[Keratosis palmoplantaris in black skin].
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Black People↗
Biomedical subjects
E Bourrat
Publications and source records attributed to E Bourrat.
[Photochemotherapy for pityriasis lichenoides: 3 cases].
BACKGROUND: Pityriasis lichenoides is a rare cutaneous disorder of unknown origin that mainly affects children and young people. The disease consists of two variants: pityriasis lichenoides et varioliformis acuta, characterized by papular lesions evolving into necrosis and pityriasis lichenoides chronica characterized by scaly papules that may last from several months to several years. Various types of therapy have been proposed for pityriasis lichenoides, to our knockledge, photochemotherapy has never been used. We report the first 3 cases of pityriasis lichenoides treated by photochemotherapy. CASE REPORTS: Three patients, 6, 15 and 18 years old, presented respectively with a 1 month, 2 years and 3 months history of numerous, papular and necrotic lesions or squally papular lesions. Clinical appearance and histopathologic examination of biopsy were consistent with pityriasis lichenoides et varioliformis acuta (patients 1 and 3) and with pityriasis lichenoides chronica (patient 2). They had received different treatments without significant effect: topical corticosteroids, antibiotic, UVB therapy and dapsone. Acitretin associated with PUVA were dramatically effective within few weeks. DISCUSSION: Pityriasis lichenoides can be a severe scarring disease with significant social, psychological and physical consequences. In our opinion, photochemotherapy, which has never been used in pityriasis lichenoides, is effective. Therapeutic modalities have to be define.
Acitretin↗
3-carboxy-4-phosphonocyclopentane amino acids: new metabotropic glutamate receptor ligands.
Two glutamic acid analogs (1 SR,3 RS,4 RS)- and (1 SR,3 SR,4 SR)-1-amino-4-phosphono cyclopentane-1,3-dicarboxylic acids (APCPD) have been synthesized. Pure E-(diethoxy-phosphoryl)-acrylic acid ethyl ester was obtained from ethyl propiolate, phenol and triethylphosphite. It was used as dienophile in a Diels-Alder reaction. Oxidation and cyclization afforded 3-(ethoxy-carbonyl)-4-(diethoxy-phosphoryl)-cyclopentanone. Bucherer-Bergs reaction and hydrolysis yielded APCPD-III and -IV which are inactive on mGlu1a receptor and antagonists on mGlu2 and mGlu8a receptors.
Amino Acids↗
[The role of the dermatologist in pediatric emergency].
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Child↗
[Cutaneous polyarteritis nodosa in children: three cases].
INTRODUCTION: Polyarteritis nodosa is a necrotizing vasculitis of small and medium-size arteries. The cutaneous form of polyarteritis nodosa follows a chronic course, characterized by recurrent episodes limited to skin, muscles and joints. This entity differs from systemic polyarteritis nodosa in the absence of visceral involvement. This form is rare in children, we describe three cases. CASE REPORTS: We describe three girls with a mean age of 11 years (range: 8-13). They presented painful subcutaneous edematous nodules, arthralgia and fever. Physical examination revealed livedo reticularis (2 cases) and pharyngeal infection (1 case). Laboratory findings showed an inflammatory syndrome. Skin biopsy supported diagnosis of polyarteritis nodosa. The course was characterized by periods of remission disrupted by exacerbations, well controlled by salicylotherapy, colchicine, dapsone or penicillin. Corticosteroid therapy was used only for invalidating symptoms. There was no systemic involvement after 2, 5 and 6 years of follow up. DISCUSSION: Cutaneous polyarteritis nodosa in children must be suspected in presence of fever, subcutaneous nodules, livedo reticularis and arthralgia. Prognosis is usually benign, so we recommend no aggressive treatment. In view of the tendency to relapse, long-term follow-up is appropriate, before confirming diagnosis.
Adolescent↗
[Pigmentary maculae of the large folds].
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Adult↗
[Eosinophilic pustulosis in an infant accompanied by immune deficit].
BACKGROUND: Eosinophilic is a skin eruption which occurs in the first years of life, progressing by pruriginous flare-ups with amicrobial papulopustulae on a hairless scalp. Eosinophil infiltration of the skin is variable (follicular or perifollicular dermal infiltration). In adults, eosinophilic pustulosis is often associated with immune deficiency, but this association has not been reported in children. We report two cases. CASE REPORTS: Two boys had a pruriginous papulopustular eruption involving the scalp and the trunk which had progressed with periods of exacerbation since birth. Search for bacteriological or mycological involvement was negative. Histology showed folliculitis with major polynuclear eosinophil infiltration. Both children had a past history of repeated skin and extracutaneous infections strongly suggesting an immune deficit. Buckly syndrome was suspected in the second case. DISCUSSION: Juvenile eosinophilic pustulosis belongs to the spectrum of childhood eosinophilic dermatoses. The presence of eosinophil infiltration in the skin demonstrates localized or systemic immune dysfunction. A hematology and immunology work-up is needed in case of associated skin or deep infections.
