[Systemic lupus erythematosus and Epstein-Barr virus]. / Lupus et virus d'Epstein-Barr.

We determined the prevalence of antibodies to Epstein-Barr virus (EBV) in 44 patients with systemic lupus erythematosus (SLE) and 44 healthy blood donors matched for sex and age as controls. Of the cases, 39 were women; mean age was 33 years. Four cases (9%) and 5 controls (11%) were positive for IgM anti-viral capsid antigen (VCA) (P = 0.9). All the cases were positive for IgG anti-VCA compared with 91% of the controls (P = 0.12). The mean immunity ratio for this antibody was 2.341 in cases and 1.873 in controls (P = 0.068). Forty (91%) cases were positive for IgG anti-EBNA1 (EB nuclear antigen1) compared with 42 (95%) controls (P = 0.6).

[Systemic lupus erythematosus and Epstein-Barr virus]

We determined the prevalence of antibodies to Epstein-Barr virus [EBV] in 44 patients with systemic lupus erythematosus [SLE] and 44 healthy blood donors matched for sex and age as controls. Of the cases, 39 were women; mean age was 33 years. Four cases [9%] and 5 controls [11%] were positive for IgM anti-viral capsid antigen [VCA] [P = 0.9]. All the cases were positive for IgG anti-VCA compared with 91% of the controls [P = 0.12]. The mean immunity ratio for this antibody was 2.341 in cases and 1.873 in controls [P = 0.068]. Forty [91%] cases were positive for IgG anti-EBNA1 [EB nuclear antigen1] compared with 42 [95%] controls [P = 0.6]

[Terbinafine-induced Stevens-Johnson syndrome and aggravation of systemic lupus erythematosus]. / Syndrome de Stevens-Johnson et aggravation d'un lupus systémique induits par la terbinafine.

BACKGROUND: There have been published reports of induction and aggravation of subacute systemic lupus erythematosus of lupus by terbinafine. We report the case of female patient with systemic lupus erythematosus who, after treatment with terbinafine, presented a Stevens Johnson syndrome eruption, together with renal exacerbation of her connective tissue disease. OBSERVATION: A 25 - year-old woman was followed for 4 years for systemic lupus erythematosus was no renal involvement. The patient was stable under corticosteroids (20 mg/d) and chloroquine (200 mg/d). She was treated with terbinafine onychomycosis caused by Trichophyton rubrum. Seven days after trunk and limbs, with cheilitis affecting the mucous membrane and bilateral conjunctivitis. This rash was followed by epidermolysis involving 10% of the cutaneous area. Stevens-Johnson syndrome was diagnosed. Laboratory tests indicated massive hematuria and proteinuria, and the renal needle biopsy sample showed signs of class III lupus glomerulonephritis. The anti-histone antibodies were highly positive. The patient was treated with systemic corticosteroids (1 mg/kg/d), chloroquine was recommenced and cyclophosphamides were given in a bolus. The outcome of the patient's eruption and lupus was favorable. DISCUSSION: Although induction and worsening of lupus by terbinafine have already been reported, the present case differs through the concomitant occurrence of eruption on treatment with terbinafine and severe relapse of lupus, thus suggesting the involvement of a shared immunological mechanism.

[Precocious mediastinal lipomatosis: a rare complication of systemic corticosteroid therapy]. / Lipomatose médiastinale précoce: une complication exceptionnelle de la corticothérapie generale.

BACKGROUND: Corticosteroid-induced lipomatosis results from hypertrophy within adipose tissue; the condition is frequently asymptomatic and its incidence is underestimated. We report a case of mediastinal lipomatosis that is rare in terms of both site and presenting symptoms. CASE REPORT: A 46-year-old woman with no disease history other than obesity with a weight of 90 kg had been treated since 2002 for mixed connective tissue disease (profound lupus and dermatomyositis). She had been treated with oral corti costeroids (1 mg/kg/d). Two months after the start of treatment, she presented chest pains, resting dyspnea particularly aggravated in dorsal decubitus, chest edema in the subclavicular space and jugular turgescence. Chest x-ray revealed widening of all levels of the mediastinum. The chest CT scan showed lipomatosis throughout the entire mediastinum with no associated chest abnormalities or pericardial effusion. Rapid downward dosage adjustment ofcorticosteroids to 10 mg/d coupled with synthetic antimalarials resulted in gradual reduction of symptoms. The chest scan performed two months later short stabilization of the patient's mediastinal lipomatosis. DISCUSSION: The effects of long-term of glucocorticosteroid therapy are well-known, in particular Cushing's syndrome. Lipomatosis has been described more recently and affects different axial regions. Mediastinal localization is seen in 15% of patients treated. This presentation is less common than orbital and epidural localizations. Although often asymptomatic, as in our own report, it may present with worrying symptoms that pose real diagnostic problems. The diagnostic examinations of choice are CT scan or MRI. Regression following discontinuation or reduction of corticosteroids is inconsistent and often gradual.

