[Beyond actinic keratoses: Field cancerization of the skin]. / Au-delà des kératoses actiniques, le champ de cancérisation cutané.

Lesions occurring in actinic keratoses (AK) form erythematous, squamous, crusty and keratotic papules that appear on skin chronically exposed to the sun due to ultraviolet radiation. They are formed by the proliferation of atypical keratinocytes limited to the epidermis and may progress to squamous cell carcinoma in situ and to cutaneous squamous cell carcinoma (CEC). Although low, the metastatic risk associated with the CEC is not negligible. The concept of field cancerization was introduced in 1953 following studies of neoplastic lesions of the oral mucosa. A cancer field is a normal-looking pre-tumoral zone with subclinical, multifocal anomalies, which may constitute a base for new neoplastic lesions. Such fields are frequently seen in areas of photo-exposed skin and around the edges of AK and CEC. In this event, treatment should not be limited to visible or palpable AK lesions, and if a cancer field is suspected, treatment involving the physical destruction or elimination of atypical keratinocytes from the entire area should be considered. Such an approach may improve the long-term prognosis, reduce treatment costs and ensure optimal cosmetic outcome.

Patient and physician satisfaction in an observational study with methyl aminolevulinate daylight photodynamic therapy in the treatment of multiple actinic keratoses of the face and scalp in six European countries.

BACKGROUND: Guidelines recommend treating actinic keratoses (AKs) as they are recognized as precursors of invasive squamous cell carcinoma. OBJECTIVE: The objective of this study was to collect real-world clinical data on the use of methyl aminolevulinate daylight photodynamic therapy (MAL DL-PDT) for the treatment of face and scalp AK in Europe. METHODS: A prospective, multicenter, non-interventional study was conducted in six European countries in patients receiving a single treatment of MAL DL-PDT for face and/or scalp AK. Patient-reported outcomes were assessed by patient questionnaires at baseline and at 3 months after treatment, efficacy was assessed at 3 months using a 6-point global improvement scale, and adverse events (AE) were recorded at each visit. RESULTS: Overall, 325 patients were enrolled from 52 investigational centres, 314 of whom attended the 3-month visit. Most patients had multiple lesions (58.4% had >10 lesions) with lesions mainly located on the scalp (60.0%) and/or forehead (54.2%). AKs were predominantly grade I (39.4%) or grade II (33.2%), and 10.5% of patients had grade III lesions. The proportions of patients and physicians that were overall satisfied to very satisfied with the MAL DL-PDT treatment were 80.4% and 90.3%, respectively. The vast majority of patients (90.0%) would consider using MAL DL-PDT again if needed. Physician-assessed efficacy at 3 months was at least much improved in 83.5% of patients, with 45.9% of patients requiring no retreatment. Related AEs were reported in 15% of patients. CONCLUSION: Use of MAL DL-PDT for multiple face and/or scalp AKs resulted in high levels of patient and physician satisfaction in clinical practice in Europe, reflecting the good efficacy and high tolerability of this convenient procedure.

[Localized eruptive juvenile xanthogranuloma]. / Xanthogranulome juvénile éruptif localisé

BACKGROUND: Juvenile xanthogranuloma (JXG) is a non-Langerhans histiocytosis of young children characterized by solitary or multiple yellowish cutaneous nodules. Atypical skin lesions such as lichenoid eruptions, and pedunculated, maculopapular, plaque-like or linear lesions have been described. We report a case of eruptive XGJ en plaque in the left leg in an infant. PATIENTS AND METHODS: A 13-month-old child presented asymptomatic eruptive, yellowish papules of the leg measuring 5 to 10mm since the age of 2months. There was no cutaneous infiltration between the lesions. Darier's sign was negative. Histological examination confirmed the diagnosis of JXG. The course of the disease comprised a gradual decrease in the number of active lesions with slight residual pigmentation. DISCUSSION: Our case was suggestive of JXG en plaque. Only 7 cases have been reported in the literature, all appearing before the age of 5months. The lesions corresponded mostly to an asymptomatic erythematous plaque studded with small yellowish/red nodules of variable localisation. Spontaneous involvement was noted in all cases. No systemic involvement was found. Herein we present a unique case of localised multiple JXG without evident clinical infiltrating plaque progressing with self-resolving flares.

Childhood acquired epidermolysis bullosa.

We present the ninth case of epidermolysis bullosa acquisita (EBA) reported in children. As in most of the other childhood cases, the 7-year-old boy described herein had an acute, widespread, inflammatory vesiculobullous eruption with oral involvement. Indirect immunofluorescence on salt-split skin as well as Western immunoblot confirmed the diagnosis of EBA. The patient responded to combined prednisone and dapsone, and was maintained with dapsone alone.

[Subcutaneous rhabdomyosarcoma in children. Clinical, immunologic and ultrastructural aspects]. / Rhabdomyosarcome sous-cutané de l'enfant. Aspects cliniques, immunologiques et ultrastructuraux.

Embryonal rhabdomyosarcoma is the most frequent of tissue sarcomas in children. Its location in the subcutaneous tissue makes it a dermatological diagnosis. In childhood, the exact histological type of the tumour, sometimes difficult to determine, is absolutely necessary since prognosis and treatment differ according to the histogenetic form. Rhabdomyosarcoma is rare in adults. One must rule out malignant pleomorphic histiocytoma which has a more favourable prognosis. Among the antisera recently made available, those directed against desmin, foetal skeletal myosin and/or specific skeletal muscle myofilament seem to be most useful when associated with the anti-myoglobin antibody.

[Subcutaneous sacrococcygeal ependymoma]. / Ependymome sous-cutané sacrococcygien.

The main clinical manifestations and the natural course of subcutaneous ependymoma are reviewed in connection with a sacrococcygeal lesion in a 12 year-old girl. After complete resection, the outcome is usually favorable but the 20% risk of metastasis justifies a close surveillance.