Pulse corticosteroid therapy for alopecia areata: long-term outcome after 10 years.

BACKGROUND: Few data are available concerning the efficiency of pulse corticosteroids in alopecia areata (AA). OBJECTIVE: Our purpose was to assess the long-term outcomes of patients treated with methylprednisolone bolus. METHODS: This study included 60 patients treated between 1995 and 2000. The short-term outcomes were analyzed in 2000. The long-term assessment of 30 patients was performed in 2010 by phone questionnaire. RESULTS: Significant hair regrowth was observed in 10/30 patients at 6 months after the bolus treatment. Half of the plurifocalis AA patients were responders at 6 months, but less than one quarter of alopecia totalis (AT) and alopecia universalis (AU) patients responded. Long-term outcomes were assessed after a mean duration of 12.3 years; 8/10 initial responders had mild or no disease, and 14/20 initial nonresponders had severe AA. CONCLUSIONS: This study confirmed the low efficiency, both short- and long-term, of this treatment for AT and AU.

[Launois-Bensaude's disease: report of 17 cases]. / Maladie de Launois-Bensaude : à propos de 17 cas.

INTRODUCTION: Launois-Bensaude's disease (LBD) is a rare condition affecting patients aged between 30 and 60, and being considered as more frequent in males. It is characterized by symmetric fat deposits mainly on the neck and upper trunk that can lead to major functional and aesthetic damage. The cause of LBD is unknown but alcoholism is often associated. PATIENTS AND METHODS: The objectives were to study the epidemiological, clinical, and biological particularities, the therapeutic modalities and the evolution of 17 patients (nine males, eight females) seen between 2003 and 2005 at a medical and surgical consultation, and to compare them to the literature data. Seven patients were seen again in our out-patient department and the nine others (one was lost for follow-up) agreed to answer our questionnaire on the phone. RESULTS AND COMMENTS: The high proportion of female in our study is due to the high prevalence of female alcoholism in Northern France. Indeed, all patients were alcoholic but eight were deprived at the time of the first consultation. Mean age for the beginning of the disease was 48. Mean delay between the first signs and our medical and surgical consultation was eight years. Tumoral masses mainly involved the neck, the trunk, and the deltoid areas. Surgical treatment was performed in nine deprived patients for at least 12 months with a satisfactory functional and aesthetic result and a mean decrease of 50% was noted for the dermatology life quality index (DLQI). We observed no immediate complications but two cases with hypertrophic scar and one case with persistent dysesthesia. Four out of them presented recurrence corresponding with resumption of alcohol abuse, confessed in two cases and suspected in the two others. In the absence of surgical management, the disease was stable in three cases but evolved in four cases. No spontaneous regression was observed.

[Topical haemotherapy as treatment for necrotic angiodermatitis: a pilot study]. / Autohémothérapie locale dans l'angiodermite nécrotique: étude pilote.

INTRODUCTION: Necrotic angiodermatitis is a variety of leg ulcer, characterized by very painful and rapidly spreading lesions. Healing takes time, a mean of 4 to 11 months. Current treatment is difficult and rarely satisfactory. Only early skin grafts lead to rapid sedation of pain and stop the lesion from extending. However, this technique, performed during a progressive phase, enhances the risk of losing a certain number of grafts. We wanted to confirm the efficacy of local auto-haemo-therapy proposed in 2 non-controlled studies. PATIENTS AND METHODS: Eleven patients with necrotic angiodermatitis were included in our non-controlled prospective study. The topical hemotherapy was applied three times a week for one month, with initial hospitalization for 15 days. The criteria of assessment were: evolution of pain, surface of the ulcer, colorimetric scale and the date of disappearance of the purple halo. RESULTS: Ten patients were relieved by the treatment, with progressive withdrawal of the analgesics. The progression of the lesion had stopped and the purple halo had disappeared in general by D6 (range: 2 to 21 days). The percentage of granulation tissue with regard to the surface of the ulcer was of 45.4 p. 100 on D0, 80 p. 100 on D14 and 85 p. 100 on D28. CONCLUSION: Topical hemotherapy applied during the initial phase of treatment would reduce the pain and prepare the lesion for a secondary skin graft.

