Development and co-construction of a therapeutic patient education program for albinism.

BACKGROUND: Long-term and ongoing support in accordance with the changing needs of patients and their families is one of the main components of patient care, including therapeutic patient education (TPE). OBJECTIVE: To co-construct a TPE program for albinism with all those involved in the management of albinism patients. METHODS: Eight steps have been defined for the co-construction process: 1) identify all the relevant experts and invite them to participate in the construction of a TPE program to improve care for and support of patients with albinism, 2) review and analyse all publications regarding TPE for albinism, 3) conduct semi-structured interviews with the patients' parents, 4) conduct brainstorming meetings with the participating experts for an exchange of experience and expertise, 5) elaborate the program's concrete content with the experts, 6) draw up a TPE skills checklist, 7) create TPE educational tools to facilitate learning, 8) review and summarize each step of the co-construction protocol. RESULTS: Co-construction of a TPE program for children, adolescents, and young adults with albinism, and their parents. CONCLUSION: Strengths and advantages of the co-construction process include: i) highlighting of the experiential knowledge mentioned in the repository, ii) multiplicity of points of view and perspectives, iii) rapid improvement in TPE training both for the association and the patients, iv) awareness of the shift caregivers' position with regards to TPE and recognition of the polysemy of their discourse. The TPE program for albinism has been authorized since 2018.

[MALT (Mucosal Associated Lymphoid Tissue) lymphoma of the palpebral conjunctiva]. / Lymphome B de la zone marginale de type MALT de la conjonctive palpébrale.

BACKGROUND: MALT (Mucosal Associated Lymphoid Tissue) lymphomas of the conjunctiva belong to the extranodal marginal zone B-cell lymphomas. This site, while standard, is uncommon. CASE-REPORT: A pink papular tumor developed on the lower eyelid of a 59-year-old woman. Sarcoidosis was diagnosed 9 years earlier associated with mediastinal lymphadenopathy and erythema nodosum not requiring treatment. Histological examination yielded a diagnosis of conjunctival MALT lymphoma. No visceral involvement was demonstrated. Radiotherapy (30 Gy) induced a complete response. A remote lesion developed on the patient's arm 18 months later. No other sites were found. Further radiotherapy (26 Gy) again induced complete remission. No new lesions were seen after 24 months of follow-up. DISCUSSION: This case is interesting because of the association of a MALT lymphoma and previous sarcoidosis, described in the literature as "sarcoidosis-lymphoma syndrome". Association of sarcoidosis with MALT lymphoma is infrequent. Treatment of conjunctival MALT lymphoma is standardized. Radiotherapy offers excellent efficacy and is well tolerated at this site. Regular and long-term follow-up is required. Local and distant relapse can occur.

Loss of containment: experimental aerosol rain-out assessment.

We measured the temperature profiles and rain-out spatial distribution for flashing water jets generated from a pilot scale experimental setup. This allowed us to define the transitions between three types of jets (stable, mechanically fragmented, flashing). The present experimental data when compared to other authors' data show that the transition to flashing type occurs at lower superheat when the orifice length increases, and that homogeneous aerosol behavior could be a valid assumption for mechanically fragmented jets whereas it is not for flashing jets.

[Epithelioma cuniculatum. Apropos of a case with review of the literature]. / L'epithélioma cuniculatum. A propos d'un cas, avec revue de la littérature.

A case of Epithelioma cuniculatum arising on a leg ulcer in a 85 years old lady is described. 102 cases have been reported before, mainly located on the sole of the foot: this rare tumor may be recognized by the gross appearance of a slow-growing exophytic cauliflower growth, exuding foul smelling material from numerous sinuses, which eventually invades underlying tissue sometimes including bone, and which rarely metastasizes to the regional lymph nodes. Histological examination shows a well-differentiated squamous epithelial proliferation without cellular atypia, with numerous crypts and sinuses. The diagnosis is difficult as the cytological regularity does not allow malignancy to be evoked. It is the conspicuity of the invasion of the deep tissues with sufficiently large samples that makes it certain. The differential diagnosis includes keratoacanthoma, pseudo-epitheliomatous hyperplasia mainly represented by pyoderma vegetans and "classical" squamous cell carcinoma. From a nosological point of view the authors totally assimilate Epithelioma cuniculatum and Gottron's papillomatosis cutis carcinoïdes regrouped under the name of cutaneous verrucous carcinoma, emphasizing the analogy with verrucous carcinomas of mucous membrane, giant condyloma of Buschke-Lowenstein, oral florid papillomatosis.

[About 2 cases of "dry syndrome" associating xerophthalmy, xerostomy and cutaneous dryness. A new entity or an unrecognized diagnostic? (author's transl)]. / Sur deux cas de "syndrome sec" associant xérophtalmie, xérostomie et sécheresse cutanée. Entité nouvelle ou diagnostic méconnu?

Two cases, in children of distinct families, of a particular form of "dry syndrome", are described. This syndrom, which associates xerophthalmy, xerostomy and cutaneous dryness, is congenital and familial. He looks different from previously described diseases or syndroms which include one or several of these three components.