[Cutaneous dyschromia in three cases of phenylketonuria. Quantitative ultrastructural study of the basal layer of the epidermis (author's transl)]. / Dyschromie cutanée dans 3 cas de phénylcétonurie étude quantitative ultrastructurale de la couche basale de l'épiderme.

The authors have studied skin color modifications in 3 cases of phenylketonuria and have observed the characteristic changes; fair skin and fair hair. In addition they noted hundred of pigmented pin point or slightly larger patches in the two more affected patients, in the areas exposed to sunshine. With regard to the ultrastructural study of the epidermis basal layer, the proportion of melanocytes in the two most severe cases was slightly higher than in the normal skin of 6 control subjects. Langerhans cells could not be acertained. The more severe was the disease the greater was the tendency for a lower proportion of keratinocytes containing melanin. There is a certain parallelism between the skin color modifications the biochemical examinations (blood level of phenylalanin and tyrosine) and the ultrastructural changes. The higher the blood level of phenlalanine and/or the more pronounced the disorders of skin color, the more evident would be the ultrastructural changes: higher proportion of melanocytes that usually do not produce the melanosomes, and lower percentage of keratinocytes with melanin. On the other hand, the ultrastructure of the basal layer would suggest the seriousness of clinical manifestations and/or the intesity of the metabolic error.

[Ultrastructural aspects of the epidermis from a sun exposed and from a covered area in two cases of xeroderma pigmentosum (x. p.) and in two controls (author's transl)]. / Etude ultrastructurale de l'épiderme des parties découvertes et couvertes de deux malades noirs atteints de xeroderma pigmentosum (x. p.) et de deux sujets témoins.

Xeroderma pigmentosum is rare among Caucasoids and seems to be rarer among Negroes. Advantage was taken of the observation of two cases in the latters to study the ultrastructure of the epidermis and to verify whether the findings were similar to those observed in the formers. The skin exposed to sunlight (forearm) and that from an covered area (buttock) were studied in the two cases and also in two controls, obviously from the same race. The tissue material was coded after each biopsy and identified only when the ultrastructural study was completed. The authors have noted vacuolization and rarely necrosis of melanocytes, great increase and polymorphism of melanosomes, abnormal melanization sometimes large masses of melanosomes and pre-melanosomes, some other aspects already described in x.p. They have also observed melanosomes disposed as cluster or grapes, and large and strange shaped keratohyaline granules in the squamous cell layer. Even in the unexposed skin there were polymorphism and increased amount of melanosomes. The macroscopic and ultrastructural changes of x. p. in the two Negroes were apparently similar to those observed in Caucasoids.

Reflections on some aspects of leprosy among children in Brazil and in other countries.

The authors discuss the classification, clinical aspects, lepromin reactivity, and epidemiologic features of leprosy among children. The most frequent characteristics of each form of leprosy are described. Lepromatous leprosy is less frequent among children in countries of low endemicity and more frequent, even in the most advanced forms, in hyperendemic regions. Borderline forms are rare. In a large number of cases the initial manifestations are those of the indeterminate form and, in an even larger number of cases, of the tuberculoid pole. The evolution from indeterminate to the tuberculoid pole very frequently occurs in a few months or within less than one year. With respect to epidemiology, the authors consider the general frequency of leprosy among children and the frequency of each form of leprosy among children and adults. Data from surveys carried out in Brazil and other countries are presented. Children have a potential for the rapid development of immunoresistance and consequently of lepromin-positivity, and therefore only a few of them eventually develop lepromatous leprosy. This explains the low indices of lepromatous leprosy (approximately 5%) even in hyperendemic areas. Finally, they discuss the factors that may be responsible for the clinical manifestations of leprosy and their characteristics among children.

Blastogenic response with autologous plasma and Mitsuda's reaction in leprosy patients and their unaffected sibs.

