Biomedical subjects
M Larrègue
Publications and source records attributed to M Larrègue.
[Systemic scleroderma in children. Apropos of 5 cases. A review of the literature].
Systemic scleroderma is a rare disease in childhood. 62 cases are analyzed. Cutaneous manifestations are identical to those seen in adults. However Raynaud phenomenon is much more frequently missing but follow-up of patients is only of 4 years' duration. We want to draw attention on possible worsening of clinical signs during intercurrent infectious episodes. We report exceptional roentgenological bone anomalies. Gastro-intestinal tract is frequently involved, particularly the oesophagus. We want to draw attention on latent small intestine involvement. A normal thoracic X-ray examination cannot rule out involvement of the lungs; systematic respiratory functional tests are absolutely necessary. All parts of the cardiac wall can be involved: we underline the particular seriousness of this involvement as it was exclusively responsible of death in 10 cases out of 21. Renal involvement is rare. We are reporting 2 cases where a staturo-ponderal retardation remains unexplained; 7 other cases in the literature report on isolated weight retardation. Biological anomalies are similar to those reported in adult. Treatment is not well-codified; we can hope that a better understanding of the disease's physiopathology will lead to the discovery of an efficient therapy.
[Acute pityriasis rubra pilaris in the child. Apropos of 4 cases].
Four cases of Pityriasis Rubra Pilaris of an acute type have been studied in three to six year old children. In three cases the disease occurred after an infection; in one out of these three cases this infection was serologically confirmed as being german measles. In the beginning, only the face is affected or the face and other part of the body. The rash looks like scarlet fever and the folicular papules appear only during the second stage. The dermatosis usually spreads within a month (from twenty days to two months) and covers the whole body without becoming erythrodermic. Then clinical picture is typical of Pityriasis Rubra Pilaris. There are no associated symptoms and no hypo-vitaminemia A is evidenced. The evolution is self limited with a spontaneous regression within three months, and there is no recurrence. These cases are similar to the classical juvenile Pityriasis Rubra Pilaris type III of Griffiths, but they keep their own particularities as far as acute evolution and good prognosis are concerned.
[Omenn's reticuloendotheliosis].
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[Congenital poikiloderma (Rothmund-Thomson's syndrome)].
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[Stevens-Johnson syndrome and pulmonary infection by Mycoplasma pneumonia (author's transl)].
The case-report of an eleven-year-old boy presenting with the association of Stevens-Johnson syndrome and pulmonary symptoms is studied. The course of the disease covered two distinct periods since a one-month interval occurred between the two cutaneo-mucous and pulmonary episodes. Mycoplasma Pneumoniae infection was confirmed by positive seroconversion. The child recovered without sequelae. Indirect immunofluorescence transitorily showed anticytoplasmic antibodies directed against basal epidermic cells. These antibodies were not fixed on the lesions as shown by direct immunofluorescence. The possible connexion between Stevens-Johnson syndrome, Mycoplasma Pneumoniae infection and anticytoplasmic antibodies is under discussion.
[Diffuse poikiloderma with acrokeratosis, preceded by bullous lesions: Weary-Kindler's disease. Nosologic and histopathologic discussion].
The authors report the case of a six and a half year old girl, with extensive poikilodermatous condition and acrokeratosis, preceded by early blistering and light sensitivity. The sequence of cutaneous symptoms suggests the diagnosis of hereditary and bullous acrokeratotic poikiloderma of Weary and Kindler; forty-two cases have already been reported in the literature and our patient would better fit into Kindler's type. The original findings under electron microscopy and direct immunofluorescence (colloid bodies, IgM deposition) that are reported herein are discussed from a pathogenic point of view.
[Dermatologic semiology in childhood].
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[Bullous and hereditary Weary-Kindler's acrokeratotic poikiloderma (author's transl)].
Two observations of hereditary and bullous acrokeratotic poikiloderma of Weary and Kindler are studied. They are transmitted in dominance on two generations. The neonatal presentation of the first observation makes us discuss an epidermolysis bullosa; but the spontaneous regression, the appearance of a reticular pigmentation and the analysis of the paternal dyschromia allow the correction of the diagnosis. Forty-one observations are actually known. The blisters are present in 95 p. 100 of the cases, the dyschromia in 90 p. 100 and the keratosis on 60 p. 100. The trisymptomatic and bisymptomatic forms prevail. The mechanism of the disease is unrecognized but the vacuolization of the basal epidermic cells seems to be at the origin of the bullous eruption.
[Porokeratosis of Mibelli in two monozygotic twins (author's transl)].
Two monozygots twins had porokeratosis Mibelli and a deep mental retardation. The exploration of this nervous components is negative. The porokeratosis in one twin had in several sites the aspect of a skin's horn. The demonstration of the monozygotis was made by the same repartition of 16 characters (HLA, blood groups). The boys had the same aspect and the same skin disease. The twin method is discussed. The existence of the porokeratosis of Mibelli in two monozygotic twins is an argument for the genetic mechanism of the disease. The familial cases of porokeratose Mibelli reported by Bataillard, Civatte, Vigne, Bloom, have consistent features for an dominant autosomic transmission with variations in the penetrance and the expressivity of the skin disease.
[Silvery hair in children: a symptom of leucogranulocytic and melanocytic diseases (author's transl)].
