Aspirin-ticlopidin in Kasabach-Merritt syndrome.
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Aspirin↗
Biomedical subjects
J Maleville
Publications and source records attributed to J Maleville.
Eosinophilic pustulosis of the scalp in childhood.
BACKGROUND: Among sterile pustulosis in childhood, a pruritic relapsing eosinophilic variant beginning in infancy and located mostly in the scalp was first described as eosinophilic pustular folliculitis in infancy by Lucky and colleagues in 1984. OBJECTIVE: Our purpose is to describe such a condition in five boys and one girl and comment on differential diagnosis and relation with Ofuji's disease. METHOD: This is a clinicopathologic study. RESULTS: All patients had scalp pustules beginning in infancy or early childhood that were unresponsive to antibiotic therapy. Lesions also occurred on other areas but the scalp was the major site of involvement. Although secondary infection was demonstrated in one case, the lesions were primarily sterile. Smears of pustules showed a variable proportion of eosinophils. Histopathologic findings suggested a major role for eosinophils in this disorder because dermal eosinophilia was noted in all patients. The inflammatory pattern was not similar to Ofuji's disease. Transient blood eosinophilia was recorded in five patients. Topical steroids relieved inflammatory episodes. Dapsone was tried in one case with apparent benefit. CONCLUSION: Eosinophilic pustulosis of the scalp in childhood is a self-limited disease that can be relieved by topical steroids.
Child, Preschool↗
Perineal ecthyma gangrenosum in infancy and early childhood: septicemic and nonsepticemic forms.
BACKGROUND: Ecthyma gangrenosum is characterized by necrotic ulcerations surrounded by an erythematous halo. It is secondary to Pseudomonas aeruginosa infection. Most lesions are located in the anogenital and axillary areas, but the route of infection is generally difficult to establish. OBJECTIVE: We report six children with perineal ecthyma gangrenosum and discuss predisposing factors, origin, and route of infection. METHODS: This was a retrospective clinical study. RESULTS: Three children had blood cultures positive for P. aeruginosa, and one died. Predisposing factors were present in all cases; two had received chemotherapy (neuroblastoma, acute lymphoblastic leukemia), and two had idiopathic granulocytopenia. The last two patients previously had received treatment with systemic antibiotics and had abnormal granulocyte killing several months later. CONCLUSION: Septicemic ecthyma gangrenosum can be rapidly fatal in young children and requires aggressive antibiotic therapy. Benign ecthyma gangrenosum in healthy infants may result from a modification of bowel microflora after antibiotic therapy in conjunction with maceration in the diaper area. However, careful evaluation and long-term follow-up must be done to detect neutropenia, functional abnormalities of granulocytes, or a possible immune deficiency.
Child, Preschool↗
Production of epidermal sheets in a serum free culture system: a further appraisal of the role of extracellular calcium.
Rhenwald and Green's technique is currently the standard method for growing stratifying epidermal cell cultures. The serum free system developed in Ham's laboratory (MCDB 153) was designed to grow keratinocyte monolayers in clonogenic conditions. Our aim was to optimize conditions in serum-free MCDB 153 for culturing epidermal sheets from adult normal skin, and to assess the effect of extracellular calcium and temperature on proliferation and differentiation of cultured keratinocytes. Sixteen strains derived from plastic surgery specimens (mean age of donors 37 years; range 5-89) were used. Primary cultures were seeded at an optimal density of 8 x 10(4) cells/cm2 in primary cultures and 10(4) cells/cm2 in secondary cultures in complete medium including EGF, insulin, hydrocortisone and bovine pituitary extract, supplemented with isoleucine, tyrosine, methionine, phenylalanine, tryptophane and histidine. Amino acid (AA) supplementation allows a 5.8-fold increase in cell counts at confluency and monolayers with densely packed cells are obtained. In AA supplemented cultures, confluency is obtained in 16 +/- 3 days in primary cultures and in 13 +/- 0.5 days at first passages. Switches to 1.1 mM calcium at first or second passages resulted in a significant increase in cell counts (P less than 0.001), when compared with AA supplemented low calcium cultures. Low temperature/low calcium cultures resulted in a 50% decrease in cell counts. Low temperature/high calcium cultures gave similar cell counts as the 37 degrees C controls. AA and calcium supplemented cultures were evaluated for differentiation markers: involucrin expression was increased, keratins 5, 6, 14, 17 were expressed, and the sheets were 6-10 layers thick by electron microscopy, with keratohyalin granules and cornified envelopes appearing at layers 3-6 (from basal layer). Dispase treatment allowed an easy detachment of these sheets. These results show that the culture medium MCDB 153 can be adapted without serum supplementation to batch culture of human adult keratinocytes to produce epidermal sheets suitable for grafting. They also indicate that extracellular calcium in physiological range of concentration is not a sufficient signal for growth arrest when other growth conditions are optimized.