Adult↗
[Sporadic superficial pemphigus in the child: 2 cases].
INTRODUCTION: Excepting the endemic foliaceus form, childhood pemphigus is uncommon. We report two cases of pemphigus foliaceus in children with typical clinical manifestations. CASE REPORTS: Case n(o) 1. A 5-year-old girl was seen for a vesiculobullous crusted dermatosis involving the trunk and the face which had developed over the last 5 months, predominantly in periorificial and fold localizations. Histology showed intragranulous acatholysis. Direct skin immunofluorescence was positive for anti-intercellular substance IgG and C3. Indirect immunofluorescence was positive for anti-intercellular substance antibodies at 1/500. The diagnosis of superficial pemphigus was retained and the child was given dapsone associated with systemic prednisone (1.5 then 2.5 mg/kg/d). Dapsone was stopped on day 15 due to poor hematological tolerance. Outcome was favorable allowing withdrawal of prednisone at 18 months. Case n(o) 2. A 6-year-old had developed since the age of 18 months a generalized and polycyclic pruriginous erythemato-squamous dermatosis with oozing discharge which started and predominated on the face (periorificial zones). Trace element (copper, selenium, zinc) and vitamin (A, E and B1) assays were within the normal range. Glucagon was normal. Histological examinations of several biopsies were non-contributive. Diagnosis of pemphigus foliaceus was finally obtained after repeated direct immunofluorescence tests which revealed anti-intercellular substance IgG. Indirect immunofluorescence was negative. The child was given prednisone (2 mg/kg/d). DISCUSSION: In children, pemphigus foliaceus has an exceptional frequency and diagnosis is often made quite late (mean 8 months). The diagnosis should always be entertained in children who develop chronic extensive erythemato-squamous and crusted dermatosis, even if formation is absent. Direct skin immunofluorescence confirms the diagnosis and should be repeated if negative in cases with highly suggestive clinical presentations. It would be reasonable to attempt "minor" treatments as the first line approach. Systemic corticosteroids are however the treatment of choice despite the risk of classical side effects. Childhood pemphigus foliaceus is not an attenuated clinical form of adult pemphigus. Mortality is not negligible and is close to that in adults.
Adult↗
[Familial chronic mucocutaneous candidiasis associated with autoimmune polyendocrinopathy. Treatment with fluconazole: 3 cases].
BACKGROUND: Familial candidiasis endocrinopathy is a hereditary disease variably associated with i) a dysfunction of the parthyroid, adrenal, thyroid, pituitary or ovarian glands as well as the pancreas (diabetes), liver (hepatitis) and gastric wall cells (Biermer's disease); ii) chronic mucocutaneous candidiasis; iii) autoimmune manifestations: chronic keratitis, pelade, vitiligo, hemolytic anemia. CASE REPORTS: We cared for three pediatric patients with chronic mucocutaneous candidiasis associated with an autoimmune polyendocrinopathy. There was a familial context in all cases with an autosomal dominant inheritance in the first case and an autosomal recessive inheritance in the two others who were siblings. In all three cases, the chronic mucocutaneous candidiasis began before the age of 5 years and had preceded the development of the endocrinopathy. Fluconazole was effective in all three cases. DISCUSSION: Chronic mucocutaneous candidiasis may appear as the inaugural symptom of an autoimmune polyendocrinopathy and should lead to a familial study with an andocrinology work-up to determine any clinical manifestations and biological evidence of autoimmunity. Fluconazole does not have marketing approval for children, but provides good control of the fungal infection, particularly in the ungueal localization, and is well tolerated.
Adolescent↗
[Neonatal monoblastic leukemia revealed by transitory specific skin lesions].
BACKGROUND: Specific cutaneous involvement is frequently observed in congenital leukemia and may be the inaugural sign. Lesions may be non-specific and even regress spontaneously, misleading diagnosis and delaying care. CASE REPORT: A infant in good health had diffuse ecchymotic maculae at birth which totally regressed within 10 days. On day 20, a macular rash and three violet nodules developed. The physical examination found enlarged nodes, liver enlargement and poor general status. Blood cell counts and the myelogram led to the diagnosis of type 5 acute myeloblastic leukemia (AML 5). Search for extension revealed cutaneous leukemia and meningeal and renal involvement. DISCUSSION: The predominance of myelocyte forms is specific for neonatal leukemia (AML 5 and 4). Specific skin lesions are frequent (25 to 30 p. 100 of cases) and sometimes precede (7 p. 100) anomalies in peripheral and medullary smears. Skin biopsy with direct smear can provide rapid diagnosis. Classically subcutaneous nodules or bluish macropapulae give the blueberry muffin baby aspect. The polymorphous features and the remarkably fluctuating skin lesions in our case are unusual, especially since the infant did not have spontaneously reversible neonatal leukemia, a rare unpredictable phenomenon described in newborns with a normal phenotype which can mislead therapeutic management.
