[An atypical case of systemic juvenile idiopathic arthritis]. / Un cas atypique d'arthrite juvénile idiopathique systémique.

The systemic juvenile idiopathic arthritis (SJIA) is a rare, auto-inflammatory and chronic childhood disease. Arthritis of at least one joint is associated with a daily fever lasting more than two weeks and with one of the following signs: lymphadenopathy, hepatomegaly, serositis, or skin rash. Systemic symptoms are often initially preponderant, in the absence of arthritis than can occur weeks or months later. The typical rash is maculopapular discrete, fleeting and dew. We present an unusual SJIA case, where the rash, like urticaria, presents itself as persistent, itchy hives. An arthritis of the elbow appeared only one month after the onset of the disease. This case illustrates the difficulty of diagnosis, which can only be made after the exclusion of more common (infectious) and severe (hematooncology) diseases.

L'arthrite juvénile idiopathique systémique (AJIS) est une maladie rare, auto-inflammatoire et chronique de l'enfant. Le tableau associe la présence d'une arthrite touchant au moins une articulation, à une fièvre quotidienne d'une durée minimum de 2 semaines et à l'un des signes suivants : adénopathies, hépatomégalie, sérosite ou éruption cutanée. Les symptômes systémiques sont souvent prépondérants initialement, en l'absence d'arthrite avérée qui peut n'apparaître qu'après plusieurs semaines ou mois. L'éruption typique est maculo-papuleuse rosée discrète et fugace. Nous présentons un cas atypique d'AJIS, dont l'éruption se présente comme une urticaire persistante et prurigineuse. Une arthrite du coude n'est apparue qu'un mois après le début de l'affection. Ce cas illustre la difficulté du diagnostic qui ne peut être posé qu'après l'exclusion de pathologies plus courantes (infectieuses) et plus graves (hémato-oncologiques).

[Palmoplantar neutrophilic eccrine hidradenitis with general extension in a child in remission after acute lymphoblastic leukemia]. / Hidradénite eccrine neutrophilique palmo-plantaire avec extension généralisée chez un enfant en rémission d'une leucémie aiguë lymphoblastique.

BACKGROUND: Neutrophilic eccrine hidradenitis (NEH) is a form of neutrophilic dermatitis characterized by tender erythematous and painful papules involving the trunk, extremities and face. The generalized form is associated with malignant hemopathies. The palmoplantar form occurs in children without any context of malignancy. Histology shows a neutrophilic infiltrate surrounding and infiltrating the eccrine glands associated with vacuolar degeneration and necrosis of the epithelial secretory portion. PATIENTS AND METHODS: We report the case of a 4-year-old girl with palmoplantar HEN progressing to the generalized form while in remission from acute lymphoblastic leukemia. DISCUSSION: Progression of HEN from the palmoplantar form to the generalized form has never been published previously in the literature.

Nail destruction in pemphigus vulgaris.

Involvement of the nails in pemphigus vulgaris (PV) is rare and is usually seen when the disease is severe. The most common clinical manifestations are chronic paronychia and onychomadesis. Finger nails are more frequently involved than toe nails. We report a case of severe and persistent PV in which an exacerbation was preceded by an erosive and destructive lesion of the right hallux nail unit. A nail bed biopsy was performed to rule out a subungual tumour. The image of suprabasal acantholysis yielded the diagnosis of PV. This localisation and clinical manifestation of PV have only been reported once.

A case of early congenital syphilis.

A case of early congenital syphilis is reported in order to emphasize that syphilis is still present nowadays and that detection of syphilis in pregnant women is primordial for the prevention of this disease.