[Multiple accessory tragi and Goldenhar's syndrome].
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Biomedical subjects
Publications and source records attributed to H Lakhdar.
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UNLABELLED: In Morocco, tuberculosis is still endemic. Cutaneous tuberculosis is ranged the fifth after the pleuro-pulmonary, lymphe node, urogenital and digestive tuberculosis. It mainly affects young people. Few studies of cutaneous tuberculosis are available in this age group. The aim of our study is to emphasize its epidemiological features. PATIENTS AND METHODS: It is a retrospective study including all cases of childhood cutaneous tuberculosis observed between January 1981 and December 2004. The diagnosis was based on the confrontation of clinical, immunological, bacteriological and histological data. RESULTS: Thirty cases were collected. The mean age was 11 years. The clinical features are as follows: gumma 46.6%, scrofuloderma 36.6%, lupus vulgaris 13.3% and skin tuberculosis chancre 3.3%. The Mantoux test was positive in 67%, tuberculoid granuloma was observed in 78% and the search of Mycobacterium tuberculosis was positive in 13%. DISCUSSION: Gumma and scrofuloderma were the most frequent forms as in other Moroccan series. These results attest the endemicity of this affection in our country. The diagnosis relies on the discovery of Mycobacterium tuberculosis; as this situation is rare for the cutaneous location, the diagnosis relies then on the association of clinical and paraclinical criteria.
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.
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BACKGROUND: Localisation of epidermoid carcinoma in the intertoe space is very rare. We report a case occurring in a setting of chronic intertoe intertrigo. CASE: A 66 year-old woman presenting intertoe intertrigo for 30 years consulted for painful ulceration of the 3rd intertoe space of the left foot present for 4 years and refractory to antifungal treatment. The examination revealed ulceration with infiltrated edges occupying the entire 3rd intertoe space, with no satellite adenopathy. Histological examination of the skin biopsy sample revealed epidermoid carcinoma. Wide excision of the lesion was performed with a safety margin of 5 mm, with removal of the 3rd and 4th left toes. The outcome was favourable with no relapse after 12 months. DISCUSSION: A single publication reported a series of 22 cases of intertoe epidermoid carcinoma in which the key aetiological feature of tumour was continual maceration. In this patient, maceration was maintained by a number of factors, including cultural factors: ritual ablutions performed 5 times daily, with no drying of the intertoe spaces, and domestic cleaning involving large quantities of water several times weekly.
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.
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INTRODUCTION: Behçet disease is a multisystemic vascularitis. Ocular affection is one of the major criteria of this disease. The aim of this study is to specify the clinical, therapeutical characteristics and the prognosis factors of the ocular affection in patients having BD and admitted to the dermatology department. PATIENTS AND METHODS: It is a retrospective investigation carried out in the dermatology department of Ibn Rochd university hospital center of Casablanca, Morroco, from Jannuary 1990 until December 2003. Two patient groups have been distinguished. The first one involved 50 patients (44,2%) having BD with ocular affection, and the second group involved 63 patients having BD without ocular affection. RESULTS: The mean age was 29 +/- 8 years in the first group VS 30 +/- 7,9 years in the 2(nd) group. The ocular affection was more frequent in males than in females (P < 0.05). The ocular manifestations were marked by uveitis and retinal periphlebitis. The frequency of the cutaneomucosal and joint manifestations was similar in both groups, whereas neurologic and vascular with ocular affections. The choice of the treatment depended on the type of ocular affection. Evolution was marked by blindness in 6 patients (12%). DISCUSSION: The ocular affection comes second after the cutanous mucuous affection. Males are more clearly affected than females. This allows saying that there is a marked effect of the sexual hormones on the ocular affection. Age is not predictive of this ocular affection. The ocular affection was severe in our series and was dominated by uveitis and vascularitis. We insist on the severity of ocular Behçet and its evolution? Risk toward blindness especially concerning young man. Currently, the treatment is not codified; however, the encouraging outcome obtained with some immunosuppressive therapies would be better if this treatment was set up early. CONCLUSION: This study enebed us to re-examine the ocular manifestations of the Behçet disease in the Maroccan population by the means of a consultation of Dermatology. Il should be noted that it is worse forecast because on the one hand of its frequent association to vascular and neurological affections and other share of the delay of consultation noted at the majority of our patients.
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INTRODUCTION: Pemphigus is a frequent chronic bullous dermatosis in Morocco that predominantly affects young women. It requires serious therapeutic management and often repeated hospitalizations. The purpose of this study was to assess the particular impact of pemphigus on quality of life by comparing the quality of life in patients suffering from pemphigus with that of the general population using a generic instrument of measure of health related quality of life: the SF-36, in association with a questionnaire exploring the impact of the disease on self perception, social relationship and behaviour. PARTICIPANTS AND METHODS: A French version of the SF-36 underwent 2 translations from French to Moroccan dialect, then two retrograde translations. After a pre-test, the questionnaire survey was administered by the same investigator to 30 patients who suffered from pemphigus, followed-up in the Ibn Rochd UHC in Casablanca and to 60 healthy adults. The two samples were paired according to age and sex. RESULTS: In the pemphigus group, there was a significant decrease of mean scores of all the SF-36 dimensions, except for physical pain and alteration in general status of health. The greatest alteration concerned the impact on physical and emotional status (p<0.00001) and the smallest alteration concerned the perception of general health (p=0.02). The factors influencing this impact were: profession, face involvement and extent of lesions. Other results concerning the psychosocial impact of pemphigus are presented. CONCLUSION: Our study confirmed that pemphigus is responsible for great alterations in health related quality of life. Therefore, the management of this disease must take into account its impact on various fields of life of the patients.
