Biomedical subjects
M Larrègue
Publications and source records attributed to M Larrègue.
[Clinical markers and screening for colorectal neoplasms].
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[Neonatal Hutchinson-Gilford progeria with cutaneous sclerodermiform involvement].
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[Epidemic erythema infectiosum or fifth disease].
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[Collodion baby: 32 new case reports].
A critical study of 69 cases of collodion baby observed between 1976 and 1984 is compared with the study conducted in 1976. These 69 cases are divided into 32 examined by the authors personally and 37 published in the literature. The total number of collodion babies is 267. A collodion baby is a high risk newborn who must be nursed in a specialized intensive care unit during the neonatal period. The vital prognosis has been improved by the prevention of metabolic disorders (dehydration with hypernatraemia) and of percutaneous intoxication with topical products, and by the early treatment of infections. The mortality rate, which was 33 p. 100 in 1976, had fallen to 11 p. 100 in 1984. Skin infection with systemic spread remains the major complication (the present situation is summarized in table II). The collodion baby is the neonatal expression of different disorders of keratinization and as such, constitutes a syndrome. Several conclusions can be drawn from a comparison of the keratinization disorders observed in our two series (table VI). Congenital ichthyosiform erythroderma and lamellar ichthyosis are responsible for 60 p. 100 of collodion babies. These two lesions may be the first signs of an ichthyosis transmitted as an autosomal dominant trait in 9.7 p. 100 of the cases. Trichothiodystrophy may be heralded by a collodion baby syndrome. In 9.7 p. 100 of the cases this syndrome disappears without sequelae. Sex-linked ichthyosis never begins with a collodion baby syndrome.
[Erythema annulare centrifugum revealing linear IgA dermatitis of childhood].
Linear IgA dermatitis was diagnosed in a 13-year old girl with erythema annulare centrifugum (EAC) on the basis of the criteria laid down by Jablonska: vesiculo-bullous eruption with specific patterns on subsequent flare-ups, subepidermal vesicles and bullae with papillary eosinophilic abscesses in erythematous areas, positive linear IgA antibody response at direct immunofluorescence in the lamina basal, absence of intolerance to gluten and responsiveness to sulfapyridine and dapsone. This patient was followed up for 10 years. During the first 5 years any attempt at withdrawing dapsone resulted in quick relapse which always remained responsive to that drug. After 5 years discontinuing dapsone was no longer followed by relapse, and the girl was considered clinically cured. Yet direct immunofluorescence in healthy skin remained positive for 2 years after treatment was stopped, as has previously been reported. At the age of 23, after 5 years without treatment the patient remained cured. This case demonstrates that linear IgA dermatitis is one of the causes of EAC. Autoimmune bullous diseases, such as pemphigus with eosinophilic spongiosis, bullous pemphigoid and dermatitis herpetiformis, are known to present as EAC. Direct cutaneous immunofluorescence is necessary to the aetiological diagnosis of EAC.
[Linear scleroderma in children (apropos of 27 cases)].
Twenty-seven cases of linear morphoea are reported and compared with 218 cases collected from the literature. The various parameters studied, including clinical features and laboratory results, were identical in both series. The aetiology of linear morphoea is unknown, even though injuries or fever have been noted as triggering factors in 20 p. 100 of the cases. Linear morphoea is a childhood disease: it begins at the age of 7 or 8 and predominates in females (63 p. 100 of the cases). Its onset is marked by the abrupt occurrence of sclerosis in most cases, although a solitary morphoea or a trophic plaque may precede the disease proper. In our series, muscular or articular involvement appeared from the start in 40 p. 100 of the patients at the same time as the initial cutaneous lesions. The active stage lasts 3 years and sometimes longer. It is characterized by extension (37 p. 100) or both extension and multiplication (63 p. 100) of the initial lesions. Regional complications aggravate linear morphoea and are more frequent in patients whose lesions extend and multiply. Their incidence was lower among the published cases than in our series. Depending on the author, retractile myositis is present in 37 to 59 p. 100 of the cases, joint stiffness in 18 to 40 p. 100 and shortening of a limb in 10 to 22 p. 100. These complications often regress incompletely, leaving sequelae which persist in the steady state in 75 p. 100 of the patients. Antinuclear antibodies, present in 37 p. 100 of the cases, are either of the homogeneous or of the speckled type. Jablonska has met them more frequently (50 p. 100), and they were often of the speckled type. The significance of these antibodies in linear morphoea is unclear since they appear inconstantly and later than clinical signs. The skin lesions associated with linear morphoea show that the other forms of scleroderma--i.e. plaque morphoea, erythematous atrophic or dyschromic plaques and guttate scleroderma--belong to the same family. The same associations are found in frontoparietal "coup de sabre" scleroderma. The treatment of linear morphoea is not yet standardized. At the moment, the best and most regular results are obtained with systemic corticosteroids and local physiotherapy.
