F. Franck

Cutaneous lymphoma associated with Epstein-Barr virus infection in 2 patients treated with methotrexate.

Whether patients with rheumatoid arthritis (RA) have an increased risk of developing non-Hodgkin lymphoma is controversial, and opinions differ on the possible role of methotrexate in the occurrence of lymphomas in patients with RA. We report 1 T-cell lymphoma and 1 B-cell lymphoma restricted to the skin associated with Epstein-Barr virus infection that healed completely and spontaneously after discontinuation of methotrexate in a man with RA and a woman with dermatomyositis. Cutaneous infiltrating cells were infected by a replicative form of Epstein-Barr virus. After discontinuation of methotrexate, the cutaneous lesions disappeared completely in 15 days without recurrence. Discontinuation of methotrexate is necessary in patients with RA or dermatomyositis who have a lymphoproliferative disorder, and a follow-up period of several weeks should be observed before specific therapy is initiated.

Aggressive epidermotropic cutaneous CD8+ lymphoma: a cutaneous lymphoma with distinct clinical and pathological features. Report of an EORTC Cutaneous Lymphoma Task Force Workshop.

Aggressive epidermotropic cutaneous CD8+ lymphoma is currently afforded provisional status in the WHO classification of lymphomas. An EORTC Workshop was convened to describe in detail the features of this putative neoplasm and evaluate its nosological status with respect to other cutaneous CD8+ lymphomas.

Azo pigments and quinacridones induce delayed hypersensitivity in red tattoos.

Induction of delayed hypersensitivity reactions by red tattoos has been occasionally reported. Little is known about the inks used. Azo pigments have been implicated in some instances, but there is only one reported case involving quinacridones.

Ichthyosiform mycosis fungoides

Malignancy-associated acquired ichthyosis is well known, but the ichthyosiform subset of mycosis fungoides (MF) is rarely reported. We report on two patients with a clinical presentation for whom diagnosis of mycosis fungoides was established on histological grounds. In both cases, long term remission was obtained with non aggressive therapies. This rare condition must be added to newly described forms of MF with epidermal hyperplasia such as keratosis lichenoides chronica like MF and pilotropic MF.

Dermopathie néphrogénique fibrosante traitée par photochimiothérapie extracorporelle : rôle du gadolinium ?

Resume Introduction La dermopathie nephrogenique fibrosante est une sclerose cutaneo-systemique touchant principalement l’insuffisant renal. Observation Nous rapportons l’observation d’un homme de 68 ans, insuffisant renal transplante et dialyse qui developpait un syndrome arthro-cutane severe. Il avait recu deux injections de gadolinium (gadodiamide, Omniscan ® ) lors d’angio-IRM. La biopsie cutanee confirmait le diagnostic de dermopathie nephrogenique fibrosante. Le malade etait traite par corticotherapie orale puis par photopherese extracorporelle. Une amelioration franche etait observee apres 12 seances. Discussion Le traitement de la fibrose nephrogenique systemique n’est pas codifie. Il fait generalement appel aux methodes utilisees dans les autres scleroses systemiques. Six observations de malades ameliores par la photopherese extracorporelle ont ete publiees. La physiopathologie est mal connue. Le gadolinium pourrait jouer le role d’agent activateur dans la pathogenie de la maladie en attirant les fibrocytes circulants dans le derme des malades atteints. Les Autorites de Sante incitent donc a la prudence quant aux injections de gadolinium chez les insuffisants renaux.BACKGROUND Nephrogenic fibrosing dermopathy is a cutaneous and systemic sclerosis affecting patients with renal failure. CASE-REPORT A 68-year-old man with renal insufficiency and on dialysis developed hardening of the skin and severe joint contractions. He had previously undergone angiography with gadolinium-containing contrast agents. A skin biopsy confirmed nephrogenic fibrosing dermopathy. The patient was treated by oral steroids followed by extracorporeal photopheresis. An improvement was seen after 12 cycles. DISCUSSION Treatment of nephrogenic systemic fibrosis is not codified and is normally based on the methods used for other forms of systemic sclerosis. Six cases of patients showing improvement under extracorporeal photopheresis have been published. The physiopathology of the disease is unknown. Gadolinium could act as a triggering agent by attracting circulating fibrocytes in the dermis of patients. Medical authorities recommend avoidance of gadolinium in patients with advanced kidney failure unless strictly necessary.