Adolescent↗
Treatment of leg ulcers with allogeneic cultured keratinocyte-collagen dressings.
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Biocompatible Materials↗
Shwachman syndrome: a case report.
Cutaneous involvement is frequent in Shwachman syndrome, and includes various degrees of dry skin, and eczematous and ichthyosiform lesions. A 12-year-old boy with Shwachman syndrome had cutaneous involvement characterized by dry skin, perioral dermatitis, and follicular keratosis. Polymorphonuclear motility was decreased. A nutrition work-up showed a decrease in liposoluble vitamins, and suggested a causative link with the skin lesions.
Bone Marrow Diseases↗
Inflammatory familial palmoplantar keratoderma: Greither's disease?
A case of a familial palmoplantar inflammatory keratoderma with autosomal dominant inheritance is reported. Associated clinical features included vasomotor troubles and hyperhidrosis consistent with a diagnosis of Greither's disease. Light microscopy was nonspecific. Electron microscopy showed aggregated tonofilaments around the nucleus, without true clumps. Desmosomes were numerous and cell-cell junctions showed an imbricated pattern, well demonstrated in the stratum corneum. The diagnosis of Greither's keratoderma is discussed.
Adult↗
[Cutaneous tumors and nodules in the neonatal period].
A review of neonatal cutaneous tumors and nodules is presented, based on a 10-year experience. Pitfalls in diagnosis are especially emphasized. A biopsy should be performed in most instances when there is a clinical doubt.
Dermoid Cyst↗
[Gangrenous ecthyma of the diaper area in infants].
Ecthyma gangrenosum due to Pseudomonas aeruginosa is a skin infection in which necrotic ulcerations surrounded by a red areola develop. The diaper area is the region most often involved in infants. Typically, ecthyma gangrenosum occurs in patients with septicemia and risk factors (chemotherapy, neutropenia). However, transient bacteremia or an infection confined to the skin may be the cause in some patients, with maceration in the diaper area and previous antibiotic therapy as risk factors.
Diagnosis, Differential↗
[Epidemiology of endemic non-venereal treponematoses].
The occurring resurgence of endemic treponematoses warrant the renewal of WHO mass campaigns. These various infections, pinta, yaws, bejel and endemic syphilis, are due to a treponema apparently identical to the venereal syphilis one. These different treponematoses do act upon epidemiology of venereal syphilis. The recent outbreak of treponematoses justify the current researches in the development of a treponemal vaccine.
Bacterial Vaccines↗
[Periungual capillaroscopy patterns in normal children].
In children as well as in adults, capillaroscopy is an unsophisticated and non invasive technique which allows to investigate vascular acrosyndromes and systemic diseases. We have studied nailfold capillaroscopy patterns in 80 children without over vascular or systemic disease: pericapillary halos and haemorrhages increased with age and capillaries matured towards the typical hair pin structure seen in adults. The number of minor dystrophies increased with age and the venous subpapillary plexuses became less easily visible.
Adolescent↗
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↗
Lichen striatus: a Blaschko linear acquired inflammatory skin eruption.