[Fahr syndrome associated to hypoparathyroidy, revealed by dermatologic manifestation]. / Syndrome de Fahr secondaire à une hypoparathyroïdie à révélation dermatologique.

BACKGROUND: Fahr syndrome is defined by the presence at the brain base of strio-pallido-notched non-arteriosclerotic bilateral and symmetric calcifications. We report an observation of Fahr syndrome revealed by a growing pyodermitis in direct relation with hypocalcemia. OBSERVATION: A 17 year-old girl was admitted to the Dermatology Department for a growing pyodermitis of the large folds. The patient was known to be epileptic and had undergone surgery for a left eye cataract a year before. Microbiologic and mycologic examinations of the pus and of the cutaneous fragments were negative. During hospitalization, the patient had an attack of tetany, a laryngeal spasm and a generalized erythema thrust studded with pustules. The phosphocalcic assessment showed severe hypocalcemia and hyperphosphoremia. Parathormone serum rate was decreased. The brain tomodensitometry conveyed bilateral calcifications of the central gray nucleus. The diagnosis of a growing amicrobial pustulosis secondary to an idiopathic hypoparathyroidism and responsible for the Fahr syndrome was maintained. Treatment was composed of calcium (2 g/day) and vitamin D3. The evolution was favorable with a relapse at 2 years. DISCUSSION: We report an idiopathic hypoparathyroidism associated with Fahr syndrome conveyed by growing amicrobial pustulosis. We discuss the dermatologic manifestations of Fahr syndrome associated with hypoparathyroidism and the physiopathologic mechanisms of the dermatologic lesions during hypocalcemia.

[Tabetic arthropathy: "a forgotten disease"]. / Arthropathie tabétique: "une maladie oubliée"

INTRODUCTION: With the current sparcity of syphilitic manifestations, the once obvious aspects of the affection have been forgotten or are little known. We report 4 cases of tabetic arthropathy admitted to the Dermatology Department Ibn Rochd University Hospital of Casablanca. OBSERVATIONS: The mean age of the patients was 50 years, all were males. The diagnosis was based on clinical, radiological, and biological criteria. There was a large contrast between the extent of the articular malformation and the indolence. The radiological aspect associated destructive and constructive lesions. The syphilis serology was positive in the articular liquid, the blood and/or the cerebro-spinal fluid. Treatment was essentially medical. DISCUSSION: Tabetic arthropathy, an exceptional affection, is one of the complications of the tabes dorsalis. Its prevention relies on the treatment of the syphilis at an early stage, before the onset of the articular and neurologic forms, the management of which remains very difficult.

[Male breast cancer in Morocco]. / Cancer du sein chez l'homme au Maroc.

INTRODUCTION: Male breast cancer is a rare disease, often of late presentation and poor prognosis. The aim of this work was to analyze the different clinical and therapeutic features for this disease in men. PATIENTS AND METHODS: This was retrospective study including 12 cases of male breast cancer seen in the Dermatology department of Casablanca between 1988 and 1999. RESULTS: The mean age of the patients was 60 years and the mean delay to consultation was 27 months. The skin was involved by tumor in 11 cases. Ulceration of the skin by tumor was seen in eight patients, and direct extension of the tumor into the nipple without ulceration was seen in three patients. Axillary lymph node involvement was seen in eight patients. Seven patients with invasive disease had metastases at distance. Treatment was usually surgical. Complementary treatment included radiotherapy, chemotherapy and/or hormonotherapy. DISCUSSION: Although breast cancer in men is far less common than breast cancer in women, it is associated with less favorable prognosis because diagnosis is usually made at an advanced stage. Concerted efforts must be made to educate both the public and health professionals, in order to make earlier diagnosis and thereby improve prognosis.

[Vulvar pathology]. / La pathologie vulvaire.

