[Polymorphism and differential diagnosis of neonatal lupus erythematosus]. / Polymorphisme et diagnostics différentiels du lupus érythémateux néonatal.

INTRODUCTION: Neonatal lupus erythematosus (NEL) is a rare condition secondary to transplacental transfer of maternal anti-nuclear antibodies, generally anti-Ro/SSA. The most common signs are dermatological and cardiac. The most frequently reported clinical association is periorbital erythema, known as "owl eye", and bipolar erythematous maculopapular plaques with fine scales. However, many semiological variants can result in diagnostic errors or delays. PATIENTS AND METHODS: This was a single-centre retrospective observational study collating all cases of NEL seen at paediatric dermatology consultations between 2010 and 2018. The diagnosis of NEL was confirmed by the presence of specific antinuclear antibodies (ANA) in the mother. The aim was to describe the different clinical forms of NEL and to discuss differential diagnosis. RESULTS AND DISCUSSION: We identified ten cases of NEL, all addressed without diagnosis or with misdiagnosis. They were divided into 3 groups based on the semiology of skin lesions: 5 presented inflammatory macular papules on the cephalic extremity and head; 3 presented acquired periorbital depigmentation; 2 presented atrophic and diffuse livedoid lesions. None had heart disease and associated haematological and hepatic damage was mild. Spontaneous remission was seen in all cases before the age of 6 months. The mothers, who were generally symptom-free or paucisymptomatic, presented anti-Ro/SSA NAAs. CONCLUSION: Recognition of the different clinical forms of NEL enables early institution of suitable therapy and monitoring of subsequent pregnancies.

[Children with psoriasis in secondary care: Clinical aspects and comorbidities diverge from the generally published data]. / Psoriasis de l'enfant vu en milieu libéral : les aspects cliniques et épidémiologiques diffèrent des données habituellement publiées.

BACKGROUND: Psoriasis affects 0.2-0.7 % of children and is associated with obesity. Published studies have been conducted in hospital settings (tertiary care). The PsoLib study evaluated childhood psoriasis in private practice (secondary care) in terms of epidemiology, clinical aspects and comorbidities. PATIENTS AND METHODS: This was a non-interventional, cross-sectional, multicenter study of children with psoriasis performed by 41 dermatologists working in private practice. The clinical and therapeutic aspects and comorbidities were systemically evaluated. We compared data to the χ-Psocar study performed in hospitals using the same methodology. RESULTS: In all, 207 children (girls: 60.4 %; mean age: 10.5±4.2 years) were included. Scalp psoriasis (40.6 %) was the most frequent clinical type, while plaque psoriasis represented 26 % of cases. Nail, tongue, and arthritic involvement were rare. Less than 1 % of children suffered from hypertension, diabetes or dyslipidemia, but 16.4 % were overweight and 7.0 % were obese. Severity (PG≥4 at peak) was associated with excess weight (P=0.01). CONCLUSION: Scalp psoriasis is the most frequent clinical type of psoriasis in childhood. Comorbidities and extracutaneous localization are rare. Even in private practice, the severity of the disease is associated with excess weight.

Travelers with cutaneous leishmaniasis cured without systemic therapy.

BACKGROUND: Cutaneous leishmaniasis (CL) is a disfiguring but not life-threatening disease. Because antileishmanial drugs are potentially toxic, the World Health Organization (WHO) recommends simple wound care or local therapy as first-line treatment, followed or replaced by systemic therapy if local therapy fails or cannot be performed. METHODS: To determine the feasibility and impact of the recommended approach, we analyzed the results of a centralized referral treatment program in 135 patients with parasitologically proven CL. RESULTS: Infections involved 10 Leishmania species and were contracted in 29 different countries. Eighty-four of 135 patients (62%) were initially treated without systemic therapy. Of 109 patients with evaluable charts, 23 of 25 (92%) treated with simple wound care and 37 of 47 (79%) treated with local antileishmanial therapy were cured by days 42-60. In 37 patients with large or complex lesions, or preexisting morbidities, or who had not been cured with local therapy, the cure rate with systemic antileishmanial agents was 60%. Systemic adverse events were observed in 15 patients, all receiving systemic therapy. CONCLUSIONS: In this population of CL patients displaying variable degrees of complexity and severity, almost two-thirds of patients could be initially managed without systemic therapy. Of these, 60 were cured before day 60. The WHO-recommended stepwise approach favoring initial local therapy therefore resulted in at least 44% of all patients being cured without exposure to the risk of systemic adverse events. Efforts are needed to further simplify local therapy of CL and to improve the management of patients with complex lesions and/or preexisting comorbidities.

[Facial cellulite associated with mandibular osteomyelitis in an infant]. / Cellulite faciale révélatrice d'une ostéomyélite mandibulaire chez un nourrisson.

