[Impact of sex and age on the clinical and epidemiological aspects of childhood psoriasis: Data from a French cross-sectional multicentre study]. / Impact de l'âge et du sexe sur les aspects cliniques et épidémiologiques du psoriasis de l'enfant. Données d'une étude transversale multicentrique française.

BACKGROUND: The prevalence of childhood psoriasis is estimated at between 0.4% and 0.7%. Clinical aspects of the diseases depend on age. The aim of this study was to investigate the clinical aspects of psoriasis according to age and sex. PATIENTS AND METHODS: A cross-sectional, multicentre study of children with psoriasis was performed by investigators belonging to the Research Group of the French Society of Paediatric Dermatology. The study was conducted from April 2012 to March 2013. Inclusion criteria were age less than 18 years and clinical diagnosis of psoriasis. The children were classified into 3 groups by age: infants: <2 years; children: ≥2 years and <13 years; adolescents≥13 years. The information collected included demographic data, clinical, epidemiological, and therapeutic aspects of the psoriasis, as well as analysis of comorbidities. RESULTS: Three hundred and thirteen children were included: 27 (8.6%) infants, 207 (66.1%) children, and 79 (25.2%) adolescents. Plaque psoriasis was the most frequent clinical type of psoriasis seen in children and adolescents (>41%), but it accounted for only 25.9% of psoriasis of infants (P<0.0001). Napkin psoriasis (37.0%) and inverse psoriasis (22.2%) were the most common forms of psoriasis seen in infants and were described significantly more frequently in this group than in the two other groups (P<0.003). Nail involvement was more common in adolescents (37.2%, P=0.03) and children (32.9%) than in infants (14.8%) and affected boys more than girls (43.6% vs 22.0%, P<0.0001). Girls presented scalp psoriasis more frequently (17.7% vs 8.7%, P=0.02). Local vitamin-D treatment and systemic therapies were used more frequently in children and adolescents than in infants. There was no significant difference for treatment use, including for acitretin, according to gender. DISCUSSION: Plaque psoriasis was the most common clinical type of psoriasis in children but affected less than 50% of the children. Age had a significant impact on extra-cutaneous skin disorders and on treatment used, while sex had little incidence. The frequency of comorbidities was not affected by age. CONCLUSION: Childhood psoriasis thus presents specific characteristics dependent on the age of the child. The results of studies exclusively dealing with adults cannot be extrapolated to children.

[Allgrove syndrome]. / Syndrome d'Allgrove.

BACKGROUND: Allgrove syndrome or "Triple A syndrome" involves adrenal insufficiency as a result of resistance to adrenocorticotropic hormone (ACTH), achalasia and alacrima, often associated with neurological signs. Herein, we report a new case of this rare genetic disease, which is of interest because of its dermatological mode of discovery. PATIENTS AND METHODS: A 4-year-old child, born to parents related by first-degree consanguinity, presented oral hyperpigmentation and diffused acquired melanoderma, as well as long-standing dry-eye syndrome. Laboratory tests confirmed low adrenal insufficiency. The combination of alacrima and adrenal insufficiency prompted screening for Allgrove syndrome, which was confirmed by genetic analysis showing homozygous c.1331+1G>A mutation within intron 14 of the gene encoding for ALADIN protein. Both parents were heterozygous for the same mutation. Two years later, the onset of vomiting raised concerns about achalasia, which was confirmed by oesophageal manometry. The child received symptomatic treatment consisting of supplementary hydrocortisone and oesophageal dilatation. DISCUSSION: The present case serves as a reminder that Allgrove syndrome may be diagnosed by dermatologists. Therapy is cross-disciplinary, being based upon medical treatment for adrenal insufficiency with prescription of artificial tears in the event of alacrima. Achalasia is treated by oesophageal dilatation or by surgery.

Psoriasis and obesity in French children: a case-control, multicentre study.