We evaluated the main causes of vulvar dermatoses; in 785 patients with a vulvar diseases who visited Ibn Rochd Hospital Centre, Casablanca between January 1991 and December 2002. The average age was 31 years (range 2 months to 81 years); 362 patients (41.52%) had vulvar pruritus, 273 patients (34.77%) had warts and 157 (20%) had one or more vulva ulcers. The most common infectious pathology was papillomavirus infection, found in 273 cases (34.77%), followed by vulvovaginal candidiases in 102 cases (12.99 %), syphilitic chancre in 18 cases (2.29%) and herpes genitalis in 17 cases (2.16%). The most common non-infectious pathology was vulvar dermatosis: 259 cases (32.99%); idiopathic pruritus vulvae: 61 cases (7.7%); and tumour-related conditions: 45 cases (5.6%). The frequency of infectious conditions was over 50% and these were generally sexually transmitted infections.

[Vulvar pathology]

We evaluated the main causes of vulvar dermatoses; in 785 patients with a vulvar diseases who visited Ibn Rochd Hospital Centre, Casablanca between January 1991 and December 2002. The average age was 31 years [range 2 months to 81 years]; 362 patients [41.52%] had vulvar pruritus, 273 patients [34.77%] had warts and 157 [20%] had one or more vulva ulcers. The most common infectious pathology was papillomavirus infection, found in 273 cases [34.77%], followed by vulvovaginal candidiases in 102 cases [12.99%], syphilitic chancre in 18 cases [2.29%] and herpes genitalis in 17 cases [2.16%]. The most common non-infectious pathology was vulvar dermatosis: 259 cases [32.99%]; idiopathic pruritus vulvae: 61 cases [7.7%]; and tumour-related conditions: 45 cases [5.6%]. The frequency of infectious conditions was over 50% and these were generally sexually transmitted infections

[Vogt-Koyanagi-Harada's disease: 3 cases]. / Maladie de Vogt-Koyanagi-Harada : 3 cas.

BACKGROUND: Vogt-Koyanagi-Harada's disease is a bilateral chronic panuveitis sometimes associated with signs of meningo-encephalic, auditory and skin and nail involvement. We report 3 cases. CASE REPORTS: The first case was a 30-year-old woman who consulted for a red eye, low visual acuity, poliosis, and diffuse alopecia which had developed over 9 months. The ophthalmology examination disclosed anterior uveitis with retinal detachment. The second patient was a 9-year-old child who developed poliosis, canities, and achromic lesions over a 2 month period. The ophthalmology examination disclosed low visual acuity, irido-corneal synechiae and pigmented deposits on the anterior lens. The third patient was a 20-year-old man who consulted for alopecia, diffuse canities, and white body hair. The ophthalmology examination disclosed low visual acuity, anterior uveitis, and a serous chorio-retinal detachment. All three patients were given general corticosteroid therapy (1 mg/kg/d). The clinical course was favorable in all cases with however one case of recurrent ocular involvement and one case of pigmentation disorders. DISCUSSION: The diagnosis of Vogt-Koyanagi-Harada's disease was established in these three cases on the basis of the ocular and skin and nail signs. This rare disease usually occurs in young, often female, patients. The pathogenesis remains unknown. Among the three signs observed, ocular involvement is the most serious. Skin and nail signs are seen in two-thirds of cases. For rapid diagnosis and early treatment, this disease requires a multidisiplinary management associating the dermatologist and the ophthalmologist.

[Herpetic superinfection of pemphigus: 6 cases]. / Surinfection herpétique du pemphigus: six cas.

