Valérie Dekeuleneer

Chromate or titanium allergy – the role of impurities?

The prevalence of metal allergy is substantial in the general population, and is rising steadily within the ageing population requiring medical devices (1). Nickel, cobalt and chromium are the classic contact allergens both in adults and in children (1, 2), and titanium is recommended as an alternative because of its excellent biocompatibility, high corrosion resistance, and inert character in the human body. Allergic contact dermatitis caused by titanium has only ever been very rarely documented. We report a case of a positive patch test reaction to titanium nitride in an 8-year-old girl suffering from shoe dermatitis. Even if the sensitization route remains unclear, it could be related to impurities, such as chromium contained in the commercial allergen.

Eight Novel Mutations Confirm the Role of AAGAB in Punctate Palmoplantar Keratoderma Type 1 (Buschke-Fischer-Brauer) and Show Broad Phenotypic Variability

Punctate palmoplantar keratoderma (PPKP1; Buschke-Fischer-Brauer) is a rare autosomal dominant inherited skin disease characterized by multiple hyperkeratotic papules involving the palms and soles. Mutations have been found at 2 loci, on chromosomes 15q22-15q24 and 8q24.13-8q24.21. We recently identified mutations in 3 families, in the AAGAB gene on 15q, which encodes the alpha- and gamma-adaptin-binding protein p34. The current study examined 14 additional families, comprising a total of 26 affected individuals and identified 8 novel mutations in 9 families. In one family a mutation that was present only in the affected individuals was found, and in 4 other families, previously reported mutations were found (1, 2). These results confirm the role of AAGAB in PPKP1. Our findings suggest that there is no correlation with age, but with mechanical factors. No additional obvious genotype-phenotype correlation was observed, even when comparing different types of mutations. Rather, identical genotypes presented a very broad interfamilial and intrafamilial variability of phenotypes.

Allergic contact dermatitis caused by a nursing comfort balm: methyl glucose dioleate as the sensitizing ingredient

Methyl glucose dioleate (MGD; CAS 86893-19-8), the diester of methyl glucoside and oleic acid, is used as a water-in-oil emulsifier, emollient, humectant and skin conditioning agent in cosmetics, and may also be present in topical pharmaceutical products. Only a few cases of contact dermatitis caused by this substance have been observed. We here report a case of allergic contact dermatitis caused by MGD from a balm used during breastfeeding.

Langerhans cell histiocytosis involving the skin and the thymus.

Langerhans cell histiocytosis (LCH) is a rare neoplastic proliferation of dendritic cells resembling Langerhans cells, which can accumulate in various organs. Thymic involvement (TI) is rare, probably underestimated, and its management and prognosis are not well established. We report a 5-month-old male infant who presented with asymptomatic skin lesions on the scalp and trunk (figure 1A). Histology and immunohistochemistry of one of the trunk lesions showed a dermal and epidermal infiltration with [...]

Angiosarcoma arising from congenital primary lymphedema

We herein report the case of a 3‐year‐old girl with atypical congenital right upper limb lymphedema who developed an angiosarcoma. Only a few cases have been reported following congenital form of lymphedema and only 4 in such a young child. We also summarize all cases of angiosarcoma associated with congenital lymphedema reported in the literature.

Schnitzler syndrome associated with hairy cell leukemia presenting with chronic urticaria and arthralgias

CRP: C-reactive protein IL-1: interleukin-

F3 : Éruption bulleuse associée à un lymphome T angio-immunoblastique

Introduction Le lymphome T angio-immunoblastique (LTAI) est une forme rare et agressive de lymphome T peripherique. Il est caracterise par des symptomes generaux, des adenopathies diffuses, une hepatosplenomegalie et une hypergammaglobulinemie polyclonale. 50% des LTAI sont accompagnes de symptomes cutanes. Quelques cas d’eruption bulleuse paraneoplasique associee a un LTAI ont ete decrits. Observation Un homme de 70 ans est admis aux urgences en raison d’une eruption eczematiforme du corps associee a des necroses des ailes du nez. Le lendemain, il developpe des bulles tendues du scrotum et de la langue. Les lesions s’etendent rapidement a l’ensemble du corps. Ce patient est connu des hematologues pour un LTAI stade IVBb, traite par une cure de polychimiotherapie de type CHOP associee au lenalidomide. Plusieurs biopsies sont realisees : Un infiltrat dermique et hypodermique dense de lymphocytes atypiques a disposition peri-vasculaire et interstitielle est present a l’histologie des lesions eczematiformes et necrotiques ; le meme type d’infiltrat, surmonte par un decollement bulleux sous-epidermique, est mis en evidence a l’histologie des lesions bulleuses ; d’importants depots d’IgA le long de la jonction dermo-epider- mique (JDE), en signal intense, epais et continu, sont constates a l’immunofluorescence en peau peribulleuse. Ces elements permettent d’eliminer le diagnostic de toxidermie bulleuse initialement suspecte, et orientent vers une atteinte cutanee du LTAI avec dermatose a IgA lineaire paraneoplasique. L’absence de reponse a la premiere cure de chimiotherapie impose un changement de traitement du lymphome (gemcitabine associee a des corticoides) qui ameliore les symptomes cutanes malgre l’absence d’effet benefique sur l’etat general du patient. Discussion Les manifestations cutanees accompagnent 50% des LTAI. Outre l’eruption maculopapuleuse la plus souvent decrite, des placards erythemato-squameux, des lesions papulovesiculeuses prurigineuses, du purpura ou encore des lesions urticariennes sont egalement possibles. Quatre images histologiques sont decrites : infiltrat dense, pleomorphe, de lymphocytes atypiques du derme superficiel et profond avec des hyperplasies vasculaires ; infiltrat perivasculaire leger avec quelques lymphocytes atypiques ; infiltrat superficiel aspecifique ; vasculite leucocytoclasique. La premiere dominait largement chez ce patient. Les cas de manifestations cutanees avec depots d’IgA ne sont que rarement decrits, et seulement 2 cas de decollement sous-epidermique avec depots lineaires d’IgA au niveau de la JDE ont, a notre connaissance, ete rapportes. Conclusion La dermatose a IgA lineaire surmontant un infiltrat dermique de lymphocytes atypiques est une forme rare de manifestation cutanee associee a un LTAI. Notre patient a presente une forme a debut brutal et extension rapide, amelioree par la chimiotherapie et les soins locaux, malgre la progression du lymphome sous-jacent.