Age Factors↗
[Congenital generalized cutis laxa: 5 cases].
BACKGROUND: Congenital cutis laxa is an exceptional condition. No large scale series has been reported in the French literature. We report 5 cases observed between 1993 and 1997. PATIENTS AND METHODS: Five children with a morphotype compatible with congenital generalized cutis laxa were examined. A family study, complete visceral workup and skin biopsy with standard histology, orceine coloration and histomorphometric analysis of the collagen and elastic fibers of the dermis were performed. Karyotype and copper metabolism (cupremia and ceruloplasminemia) were available in 3 children. RESULTS: The diagnosis was clinical and proven histologically by orceine coloration of skin biopsies in all cases. There were discrete ultrastructure anomalies in the pure cutaneous form expressed in case n(o) 1 with possible autosomal dominant inheritance. Cupremia and ceruloplasminemia were normal in the 3 children explored; this corresponds to absence of the Elhers-Danlos type IX phenotype. The karyotype was normal in 3/3 children, in agreement with the absence in these three children of marfanoid cutis laxa phenotype. Patients n(o) 2, 3, 4 and 5 had common features: probable autosomal recessive inheritance and severe prognosis. Patient n(o) 2 died at the age of 3 weeks and had severe pulmonary emphysema. This child's sister also had cutis laxa but with no visceral component (autosomal recessive inheritance with variable expression). Patients n(o) 3, 4 and 5 had a severe multiple malformative syndrome with facial dysmorphism, growth retardation, unexplained digestive disorders and psychomotor retardation. DISCUSSION: Our series of 5 patients and data in the literature confirm that primary cutis laxa is a heterogeneous group of conditions both clinically and genetically. The anomalies associated in patients n(o) 3, 4 and 5 were not directly related to anomalous elastic tissue as was also the case for the craniostenosis in patient n(o) 3 reported in other cases in the literature.
Abnormalities, Multiple↗
[Actinic prurigo of childhood. 3 familial cases associated with HLA-DR 0407].
BACKGROUND: Actinic prurigo, as idiopathic skin reaction involving light-exposed areas, was first described in American Indians. Actinic prurigo was early considered to be a particular form at polymorphous phototoxicity, but can be identified as a specific entity on the bases of clinical features and epidemiological characteristics. CASE REPORTS: Three children in the same family developed photosensitive reactions early in childhood with characteristic polymorphous and persistent eczema-like or papulo-nodular pruriginous lesions which predominated in light-exposed areas and appeared several hours after exposure to sun. The lesions persisted during the winter season. The lesions followed a chronic course but tended to improve at puberty. Clinical laboratory tests, serum and urine porphyrin levels and antinuclear factors were normal. Histology and photobiology explorations gave non-specific results. DISCUSSION: These observations have three points in common with actinic prurigo observed in American Indians. HLA typing showed that our three patients, as in white patients in Great Britain, had a significant association with a specific HLA DR1 subtype: DRB1*0407. This DRB1*0407 alleles could play a role in initiating the immune response to a light-induced peptide antigen. This particular genetic predisposition, if confirmed in other studies, would be an additional argument for distinguishing actinic prurigo as a specific polymorphous phototoxicity entity.
Adolescent↗
[Anitis, vulvar edema and macrocheilitis disclosing Crohn disease in a child: value of metronidazole].
BACKGROUND: We report a case of oro-genital and perianal Crohn's disease which progressed for 4 years with no digestive involvement in a 12-year-old girl. CASE REPORT: At the age of 8 years, a young girl developed recurrent anal fissures. Voluminous vulvar edema developed at 12 years with fissurar macrocheilitis. There were no digestive signs and the diagnosis of Crohn's disease was obtained on the basis of granulomatous epithelioid infiltration of biopsy specimens (Bauhin valve, anus, vulva). Metronidazole given per os at the dose of 25 mg/kg/day for 6 months led to partial significant symptomatic remission. DISCUSSION: Unilateral or bilateral vulvar edema is highly suggestive of Crohn's disease even if the classical digestive inflammatory signs are absent. Demonstration of perianal lesions (erythema, pseudocondylomatous formations) must not mislead the diagnosis (sexual abuse). Simultaneous granulomatous lesions in the genital and labial regions is exceptional in Crohn's disease. Ileocolonoscopy is indicated in such cases and alone can demonstrate latent digestive inflammatory processes. Different agents have been proposed for treatment of local Crohn's disease skin lesions. It is difficult to evaluate their efficacy due to the spontaneous variability of disease expression.
