PubMed Health⌕ Search

Biomedical subjects

F Nejjam

Publications and source records attributed to F Nejjam.

10 recordsLinked to original sources
PubMed2001

[Allgrove's syndrome].

INTRODUCTION: Triple A syndrome is an autosomal recessive disease causing achalasia, alacrima and adrenal involvement with isolated glucocorticoid deficiency. Less than 70 cases have been reported worldwide. We report a case of familial adrenal insufficiency with hyperpigmentation diagnosed in a 14 month-old child. CASE REPORT: A 2 year-old boy, a product of consanguineous parents, was referred to our institution for evaluation of melanoderma. Since birth the boy had suffered from vomiting and diarrhea. Aged 6 months, mucosal erosive lesions had appeared associated with fever and further complicated at the age of one year by alacrima. At the age of 14 months, hyperpigmentation of all the skin had occurred. The family history is significant: two siblings (a girl and a boy) died of hypoglycemia with melanoderma and alacrima at the age of 5 and 3 respectively. Physical examination showed generalized hyperpigmentation particularly marked on the lips and genitalia. The genitalia of our patient were normal. Cortisol was 23.7 microns/l (normal: 193-772); ACTH was 11,722 pg/ml (normal: 10-60) and computed tomography of the abdomen confirmed adrenal gland hypoplasia. Treatment was initiated with hydrocortisone at the dose of 10 mg/day. At the age of 3, the boy developed plantar hyperkeratosis. When solid food was introduced, vomiting and regurgitation increased. An oesophagogram with fibroscopy revealed achalasia of the cardia. This was successfully corrected by surgery. Schimer's test confirmed alacrima. DISCUSSION: Our case report, characteristic of triple A syndrome, is unusual in view of the presence of plantar keratoderma and absence of any neurological abnormalities.

Adrenal Insufficiency↗
PubMed2000

[Icthyosiform erythroderma: atypical manifestation of sarcoidosis].

BACKGROUND: Specific cutaneous manifestations of sarcoidosis can be extremely varied. We report one observation of cutaneous sarcoidosis with an exceptional clinical presentation: icthyosiform erythroderma. CASE REPORT: A 33-year-old woman had a dry icthyosiform erythroderma associated with arthralgia and muscular deficit of the pelvic belt in a context of fever and weight loss. Skin biopsy revealed the presence of an epithelioid giant-cell granuloma. This granuloma was also found in the salivary glands and in the bronchial mucosa. The chest X ray showed diffuse reticularis opacities. The diagnosis of sarcoidosis was retained. The patient was treated with oral prednisone (0.75 mg/kg/day). Total skin clearing was obtained after 15 days. DISCUSSION: This observation points out the heterogeneous nature of clinical manifestations of cutaneous sarcoidosis. The icthyosis erythroderma form is exceptional. Correct diagnosis is difficult and must be based on clinical, histological, radiological and biological criteria of sarcoidosis.

Adult↗
PubMed2000

[Vogt-Koyanagi-Harada's disease: 3 cases].

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.

Adult↗
PubMed1996

[Closed spinal dysraphism. Apropos of 3 cases].

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.

Child↗
PubMed1995

Pilot study of terbinafine in children suffering from tinea capitis: evaluation of efficacy, safety and pharmacokinetics.

In an open pilot study, 12 children with tinea capitis were treated for 6 weeks with oral terbinafine (125 mg/day), and followed up 2 weeks later. The study was conducted to evaluate the efficacy, safety and pharmacokinetics of terbinafine. All patients were completely cured at the end of the treatment period, and there was no evidence of relapse at follow-up. Seven had a negative culture after 3 weeks of treatment. The time to obtain culture conversion from positive to negative did not appear to be related to body weight, but to clinical severity at baseline. Terbinafine is well tolerated and safe over a 56-day period. The kinetic data show a higher clearance of terbinafine in children compared with adults, with shorter alpha- and beta-phase elimination half-lives. However, a longer terminal gamma-phase (at least 6 days) is observed, as in adults, after multiple dose administration, and this is related to elimination from the tissues. The plasma concentrations are comparable between children and adults at a steady state (125 mg/day).

Adolescent↗
PubMed1994

[Lichen planus and hepatitis 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.

Female↗