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Biomedical subjects

P Plantin

Publications and source records attributed to P Plantin.

At least 19 recordsLinked to original sources
PubMed2002

[Trumpet nail deformity during the course of Dowling-Meara type epidermolysis bullosa simplex. A report of two cases].

INTRODUCTION: We report two patients with Dowling-Meara type epidermolysis bullosa simplex: who had trumpet nail deformity. CASE REPORTS: Two 75 and 72 year-old sisters, had flare-ups of epidermolysis bullosa simplex since childhood. The aspect of the lesions and electron microscopy were in favour of the diagnosis of the Dowling-Meara variant of epidermolysis bullosa simplex. Both women had several fingernails with pincer and trumpet nail deformities. There were no such signs among the other siblings, who had never had epidermolysis. DISCUSSION: Nail involvement is not rare during the course of superficial epidermolysis bullosa. The trumpet lesions appear to be relatively specific to the present two cases of epidermolysis bullosa simplex. The electron microscopic findings of these two cases were also noteworthy, with whisk-like but not round clumping of the perinuclear tonofilaments.

Aged↗
PubMed2000

[Warts and molluscum contagiosum: practical point of view].

Warts and molluscums contagiosums are two benign viral skin diseases that commonly affect children. Contamination occurs by autoinoculation or during skin to skin contact. Molluscums contagiosums are more frequent in immunodeficient and atopic children. Swimming-pool practice and contact sports favour warts transmission. The choice of treatment depends upon the age of the child and the number and location of the lesions. Natural resolution can be awaited when lesions are limited. In first intent, curettage of the lesions under local anesthesia for molluscums contagiosums, salicylic acid preparation or cryotherapy according to location for warts, are the treatment of choice. In neither affection school ousting is necessary.

Adjuvants, Immunologic↗
PubMed2000

[Pili multigemini: a pilar dysplasia with linear disposition].

BACKGROUND: Pili multigemini is an uncommon pilar dysplasia with linear disposition which could be explained by the pattern of Blaschko's lines. CASE REPORT: A 37-year-old man with no medical history developed pili multigemini over a heavily bearded chin. DISCUSSION: Mili multigemini is an uncommon developmental defect of hair follicles resulting from hairs with multiple matrices and papillae emerging through a single pilosebaceous canal. This defect has a linear distribution on the chin we found to follow Blaschko's lines. Pili multigemini has been observed in association with a few rare malformations. Treatment is difficult.

Adult↗
PubMed2000

[Fibrillin network in normal bone tissue].

The molecular basis for Marfan's syndrome is known to reside in mutations in FBN1, the gene for fibrillin 1. The skeletal manifestations of Marfan syndrome include morphologic abnormalities and osteopenia. Presence and distribution of fibrillin 1 in adult bone (healthy or with Marfan syndrome) has not been studied extensively. We evaluated distribution of fibrillin and type III collagen in bone and cartilage of children and adults without bone disease, using monoclonal antibodies. Fibrillin is mostly present in attachment sites for tendons. In cartilage and bone tissue, fibrillin is identified at the junction between cartilage and bone in children, and in the areas with intense osteoblastic activity. These data suggest participation of fibrillin in bone formation and growth during youth and in bone mineralisation in adult.

Adolescent↗
PubMed1999

Bone mineral density in sixty adult patients with Marfan syndrome.

Sixty adult patients (40 women, 20 men) with Marfan syndrome (MFS) according to the Berlin criteria had a full clinical examination and bone mineral density (BMD) measurement by dual-energy X-ray absorptiometry of the hip and nondominant forearm. BMD was expressed as a Z-score and compared with the reference population of the Hologic database. In MFS men, BMD (g/cm(2)) was compared with the BMD of 45 normal tall Caucasian adults. Osteocalcin was measured by radioimmunoassay. In patients with MFS, BMD was compared between patients with and without previous fractures and according to the phenotypic severity of MFS. The mean age of the patients was 32.9 +/- 9.3 years (women 32.5 +/- 9.7, men 33.4 +/- 8.6), mean height was 180.3 +/- 10.3 cm (women 176.3 +/- 9.2, men 188.1 +/- 7.5) and mean body mass index 20.9 +/- 3.6 kg/m(2) (women 20.8 +/- 3.4, men 20.95 +/- 3.97). Hyperlaxity score (Beighton criteria) was 6.9 +/- 1. 1. Six patients (10%) had a previous fracture. Thirty per cent of patients had had at least one previous operation for scoliosis, aortic dilatation or eye problems. BMD values in the 60 patients were as follows: Z-score of the hip, -1.26 +/- 0.93, p<10(-9) (neck, -0.93 +/- 1.09, p<10(-9); trochanter, -1.31 +/- 0.85, p<10(-9); intertrochanter, -1.39 +/- 0.99, p<10(-9); Ward's triangle, -0.93 +/- 1.88, p<10(-9)); Z-score of the radius: -1.6 +/- 1.06, p<10(-9) (1/3 proximal, -1.29 +/- 1.03; mid-radius, -1.94 +/- 1.04; ultradistal, -0.68 +/- 1.1, p<10(-9)). The decrease in BMD was similar in men and women at both the hip and the radius. BMD in MFS patients was significantly decreased at cortical compared with trabecular sites (radius 1/3 proximal vs ultradistal, p<0.0001; total femur vs Ward's triangle, p<0.0005). No difference in BMD was found between MFS patients with or without previous fractures and those with severe or less severe phenotypic expression of MFS. An influence of height and weight in MFS on BMD is suspected. Osteocalcin was not increased in our group of MFS patients. Thus both men and women with MFS have a significant deficit of BMD at the hip and radius. The decrease in BMD is present equally in both sexes and is more pronounced at predominantly cortical sites. In our group of patients we found no increase in fractures and no relation between decreased BMD and phenotypic expression of the syndrome.

