Validation of a non-contact technique for local skin temperature measurements.

UNLABELLED: Here we propose to quantify local temperature variations using thermal imaging to assess the effect of dermatological lasers. OBJECTIVES: To quantify the temperature raise induced by laser application and to differentiate the effects of a potassium titanyl phosphate (KTP) laser and an intense pulsed light (IPL). METHODS: A randomized comparative study was performed on 10 adult volunteers with symmetrical rosacea treated by KTP laser or IPL. Skin temperature measurements were performed on inclusion, immediately after laser treatment and 3 min after thermal water application, using a high-resolution (0.08 degrees C) infrared thermal video camera. RESULTS: KTP laser treatment induced a significant rise in local skin temperature whereas no significant change was revealed by the IPL treatment. The infrared camera is a reliable and reproducible technique that allows a follow-up of skin temperature without skin contact. CONCLUSION: Thermography using an infrared camera could potentially be applied in clinical pharmacology for inflammatory reactions or scarring processes.

Acne-like eruption induced by pseudoxanthoma elasticum: effectiveness of liquid nitrogen cryotherapy.

We report a case of pseudoxanthoma elasticum associated with an acneiform eruption involving the cervical area. When she was 16 years-old our patient was diagnosed with pseudoxanthoma elasticum, affecting the skin (flexural, cervical and neck areas) and the eyes (bilateral ocular angioid streaks). Ten years later, acneiform lesions (inflammatory and comedones) developed on these lesions: - the inflammatory lesions were characterized by phagocytosis of pathological elastic fibers inducing granuloma - the histological aspects of pseudoxanthoma elasticum were observed around large comedones. This association is rarely reported and we think that atypical, cervical acneiform lesions may be an indication of pseudoxanthoma elasticum. The mechanism for this association is not clearly understood. In our case, Von-Kossa staining was negative for the granulomatous lesions and positive for the comedones: calcification could protect elastopathic fibers from phagocytosis. Treatment is difficult: anti-acneic treatments are not effective except for tetracylines, the anti-inflammatory effects of which can improve granulomatous lesions. As its efficacy has been reported in elastosis perforans serpiginosa, we used liquid nitrogen cryotherapy on the inflammatory lesions with good results (separation of epidermis from dermis perhaps promoting transepithelial elimination of the abnormal dermal elastic tissue).

Cutaneous manifestation of left atrial myxoma.

A 53-year-old woman had a left hemiplegia with suspicion of cerebral metastases. Thoracic and abdominal computed tomography revealed renal and splenic infarction features and she presented violaceous papulosis on her fingers corresponding to thrombosis of dermal vessels. Echocardiography showed a left atrial tumor evoking myxoma. The clinical features of left atrial myxomas are intracardiac obstruction, extracardiac embolism and general symptoms. Cutaneous manifestations are frequently reported and can correspond to cutaneous manifestations of emboli, symptoms related to auto-immune disorders and specific cutaneous findings that suggest atrial myxoma as part of more complex syndromes.

Panniculitis revealing qualitative alpha 1 antitrypsine deficiency (MS variant).

A 16-year-old girl presented painful, red, nodular lesions on the abdomen. A cutaneous biopsy showed inflammatory cell infiltrate and fibrosis in the dermis and in the septa with isolated adipocyte lobules. alpha1-antitrypsin level was found to be normal but M1S phenotype of alpha1-antitrypsin was determined by isoelectric focusing in polyacrylamide gel. alpha1-antitrypsin level was normal for her family but M2S phenotype was found in her father. Alpha 1-antitrypsin (alpha1 AT) deficiency is a common hereditary disorder of Caucasians. The locus is pleiomorphic and 75 alleles have been identified. Numerous pathological mutations can be classified by the mechanisms which cause the deficiency. The major clinical importance of this deficiency is emphysema and liver disease. Panniculitis is rarely reported and seems to occur principally for the ZZ or MZ phenotype and for low levels of alpha1 AT. MS phenotype has been more rarely reported and triggering agents such as trauma and infections must be present. However, normal levels of alpha1 AT in the serum have previously been reported as in our case, and we suggest the study of alpha1 AT phenotype even if the plasma level is normal.

L-carnitine in cardiogenic shock therapy: pharmacodynamic aspects and clinical data.