Many investigators have evaluated the influence of genetic constitution on the susceptibility to leprosy in studies linked to different types of research. To determine the possible existence of a family trait linked to the lymphoproliferation and to lepromin reactivity we studied the blastogenic response to phytohaemagglutinin, lepromin and M. leprae and the Mitsuda's reaction in leprosy patients and their unaffected sibs. Sixty-eight individuals were studied, 34 were leprosy patients (17 lepromatous and 17 tuberculoid leprosy) and the remaining were their sibs previously matched by sex and age. The indices of blastogenesis and lepromin reactivity were lower in lepromatous than in tuberculoid patients, that confirmed the immunological polarity of the two types of leprosy. Both the lymphoproliferation and Mitsuda's reaction results suggest different cell immune responses in leprosy patients and their unaffected sibs, so that the hypothesis of a family trait favouring the similarity of responses to these tests among sibs becomes unlikely.

Ultrastructure of lymphocytes from patients with paracoccidioidomycosis in the lymphocyte transformation test by phytohemagglutinin.

The morphology and ultrastructure of peripheral blood lymphocytes from patients with paracoccidioidomycosis (PCM) and from unaffected individuals (controls) were studied before and after Ficoll-Hypaque separation and at the end of culture, stimulated with phytohemagglutinin. Patient lymphocytes were cultured in medium with autologous plasma (from the patient himself) and with homologous plasma (from an unaffected donor), while donor lymphocytes were cultured in medium with plasma from a patient or with plasma from the donor himself. The Ficoll-Hypaque mixture caused no morphological or ultrastructural changes in the lymphocytes of patients or of unaffected donors. Patient lymphocytes cultured in medium with autologous plasma showed different degrees of cytoplasmic and nuclear alterations, such as organelle dissolution, vacuoles, amorphous masses, deformed nuclei, and absence of nucleoli. Lymphocytes from control individuals cultured in patient plasma also showed ultrastructural alterations, though they were less marked, and a reduced number of 'blasts'. Patient lymphocytes cultured in medium with homologous plasma (from a control individual) showed a morphology similar to that of lymphocytes from control individuals cultured in medium with their own plasma, although with a lower number of 'blasts'. On the basis of the results obtained using that methodology, we draw the following conclusions: separation by Ficoll-Hypaque does not seem to alter the ultrastructure of patient or donor lymphocytes; patients with diffuse PCM and more markedly impaired general condition can exhibit lymphocytes with morphological and ultrastructural alterations capable of affecting their biological systems and functionality.(ABSTRACT TRUNCATED AT 250 WORDS)

[Correlation between early (Fernandez) and late (Mitsuda) reactions in non-contact children and adolescents without manifestations of leprosy, tested with human lepromin and different concentrations of armadillo antigen in 3 double-blind trials]. / Corrélation entre les réactions précoces (Fernandez) et tardives (Mitsuda) chez des enfants et adolescents non-contacts et sans manifestations de lèpre, testés avec lépromine humaine et différentes concentrations de l'antigène du tatou en trois essais à double insu.

The authors carried out 3 double blind trials to determine the correlation between the early and the late lepromin reactions and to verify the practical and control values of the 48 hours or Fernandez reaction. Children and adolescents non-contacts were tested with human lepromin (40 x 10(6) bacilli/ml) and different concentrations of armadillo lepromin (160, 40, 20, 10, 5, 2,5, and 1 x 10(6) bacilli/ml). The conclusions are as follows: 1. The coefficients of correlation (r values) point out that the correlation between the two reactions is poor or almost null. 2. The negative or doubtful reactions, the most often observed, have no practical value because they are almost always followed by a positive Mitsuda reaction. 3. The positive Fernandez reaction was always followed by a positive Mitsuda reaction. Thus it has practical importance. However as the frequency of early positivity is low with human and armadillo lepromin of lower concentrations, recommended in routine testing, the 48 hours reading is unnecessary and without control value. It would represent a loss of time and money.

Lymphocyte blastogenesis and lepromin reactivity in leprosy patients and their parents.