[Cutaneous expansion by inflatable implant].
The capacity of the skin to stretch and expand, as shown in pregnancy, has been used by Radovan who, in 1976, introduced skin expanders made of silicone. These are empty balloons of different sizes and shapes which are surgically placed under the skin and gradually filled with saline. The saline solution is introduced through a valve with a syringe and a 21 G needle. Skin expansion usually requires 6 to 8 weeks to be completed. Transient redness of the skin is frequently observed. A thin capsule can be found around the expander but does not need to be removed. The excess skin can be utilized as advancement flap, rotation flap or even two-stage distant flap. Our experience is based on 33 expansions performed in 26 patients (table I). Giant naevi were the most frequent lesions treated (20 cases), and face, scalp and neck were the most frequent localizations (27/33 cases) (table II). Complications occurred in 21 p. 100 of the cases, and 5 expanders had to be removed. Nevertheless, expansion could be completed in most cases, sometimes after replacement of the prosthesis. The results of repair surgery using expanded skin have always been satisfactory: in colour and texture the expanded skin was similar to that of adjacent areas, and sensation was preserved. Skin expansion in fact made it possible to perform local flaps--which usually represent the best solution for skin replacement--by creating hyperlaxity in areas adjacent to the pathological areas. There has been no retraction of the expanded skin, and the scars have shown no tendency to hypertrophic reaction or distension.(ABSTRACT TRUNCATED AT 250 WORDS)
[Caustic diaper dermatitis and toxic encephalopathy following the application of talc contaminated by hexachlorophene].
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[Radical excision of giant abdominal nevi in infants. Apropos of 3 cases].
Total removal of giant naevi with a minimal residual scar is of course ideal for prevention of malignancy and obvious psychological reasons. One can take advantage of the excess of skin existing in infants to be able to perform such wide excisions with immediate closure. Three cases of abdominal wall excisions in infants are presented, one of the upper abdomen at 9 months, two of the lower abdomen, at 3 months and 6 months. In all cases, a wide undermining was performed to allow the immediate closure. For the two lower abdominal excisions, preservation and transposition of the umbilicus were performed, like on an aesthetic abdominoplasty in adults. The blood loss was minimized by infiltration with epinephrine and meticulous hemostasis. Healing was uneventful. The scars, with a one to two years follow-up, are smooth and fine, and there are no change of contour. Progress in pediatric anesthesiology has resulted in the fact that a large excision and repair in infants presents no more potential dangers than operation performed later. Feasibility of such early wide excisions has been explored in other areas of the body; however, the abdominal wall is the area of choice.
[Vitiligoid achromias and severe atopic dermatitis. Apropos of 4 cases].