BACKGROUND: Herpes simplex infection should be searched for in patients who experience unexplained aggravation of acantholytic dermatosis. Epidermal alterations appear to play an important favoring role. PATIENTS AND METHODS: We report 6 cases of Herpes simplex infections in patients with pemphigus observed at the Ibn Rochd hospital dermatology unit, Casablanca, over a 3-year period (1995-1998). RESULTS: There were 4 men and 2 women, age range 27-76 years. Superficial pemphigus was observed in 5 cases and pemphigus vulgaris in 1. In all cases, histology showed cytopathogenic Herpes simplex infection. The Herpes simplex infection appeared when the patients were given general corticosteroid therapy at doses from 1 to 1.5 mg/kg/d for 15 days to 1 month. In two cases, the corticosteroids were associated with an immunosuppressor (azathioprine, cyclophosphamide). Cure was rapid with systemic aciclovir, achieved in 5 to 20 days. Recurrence was observed in 2 cases. DISCUSSION: Herpes simplex superinfection in patients with pemphigus is classically described but uncommonly observed. In our experience, it has become more frequent over recent years since, before 1990, we observed no cases. Its prevalence may be underestimated. The diagnosis of Herpes simplex superinfection is sometimes difficult and should be suggested in case of relapse or resistance to treatment. The Herpes virus simulates most cases of pemphigus relapse, with a preferential localization on the bullous lesions. The painful nature of the eruption is highly suggestive of Herpes simplex infection as is the presence of vesicles grouped in bouquets. Both type 1 and type 2 viruses are observed. Cure is rapidly achieved with aciclovir in 5 to 15 days. Spontaneous cure has been reported in the literature as well as recurrent herpetic infection. In two cases in the literature, the Herpes eruption was fatal due to severe herpetic hepatitis and disseminated intravascular coagulation. Herpes simplex infection must be rapidly recognized in pemphigus patients so curative aciclovir treatment can be given early.

Neuronal responses to cannabinoid receptor ligands in the solitary tract nucleus.

Our previous study showed many neurons in the subpostremal division of the nucleus tractus solitarii to be cannabinoid-sensitive. In order to further investigate this sensitivity, single unit activity was recorded extracellularly in rat hindbrain slices, and the effects of bath application of delta9-tetrahydrocannabinol and of two synthetic cannabinoid receptor agonists were analysed and compared to each other. Approximately half the recorded neurons responded to agonists, and most of the neurons exposed to two of the agonists reacted similarly to both. The involvement of cannabinoid CB receptors in neuronal sensitivity to delta9-tetrahydrocannabinol is supported by these data and by the effects of N-piperidin-5-(4-chlorophenyl)-1-(2,4-dichlorophenyl)-4-methyl-3-p yrazole-carboxamide, hydrochloride (SR 141716A), a compound which is considered to be a selective antagonist and/or a selective inverse agonist of this receptor type.

[Repair of substance losses of the nose: apropos of 45 cases]. / La réparation des pertes de substance du nez: à propos de 45 cas.

The authors report 45 cases of tissue loss in the nose, in most cases secondary to cancer ablation, and in a few (2 cases) to trauma. The reconstructive techniques were mainly the classical ones using local flaps, usually at the same time as the tumour removal. Where the reconstruction was a total one, this involved the use of a compound extended Converse flap, which has the advantage of bringing in sufficient tissue associated with the bone graft, and also avoids the risks of thinning or exposure of the graft. The indications depend on anatomical factors, and on the extent and depth of the tissue loss. The cospetic results are very satisfactory, but it is necessary to revise the flaps in order to obtain the best final results.

[Closed spinal dysraphism. Apropos of 3 cases]. / Dysraphisme spinal fremé. A propos de trois cas.

CASE REPORT: Closed spinal midline developmental defect was observed in three children, two girls aged 7 and 10 years and one boy aged 9 years. There were no similar cases in the families. The first signs appeared at approximately 6 months, 9 years and at birth. In the first two cases, developmental defects of the toes were followed a few months or a few years later by chronic ulcerations of the plantar surface of the foot. On physical examination there was a greater toe, amputation of the toes, perforating plantar ulceration, prolonged cauda equin and neurological disorders. Radiology revealed defective closure of the dorsal and/or lumbar vertebrae and lysis of the third phalanxes and magnetic resonance imaging showed diastematomyelia. The third child was born with a pilous nevus and a prolonged cauda equin. A soft subcutaneous tumor developed 3 years later over the lumbar area, increasing in size becoming quite voluminous and painful. In this case, there was no amputation of the toes or perforating plantar ulceration or neurological disorders. Radiography showed hemisacralization of L5 and spina bifida of S1. Magnetic resonance imaging showed a normal spinal cord with a tumor independent of the neural canal. Biopsy exeresis led to the diagnosis of neurofibroma. DISCUSSION: Closed spinal midline development defect occurs more readily in girls than in boys. Multiple and complex abnormalities are observed: diastematomyelia, diplomyelia, dermal fistulae, neuroenteral cysts, dysembryoplasic tumors, abnormal position of the spinal cord, and usually vertebral malformations. There are three types of clinical signs involving skin, bone and neurological manifestations. Radiological explorations should be completed with myelography and a computed tomography as well as magnetic resonance imaging to determine the extent of the malformations and possible surgical indications.

