Introduction. La periarterite noueuse est une vascularite necrosante des arteres de petit et moyen calibre. La forme cutanee pure se caracterise par une evolution prolongee, marquee par des poussees limitees a la peau, aux muscles, aux articulations et par l'absence d'atteinte viscerale. Cette forme est exceptionnelle chez l'enfant. Nous en rapportons trois observations. Observations. Trois fillettes âgees de 8 a 13 ans souffraient de nodules sous-cutanes inflammatoires et douloureux associes a des arthralgies et a une fievre. Il existait un livedo ramifie chez deux d'entre elles et une pharyngite chez la troisieme. Elles avaient toutes un syndrome inflammatoire biologique. Les examens histologiques cutanes ont confirme le diagnostic de periarterite noueuse. L'evolution a ete marquee par des recidives cutanees controlees par un traitement par anti-inflammatoires non steroidiens, colchicine, dapsone, penicilline, ou par corticotherapie en cas de poussees invalidantes. Il n'y a pas eu d'evolution vers une forme systemique apres 2, 5 et 6 ans d'evolution. Discussion. Le diagnostic de periarterite noueuse cutanee doit etre evoque chez un enfant ayant de la fievre, des nodules sous-cutanes, un livedo et des arthralgies. Son traitement doit etre symptomatique etant donne le pronostic habituellement benin mais une surveillance au long cours est necessaire avant d'affirmer son caractere purement cutane et en raison de la tendance a la recidive.

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.

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.

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.

Background. We report a case of a large blue mongolian spot which led to early diagnosis of Hurler's syndrome. This association is uncommon and should be recognized by dermatologists for early diagnosis and management Case report. A male infant from Guinea, born to first-cousin parents, was seen at the age of 4.5 months for multiple, particularly extensive blue mongolian spots. Growth was + 2 SD for age and the infant's psychomotor development was normal. A slight thickening of the skin was noticed without real dysmorphism. The blue spots extended over the entire posterior aspect and part of the anterior aspect of the trunk and involved all four limbs and the eyelids. The elbow and knee joints were moderately stiff and liver enlargement was palpated. The skin biopsy showed fusiform cells with melanin pigment tattooing the cytoplasm. No vacuolized epidermal cells were observed. Blood cell counts and liver and kidney tests were normal. Tests were positive for vacuolized lymphocytes and Gasser lymphocytes. Urine was positive for mucopolysaccharides and the enzymology study showed an alpha-L-iduronidase deficiency in serum and leukocytes, confirming the diagnosis of Hurler's disease. As no HLA compatible donor was available, no bone marrow graft was attempted. The child is a candidate for organoid gene therapy. Discussion. Mongolian spots predominate in Asian, American Indian and black population (90% of the cases) compared with Caucasians (10%). The pathogenesis and pathogenic associations are unknown. The incidence of large widespread mongolian spots is also unknown and no precise criteria are available to define this entity. A few cases of extended mongolian spots associated with type 1 gangliosidosis and about 20 cases associated with Hurler's disease have been reported in the literature. The association with Hurler's disease is probably not fortuitous and several hypotheses have been put forward. Bone marrow transplantation can improve prognosis if performed early before onset of irreversible visceral disorders,emphasizing the importance of early diagnosis in children.

BACKGROUND:We report a case of a large blue mongolian spot which led to early diagnosis of Hurler's syndrome. This association is uncommon and should be recognized by dermatologists for early diagnosis and management.CASE REPORT:A male infant from Guinea, born to first-cousin parents, was seen at the age of 4.5 months for multiple, particularly extensive blue mongolian spots. Growth was +2 SD for age and the infant's psychomotor development was normal. A slight thickening of the skin was noticed without real dysmorphism. The blue spots extended over the entire posterior aspect and part of the anterior aspect of the trunk and involved all four limbs and the eyelids. The elbow and knee joints were moderately stiff and liver enlargement was palpated. The skin biopsy showed fusiform cells with melanin pigment tattooing the cytoplasm. No vacuolized epidermal cells were observed. Blood cell counts and liver and kidney tests were normal. Tests were positive for vacuolized lymphocytes and Gasser lymphocytes. Urine was positive for mucopolysaccharides and the enzymology study showed an alpha-L-iduronidase deficiency in serum and leukocytes, confirming the diagnosis of Hurler's disease. As no HLA compatible donor was available, no bone marrow graft was attempted. The child is a candidate for organoid gene therapy.DISCUSSION:Mongolian spots predominate in Asian, American Indian and black population (90% of the cases) compared with Caucasians (10%). The pathogenesis and pathogenic associations are unknown. The incidence of large widespread mongolian spots is also unknown and no precise criteria are available to define this entity. A few cases of extended mongolian spots associated with type 1 gangliosidosis and about 20 cases associated with Hurler's disease have been reported in the literature. The association with Hurler's disease is probably not fortuitous and several hypotheses have been put forward. Bone marrow transplantation can improve prognosis if performed early before onset of irreversible visceral disorders, emphasizing the importance of early diagnosis in children.

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.

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.

Background. Actinic prurigo, as idiopathic skin reaction involving light-exposed areas, was first described in American Indiens. Actinic prurigo was early considered to be a particular form at polymorphous phototoxicity, but can be identified as a specific entily on the bases of clinical features and epidemioiogical 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. Rootline 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 allels 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 entiry.

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.

OBJECTIVE:Lichen planus is in children uncommon and poorly understood. The classical description is comparable to lichen planus in adults. We conducted a retrospective analysis of 12 cases in children.PATIENTS AND METHODS:Twelve children with lichen planus consulted the Saint-Louis or Robert-Debré hospitals between February 1994 and March 1996. Data collected included: age, sex, ethnic origin, drug use, anti-hepatitis vaccination status, disease history, physical examination, skin histology, liver tests, hepatitis B and C serology, treatment and outcome. Histological proof was obtained in all cases but one (a child with isolated ungueal involvement whose sister had histologically proven ungueal lichen planus).RESULTS AND DISCUSSION:The clinical features classically described in adults were atypical in all our childhood cases. A rapidly extensive eruption was the main sign in 6 cases. The localizations were unusual with lesions involving all four limbs and the trunk as well as the face in 5 cases and the scalp in 1. Mucosal involvement, observed in 65 p. 100 of adult cases was only found in one of our children. Unguel involvement also appears to be uncommon in children. The etiological pattern was also unusual since we did not observe a single case related to drugs or hepatitis B or C infection. Three children developed a lichen eruption after anti-hepatitis B infection. Four other cases of lichen planus after anti-hepatitis B vaccination have been reported in the literature. Mean delay between the booster vaccination and onset of eruption is reported to be 40 days. The increased incidence of childhood lichen planus in tropical zones suggests ethnic, genetic and climatic factors may be involved. Prognosis is poorly defined in the literature. Certain authors emphasize the long duration of the disease and resistance to treatment in cases of childhood lichen planus. Currently, there is no consensus on treatment. Dermocorticoids in combination with antihistaminics are usually prescribed. General corticosteroid therapy would appear to be warranted in extensive progressive forms with important functional and esthetic impact (scalp involvement with cicatricial alopecia, pigmentation sequellae). The role of other drugs, particularly retinoids, remains to be defined. This retrospective series was not statistically significant. Data in the literature are rather discordant, emphasizing the need for a prospective analysis to acquire a better understanding of the real incidence of childhood lichen planus and better define the therapeutic strategy.

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.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.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.

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 cole-anal anastomosis followed rapidly by perineal rash. The rash did not respond to local treatments and pseudo-condylomatous papulo-nodules 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 pseudo-verrucosis 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.