To determine whether there is an inherited familiar trait linked to the lymphocyte blastogenesis test (LTT), under stimulation with PHA, lepromin and Mycobacterium leprae in culture medium containing autologous plasma, this test was carried out in patients with the polar forms of leprosy and their parents. The lepromin reaction was also studied in the patients and their parents because, since the test is negative in lepromatous (L) patients and a greater proportion of negativity is detected among their relatives, it might be assumed that the lymphocytes of these individuals could have a lower tendency towards blastogenesis than lymphocytes of tuberculoid (T) patients and their relatives. Thirty individuals were studied, ten of them being leprosy patients (4 L and 6 T) and the remaining their parents; 115 LTT, including control and stimulated cultures, were performed. In the limited number of patients and parents studied, the results showed that mothers of either L or T patients displayed a similarly low response to the stimulants M. leprae and lepromin. The lepromin reaction was negative in all L patients and positive in 3 out of 8 parents, as well in all the T cases and their fathers. Fathers and their T descendants were lepromin positive and there was a certain relationship between this reactivity and blastogenesis. This might suggest a possible inherited familiar trait related to a relative degree of resistance. However, further evidences from studies with larger number of subjects are required to support this hypothesis.

Lymphocyte transformation test in patients with American leishmaniasis.

To determine the correlation between the lymphocyte transformation test (LTT) with phytohemagglutinin (PHA) and the clinical forms of American leishmaniasis (AL), duration of the disease and reaction to leishmanin, the authors studied 12 patients and compared them to 25 unaffected individuals. The ulcerated cutaneous form of the disease was observed in 4 patients, the ulcero-nodular lymphangitic form in 1, the mucous cutaneous form with destruction of the nose in 6, and the verrucous form in 1. General patient condition was satisfactory and the Montenegro reaction macroscopically positive with infiltrations of 5-15 mm in diameter. The results revealed no evidence of immunodeficiency among patients. No correlation was observed between clinical forms, duration of the disease and Montenegro reaction and LTT results. LTT with PHA does not appear to be an adequate indicator of the cell immune system in AL cases.

Lymphocyte transformation test in patients with paracoccidioidomycosis.

The lymphocyte transformation test (LTT) by phytohemagglutinin was carried out on lymphocyte cultures from patients with paracoccidioidomycosis, in medium with autologous plasma (from the patient) or homologous plasma (from an unaffected individual), and lymphocyte cultures from unaffected and apparently normal individuals, in medium with autologous plasma (from the individual) or homologous plasma (from a patient with paracoccidioidomycosis). Blastogenesis was evaluated morphologically by 'blast' percentage, and the results analyzed according to clinical form of the disease and general condition of the patient. In the medium containing autologous plasma, percentages below the lower limit of the confidence interval for the distribution of the values for apparently normal individuals were encountered more frequently among patients with diffuse extrapulmonary paracoccidioidomycosis and in poor general condition. When the lymphocytes from patients with the disease were cultured in medium containing homologous plasma, blastogenesis increased in most cases. The lymphocytes of unaffected individuals exhibited a lower response more frequently when cultured in medium containing plasma from patients with the disease than when cultured in medium containing their own plasma. These results suggest that existence of factor(s) inhibiting blastogenesis in the plasma of these patients. In addition to such factor(s), an intrinsic lymphocyte defect may also occur in some patients, which might prevent a greater response to phytohemagglutinin even in homologous plasma. Smears of lymphocytes cultured in autologous plasma and obtained from patients, especially those with diffuse extrapulmonary disease and in regular or poor general condition, and smears from most of the controls whose lymphocytes were cultured in the plasma of these patients revealed deeply stained cells with altered morphology and considerably reduced in number.(ABSTRACT TRUNCATED AT 250 WORDS)

[Comparative analysis of the clinical diagnosis of hanseniasis and the histopathological examinations performed according to the criteria of the Madrid and Ridley-Jopling classifications]. / Análise comparativa entre o diagnóstico clínico da hanseníase e os exames histopatológicos realizados segundo os critérios da classificação de Madrid e a de Ridley-Jopling.

