Biomedical subjects
M Larrègue
Publications and source records attributed to M Larrègue.
[Tetanus and leg ulcer (author's transl)].
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[Gluteal dermatitis in infants].
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[Pachydermatoglyphy. A sign of malignancy for acanthosis nigricans (author's transl)].
The pachydermatoglyphy corresponds from the clinical point of view to a diffuse palmo-plantar keratodermia, of a yellowish color, characterised by a rough thickening of the finger prints. The presence of the pachydermatoglyphy has been pointed out in six cases of malignant acanthosis nigricans. This was already found in previous descriptions. Pachydermatoglyphy is specially found in malignant acanthosis nigricans, but we have also observed such cases in erythrokeratodermia with pilar keratosis and deafness.
[The mycosis fungoides group of diseases].
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[Richner-Hanhart's syndrome or oculo-cutaneous tyrosinosis (about one case) (author's transl)].
Richner-Hanhart's syndrome correspond to an hypertyrosinemia due to a deficiency of a soluble tyrosine amino-transferase. This recently described tyrosinosis has been called oculo-cutaneous tyrosinosis. This disease transmitted on a recessive way in amenable to a treatment by a low tyrosine diet. In an infant, 18 months old, presenting a bilateral dendritic keratitis, a punctiform keratosis of the extremities, a patchy leucokeratosis of the tongue and a mental ketardation, the hypertyrosinemia reached 52 mg per 100 ml and the urine demonstrated the presence of phenyl-atonic acids. There was no hepato-renal involvement. The deferency of soluble amino-transferase was studied on the hepatocytes and confirmed. The low tyrosine diet made the clinical and biological signs disappear. The improvement was noticeable from the first week on and continued during the 16 months of the follow-up. There was no ill effect of the special diet on the weight and height growth. The oculo-cutaneous tyrosinosis is similar to the experimental form obtained by Schweizer on the rat. The occurrence of intracellular tyrosine crystals probably damages lysosine membrane and the release lysomie proteases induce the cellular lesions.
[Association of ectodermal dysplasia, cleft of the lip palate and "scrubbing-brush hair". Its situation in "D. E. F. syndromes" (ectodermal dysplasia, cleft of the lip and/or palate (author's transl)].
The "D. E. F.-syndromes" consist of ectodermal dysplasia, cleft of the lip and/or palate (fente labiale et/ou palatine). This group includes the A. E. C.- and the E. E. C.-syndromes. We are reporting two cases of D. E. F.-syndrome, in which there was a very particular hair dysplasia, which we named "scrubbing-brush hair". The first case was a boy. The disease was probably transmitted on the dominant autosomal mode. The ectodermal dysplasia was of hypohidrotic type. The second case was also observed in a boy. There was no similar genetic abnormality in the family. The ectodermal dysplasia was of hidrotic types. The embryological findings account for the association between the ectodermal dysplasia and the medial dysraphia of the face.
[Systematized papulosis of the lower scapular areas with ultrastructural anhist bodies: specific cutaneous symptoms of Hunter's disease (author's transl)].
Papulosis of the scapular areas is a characteristic dermatologic sign of Hunter's disease. It has not been described in the other mucopolysaccharidoses. This sign had been detailed by Hunter in 1917 in his princeps observation. We noted it in two cases of the disease. The ultrastructural study showed an accumulation in the connective interstitial substance of anhist bodies of intra-cellular origin.
[Acrodermatitis enteropathica. Anatomo-clinical study of 2 familial cases treated with zinc sulfate].
Two familial cases of acrodermatitis enteropathica began at the time of weaning, with characteristic dermatologic lesions and digestive signs, resulting in lack of weight gain: at 13 months of age growth future was evident in the elder child. The anatomic lesions were those of an exophagitis and, in the duodenal mucosa, a dilation of the capillaries of the chorion in the absence of villous atrophy. The ultrastructural study showed inclusions in Paneth's cells, and changes in keratin cells, with intra-cytoplasmic vacuoles and abnormal melanin pigment in the skin. The administration of zinc sulphate induced a rapid recovery of cutaneous lesions and resumption of growth. The interruption of treatment resulted twice in a quite immediate relapse of cutaneous signs in the first patient, which was rapidly reversible with the reintroduction of zinc sulphate.
[Contact dermatitis caused by dandelion, laurel oil and frullania by cross-allergy].
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[Congenital dermatoses in the newborn infant].
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[Toxic staphylococcal cutaneous necrolysis (scalded skin syndrome) (author's transl)].
The exfoliating exotoxin of serotype II staphylococci is responsable of three diseases with prevailing dermatologic symptoms: staphylococcal scarlet fever, staphylococcal Lyell syndrom and Ritter-Lyell disease. Melish demonstrated the effect of staphylococcal exotoxin on new-born mice less than five days old. This experimental model allowed to study the characteristics of the toxin. Precocious antistaphylococcal antibiotherapy is necessary.
[Centrifugal annular erythema of Colcott Fox type (erythema gyratum perstans)].
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[Benign rheumatoid nodules. Report of one case].
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[Dermo-neuro-hypsarrhythmia. Association of a congenital dermatosis and hypsarthymia. 9 cases (author's transl)].
These 9 cases confirm that tuberose sclerosis, von Recklinghausen's neurofibromatosis, incontinentia pigmenti, linear warty sebaceous naevus and alopecic naevus resulting in a woolly naevus of the scalp may be complicated by flexion spasms with hypsarhythmia. Early diagnosis of hypsarhythmia makes possible specific treatment with ACTH or hydrocortisone.
[Esophageal localization of Darier's disease. Radiological description. Apropos of 4 cases].
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[A rare association of hamartomas of the kidney, renal insufficiency, Bourneville disease and arterial hypertension (author's transl)].
On the basis of a clinical case, the authors review the principal signs of Bourneville tuberose sclerosis and show the renal manifestations which may be encountered. The most common is the presence in both kidneys of tumours which are hamartomas. In addition, this patient suffered from hypertension and renal insufficiency. Their frequency and pathogenesis are discussed in the context of this disease.
[Staphylococcal Lyell's syndrome. Apropos of 4 cases].
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