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Biomedical subjects

A Taïeb

Publications and source records attributed to A Taïeb.

At least 145 records · Page 8Linked to original sources

Infantile acute hemorrhagic edema of the skin: study of ten cases.

Infantile acute hemorrhagic edema of the skin is not included as a separate entity in the current English-language literature as it is in continental Europe. Therefore we have attempted to clarify the nosologic position of acute hemorrhagic edema among cutaneous vasculitides in children, on the basis of our experience in 10 cases. Our study confirms that acute hemorrhagic edema affects infants between 4 and 24 months of age. The two main features are an ecchymotic purpura, often in a cockade pattern, and an inflammatory edema of the limbs and face. Visceral involvement is uncommon. Spontaneous and complete resolution occurs within 1 to 3 weeks; one to four attacks may occur. Histopathologic examination demonstrates a leukocytoclastic vasculitis. Perivascular IgA deposits can occasionally be found. Besides typical acute hemorrhagic edema, some cases in 2- to 4-year-old children appear to overlap with Schönlein-Henoch purpura. We suggest that typical acute hemorrhagic edema should be regarded as a separate clinical entity. This allows an appropriate prognosis to be made for this generally benign disease of infants.

Acute Disease↗

Treatment of leg ulcers with an allogeneic cultured-keratinocyte-collagen dressing.

A living cellular allogeneic dressing made up of cultured keratinocytes adhering to a collagen film was used to treat 20 leg ulcers of various aetiologies in 16 patients. A reduction in pain was noted in 80% of cases, and promotion of granulation tissue in the ulcer bed in 70% of cases. In 10 patients, epithelialization of 71 +/- 29% of the ulcer was noted at Day 30.

Aged↗

Urticaria in infants: a study of forty patients.

A retrospective study was carried out in 40 infants, age 1 to 24 months, with urticaria. Acute urticaria was seen most frequently (85%), followed by recurrent (10%) and chronic disease (5%). Several clinical features such as frequency of angioedema and hemorrhagic lesions appeared to be specific to urticaria in infants. An underlying cause was identified or suspected in 65% of cases: foods in 25%, and drugs and infections in 37.5%. Under 6 months of age, all infants had acute urticaria and 75% had cow's milk allergy. After 6 months of age, the main causes were drug intake (mostly aspirin and amoxicillin) and/or infections (mainly viral) (50%). Atopy was not overrepresented (20%), although a possible link between atopy and recurrent urticaria was noted. After a follow-up of 2 to 7.5 years, 96% of patients were symptom free. Thus, our results indicate that clinical and etiologic features of urticaria in infants are somewhat different from those of adults and children.

Acute Disease↗

Generalized epidermolysis bullosa with congenital synechiae, associated malformations and unusual ultrastructure: a new entity?

A male newborn presented at birth with marked skin fragility and widespread erosions of the face, scalp, trunk and extremities. Abnormal associated findings included: cleft palate, hypoplastic low-set ears, bilateral synechiae of the first and second toes, incomplete fusion of both eyelids, and dystrophic nails. There was no evidence of consanguineous parentage. The karyotype was normal. The baby died at 3 days of age because of pulmonary distress. As demonstrated by light and electron microscopy, there were both epidermolytic and dermolytic features. Desmosomes and tonofilaments were decreased in most basal cells. Hemidesmosomes were normal. Anchoring fibrils were rare. This type of epidermolysis bullosa does not fit any known categories of disease.

Abnormalities, Multiple↗

[Immunologic changes in linear scleroderma in children. Apropos of 11 cases].