Dermatose pustuleuse érosive des jambes : rôle de la carence en zinc ?

Resume Introduction La dermatose pustuleuse erosive des jambes est une entite recemment decrite, rare, d’etiologie inconnue touchant les sujets âges. Elle se caracterise par des erosions symetriques des jambes resultant de la confluence de pustules steriles survenant, le plus souvent, apres un traumatisme minime. Le traitement par dermocorticoides est rapidement efficace mais il n’est pas codifie et les rechutes sont frequentes. Observations Trois malades âges respectivement de 74, 84 et 92 ans avaient des erosions des jambes et des chevilles associees a des pustules en peripherie apparues a la suite d’un traumatisme minime. Il s’agissait de pustules steriles d’aspect spongiforme a l’examen histologique. Aucun n’avait d’antecedent de psoriasis. Tous avaient une carence en zinc. Un traitement par dermocorticoides associe a du gluconate de zinc oral a permis, pour deux des malades, une resolution totale en quelques jours et a ete efficace lors d’une rechute chez le troisieme. Discussion La dermatose pustuleuse erosive des jambes est une dermatose benigne vraisemblablement de frequence sous-estimee a integrer dans le cadre des dermatoses neutrophiliques inflammatoires. Nos observations suggerent le role declenchant ou favorisant de la carence en zinc.BACKGROUND Erosive pustular dermatosis of the legs is a rare and recently described condition seen in elderly subjects. It is characterised by symmetric erosions of the legs resulting from a confluence of sterile pustules, usually following minor trauma. Treatment with dermal corticosteroids is rapidly effective but is not codified and relapse is common. CASE-REPORTS Three patients aged respectively 74, 84 and 92 years presented ulcers of the legs and ankles associated with peripheral pustules following minor injury. These pustules were sterile and exhibited a spongiform appearance on histological examination. None of these patients had a prior history of psoriasis. However, all presented zinc deficiency. Treatment with dermal corticosteroids combined with oral zinc gluconate resulted in complete resolution in two of the patients after several days of therapy and proved effective during relapse in the third patient. DISCUSSION Erosive pustular dermatosis of the legs is a benign dermatosis that is probably under-reported that should be included in the category of inflammatory neutrophilic dermatoses. The cases we present suggest a triggering or enhancing role of zinc deficiency.

Absence de valeur prédictive des signes de régression histologique sur l'envahissement du ganglion sentinelle

Resume Introduction La valeur pronostique de la presence de signes histologiques de regression dans la tumeur primitive de melanome est discutee. Objectif Determiner s’il existe une association entre la presence de signes de regression dans la tumeur primitive de melanome et la presence de micrometastases ganglionnaires. Malades et methode Il s’agissait d’une etude monocentrique, retrospective, portant sur 84 tumeurs primitives de melanome d’epaisseur superieure a 1 mm et le ganglion sentinelle correspondant. L’analyse des signes de regression a ete effectuee par deux examinateurs independants. L’indice de Breslow, l’âge, le sexe, la localisation du melanome ont ete notes pour chaque tumeur. Resultats Parmi les 84 melanomes etudies, 40 avaient des signes de regression et 24 etaient associes a des micrometastases ganglionnaires. Parmi ces derniers, seules 10 avaient des signes de regression. Il n’y avait pas d’association entre signes de regression et micrometastases (RR = 0,5 ; p = 0,5). Seuls un indice de Breslow superieur a 2 mm (RR = 4,6, p = 0,03) et le sexe masculin (RR = 7,6, p = 0,006) apparaissaient comme predictifs de l’atteinte du ganglion sentinelle. En analyse multivariee, ces deux derniers facteurs etaient independants avec, pour RR respectifs 3,2 (IC95 p. 100 : 1,1-9,1.) et 4,6 (IC95 p. 100 : 1,5-13,8.). Discussion Ces resultats suggerent qu’il n’y a pas une forte association entre la regression au sein de la tumeur primitive et la presence d’une micrometastase dans le ganglion sentinelle. Ils confirment que l’indice de Breslow est significativement predictif de la presence de micrometastases.

A case of severe pityriasis rubra pilaris with a dramatic response to apremilast

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