An illustrative case report and a series of 18 well-documented cases of lichen striatus are presented. The mean age at diagnosis was 3 years (6 months to 14 years; median 2 years). The lesions were predominantly distributed on the trunk in 33% of cases and on the limbs in the remaining two thirds (upper limb: 48%; lower limb: 19%). Pruritus was noted in only 1 of 18 cases. Six cases were associated with clinical features of atopy and/or minor signs of atopic dermatitis (e.g., pityriasis alba). Two cases were considered to be clinically associated with lesions consistent with psoriasis. The mean duration was 9.5 months (4 weeks to 3 years; median 6 months). In one patient, two relapses occurred in 4 years. Hypochromic sequelae were noted in 50% of cases. Lichen striatus is the most common acquired self-limited linear eruption in childhood that follows Blaschko's lines. A new acronym is proposed to emphasize the developmental background of the disease: BLAISE for Blaschko linear acquired inflammatory skin eruption.
Adolescent↗
Linear IgA dermatosis of childhood: case report with an immunoelectron microscopic study.
A 7-year-old girl developed a cutaneous bullous eruption with genital and nasal mucous membrane involvement. Direct immunofluorescence revealed linear deposits of IgA and IgM at the basement membrane zone. No circulating antibasement membrane zone antibodies were detected. Small bowel biopsies showed a partial villous atrophy. The clinical, histologic, and immunopathologic findings were consistent with a diagnosis of linear IgA dermatosis of childhood. Immunoelectron microscopy revealed IgA deposits in the lamina lucida in association with hemidesmosomes, confirming results of two recent studies.
Basement Membrane↗
Porphyria cutanea tarda associated with human immunodeficiency virus infection. A study of four cases and review of the literature.
We report the cases of 4 male subjects, 29, 32, 41 and 44 years old, presenting isolated seropositivities for the human immunodeficiency virus (HIV), or full-blown acquired immunodeficiency syndrome, associated with a typical porphyria cutanea tarda (PCT). The 4 patients are in the usual risk groups for HIV infection. Viral hepatitis was observed in 3 of the 4 cases. Over the past 3 years, 15 cases associating HIV infection and PCT have been reported; almost all had the usual risk factors for HIV infection and hepatopathy. We speculate that HIV infection may have favored the occurrence of early PCT in these cases by altering the metabolism of the porphyrins, either directly or by means of the associated hepatopathy.
Adult↗
Proteus syndrome in 7 patients: clinical and genetic considerations.
The Proteus syndrome is a congenital hamartomatous disorder delineated in 1983. Because of its polymorphic appearance, the syndrome was named after the greek god Proteus whose name means much less than the polymorphous much greater than. Major clinical findings include hemi hypertrophy, macrodactyly, exostoses, scoliosis, epidermal nevi, haemangiomas, deeply rugated soles of the feet and a variety of deep and subcutaneous masses. We report on 7 new cases of Proteus syndrome. All reported cases have been sporadic. Therefore this syndrome could be due to the action of a dominant lethal gene surviving by mosaicism.
Adult↗
[Diverse aspects of congenital syphilis].
Together with the new rise of recent syphilis in the adult woman, congenital syphilis reappears sporadically in our countries, where prophylactic measures (prenatal serology) are sometimes defeated. It remains much more frequent in developing countries. One should know how to interpret a positive serology in a newborn, as it may only reflect passive transplacental transfer of maternal antibodies. One should on the other hand think of syphilis again when the clinical signs are more or less suggestive, the typical picture not being, by far, the most frequent. In spite of the efficacy of Penicillin G: 50,000 U/kg/day during 10 days, neonatal death rate for congenital syphilis is in the neighbourhood of 10%. Our major effort should therefore be aimed at prevention.
Female↗
[Linear porokeratosis of Mibelli in monozygotic twin girls].
Two monozygotic female twins with linear porokeratosis of Mibelli are described. One had only minimal lesions of the right elbow. The other had a linear lesion of the right arm following Blaschko's lines. Monozygotism was established using DNA fingerprinting. A single-gene defect of variable expressivity involving the clonal development of epidermal cutaneous cells according to the lines of cutaneous embryogenesis is suspected. Dithranol and carbon dioxide laser treatment are discussed.
Anthralin↗