Cutaneous granulocytic sarcoma arising at the site of radiotherapy for breast carcinoma.

Carcinogenic effects of radiotherapy in breast cancer are well-known. Long-term follow-up of these patients shows a significantly increased risk of leukemia. Cutaneous granulocytic sarcoma is an uncommon leukemia cutis that usually occurs in association with acute myelocytic leukemia or myeloproliferative disorders. We report a case of cutaneous granulocytic sarcoma in a 44-year-old woman who had been treated six months earlier for breast adenocarcinoma. The treatment had associated lumpectomy, axillary lymph node dissection and radiotherapy. Skin lesions appeared firstly and predominantly on the irradiated area. Haematological investigations were normal and the diagnosis of isolated sarcoma was made. The uncommon features of this case were the short interval between radiotherapy and the occurrence of leukemia skin lesions and the fact that, to our knowledge, this is the first report of leukemia cutis localised on the irradiated area. The responsibility of radiation in the distribution of cutaneous lesions of granulocytic sarcoma is discussed.

[Amelioration of the diagnostic possibilities of irrigraphy by a new index, I0]. / Amélioration des possibilités diagnostiques de l'irrigraphie par un nouvel indice I0.

Irrigraphie (segmental impedance plethysmography) is a reliable non invasive method for the positive and topographic diagnosis of arterial occlusive disease and for its prognosis (5, 8, 9, 12, 13). The method is based on pulse volume recordings at six different positions of the leg (from 1 = proximal to 6 = distal). The amplitude of the signal is related to heart rate and to the basic impedance of the segment of the leg. The diagnosis of aorto-iliac disease is proposed when the upper extremity index (I1MS) over the lower extremity proximal index (I1) ratio (ITA) is increased (greater than 1.4), or when the I1 value is lower than 1.9. The diagnosis of femoro-popliteal disease is proposed when there is a significant drop between I1 and the above knee index I3. However in a recent study we found that I1 value drops when there is a severe femoro-popliteal lesion. In this case, irrigraphie proposes erroneously the diagnosis of aorto-iliac and femoro-popliteal diseases. In order to improve the method, we thought that a new electrod position (I0) over the iliac region would give to irrigraphie a more reliable topography diagnosis possibility. In this study 32 patients were analysed with angiography and irrigraphy, and divided into 3 groups: group I = isolated iliac disease (19); group F = isolated femoral diseases (22); group IF = ilio-femoral diseases (12). We observe from the ROC analysis, that I0 has a sensitivity of 84% and a specificity of 86%, when the iliac lesions are isolated whereas I1 and ITA give a lower specificity (52%). However, when aorto-iliac lesions are combined with femoro-popliteal lesions the irrigraphie is a little less sensitive (71%). In conclusion I0 index is more reliable for the topographical diagnosis of obstructive disease of the iliac artery than I1 index and we suggest to replace I1 by I0 whenever it is possible.

[Treatment of solar urticaria by intravenous immunoglobulins and PUVA therapy]. / Urticaire solaire traitée par l'association immunoglobulines polyvalentes et PUVAthérapie.

BACKGROUND: Solar urticaria is a rare form of urticaria occurring a few minutes after solar exposure. It is particularly incapaciting because it limits outdoor activities. Antihistamine and phototherapy are sometimes disappointing. CASE REPORT: A 32 Year-old woman had been suffering from severe solar urticaria since November 2000, which was confirmed by photobiological data. High-dose antihistamine treatment (fexofenadine 180 mg twice a day) was inefficient. Despite a first UVA desensitization, PUVAtherapy produced only a partial improvement and short lasting for protection, with an important handicap in daily life. In March 2002, among the others treatments, we chose intravenous immunoglobulins: 0.5 g/kg the first day then 1 g/kg the second and the third days. The minimal urticaria dose was raised from 1 J/cm2 in UVA before perfusion up to 15.6 J/cm2 48 hours later and in UVB from 100 mJ/cm2 up to 2,200 mJ/cm2. Clinically the improvement was significant but partial in daily activities. It was possible to reintroduce PUVAtherapy without UVA-desensitization and, for the first time, to obtain complete remission for more than 2 Months with an association of intravenous immunoglobulins, PUVAtherapy and antihistamine treatment. In July 2002, treatment was successfully repeated. DISCUSSION: First intention treatments (antihistamine and PUVAtherapy) are sometimes inefficient. Others treatments (plasmapheresis, ciclosporin, doxepin, cimetidine) are restrictive, not always efficient and can induce severe side-effects. We report the second case of solar urticaria improved by intravenous immunoglobulins. In spite of the cost, intravenous immunoglobulins seem to be an interesting treatment, at least by avoiding UVA-desensitization in severe cases of solar urticaria.