A comparative study of the Ridley-Jopling's (RJ) and of the Congress of Madrid's (CM) pathological criteria was made in the different clinical types and groups of hanseniasis. A concordance between both criteria was found in the Indeterminate group and in the regressive phases of the Virchowian (V), Tuberculoid (T) and Reactional tuberculoid (RT) types. Clinical RT was confirmed by pathology in 81.2% of the cases according to CM, whereas 46.2% were considered "Borderline" according to RJ. Out of the 48 clinically V patients, 17 (35.4%) were "Borderline" (BL-2, BL-1 and BB), but practically all were also pathologically V according to CM. It is concluded that there is no practical convenience in the establishment of histopathological sub-groups that do not perfectly agree with clinical criteria. The Authors stress the importance of the study of the plasmocytes in the V infiltrates, of the lymphocytes in all granulomas and of the differences in the involvement of the neural ends, specially between the T and V poles. The dyeing of lipids by the Sudan III is useful to perfectly characterize the V pole, recognize residual V structures, separate the sub-groups BT, BB and BL, help in the early diagnosis of V infiltrations and differentiate the edematous, diffuse, non-granular cytoplasmatic vacuolization of RT.

[Analysis of inter and intrapathologist concordance and discordance in a comparative study of the Madrid and Ridley-Jopling classifications of leprosy]. / Analyse de la concordance et de la discordance interet intrapathologistes dans une étude comparative des classifications de la lèpre de Madrid et de Ridley-Jopling.

Eighty-six patients of leprosy have been examined by three leprologists; they have been classified according to the Madrid classification and their lesions biopsied and sent to two pathologists for independent histopathological examination. The pathologists have not received any information regarding the cases. Firstly the "senior" pathologist (A) utilized the Madrid classification and the "junior" pathologist (B) the Ridley-Jopling classification. In a second phase, the "senior" pathologist utilized the Ridley-Jopling classification and the "junior" pathologist the Madrid classification. Both pathologists did not know their own previous histopathological diagnosis at the second phase. The analysis of concordance and discordance between the histopathological diagnosis of the two pathologists and for the same pathologist, on utilizing the two classifications, have elicited the following conclusions: 1. There has been more concordance between the two pathologists on utilizing the Madrid classification than the Ridley-Jopling classification for the lepromatous, borderline and tuberculoïd in reaction patients. 2. The comparison of the two classifications for each pathologist on "blindly" examination of the material, has shown more concordance for the "senior" pathologist than for the "junior" pathologist. 3. These observations have convoyed to the final conclusion that the Ridley-Jopling classification brings some difficulties to the pathologists with limited experience in leprology and therefore it should be utilized only by experienced pathologists. 4. This preliminary study shows the necessity of performing another one with larger number of patients, in the active phase, with larger number of pathologists, preferably from different countries, and by utilizing the same methodology of "blind" examination of the material.

Epidemiological survey of skin diseases in schoolchildren living in the Purus Valley (Acre State, Amazonia, Brazil).

A total of 9,955 schoolchildren aged 6-16 years have been examined in a tropical region. The prevalence of dermatosis varied from 21 to 87% in the municipalities surveyed. The most common dermatoses were pediculosis (prevalence 50%), nevi (16.8%), pityriasis versicolor (13.2%), pyoderma (12.2%), pityriasis alba (9.9%), dermatophytosis (6.2%), viral dermatosis (6.2%), scabies (3.0%) and acne vulgaris (2.7%). The prevalence of angular stomatitis, miliaria rubra, candidiasis, piedra nigra, keratosis pilaris, ephelides and geographic tongue is lower but still relatively high. Females had higher rates of pediculosis capitis and males higher prevalence of pityriasis alba. The prevalence of pityriasis versicolor, pigmented nevus and scabies was similar in males and females. Folliculitis, macular pigmented nevi and especially pityriasis versicolor tended to increase with age. Leprosy is hyperendemic in the surveyed area and its rate in the schoolchildren examined was 0.08%. Population movement (urbanization), socioeconomic situation, living conditions, promiscuity, and lack of hygiene may be the cause of such high prevalence and of association of two or more skin conditions. Climatic conditions might have enhanced the prevalence of certain dermatoses (pityriasis versicolor, dermatophytosis, piedra nigra, candidiasis, miliaria rubra).