Immunologic data collected in 11 children (6 girls and 3 boys under fourteen) presenting with linear scleroderma were analysed in a retrospective study: 2 children presented with superficial linear scleroderma, 6 with monomelic scleroderma, 1 with dimelic scleroderma, 1 with the "en coup de sabre" variety associated with dimelic homolateral involvement, and another with "en coup de sabre" scleroderma combined with facial hemiatrophy: Antinuclear antibodies (ANA) were demonstrated in 9/11 cases (i. e. 81 p. 100). The immunofluorescence staining pattern was homogeneous in all nine with a low titer (less than 250 in 5 of them). ANA to single stranded DNA was present in 1/3. The demonstration of ANA in these 9 children was correlated with deep or extensive sclerosis with muscular involvement in 7. But neither the presence nor the titer of ANA were correlated with the subsequent development of osteoarticular sequelae. The level of total complement appeared to be lowered in 3/8 cases. No renal involvement was demonstrated. Blood tests for circulating immune complexes were positive in 4/8 patients. Skin biopsy for direct immunofluorescence was performed in 6 children and demonstrated immunoglobulin deposits in 4: three had IgM fixation on the dermo-epidermal junction, and one had speckled fixation of IgG on epidermal nuclei (this has not previously been reported in localized scleroderma). There data highlight: a--the high frequency of ANA in linear scleroderma.(ABSTRACT TRUNCATED AT 250 WORDS)

Adolescent↗

Postgranulomatous anetoderma associated with Takayasu's arteritis in a child.

Takayasu's arteritis (TA) is a rare chronic inflammatory arteriopathy affecting mainly the aorta and its branches. Many skin manifestations have been reported in association with this disease. Pyoderma gangrenosum and subcutaneous inflammatory lesions of the leg are the most frequent. We studied a boy with TA in whom a papular rash of the trunk preceded the onset of vascular symptoms by many years. Histologically, the lesions were superficial and consisted of middermal noncaseating tuberculoid granulomas, which progressed to atrophy and anetoderma because of elastic network disruption. Granulomas were also found in synovial tissue but not in a temporal artery biopsy specimen, which showed only intimal hyperplasia. Our observations suggest that vascular and skin lesions with elastic tissue may both result from a common granulomatous hypersensitivity process.

Aortic Arch Syndromes↗

Collagen studies in congenital cutis laxa.

A case of congenital cutis laxa (CCL) of unclear mode of inheritance associated with multiple pulmonary artery branch stenosis was extensively investigated to assess possible correlations between clinical, ultrastructural, and biochemical features. Light microscopy revealed that elastic fibers were absent in the papillary dermis, while hypoplastic elsewhere. Transmission electron microscopy showed a poor elastin matrix content in some elastic fibers, variable diameters of collagen fibrils, and abundant glycogen granules in most dermal cells. Measurement of collagen fibril diameters, using an image analyzer, was carried out in the patient and two age- and site-matched controls. A bimodal distribution was found in the upper reticular dermis of the patient. In vitro analysis of collagen in skin fibroblast cultures of the patient showed increased collagen synthesis with a balanced production of type I and type III procollagens. Our study confirms that CCL represents a disorder both of collagen and elastic connective tissue.

Cells, Cultured↗

Lupus erythematosus panniculitis with partial genetic deficiency of C2 and C4 in a child.

A 7-year-old girl developed recurrent febrile nodules and subcutaneous plaques on her arm, shoulder, and face. Primary acquired toxoplasmosis was diagnosed at onset, because of associated lymphadenopathy and detection of specific IgM. Skin lesions were responsive to therapy with systemic steroids but progressed to atrophy and poikiloderma within a few months. Subsequently, chloroquine therapy has been instituted and no relapse has occurred. Histopathologic findings and direct immunofluorescence studies were diagnostic of lupus erythematosus panniculitis. Electron microscopy showed lamellar inclusions within mast cells. Results of laboratory investigations disclosed leukopenia and hypocomplementemia with low C2 serum levels. A family study of the complement system, including C4 and Bf phenotyping and HLA A, B, and DR haplotyping, revealed the carriage of both C2 and C4A null alleles in the propositus. This observation suggests an additional link between lupus erythematosus panniculitis and the remainder of the lupus erythematosus clinical spectrum.

Adolescent↗

Solitary Langerhans cell histiocytoma.

Biopsy of a solitary tumor of the buttock in a 3-month-old girl was diagnosed as a histiocytic proliferation suggestive of histiocytosis X. Electron microscopy showed Birbeck granules and dense myelinlike bodies within the cytoplasm of the tumor cells. An immunoperoxidase study, using a panel of monoclonal antibodies (OKT6, OKT4, and OKM1) and a polyclonal anti-S100 protein antibody, showed positive staining for OKT6 and OM1 and moderately positive staining for OKT4 and S100. After surgery to remove the tumor, visceral involvement could not be demonstrated during a 20-month follow-up.

Buttocks↗