Sensitivity of nucleus tractus solitarius neurons to induced moderate hyperglycemia, with special reference to catecholaminergic regions.

Extracellular single-unit recordings have been carried out in the caudal part of the nucleus tractus solitarius of male rats anesthetized by i.v. infusion with Ketamine. The sensitivity of 38 cells to 0.2 ml of 0.55 M glucose i.v. administration has been studied. The spike frequency of 75% of these cells was affected by the induced moderate hyperglycemia and a majority of them was activated. Immunohistochemical data on 9 glycemia-sensitive neurons showed that all recording sites were within TH-immunoreactive regions of the caudal part of the nucleus (either in the A2 noradrenergic group, or the C2 adrenergic group). These results confirm the existence of glycemia-sensitive neurons in the nucleus tractus solitarius, and suggest that these cells might mediate the effects of physiological fluctuations in blood glucose level on the control of food intake and metabolic regulations. The localization of these cells in catecholaminergic regions suggests that adrenaline and/or noradrenaline efferents might convey feeding relevant information concerning glycemic level or satiety signal from the nucleus tractus solitarius to forebrain structures involved in food intake, such as the perifornical lateral hypothalamic area and the paraventricular nucleus.

Inotropic effects of ouabain and Na, K-ATPase activity in failing rabbit hearts.

Left ventricular (LV) function and Na+, K(+)-ATPase activity were studied in rabbit failing heart (FH) obtained by a double pressure plus volume overload which increased LV/body weight ratio by 58%. Ouabain (10(-6) M) increased peak dP/dt by 69% in control hearts (CH) but not in FH. Na+, K(+)-ATPase activity was similar in FH and in CH but the affinity of Na+, K(+)-ATPase for ouabain was significantly higher in CH than in FH (IC50: 2 microM vs 90 microM). The blunted LV contractile response to ouabain in FH is attributed to a decreased Na+, K(+)-ATPase affinity to ouabain and not to a decreased receptor density.

Timing and velocity of ejection as major determinants of end-systolic pressure in isolated rabbit hearts.

BACKGROUND: Systolic shortening is known to produce muscle deactivation. The present study was designed to analyze whether the velocity and the timing of ejection play a role on end-systolic pressure-volume relations (ESPVR). METHODS AND RESULTS: In isolated rabbit hearts, left ventricular pressure and volume were recorded and digitized, and left ventricular volume was controlled by a servosystem (4-millisecond cycles) to alter the timing of ejection. A significant deficit in end-systolic pressure was observed when ejection was late in systole with respect to earlier ejection. This was associated with a significantly reduced end-systolic elastance. End-systolic pressure of beats with slow ejection was intermediate between that of the beats with early ejection and that of beats with late ejection with a significantly increased end-systolic volume compared with beats with early rapid ejection. The same results were obtained with hypertrophied hearts (abdominal aortic stenosis). Pressure-volume loop areas were significantly increased in beats with slow ejections and with rapid delayed ejections versus early rapid ejections. No change in the positive peak of dP/dt was observed when the timing and the velocity of ejection were modified. CONCLUSIONS: ESPVR is modified by the ejection profile, with a decreased end-systolic pressure and an increased pressure-volume loop area related to the velocity and the amount of shortening during the end-systolic phase. These indices of ventricular function thus must be used with caution when the timing of ejection is altered, and the end-diastolic volume-peak dP/dt relation may be a better index of ventricular function.

Electrophysiologic properties and role of the vagal thermoreceptors of lower esophagus and stomach of cat.

Vagal unitary discharges were elicited in anesthetized cats by thermal stimulation of the lower thoracic esophagus and stomach. Discharges were recorded from the nodose ganglion, using extracellular glass microelectrodes. Three types of receptors were distinguished according to the temperature ranges at which they discharged: the cold receptors (10 degrees-36 degrees C), the warm receptors (39 degrees-50 degrees C), and the mixed receptors (10 degrees-35 degrees C and 40 degrees-50 degrees C). All endings were connected to unmyelinated fibers (conduction velocities around 1 m/s). These receptors are stimulated neither by mechanical (strong distention, localized stroking of mucosa) nor by chemical (acid and glucose solutions) stimuli. Therefore they must be considered as true thermoreceptors. Stimulation of the esophageal and gastric thermoreceptors produced changes both in esophageal motility and in respiratory frequency. It was concluded that they are involved in coordination of digestive activity as well as in thermoregulation.