Four patients with important and disabling atopic dermatitis persisting during adulthood have presented leucodermia in certain areas of eczema. As melanosomes and melanocytes have totally disappeared in these regions, this depigmentation corresponds thus to an achromia. Clinically, we noted macular achromia with hyperpigmented border of special topography since touching pleat regions initially present eczema lesions: anterior face of wrists and footnecks. Slight lichenification is noted in achromic regions. In spite of many analogies with vitiligo, we differentiate this achromia from vitiligo on the basis of absence of familial post-history and absence of new localisations after 5 to 6 years cause. A review of depigmentations described during atopic dermatitis does not show similar facts. Depigmentation induced by local steroid therapy does not give such clinical and ultrastructural aspects. In 3 patients, there was no local application of depigmenting agent. In one case, 8-oxyquinoleine was applied transiently. Vitiliginous achromias of pleat regions initially presenting important atopic dermatitis are probably due to multifactorial factors: possible factors are inflammation and secondary epidermal modification due to local steroid therapy and may be some excipients. Being a rare situation in atopy, we have registered it only 14 times in 860 followed atopic dermatitis. The study of series of atopic dermatitis followed on a long-term basis should allow to evaluate the frequency of such incidents and the respective role of aetiological factors with we suspect.
[Type II oculo-cutaneous tyrosinosis].
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[A syndrome of hyper-IgE and recurrent infections. Developmental variants? A familial study].
The first patient suffered from a very severe atopic dermatitis with intense pruritus and thickened skin. He had also recurrent infections, particularly related to Staphylococcus coagulase +, and axillary and inguinal lymphodermopathy. The use of tetracosactide given intramuscularly allowed controlling the evolution of his atopic dermatitis. After several months of treatment, the skin became less infiltrated, lymphodermopathy disappeared and no severe infection had happened. The second patient had a less severe atopic dermatitis and recurrent infections without any particular severity. Topical corticosteroids allowed to control the atopic dermatitis. These two patients had high levels of circulating IgE and an important deficiency of polymorphonuclear chemotaxis which was evaluated by migration through boyden room. Study of the family showed atopic manifestations in several members, but with lower levels of IgE. The most characteristic abnormality of this syndrome is the according to considerable increase of IgE. The deficit in polymorphonuclear chemotaxis may vary according to time and even become normal. The prognosis over long periods remains to be determined.
[Kindler syndrome. Case report with ultrastructure study].
A case is reported of a 20 year-old man who had progressive poikiloderma, cutaneous atrophy, plantar keratosis, and, from birth, blister formation regressive in infancy. Ultrastructural study performed on poikilodermic and atrophic lesions showed that dermoepidermal "clefts" or " vacuolizations " observed in optical microscopy did correspond to a cleavage between lamina densa and undamaged basal cells membrane. Sixty cases of congenital poikiloderma with blisters and keratosis have been reviewed in the literature; 13 out of these, are as the present case, similar to the original observation of Th. Kindler ; the common clinical features of these cases (cutaneous atrophy, mucous membrane involvement) and probably autosomic recessive inheritance, allow to consider, according to the authors opinion, Kindler 's syndrome as a peculiar entity.
[Papular tuberculid in an infant following BCG vaccination].
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[Caustic diaper dermatitis and encephalitis secondary to the application of talcum contaminated with hexachlorophene].
Five observations of infants with transcutaneous intoxication by 6,3 p. 100 hexachlorophene contaminated talcum powder are reported. The diaper dermatitis is particular because of its topography (red pants shape), of its sudden occurring, of its papyraceous aspect evoking caustic origin, and of its association with severe encephalopathy. The neurological signs start with epileptic fits leading rapidly to coma. Prognosis is serious leading either to death or to paraplegia. Enquiry on that epidemic shows that mortality raised up to 18 p. 100. The neurological signs with oedematous degeneration of myelin are characteristic of hexachlorophene toxicity. Plasma levels of toxics range up to 15,94 mg/ml i.e. 30 times more than the rates observed by Curley in a premature washed with a commercial solution containing 3 p. 100 hexachlorophene. During a toxic neurological syndrome, the existence of diaper dermatitis with red plants shape must lead to an aetiological diagnosis of the possibility of transcutaneous intoxication even if the product seems as harmless as talcum powder.
[Anodontic anhydrotic ectodermal dysplasia].
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[Lateral nail-pad of the big toe in infants. Apropos of 2 cases].
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