Following our previous work on biochemical and clinical aspects of cardiogenic shock, we carried out an open study on 27 patients hospitalized in shock condition and investigated for the entire period of permanence in intensive care units (ICU). The subjects were treated with high doses of L-carnitine following previous results on the use of this molecule in conditions of oxidative damage due to acute cellular hypoxia. When compared with the data reported in the literature, the results obtained in this study show a surprisingly positive trend for the carnitine-treated patients in terms of survival rate to the cardiogenic shock. This finding and statistical analysis of the clinical parameters confirm the suggestion that L-carnitine could be credited with a new and interesting role in the therapy of cardiogenic shock.

[Celiac disease associated with cutaneous sarcoidosic granuloma]. / Maladie coeliaque associée à un granulome sarcoïdosique cutané.

Coeliac disease can be associated with numerous internal, skin and mucosa involvements: their physiopathology is often obscure. We report the case of a 14-year old female patient who suffered from a coeliac disease diagnosed in 1988 with considerable improvement with a gluten-free diet. Her two daughters also presented coeliac disease and her sister suffered from nevoid basal cell carcinoma syndrome. Four years later, she presented non pruriginous small nodules over both lower extremities. Skin biopsy revealed a non-caseating granuloma into the derm: we only could evocate sarcoidosis affecting the skin. The dermatological lesions improved during the following weeks with a gluten free diet and relapsed each time this diet was stopped. Many clinical associations with coeliac disease have been described with numerous visceral and skin-mucosa involvements. Eight cases of coeliac disease associated with sarcoidosis affecting the lung have been reported: in five cases, coeliac disease preceded sarcoidosis and in one case sarcoidosis relapsed each time gluten was reintroduced like in our case. This two diseases seem to share immunological and genetic disturbances.

[Hypersensitive urticarial vasculitis after natisedine intake]. / Vasculite urticarienne d'hypersensibilité après prise de Natisédine.

BACKGROUND: Various skin and mucosal reactions can be observed after administration of quinidine derivatives. CASE REPORT: A patient who was taking Natisédine (quinidine phenylethyl barbiturate) intermittently and at reintroduction developed a papulopurpuric eruption (without thrombopenia) producing extensive centrifugal annular infiltration and central healing which regressed approximately one week after drug withdrawal. This eruption was associated with moderate 24 h proteinuria. The clinical aspect was that of vasculitis purpura as confirmed histology. Direct immunofluorescence only demonstrated C3 deposits in the vessel walls of the superficial dermis. The quinidine moiety of this drug (currently removed from the formulation) appears to be the responsible agent (imputability score: 13 B3). DISCUSSION: Thrombopenic purpura by synthesis of anti-platelet antibodies induced by quinidine derivatives is well known. Inversely, cases of non-thrombopenic purpura imputable to these same derivatives is uncommon (7 reported cases). The pathophysiological mechanisms involved might be similar (antigenic similarity between the platelet surface and endothelium).

[Multiple cutaneous reticulohistiocytosis]. / Histiocytose multicentrique avec atteinte cutanée diffuse.

BACKGROUND: Multicentric reticulohistiocytosis is a non Langerhans cell histiocytosis. This rare disease is characterized by cutaneous papules and nodules and a destructive polyarthritis; multisystem involvement may occur. Multiple and diffuse cutaneous reticulohistiocytosis have been more rarely reported. We present a case which was distinctive by the existence of multiple cutaneous plaques. CASE REPORT: A 65-year-old woman presented cutaneous papules and nodules associated with a destructive arthritis affecting the hands. Histological examination of a cutaneous biopsy associated with immunophenotyping and electronic microscopy permitted us to make the diagnosis of multicentric reticulohistiocytosis. The search for visceral involvement or underlying neoplasia was negative. Rapidly, cutaneous aggravation occurred with multiple and diffuse infiltrated plaques on the back, the face, the ears, the thighs and the forearms. The same histological aspect was found for these lesions. Treatment with corticosteroids and cyclophosphamide was successful. DISCUSSION: This case report is the first one with diffuse cutaneous lesions of multicentric reticulohistiocytosis with aspect of infiltrated plaques. Diffuse cutaneous lesions in multicentric reticulohistiocytosis have been rarely reported with diffuse papules or nodules pattern. A visceral involvement seems to be more frequent for diffuse cutaneous involvement. In all cases, the association of multicentric reticulohistiocytosis with neoplasia in up to 25 p. 100 is of interest. Treatment of multicentric reticulohistiocytosis consists in corticosteroids at the initial phase associated with alkylants agents or methotrexate.

