[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.

[Comparison of diagnostic criteria in Moroccan patients with Behçet's disease]. / Comparison des critères diagnostiques de la maladie de Behçet chez des malades marocains.

BACKGROUND: Behçet's disease is a vasculitis with diverse systemic manifestations usually involving the skin, eyes, joints and nerves. Clinical diagnosis is based on several signs, generally using the criteria established by the International Group for the Study of Behçet's disease, by O'Duffy, by Hamza, by Mason and Barnes and by the Japanese Committee. The purpose of this retrospective study was to assess the frequency of clinical manifestations in Behçet's disease and to apply the five diagnostic criteria scorings cited above to our patients to determine their sensitivity. PATIENTS AND METHODS: Between January 1988 and December 1997, 110 cases of Behçet's disease were seen at our Dermatology Unit in Casablanca as inpatients or outpatients. Complete history and clinical data including results of the ophthalmology exam and skin tests were available for 70 patients (29 men and 41 women, mean age 31 years). RESULTS: All 70 patients had buccal aphtosis and 88 p. 100 had genital aphtosis. Ocular signs were found in 36 p. 100 of the patients and the skin test was positive in 57 p. 100. According to the criteria of the International Group for the Study of Behçet's disease, 65 patients (93 p. 100) had Behçet's disease. For the Japanese Committee criteria there were 63 (90 p. 100), for the O'Duffy criteria 48 (68 p. 100), for Hamza criteria 45 (64 p. 100) and for Mason and Barnes criteria 35 (50 p. 100). DISCUSSION: Our series is comparable to other Mediterranean series observed in dermatology units, but our patients had fewer systemic manifestations compared with another Moroccan series reported from an internal medicine ward. This could be due to recruitment bias and different medical education. For positive diagnosis of Behçet's disease, the criteria established by the International Group for the Study of Behçet's disease, the Japanese Committee, O'Duffy and Hamza enabled the diagnosis in the majority of the cases. Fifty percent of the patients had all the criteria described in these scores. These criteria would appear to be both overly descriptive, with the risk of confounding other disease states such as enterocolopathy, or underly descriptive, making it impossible to classify certain patients as having Behçet's disease.

[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.

[Lichen planus and hepatitis C]. / Lichen plan et hépatite virale C.

Cases associating lichen planus and hepatitis C are increasingly being reported. We observed three new cases, two males and one female aged 46, 63 and 56 years respectively. In all three cases, the lichen planus was limited to the oral cavity with erosion in two cases. Hepatitis C was confirmed by 2nd generation ELISA. Two of the patients consulted for lichen planus during the recovery phase after hepatitis C. In the third patient, lichen planus preceded the hepatitis. These three cases are further evidence favouring the hypothesis of a non-fortuitous association between lichen planus and hepatitis C. The hepatitis C virus might induce an immune reaction leading both to lichen planus and chronic hepatitis.

[Neurosyphilis: 53 cases]. / La neurosyphilis: a propos de 53 cas.

PIP: This study reported on 53 cases of neurosyphilis which were seen in the Dermatovenerology Department of the Ibn Rochd University Hospital Center in Casablanca from January 1983 to December 1988. The classification was as follows: 26 general paresis, 8 tables, 6 myelitis and cerebral syphilitic arteritis, 7 optic atrophy, 2 early syphilitic meningoencephalitis, 2 unclassifiable forms, and 1 asymptomatic neurosyphilis. A history of syphilis was found in 43 or 3% of the cases. Patients ranged in age from 22 to 61 years, with an average of 40.8 years. There was a clear predominance of males (81.1%). All of the patients were negative for HIV. The diagnosis of neurosyphilis was highlighted along with the clinical manifestations associated with a positive serology (VDRL, TPHA) in the serum and the cerebrospinal fluid (CSF). There was a pleocytosis of CSF in 52.8% of the cases and increased CSF protein in 35.8% of the cases. Treatment consisted of intravenous aqueous penicillin G in 52 patients with a follow up of between 2-66 months. Improvement of lesions was noted in 48% of the cases and sequelae were definitive in 50% of the cases. 1 patient (2% of the cases) died. (author's modified)^ieng