcinoma and corresponding to a tumour of the follicular infundibulum. Remarkable features of this case were the large number of lesions, their photosensitivity (they became pruriginous and increased in size after exposure to the sun), the presence of eccrine sweat glands beneath a skin lesion of the thigh, the basal cell degeneration of two lesions and the slight improvement observed after etretinate therapy. To our knowledge, such findings have not yet been reported in tumours of the follicular infundibulum.

[Retinoids and the prevention of cutaneous epitheliomas: 1977-1987]. / Rétinoïdes et prévention des épithéliomas cutanés: 1977-1987.

In 1977 we undertook a prospective study aimed at evaluating the preventive effects of a retinoid (etretinate, Roche) on the occurrence of skin carcinomas in dermatoses carrying a high risk of malignancy. Ten patients were included in the study: 4 had Xeroderma pigmentosum (XP), 4 had basal cell naevus syndrome (BCN), and there was 1 case each of porokeratosis of Mibelli (PM) and familial epitheliomatosis of Ferguson-Smith (EFS). All recorded carcinomas had been cured before treatment was initiated. The number of epitheliomas which developed during treatment with etretinate was compared with the number of epitheliomas recorded during the months or years preceding this treatment or during trial periods without treatment. The results obtained were very encouraging: 1. In patients with XP the actinic keratosis rapidly regressed, and the dryness of the skin was markedly reduced during the 5 years under etretinate. During the period without etretinate skin dryness and multiple actinic keratosis soon reappeared, as did, in one case, numerous basal cell carcinomas (BCC). However, etretinate did not prevent the occurrence of a malignant melanoma and of a few lesions of lentigo and naevus. 2. In the first case of BCN concerning two sisters, one treated the other untreated, less BCC lesions and, mainly, less BCN lesions were recorded in the sister treated than in the untreated sister. In the second case of BCN 28 BCC lesions developed while the patient was under observation without any treatment during 6 months, whereas only 31 BCC lesions had been recorded during 6 years under etretinate.(ABSTRACT TRUNCATED AT 250 WORDS)

Radiation induced formation of giant cells (Saccharomyces uvarum). I. Budding process and chitin ring formation.

X-irradiated yeast cells (Saccharomyces uvarum) grown in liquid media stop mitosis and form giant cells. Chitin ring formation, being a prerequisite for cell separation, was studied by fluorescence microscopy using calcofluor white, a chitin specific dye. Experiments with inhibitors of DNA synthesis (hydroxyurea) and chitin synthesis (polyoxin D) demonstrate chitin ring formation to be dependent on DNA synthesis, whereas bud formation is independent of DNA synthesis and chitin ring formation respectively. Basing on these results the formation of X-ray induced giant cells implies one DNA replication which in turn induces the formation of only one chitin ring between mother cell and giant bud. Obviously no septum can be formed. Thus cell separation does not occur, but the bud already formed, produces another bud demonstrating that bud formation itself is independent of DNA synthesis.

[Cutaneous responses to an injectable collagen implant (zyderm). Clinical, histological and ultrastructural correlations after intradermal tests]. / Réponses cutanées à l'implant de collagéne injectable (zyderm). Corrélations cliniques, histologiques et ultrastructurales aprés tests intradermiques.

A test implant of Zyderm collagen was carried out on 250 patients using the volar forearm site. The behaviour of the implant in the host tissue was followed-up in 6 cases by both direct and electron microscopy. There were 14 positive reactions. Biopsy was carried out in 3 cases, all of which exhibited severe reactions; foreign body granulomata were found in 2 of these, perivascular lymphocytic infiltration in the third. All 3 had a follow-up for 3 months and the biopsy sites healed normally. Trial implants are always advisable with collagen material. In order to avoid the possibility of such delayed hypersensitivity reactions, the test implant should be investigated at 72 hours and kept under observation for 4 weeks prior to carrying out the full corrective procedure. In two cases, reactive adverse reactions at the collagen implant treatment site were observed in spite of negative reaction at the test site. Nevertheless evidence suggests that Zyderm injectable collagen is a suitable material, leading to good cosmetic results; it carries minimal risk and is well tolerated even after a long period of time.

[Diagnostic value of electron microscopy in dermatology]. / Intérêt diagnostique de la microscopie électronique en dermatologie.

Basing themselves on their personal experience, the authors stress the main indications and the value of electron microscopy in dermatology. In some cases it provides an indispensable adjunct to standard light microscopy (tumor pathology, cutaneous lymphoma, collagenosis, storage diseases, etc). In other cases, it provides greater insight into dermatological affections, such as pigmentation disorders or bullae. Ultrastructural studies should therefore often be included in a thorough morphological examination of a skin biopsy.