[Intravascular papillary endothelial hyperplasia: multiple lesions appearing in the course of treatment with interferon beta]. / Hyperplasie endothéliale papillaire intravasculaire: multiples lésions apparues au cours d'un traitement par interféron bêta.

INTRODUCTION: Intravascular papillary endothelial hyperplasia is a benign and rare vascular lesion. We report a case of multiple vascular lesions of the hand following 3 months treatment with beta-interferon injections for multiple sclerosis. OBSERVATION: A 50 year-old man had multiple vascular nodules of the hands. He was treated with beta interferon injections for multiple sclerosis for 3 months. Histology showed typical changes of intravascular papillary endothelial hyperplasia: papillary endothelial proliferation in a dilated cavity associated with thrombosis. DISCUSSION: Intravascular papillary endothelial hyperplasia is a benign and rare vascular lesion usually presenting as a simple nodule. It may be painful. Diagnosis is histologic, characterized by papillary endothelial proliferation associated with a thrombus within a vessel. It may be confused with hemangiosarcoma. Treatment is surgical and recurrence after treatment is rare. Intravascular papillary endothelial hyperplasia is generally considered as an unusual form of thrombus organization. Intravascular papillary endothelial hyperplasia is divided into two groups: a pure form occurring within a dilated vessel and a mixed form appearing in benign vascular lesions. The originality of this case is the rarity and the multiplicity of the lesions. The possible pathogenesis of interferon-induced cutaneous vascular lesions is discussed.

[Brooke-Spiegler syndrome]. / Syndrome de Brooke-Spiegler.

BACKGROUND: Brooke-Spiegler syndrome is an association of multiple trichoepitheliomas and cylindromas, sometimes accompanied by other adnexal tumors. CASE REPORT: A 44-year-old woman with trichoepitheliomas involving the naso-genal and mental areas associated with cylindromas and spiradenomas on the forehead and pretragal regions creating a turban effect. Other complete or diassociated syndromes were found in family members. No neoplastic tumor was identified. DISCUSSION: Brooke-Spiegler syndrome is an hereditary disease with autosomal dominant transmission. Both benign and malignant neoplasias can be associated. The concomitant existence of different tumors could be helpful in understanding the pathophysiology. There is some debate about the exact origin of the trichoepitheliomas, cylindromas and spiradenomas. Several single-cause theories have been put forward but remain to be confirmed as the genetic anomalies identified for trichoepitheliomas and cylindromas map to different sites. Patients with Brooke-Spiegler syndrome should be explored for malignant neoplasia. A family study is indicated.

[Photoaggravated contact allergy and contact photoallergy caused by ketoprofen: 19 cases]. / Allergies de contact photoaggravées et photoallergies de contact au kétoprofène: 19 cas.

INTRODUCTION: Between September 1994 and September 1999, we observed 19 cases of photoaggraved contact allergy or contact photoallergy to ketoprofen (non steroidal anti-inflammatory derived from arylpropionic acid). We present a clinical and photobiological retrospective study of these 19 cases with investigation of cross-reactivity between benzophenone-containing molecules. PATIENTS AND METHODS: On clinical level, we investigated the type of eruption, the delay of appearance, the initial area of eruption and areas of diffusion. Phototesting included patchtests and photopatchtests performed with the gel containing ketoprofen (17 patients), ketoprofen 2 p. 100 petrolatum (14 patients), fenofibrate 10 p. 100 petrolatum and 10 p. 100 water (15 patients), 3 benzophenones (19 patients): oxybenzone 10 p. 100 petrolatum, mexenone 2 p. 100 petrolatum, sulisobenzone 10 p. 100 petrolatum and the other arylpropionic derivatives (4 patients). Three identical series were applied: one was irradiated with 3/4 polychromatic minimal erythematosus dose, a second was irradiated with UVA 13 J/cm2 until January 1997, then 5 J/cm2, the third series was not irradiated (control series). RESULTS: Patients were 9 men and 10 women with an average age of 41.2 years. The type of eruption was an eczema. The delay of appearance of the eruption was one day to 3 months. For 10 patients, the delay was between 4 and 18 days. The eruption was localized to the application area in 1 case, to the application area then to the same contralateral area in 3 cases, to the application area then to all photoexposed areas in 13 cases, to the application area then to the photoexposed areas and then to non-sun-exposed areas in 2 cases. Evolution showed prolonged photosensitivity in 3 cases after withdrawal of the contact and the contact photoallergy to ketoprofen was severe. Gel-containing ketoprofen photopatchtests showed 9 photoaggravated contact allergy, 6 contact photoallergy and 2 contact allergy. Ketoprofen photopatchtests showed 12 contact photoallergy and 2 photoaggraved contact allergy. Tiaprofenic acid photopatchtests were positive in all performed cases (4/4), but photopatchtests with the other arylpropionic derivatives, without benzophenone structure, were negative. Fenofibrate photopatchtests were always positive (15/15). Benzophenones photopatchtests only showed 4 cases of contact photoallergy to oxybenzone (4/19). In 68 p. 100 of cases, patients presented a contact allergy or photoallergy to fragrances. CONCLUSIONS: This study shows the actual frequency of contact allergy and contact photoallergy to ketoprofen with a higher frequency of contact photoallergy. Thus, photopatchtesting is essential. In cases of contact photoallergy to ketoprofen, ketoprofen, tiaprofenic acid but not the other arylpropionic derivatives, fenofibrate and benzophenones have to be withdrawn.