[Complete supernumerary breast on the thigh in a male patient]. / Sein surnuméraire complet de la cuisse chez un homme.

BACKGROUND: Supernumerary nipples are not rare but the developement of a complete supernumerary breast is exceptional. CASE REPORT: A 59-year-old man presented a progressively increasing ancient right- groin-masse. The histopathologic examination of the lesion confirmed the diagnostic of polymastia. DISCUSSION: The interest of this observation results from the very unusual occurence of polymastia (less than 1 p. 100 of supernumerary nipples), especially for a caucasian man. The prevalence appears to be higher in women and oriental people. Diagnostic of supernumerary nipple is difficult because of its atypical appearance and ectopic location. However, this diagnostic is important because ectopic breast tissue is subject to the same pathologic changes that occur in normally positioned breasts and it can be a marker for associated diseases such as urologic malformations or urogenital malignancies.

[Chronic lupus erythematosus presenting as acneiform lesions]. / Lésions acnéiformes révélatrices d'un lupus érythémateux chronique.

BACKGROUND: Cutaneous manifestations of lupus erythematosus are numerous but usually permit easy diagnosis. However, there are atypical lesions that can mimic benign dermatologic disorders. We report on a patient with lesions of acne leading to the diagnosis of chronic lupus erythematosus, and who subsequently developed systemic lupus erythematosus. CASE REPORT: A 30-year-old woman presented with inflammatory lesions and comedos on the face. The eruption started after her last pregnancy and was refractory to local and general treatment. She also complained of arthralgia, Raynaud's phenomenon and diffuse alopecia. Cutaneous biopsy was characteristic of chronic lupus erythematosus. Immunofluorescence microscopy of lesional skin showed a lupus band deposit. Antinuclear antibodies were highly positive. The patient was successfully treated with chloroquine. Three years later, the patient presented with photodistributed eruption. Antinuclear antibodies were still positive and in addition anticardiolipin antibodies were found. Final diagnosis was systemic lupus erythematosus. DISCUSSION: Acneiform lesions are rarely reported in lupus erythematosus. Only three similar cases were reported in literature. Atypical and treatment-resistant eruptions should attract attention. Furthermore, the occurrence of systemic lupus in chronic lupus erythematosus is not an unfrequent phenomenon and the oestrogen-dependance of chronic lupus lesions may predict this association.

Wells' syndrome associated with recurrent giardiasis.

Wells' syndrome (WS) is a rare, recurrent, inflammatory dermatosis of uncertain pathogenesis, although a hypersensitivity response to different stimuli has been postulated. Several cases in association with parasitic infections have been described. We present a patient with WS associated with recurrent giardiasis. The course of the dermatosis was strictly related to the course of the parasitic infection. Both diseases disappeared with antiparasitic treatment. To our knowledge, this association is here reported for the first time.

Doppler power frequency spectrum analysis in the diagnosis of carotid artery disease.

Power Frequency Spectral Analysis (PFSA) which evaluates the power of the frequency components at a selected interval, was applied in this preliminary study using a 5MHz Doppler continuous wave flow velocity meter and a modified Edwards Spectraview 500 analyzer. PFSA was used to study the common, internal and external carotid arteries of ten young adult control subjects and 31 patients with angiographically documented carotid bifurcation lesions. Two useful indices are described: (1) the frequency band width at 50% of maximum power (f50%) and (2) the highest frequency recorded (fmax). An f50% wider than 1900 Hz (corresponding to spectrum broadening) identified stenoses as low as a 15% diameter reduction. An fmax higher than 6500 Hz practically always indicated a 50% or greater diameter reduction. Eighty two percent of stenoses with 11-25% diameter reduction were detected, as were 95% of stenoses from 36-50%. All stenoses from 51-99% were identified.