INTRODUCTION: Xeroderma pigmentosum is a rare recessive and autosomically transmitted genodermatosis. Its cutaneous manifestations are dominated by skin cancers. This investigation aims at studying the epidemiologic, clinic, histologic, therapeutic and evolutive aspects of the skin tumors during xeroderma pigmentosum. PATIENTS AND METHODS: A retrospective monocentric study was carried out in the Dermatology and Veneorology Department of Ibn Rochd University Hospital of Casablanca, Morocco. It included all the xeroderma pigmentosum admitted to hospital or followed-up from 1990 to 2000. All the dossiers were included. The anatomopathologic study was carried out in all the skin tumor cases. RESULTS: One hundred and twenty xeroderma pigmentosum were admitted in 10 Years. Fifty-four percent of the cases were females and 46 p. 100 were males. The mean apparition of the first tumor was 7.7 Years. One hundred and fifty-three skin tumors were diagnosed in 96 patients (80 p. 100). These tumors were of basocellular carcinoma type in 32.6 p. 100, of squamous cell carcinoma type in 33.9 p. 100 and of melanoma type in 11 p. 100. Ocular tumors were found in 31 cases (25.8 p. 100) and buccal in 8 cases. Therapeutically, the surgical exeresis of one or many tumors was performed in all cases. Electrocoagulation was associated in 42 p. 100 of the cases and skin graft in 52 p. 100. Cutaneous relapses after surgery were noticed in 55 cases (57.2 p. 100). Twenty-five patients died and 31 were lost to follow-up. DISCUSSION: Our series is characterized by a large frequency of mainly cutaneous tumors (80 p. 100) in comparison with the largest review of literature (45 p. 100). These tumors were mainly represented by basocellular and squamous cell carcinoma with onset at an early age (7.7 Years). Our series is also characterized by a large frequency of cutaneous relapses after surgery (51.6 p. 100). Neglecting advice on photoprotection and the lack of regular control visits lead to the proliferation of large size tumors, making therapeutic strategies difficult or even impossible.
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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.
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The renal failure-related pruritus is frequent (10-30%) and this frequency increases in dialysed patients (60-80%). Its physiopathology remains badly known. The prurit is variable in intensity and surface. It predominates on the forehead, the back and the forearms. The skin is normal on it presents non specific lesions. The best treatment would be the renal graft. When it can't be done, phototherapy represents a good therapeutic alternative. We report the experience of our patients from December 1993 to January 2002. The patients could benefit from a steady treatment and be well followed-up. The mean age of our patients was 51 years with a masculine predominance (12 men to 7 women). The mean duration of hemodialysis was 3 years and the one of prurit was 10 months. Between December 1993 and January 2001, UVB phototherapy with a spectrum was used in 14 cases. After this period a phototherapy with a narrow spectrum was used. The mean dose was 2 Joules with a number of sessions. Eventually, the total body irradiation with UVB was the most regularly effective therapy of the hemodialysed prurit. It allows an important reduction, which is a significant result in comparison with UVA irradiation.
INTRODUCTION: Juvenile hyaline fibromatosis is a rare hereditary genetic disease characterized by a cutaneous tumoral fibroblastic proliferation with joint involvement. CASE-REPORT: We report the case of 2 homozygous twin brothers both presenting a diffuse and invalidating form of juvenile hyaline fibromatosis. The 2 boys' born of a twin pregnancy' presented at the age of 2 months a clinical picture made up by the classic association: infiltrated papulo-nodules, gingival hyperplasia and joint contracture. The diagnosis of juvenile hyaline fibromatosis suggested clinically was confirmed by the histological study of the skin. DISCUSSION: These observations of juvenile hyaline fibromatosis's are particular in the involvement of 2 twin brothers, which to our knowledge has never been reported, the resemblance of the clinical features, and the severity of the functional handicap.
The prevalence of pruritus varies from 60% to 90% in patients receiving hemodialysis. The aim to this study was to evaluate the frequency of pruritus and its characteristics among moroccan patients receiving hemodialysis. Pruritus related to primary skin disease or other identified cause than chronic renal failure was excluded. Biological parameters were compared between patients with pruritus and those without. Pruritus occurred in 74.4% of hemodialysis patients and concerned 66 men and 68 women, mean age 44.8 + 14 years. Duration of hemodialysis varied between 5 months to 10 years. Main characteristics of pruritus were a general pattern in 70%, moderate intensity in 50%, appearance after dialysis in 81.2% and severe psychologic repercussion in 20.8%. Antihistamines first-line treatment was rarely effective. UVB radiation used in 7 cases leaded to a marked improvement. This study underlined the high frequency of pruritus in moroccan patients with chronic renal failure on maintenance hemodialysis. Hemodialysis can initiate this symptom as well as improve it. Biological parameters were not different between patients with or without pruritus. It also pointed out on therapeutic challenges. However, UVB radiation seems to be an effective therapy in intractable itching as well as increased frequency of weekly courses of hemodialysis. Long term skin hydratation should be also highly recommended.