Cheilitis↗
[Cutaneous pathology of misery].
Cutaneous disease is the most common health problem found in the homeless, in out patient clinics as well as in hospitals. They are related to life style characteristics. Fungal, bacterial infections, scabies and pediculosis are mainly observed. Other frequently observed skin diseases are caused by physical violence and trauma, drug abuse and nutritional deficiency In addition to limited access to health care, homeless patients tend to neglect their skin until problems become serious and disabling. The patient's condition will worsen without hospitalisation.
France↗
[Perianal papulonodular dermatitis in Hirschsprung disease].
INTRODUCTION: Macerations resulting from anal and/or urinary incontinence can lead to perineal or peristomal pseudo-verrucosis. The papulo-nodular perianal lesions observed in children after surgery for Hirschsprung syndrome is an example. CASE REPORT: A 28-month-old boy with rectosigmoid Hirschsprung disease developed chronic diarrhea after surgery with colo-anal anastomosis followed rapidly by perineal rash. The rash did not respond to local treatments and pseudo-condylomatous papulonodules developed. Infection, deficiency and tumoral causes were eliminated. Skin biopsy showed benign epidermal hyperplasia. The lesions regressed spontaneously within a few weeks after the diarrhea had been controlled and diapers were no longer used, confirming the diagnosis of perianal papulo-nodular dermatosis following surgical treatment for Hirschprung disease. DISCUSSION: The presentation of the perianal rash was similar to Sevestre and Jacquet dermatitis and infantile gluteal granuloma. The pathogenesis is similar to perianal or peristomal pseudoverrucosis seen in incontinent or stomy patients. Diagnosis is based on the clinical presentation, the periorificial localization and the pseudo-tumoral aspect of the lesions. Removal of the cause of maceration, when possible, is the basis of treatment.
Anus Diseases↗
[Scleroderma-like patch on the thigh in infants after vitamin K injection at birth: six observations].
INTRODUCTION: Texier's disease or pseudosclerodermatous reaction after intramuscular injection of vitamin K1 is well known in adults although only 1 report of a case in a newborn was found in the literature. We report 6 cases. CASE REPORTS: Six infants (4 boys, 2 girls) developed "peau d'orange" skin lesions after the age of 6 months which was localized in the lower third of the medial aspect of the thigh. Initial rapid locoregional extension was followed by stabilization and then regression. In all 6 cases, histology showed lesions of the fascia and/or the deep hypoderma associated with variable mononuclear inflammatory infiltration and hyalin fibrosis. When performed, immunological studies (complement fixation, search for autoantibodies) were always negative or normal. No visceral involvement was found. DISCUSSION: A pseudosclerodermatous lesion of the lower third of the thigh occurred in 6 infants at the site of an intramuscular injection of vitamin K1 administered at birth. The history, clinical manifestations, histology and outcome of these cases are compatible with the diagnosis of Texier's disease. We discuss the role of the solvent in the Roche vitamin K1 injection. The pathogenesis of this side effect remains unknown. CONCLUSION: Texier's disease in infants after injection of vitamin K1 at birth is a stereotypic dermatosis. Diagnosis is based on history and clinical presentation. The causal effect of injectable vitamin K1 should be entertained whenever pseudosclerodermatous lesions are observed in a young child.
Antifibrinolytic Agents↗
[Rosacea with ocular involvement in a child].
INTRODUCTION: Rosacea ia an uncommon facial eruption in children. We report a case of rosacea associated with a specific episcleritis. CASE REPORT: A 10-year-old girl consulted for an erythematous papular and pustular eruption of the mid face of 1 month duration. The child complained that she had had a red painful right eye for 6 months. There was no argument for acne, periorificial granulomatous dermititis or for sarcoidosis. The ophthalmological examination gave the diagnosis of ocular and cutaneous rosacea. Oral antibiotics followed by erythromycin gave favorable results for both skin and ocular lesions. DISCUSSION: Ocular involvement is a frequent complication of rosacea in adults. It is exceptional in children in whom rosacea is in itself rare. The presence of blepharitis, keratoconjunctivitis or episcleritis may suggest the diagnosis in a child with a mid facial eruption. As in the adult, skin and ocular rosacea responds well to prolonged oral antibiotics. The choice of the antibiotic depends on the age of the child. Cyclins are not authorized in children under the age of 8 years.
Anti-Bacterial Agents↗