BACKGROUND: Obesity is more common in adults with psoriasis than in the general population, but there is a lack of data available regarding this association in children. OBJECTIVES: To evaluate whether obesity was more common in French children with psoriasis of any clinical type or severity. METHODS: A multicentre case-control study was performed in 23 French dermatology centres. Children without chronic or genetic inflammatory disease were selected as controls and matched for age, sex and dermatology centre. We used three weight cut-off categories to compare the two groups: overweight, overweight with abdominal obesity and overweight with obesity according to the French Health Authority guidelines. RESULTS: A total of 261 children with psoriasis were included. The mean age was 9.8 years, 126 were boys and 135 were girls. Overall, 42.5% of these children had plaque psoriasis and 32.2% had severe psoriasis. There was no difference between the psoriasis and control groups when the frequency of children who were overweight was compared (20·7% in psoriasis group vs. 17·1% in control group; P = 0·18). Overweight with abdominal obesity including obesity (18·4% vs. 10·4%; P = 0·009) and obesity alone (10·0% vs. 3·1%; P = 0·001) were more common in the psoriasis group. CONCLUSIONS: This study shows that being overweight with abdominal obesity and being obese is more common in children with psoriasis than in controls. The risk factors are the same as those that affect the French general population, i.e. female sex and having a parent who was overweight. The severity and clinical type of psoriasis do not affect overweight and obesity.

Antileukemic and long-term effects of two regimens with or without TBI in allogeneic bone marrow transplantation for childhood acute lymphoblastic leukemia.

Between September 1986 and June 1997, 24 children with high-risk ALL in CR1 were allografted after TAM (fractionated TBI, high-dose Ara-C, and melphalan; n = 10) or BAM protocol (busulfan, high-dose Ara-C, and melphalan; n = 14). The EFS for transplants from sibling donors was 33% with TAM and 62% with BAM (P = 0.148). The probability of acute GvHD was 70% with TAM and 15% with BAM (P = 0.003). Four of 17 evaluable patients relapsed: one after TAM and three after BAM. In all, 46 other children transplanted in CR beyond CR1 were studied for sequelae. Long-term side effects were more frequent in TAM vs BAM. In children with ALL, busulfan may be a good alternative to TBI to improve the quality of life.

[Eccrine neutrophilic hidradenitis: idiopathic plantar form in children]. / Hidradénite eccrine neutrophilique: forme plantaire idiopathique de l'enfant.

UNLABELLED: In this study, two cases have been reported of idiopathic plantar hidradenitis, an uncommon dermatological pathology with a spontaneous favorable outcome. OBSERVATIONS: Two children aged 12 and 14 years presented with a painful papulo-nodular plantar rash with major functional impairment. The diagnosis of idiopathic plantar hidradenitis was considered, and then confirmed in one case by plantar biopsy. Non-steroidal antiinflammatory drugs, associated with paracetamol in one case were administered. The symptoms disappeared spontaneously within a few days in both cases, without any recurrence. CONCLUSION: A knowledge of the symptoms connected with plantar hidradenitis in the child allows a rapid diagnosis to be made without hospitalization or further medical examination. Analgesic treatment and rest seem to be the only useful approaches. Biopsy to investigate eccrine gland infiltration by neutrophils can only be proposed in the case of an abnormally prolonged duration or an atypical presentation of this pathology.

[Familial hyperchylomicronemia with a new mutation of the lipoprotein lipase gene]. / Hyperchylomicronémie familiale avec nouvelle mutation du gène de la lipoprotéine lipase.

INTRODUCTION: Familial hyperchylomicronemia is a rare autosomal recessive disease caused by lipoprotein lipase deficiency. CASE-REPORT: A nine month-old girl presented with eruptive xanthomas revealing a familial hyperchylomicronemia. No lipoprotein lipase activity was found. DNA analysis revealed a novel homozygous non-sense mutation of the lipoprotein lipase gene at the codon 288. The parents were heterozygous carriers. DISCUSSION: Familial hyperchylomicronemia usually presents with eruptiva xanthomas, abdominal pain, pancreatic manifestation and lipemia retinalis. Papulo-nodular xanthomas occur more frequently in children as in our case. Eighty lipoprotein lipase gene mutations have been recorded to date. The gene locates on chromosome 8. Only 9 non-sense mutations have been described which lead to a truncated protein. In our case, no enzymatic activity was detected probably due to an absence of secretion of the enzyme, even though catalytic activity persisted. The homozygous carrier status leads to hyperchylomicronemia whereas the heterozygote status may lead to mixed hyperlipidemia with an increased risk of atherosclerosis. The screening of lipoprotein lipase gene mutations should be carried out in all families with hyperchylomicronemia, regardless of the presence or absence of xanthomas.

