Idiopathic facial aseptic granuloma in children: Management and long-term follow-up.

AIMS: The primary objective was to study long-term outcomes in children with idiopathic facial aseptic granuloma (IFAG). The secondary objectives were to describe the clinical polymorphism of this entity, the different therapeutic options, and the occurrence of rosacea in this population. METHODS: We selected patients with a clinical diagnosis of IFAG seen in two different hospitals in Normandy between March 2016 and February 2021, and we collected clinical and therapeutic data from computerized medical records. A remote consultation based on recent photographs was performed to collect new data, and the children's parents were asked to complete a questionnaire between February and August 2021. RESULTS: Ten children were included in this study. Nine patients presented with a single red to purplish nodule. One patient presented multiple papulopustular plaques. IFAG healed spontaneously without major sequelae, and this outcome was not influenced by any treatments. During follow-up, two patients developed childhood rosacea. CONCLUSION: Although some clinical heterogeneity exists, early diagnosis and follow-up of IFAG is necessary to avoid unnecessary topical or systemic antibiotic treatments that do not shorten the disease course. IFAG appears to be a possible mode of entry for infantile rosacea.

[A case of "juvenile" self-healing cutaneous mucinosis in an adult]. / Mucinose cutanée « juvénile ¼ auto-involutive chez l'adulte.

INTRODUCTION: Self-healing juvenile cutaneous mucinosis (SHJCM) is a stereotypical disease in children characterized by the acute onset of subcutaneous papules and nodules on the face, dorsum of the hands and peri-articular regions that disappear spontaneously within a few months or years. A few cases have been reported in adults, but these display more heterogeneous clinical and histopathological features. Herein we report a case with a juvenile clinical presentation in an adult woman. OBSERVATION: A 36-year-old patient with a history of Von Willebrand disease was referred to our dermatology department following the rapid development of subcutaneous nodules on her face, hands and large joints, together with periorbital edema. Three nodules were surgically removed and histology demonstrated mucin deposition in the dermis with dissociation of collagen fibers. Autoimmune disease, neoplasia, infection and dysthyroidism were ruled out. Bilateral carpal tunnel syndrome was confirmed by electromyogram in this patient carrying out manual work. Treatment with hydroxychloroquine proved unfruitful. After 1.5 years of follow-up, her lesions showed partial regression. CONCLUSION: The form of SHJCM described in pediatric populations may occur in rare cases in adults. Should the name of juvenile mucinosis still be used in this event?

[Extensive connective tissue nevus in children]. / Hamartome conjonctif étendu de l'enfant.

BACKGROUND: Connective tissue nevus (CTN) is a rare condition of the extracellular matrix components that generally presents as papulae of normal skin colour. This condition may be syndromic or sporadic. PATIENTS AND METHODS: We report herein two isolated cases of extensive and infiltrative CTN in children at risk for subsequent joint stiffening. The pathology samples displayed respectively mixed hamartoma and a collagenoma. DISCUSSION: The onset of these lesions is often difficult to establish, since they are usually unnoticeable at first. When confronted with extensive CTN, the main differential diagnoses are eosinophilic fasciitis and morphea, and these must be ruled out by skin biopsy. CTN is associated with osteopoikilosis in Buschke-Ollendorf syndrome. Skeletal lesions are asymptomatic and are detected by means of iterative X-ray. Their management comprises symptomatic care.

[Optimization of the purification of human plasma fibronectin by double affinity chromatography using gelatin and heparin Trisacryl LS]. / Optimisation de la purification de la fibronectine plasmatique humaine par double chromatographie d'affinité sur gélatine et héparine Trisacryl LS.

The authors described an optimized method of preparation to obtain pure human plasma fibronectin. The new performances related to the chromatographic and concentration steps, where the saving of time is about 40% and the lyophilization step where the saving of energy is also about 40%. The final product is without any less of either physical and biological activities. The size of the columns, the volumes of the chromatographic supports (gelatin and heparin-Trisacryl LS) and the quantity of the treated plasma are very much important and also the quantities of the final product are very increased.