INTRODUCTION: The discovery of a jugular tumefaction in an infant evokes several diseases. We report the case of a 4-month-old infant whose jugular cellulite revealed mandibular osteomyelitis. CASE REPORT: A 4-month-old boy was referred for hard, hot tumefaction of the right cheek and multiple cervical adenopathies. The suggested diagnosis was cellulite of cutaneous origin. He presented 21 900/mm(3) hyperleukocytosis associated with an inflammatory biological syndrome. Standard x-ray of the facial mass was normal. Sonography of the face showed thickening of the soft subcutaneous tissues and retro and sub-mandibular adenopathies with abcedation. Antibiotherapy with amoxicillin and clavulanic acid led to rapid improvement. Three days after withdrawal of the antibiotherapy, the tumefaction recurred without fever. A facial scan eliminated cystic lymphangioma and showed osteolysis of the external plateau of the ascending branch of the mandible with periosteal appositions. Histological examination of a surgical bone biopsy showed infectious osteitis and culture revealed hemolytic beta streptococci. Six weeks of antibiotherapy (initially with amoxicillin and gentamycin, then amoxicillin in monotherapy) led to the regression of all cutaneous signs. COMMENTS: When confronted with a tumefaction in this area, malignant or benign tumoral causes such as cystic lymphangioma must be eliminated. Infectious causes (abscess, parotid inflammation and osteomyelitis) must be evoked and distinguished from infantile cortical hyperostosis (Caffey-Silverman's syndrome). Standard radiological imaging, scan or scintigraphy are useful diagnostic tools. If osteolysis is discovered, a biopsy must be taken for anatomopathological and biological examination.

[Cutaneous hydration of the premature and new born]. / Hydratation de la peau du nouveau-né, du prématuré

The barrier function of the stratum corneum is a fundamental element in maintaining cutaneous hydration. Alteration in the stratum corneum leads to the loss of this barrier function, with increased transdermal water loss, decreased water content and installation of xerosis. Moisturizers correct cutaneous xerosis by restoring the stratum corneum. In the new born, the architectural and biochemical structure of the stratum corneum is identical to that of infants and adults. However, 60 p. cent of new born exhibit physiologic desquamation, demonstrating a transitory functional deficit in the stratum corneum, justifying emollient treatment. After this physiological desquamation period, emollients are reserved for atopic infants or those presenting keratinization disorders. In the premature, the epidermis and particularly the stratum corneum are immature; trans-epidermal water loss is elevated and the skin is dry or even fissural. The interest of applying emollients to the skin of premature new born was recently demonstrated. Emollients improved the aspect of the skin and also decreased the number of infectious episodes.

[Pemphigoid gestationis: treatment by topical class I corticosteroid]. / Pemphigoïde de la grossesse: traitement par corticothérapie locale de classe I.

BACKGROUND: Systemic corticosteroid is the main treatment of severe forms of pemphigoid gestationis. We report a case of generalised pemphigoid gestationis successfully treated with very potent topical corticosteroid. CASE: A 37-year-old woman developed during her third pregnancy with a new partner an urticated generalised eruption associated with bullous lesions. The diagnosis of pemphigoid gestationis was confirmed by direct immunofluorescence which detected a linear C3 deposition along the basement membrane zone and the positivity of Herpes Gestationis Factor (10 units). Local treatment with potent corticosteroid (betamethasone dipropionate 0.05 p. 100) failed and the patient was successfully treated by clobetasol propionate 0.05 p. 100 cream. The infant, in good health, was not delivered prematurely. DISCUSSION: Severe form of pemphigoid gestationis are currently treated with 0.5 to 1 mg/kg/day of systemic corticosteroids, with maternal and pediatric possible side effects. As in bullous pemphigoid, this observation underlines the efficiency and good tolerance of very potent corticosteroid in severe forms of pemphigoid gestationis.

High levels of patched gene mutations in basal-cell carcinomas from patients with xeroderma pigmentosum.

Recently, hptc, a human gene homologous to the Drosophila segment polarity gene patched (ptc), has been implicated in the nevoid basal-cell carcinoma (BCC) syndrome, and somatic mutations of hptc also have been found in sporadic BCCs, the most frequent cancers found in the white population. We have analyzed the hptc gene, postulated to be a tumor suppressor gene, in 22 BCCs from patients with the hyperphotosensitive genodermatosis xeroderma pigmentosum (XP). Patients with XP are deficient in the repair of UV-induced DNA lesions and are characterized by their predisposition to cancers in sun-exposed skin. Analysis using PCR-single-strand conformation polymorphism of the hptc gene identified 19 alterations in 16 of 22 (73%) of the BCCs examined. Only two (11%) deletions of the hptc gene were found in XP BCCs compared with >30% rearrangement observed in non-XP sporadic BCCs, and 17 of 19 (89%) were base substitutions. Among the 17 base substitutions, 11 (65%) were CC --> TT tandem mutations, and 4 (23%) were C --> T substitutions, all targeted at bipyrimidine sites. Hence, a significantly higher number (15 of 19; 79%) of UV-specific alterations are seen in XP tumors, in contrast to non-XP sporadic BCCs. Interestingly, we have found that in 7 of 14 (50%) XP BCCs analyzed, both hptc and the tumor suppressor gene p53 are mutated. Not only have our data indicated the key role played by hptc in the development of BCCs, they also have substantiated the link between unrepaired UV-induced DNA lesions and skin carcinogenesis, as exemplified by the UV-specific alterations of different genes in the same tumors.