Absorptiometry, Photon↗
PubMed1999

[Neonatal toxic erythema: 3 atypical cases].

UNLABELLED: Diagnosis of pustular dermatosis occurring during the first days of life is based on clinical findings. Erythema toxicum neonatorum (ETN) is the more frequent benign self limiting eruption in the newborn. CASE REPORTS: Three cases of ETN with localized pustules to the genitals and perineal area are described. COMMENT: When encountering a newborn with a localized pustulosis rash, it is important to separate benign condition as ETN from those that require prompt diagnosis and therapy. Atypical ETN and pustular dermatosis due to bacterial or viral infections or inflammatory diseases (e.g., eosinophilic pustulosis) can be differentiated by cytological and bacterial samples.

Diagnosis, Differential↗
PubMed1998

Pyodermitis of genital areas: an atypical manifestation of eosinophilic pustulosis of childhood.

Eosinophilic pustulosis of the scalp was first described in 1984. It has also been described in other sites than the scalp. We report a case in which the lesions exclusively involved the genitals. A 4-month-old boy presented with papulopustular lesions of the genitals in the form of pyodermitis with a favourable course over several days but which subsequently recurred. A smear of a pustule revealed no signs of scabies or viral, fungal or bacterial infection. Histology showed a non-follicular eosinophilic pustulosis. This case emphasizes the ubiquitous and sometimes misleading nature of eosinophilic pustulosis and the non-follicular nature of the lesions.

Biopsy↗
PubMed1998

[Universal dyschromatosis: a familial case].

INTRODUCTION: Universal dyschromatosis is a generalized leucomelanodermia recognised in Japan in 1933. We report a family with universal dyschromatosis, demonstrating the mode of transmission. The ultrastructural aspects are compatible with a functional melanogenesis anomaly. CASE REPORT: A 9-year-old girl was hospitalized for recently diagnosed insulin-dependent diabetes mellitus. She was born to non-consanguinous parents and her past medical history was uneventful. Her father was of mixed ethnic origin. The physical examination revealed generalized leukomelanoderma identified since the first year of life. Zones of small achromatic maculae alternated with zones of pigmented maculae of variable size and color. Lesions were diffuse but predominated on the trunk and did not involve the face, the hands or the feet. Neither the child nor her father who also has leukomelanoderma were photosensitive. A skin biopsy from the gluteal region revealed alternating zones of hyper- and hypopigmentation. The ultrastructural analysis showed that the number of melanocytes was not significantly different in the different pigmented zones and the pigment transfer to adjacent keratinocytes was intact. There were three other girls in the kinhood and two, as well as a few other individuals in the family, had a localized form of the disease. DISCUSSION: Universal dyschromatosis is a rare genodermatosis. The familial cases reported here illustrate the variable clinical presentations of this pigmentary abnormality. The pedigree in this family demonstrated incomplete penetrance of hereditary leukomelanoderma with autosomal dominant inheritance. The localized forms reported to date under different names would actually appear to correspond to incomplete expression of the dermatosis. The skin manifestations in universal dyschromatosis would appear to be similar to those in a few other skin diseases, mainly xeroderma pigmentosum, especially the localized forms; for generalized forms however, there is little room for confusion as photosensitivity is absent and lesions predominate in unexposed zones. The ultrastructure investigations showed different levels of melanocyte activity without abnormal pigment production or transfer. This abnormality has variable expression, explaining the multitude of clinical presentations.

Adult↗
PubMed1997

[Cutaneous semiology of febrile rashes in children].

Generalized eruptions with fever are a frequent problem in paediatric practice. Clinical type of skin lesions, distribution and associated signs and symptoms are sometimes specific enough for a definitive diagnosis. However non specific clinical findings do not allow an aetiologic conclusion in some instances. More than 50 infectious agents, drugs and numerous inflammatory diseases are known to cause rashes in childhood.

Adolescent↗