Nine children with silvery hair have been dermatologically studied: eight out nine presented a context of abnormal sensitivity to infections. The study of pigmentation, granulocytes functions and immunity system allows a separation in several types: 1) 4 patients had a characteristic Chediak-Higashi syndrome, this diagnosis being based on pigmentary and immunohematological grounds. 2) Two siblings exhibited functional disturbance of the Chediak-Higashi type, but without its cytological abnormalities. A third patient resembled closely to this picture, but was insufficiently investigated. 3) The eighth patient has been studied by Drs. Griscelli and Pruniéras who had previously described a new disease on immunological, hematological and pigmentary grounds. All these case with defective polymorphonuclear functions have febrile peaks with accelerated aggravation close to Chediak-Higashi syndrome. 4) The ninth patient with silvery hair does not have any immunological abnormalities, nor any accelerated phase at the age of eight but suffers from diffuse epilepsy, cerebellar and chorioretinal atrophy.
[Stevens-Johnson syndrome and pulmonary infection by Mycoplasma pneumoniae (author's transl)].
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[Native favus in Poitou. About 3 cases (author's transl)].
Three cases of tinea favus of pityriasic aspect and without a "scutulum" sign are diagnosed in a small town of "Poitou" region. They correspond to an autochtonal focus. The various enquiries show that favus has become rare in France but the local focus as are still active, since 43 p. 100 of recorded favus are of local origin.
[Dermatological manifestations of synovial cysts of the lower limbs (author's transl)].
Two cases of synovial cysts of the knees are reported. Thier emergeing point located far away from the joint they originate from can make the diagnosis be difficult. Puncture allows to remove synovial fluid and to inject a radio-active iodine compound to realise a cystography. The radiography of the joint demonstrates an already existing joint disease. The atypical aspects of synovial cysts are emphasized on the basis of then two cases such as pseudo-phlebitic, pseudo-arteritic, inflammatory and fistulised. The presentation of synovial cysts of the legs is frequently dermatological. The diagnosis must be suspected in order to avoid a dermatological biopsy.
[Profuse infiltrating erythema marginatum in a 2-year-old girl].
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[Pemphigus foliaceus in a child. A two years follow-up under steroid. An immunosuppression therapy (author's transl)].
The observation of pemphigus foliaceus out of endemic zone in a six-year-old child is reported. The rate of anti-ICS antibodies is very high (1/6,400). The direct immunofluorescence shows the common aspect of epidermic network between the cells. Fluorescence basement membrane has been found with monospecific anti-C3 sera, and after remission with the monospecific anti-IgG sera. The clinical, histological and immunological relationship between pemphigus foliaceus in its sporadic and endemic forms and pemphigus erythematosus are discussed. The clinical and immunological regression is obtained by association of prednisone and immunodepressive therapy. After two years of treatment the maintenance dosis is not yet reached. A review of ten previous reported cases of pemphigus foliaceus in children in its sporadic form is presented.
[Acute haemorrhagic edema of the skin of the new-born with lethal intestinal complication (author's transl)].
A child, eleven months old, presented with an A. H. O. after an otitis. The dermatologic aspect is typical with oedema and target-like hemorrhagies. The disease evolved in three successive episodes. During the second one two intestinal intussuceptions occurred. It was necessary to proceed to an intestinal resection of twenty centimeters of the small bowel and of the right part of the large bowel. The post-operative period has been complicated by an intravascular coagulation, perforation of small bowel and peritonis due to Proteus bacillus. The child died during these complications. This evolution looks like an anaphylactoid purpura of Schönlein-Henoch. But this does not allow to affirm that these two allergic vasculitis are related to the same etiology. Our own observation suggests the necessity of watching the coagulation rate when an acute haemorrhagic oedema is complicated by bowel's intussusception.
[Changes in the keratinocytes in oculo-cutaneous tyrosinosis: Richner-Hanhart syndrome (author's transl)].
The Richner-Hanhart syndrome corresponds to a tyrosine elevation in serum due to a defect in soluble tyrosine amino-transferase in liver cells. This new enzymopathy which is transmitted in an autosomal recessive mode is called oculo-cutaneous tyrosinosis. It is curable by a low diet in tyrosine and its precursors. The diagnosis has been clinically suggested in an 18 months old girl, by the association of punctate palmar and plantar keratosis, dendritic ulcerated keratitis, and mental retardation. The diagnosis was established by elevation of tyrosinemia up to 52 mg/100 ml associated with a high urinary elimination of tyrosine and phenylcetonic acid. Absence of anomaly in the metabolism of methionin and hepatorenal lesion is characteristic. The diagnosis was confirmed by the absence of soluble tyrosine aminotransferase in liver cells and by the effectiveness of the diet. The clinical keratosis corresponds histologically to a orthokeratotic hyperkeratosis. The keratinocytes show 2 types of anomalies ranged in the epiderm. Intracytoplasmic vacuoles which include or lead to pseudomyelinic formations extend progressively from the mitochondrial alterations in the epidemial basal layers. Bulky polyhedral electron dense particles are found in the cytoplasm of the superficial keratinocytes. Most of these images have been demonstrates anteriorly in the keratinocytes ant the corned; on the other hand, signs of mitochondrial anomaly had not been observed. The genesis of these cellular alterations based on the liberation of lysosomial enzymes by the action of crystals of tyrosine has been suggested by Goldsmith from experimental facts. However, it seems that the mitochondrial defect occurs outside this mechanism.