[Azatropin in severe atopic dermatitis: 24 cases].
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Biomedical subjects
Publications and source records attributed to M Larregue.
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BACKGROUND: Mammary Paget's disease unfrequently occurs in males, and may be pigmented in rare instances. Differential diagnosis with malignant melanoma relies on immunohistochemical studies. CASE REPORT: A case of Paget's disease of the nipple in a 76 year-old male is reported, clinically mimicking a malignant melanoma because of massive pigmentation. Histologically, large Paget's clear cells were intermingled with numerous melanin-rich dendritic melanocytes. An underlying ductal carcinoma was found. After differential immunohistochemical staining, diagnosis of Paget's disease could be unequivocally substantiated since Paget's cells stained for epithelial markers, c-erbB-2 and hormonal receptors, whereas protein S100 and HMB45 were negative. DISCUSSION: Pigmentation in mammary Paget's disease occurs preferentially in males. Pigmentation results from numerous melanocytes with abundant melanin in close contact with Paget's cells. An increased number of melanocytes may also be observed in cutaneous metastatic breast carcinomas. It could result from a chemotactic factor produced by neoplastic cells.
BACKGROUND: Localized cutaneous leishmaniasis acquired in France is rarely reported. Diagnosis usually relies on detection of leishmania on smears or by culture. CASE REPORT: A 9-year-old child living outside endemic French Mediterranean areas had long-lasting crusted papules on the face for several months. Although the lesions were suggestive of cutaneous leishmaniasis, smears and culture were negative for Leishmania. Skin biopsy showed epithelioid and giant cell granuloma, but Leishman bodies were absent. Western Blot analysis of the patient's serum revealed antibodies directed against Leishmania infantum antigens, thus confirming the diagnosis of cutaneous leishmaniasis. Intralesional injections of meglumine antimoniate yielded complete regression of lesions. DISCUSSION: Localized cutaneous leishmaniasis in France is caused by Leishmania infantum and may be diagnosed outside endemic Mediterranean areas, following transmission from a sandfly bite during summer holidays in Southern France. Serum analysis by Western Blot assay distinguishes between clinically active and asymptomatic Leishmania infections, the latter being common in endemic areas. Western Blot analysis is useful for the diagnosis of cutaneous leishmaniasis when parasites cannot be detected by direct techniques.
Three patients with malignant blue nevus are reported-one on the right side of the body, one on the right arm, and one on the face. The criteria and difficulty of histopathological diagnosis are considered as well as the differential diagnoses for this tumor. The therapy is described, and the possible relations between malignant blue nevus and certain other tumors (e.g., atypical or locally aggressive cellular blue nevus) are explored. A review of the literature reveals that there is current disagreement about the exact prognosis for these tumors and indicates the need to collect data for all patients observed.
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.
Usually, recorded cases of allergic contact dermatitis to tobacco are confined to occupational diseases and involve agricultural workers and those engaged in manufacturing or selling the products, all of whom are in contact with tobacco leaves. We have found three cases of contact dermatitis caused by cigarette smoke, which are not occupational disease. We do not know what offending agent is, but in one case the patient is allergic to perfume and this may be the factor responsible, since she smokes only flavoured cigarettes. In addition, we need to know whether the allergen only appears during combustion and is therefore present only in cigarette smoke.
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The skin symptoms of trypanosomiasis in children are reviewed following report of one case of African trypanosomiasis (T. Gambiense). Trypanids, existing in 10-20% of patients during blood and lymphatic dissemination, appear are transient centrifugal annular erythemas made of few elements located on the trunk and roots of arms and thighs. Centrifugal annular erythema in a context of severe infections disease in a exposed subject coming from and endemic zone constitutes a characteristic diagnostic element.