[Perianal streptococcal dermatitis]. / Anite périanale streptococcique.

BACKGROUND: Pediatric perianal streptococcal dermatitis (PSD) is a well-defined clinical entity. However, its highly uniform presentation remains surprisingly unrecognized by many practitioners 33 years after its first description. CASE REPORT: A seven-year-old girl had a three-week history of perianal and vulva redness with well-defined margins. Functional symptoms associated perirectal tenderness and pain during defecation, which was responsible for constipation. At onset she also presented with a sore throat, which resolved spontaneously, and she had been complaining for a few days about a perioral impetigo. She received mycostatin unsuccessfully for an alleged candidiasis. Positive cultures for group A beta-hemolytic streptococci from both perirectal and perioral swabs confirmed the diagnosis of PSD. Therapy with amoxicillin (50 mg/kg/d) was prescribed for ten days. Perianal lesions were cleared by day 2. CONCLUSION: Since PSD can masquerade as candidiasis, psoriasis, seborrheic dermatitis, inflammatory bowel disease or even sexual abuse, it remains an underdiagnosed entity. This situation leads to delayed diagnosis and treatment which in turn might increase the frequency of secondary complications related to streptococcal infections (i.e., post-streptococcal acute nephritis and rheumatism, guttate psoriasis, etc.).

Acyclovir-resistant varicella infection with atypical lesions in a non-HIV leukemic infant.

UNLABELLED: An HIV-negative infant presented with VZV primary infection during the maintenance therapy for megakaryoblastic leukaemia. The lesions were initially vesicular and necrotic but became verrucous and hyperkeratotic. A clinical resistance to acyclovir was suspected and confirmed by histologic and virologic studies. The patient was successfully treated by foscarnet. CONCLUSION: resistance of VZV to acyclovir may occur after a short treatment in a non-AIDS patient.

[Ofuji's papulo-erythroderma: treatment with cyclosporin A]. / Papulo-érythrodermie d'Ofuji: traitement par ciclosporine-A.

BACKGROUND: Papuloerythroderma of Ofuji is an uncommon condition characterized by diffuse pruriginous eruptions of infiltrating papulae. The large folds are not involved. The eruptions are associated with lymphopenia and eosinophilia. Very few cases have been reported in the literature and because of their heterogeneous nature, there is some debate as to whether this is a single clinical entity or a particular presentation of erythrodermia; Immunomodulation is recommended. CASE REPORT: A 73 year-old man of asian origin living in France presented chronic pruriginous erythrodermia with flat purplish-brown confluent papulae which did not involve the large folds. Eosophilia and lymphopenia were present. The biopsy specimen evidenced dermal infiltration with CD4+CD45+RO+ T cells, eosinophils and DC1a+ dendritic cells. Further explorations did not reveal any sign favoring lymphoma, carcinoma or underlying infection. Dermocorticoids, PUVA-therapy and interferon-alpha were ineffective. Considerable clinical improvement was obtained with cyclosporine A. DISCUSSION: We used ciclosporine A in our patient after repeated failure of other therapies reported to be effective in this dermatosis. Despite the favorable outcome, this therapeutic approach should be used with prudence.

Coagulation factor XIII in scleroderma.

The ability of blood coagulation factor XIII (FXIII) to affect collagen synthesis and degradation led to its use in the treatment of scleroderma. Encouraging initial results were achieved principally in terms of skin sclerosis, musculoskeletal involvement and weakness. Further assessment of this treatment in scleroderma was abandoned when, following the HIV epidemic, FXIII use became strictly regulated. Safer concentrates are now available which may allow us to reconsider this therapy. This paper, which briefly reviews available data related to FXIII use in scleroderma and which proposes general rules for prescribing, is aimed at generating an open debate as to the need to widen the regulated use of FXIII to scleroderma.