[CD30 positive pilotropic lymphoma]. / Lymphome pilotrope CD30 positif.

BACKGROUND: We report an unusual case of cutaneous CD30-positive lymphoma with pilar tropism and circulating Sezary cells which had a rapidly fatal course. CASE REPORT: A 78-year-old man presented erythematous infiltration of the face, a pruriginous eruption on the trunk and proximal portions of the limbs with small erythematopurpuric follicular papulae, and node enlargement in the inguinal and axillary areas. The rest of the clinical examination was normal. Circulating Sezary cells were found in significant numbers on two different blood smears. Histologic and immunohistochemistry examination of a skin biopsy evidenced medium to large sized lymphoid cell infiltration in a perifollicular localization. A few small cells penetrated the pilar apparatus. There was no follicular mucinosis. The tumoral cells expressed CD2, CD3, CD4 and 75 p. 100 were positive for CD30. Node aspiration showed lymphomatous cells and CD3+ and CD30+ lymphomatous infiltration was found on marrow smears. A T clone was evidenced both in blood and bone marrow leading to the diagnosis of pilotropic CD30-positive lymphoma. Chlorambucil and prednisone were given. The patient died 5 months later. DISCUSSION: The cytology findings suggest medium to large cell pleomorphic lymphoma. The circulating Sezary cells, the pilotropic eruption, and the rapidly fatal outcome suggest transformation of a Sezary syndrome into CD30-positive large cell lymphoma which has been described in fungoid mycosis.

[Relationship between clinical findings of patients with bullous pemphigoid and antigens recognized by their circulating antibasement membrane zone antibodies]. / Analyse des signes cliniques des malades atteints de pemphigoïde en fonction des antigènes reconnus par leur sérum en immunotransfert.

INTRODUCTION: Clinical features and extent of bullous pemphigoid lesions differed widely among patients. The pathogenic role of anti-BPAG2 antibodies has been recently demonstrated. The aim of this study was to analyze the relationship between clinical features of bullous pemphigoid patients and the antigens recognized by their serum. PATIENTS AND METHODS: One hundred and twelve bullous pemphigoid patients were included in this prospective multicenter study. Inclusion criteria were the following: 1) diagnosis of bullous pemphigoid established on the presence of 3 of the 4 clinical features of bullous pemphigoid, histological picture of bullous pemphigoid and positive direct immunofluorescence; 2) serum available for immunoblotting studies. The clinical and biological findings were prospectively recorded on standard forms. Sera were collected and analyzed using indirect immunofluorescence and immunoblotting on human epidermal extracts. RESULTS: Analysis of patient's clinical features depending on the antigens recognized by their serum showed that patients whose serum contained anti-BPAG1 antibodies had more frequently pruritus, blisters on the lower limbs and a positive indirect immunofluorescence. Patients whose serum contained anti-BPAG2 antibodies had blisters more frequently localized on the head, and a more frequently negative indirect immunofluorescence. Patients whose serum was negative by immunoblotting had less frequently urticarial and/or eczematous lesions, bullae less frequently localized on the lower part of the trunk, abdomen and lower limbs, lower eosinophilia and a more frequently negative indirect immunofluorescence. CONCLUSION: Patients with circulating anti-BPAG1 antibodies exhibited the most typical, clinical and biological features of bullous pemphigoid.