After successful ascorbate and manganese treatment of a female patient with prolidase deficiency and iminodipeptiduria, we attempted to explain the mechanism of action of these drugs in vitro, using them preferentially on skin fibroblasts. Since in vivo, ascorbate and manganese seemed to be responsible for both biochemical and clinical improvement, they were also expected to activate prolidase activity in vitro. Cell growth and prolidase activity were accordingly observed in fibroblast cultures supplemented with these compounds. It seemed that only ascorbate accounted for the successful in vivo response. To understand the mechanism involved, we studied collagen metabolism and found a decreased proline pool, a massive increase of rapidly degraded collagen and moderate enhancement of type III collagen and type I trimer in the patient's fibroblasts. We believe that ascorbate allowed the prolidase-deficient cells to maintain a normal collagen pool by increasing collagen synthesis. Both the massive increase in cell growth in response to ascorbate and the bad response as regards the quality of the collagen produced confirm the secondary nature of this mechanism. However, the relationship between accelerated collagen catabolism and prolidase deficiency remains unclear.
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A retrospective study was undertaken in 47 children with congenital capillary naevus of the face in order to appreciate the predictive value of the topography of the facial angioma for the diagnosis of associated pial angioma. In patients with Sturge-Weber disease, facial angioma concerned the whole upper eyelid, extending to the forehead and/or cheek. Less often, it was localized to the root of the nose; in such cases it was a port-wine, homogeneous angioma, with clearcut outlines, quite different from the angiomatous dots frequently seen in this area in neonates. Except for these rare cases, angiomas concerned only half or less of the upper eyelid and no pial angioma was associated. Finally, congenital capillary naevi with this topography typical of Sturge-Weber disease were frequently associated with pial angioma.
A 33-year-old female patient with chronic recurrent leg ulcerations was shown to present a massive iminodipeptiduria which seemed to be attributable to disturbance of collagen metabolism. Biochemical investigations confirmed an hereditary prolidase deficiency. A treatment was tried for the first time and showed a good biochemical result and a clinical improvement.
An 8-year old girl suffering from chronic relapsing staphylococcal skin infection since the first days of life was found to have extremely high (up to 26,500 units/ml) serum IgE levels and impaired polymorphonuclear chemotaxis. The other immunological tests appeared to be normal. There seemed to be a correlation between impaired chemotaxis and rise in IgE, the latter perhaps being related to a deficiency in some thymus-dependent lymphocyte functions.
The authors describe the surgical procedure employed in a case of bilateral cicatricial ectropion (superior and inferior), occurring during the course of a lamellar icthyosis. Total skin homografts should be applied before the appearance of severe corneal lésions on both eyelids, inferior and then superior. Two factors have to be emphasized: the apparent appearance of rejection of the grafts when the first dressings are made, and the progressive retraction of the homografts which requires repeated graft applications.
The Richner-Hanhart syndrome corresponds to a tyrosine elevation in serum due to deficit in soluble tyrosine aminotransferase in liver cells. This new enzymopathy which is transmitted in an autosomal recessive mode is called oculocutaneous tyrosinosis. It is curable by a poor diet in tyrosine and its precursors. The diagnosis has been invoked in a 18 months old girl, on the association of punctuate palmar and plantar keratosis, dentritic ulcerated keratitis, and mental retardation. The diagnosis is confirmed by elevation of tyrosinemia to 52 mgs/100 mls associated with a high urinary elimination of tyrosine and plenylcetonic acid. Absences of anomaly in the metabolism of methionin and hepatorenal absence of disturbance of hepatorenal system is characteristic. The keratosis accompany orthokeratotic hyperkeratosis. The keratinocytes show 2 types of anomaly ranged in strates 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 aspects have been demonstrated anteriorly in the keratinocytes and the cornea; on the other hand, signs of mitochondrial sulferance 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 the mitochondrial defect occurs outside this mechanism.
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A case of Richner-Hanhart syndrom with tyrosinemia is being reported. The diagnosis was suggested from clinical manifestations of this syndrom : superficial opacities of the cornea in an infant preceeding hyperkeratosis of the palms and soles and mild mental retardation. It has been confirmed by the high levels of blood tyrosine. The introduction of a diet low in tyrosine and alanine has lead to a rapid improvement and finally a complete cure of the ophthalmological and dermatological symptoms. The normal metabolism of tyrosine is recalled as well as the specific metabolic aberration responsable for this syndrom (deficiency of cytosol tyrosine amino-transferase). This case is being compared with those which have been previously reported.
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