[Successful use of combined corticosteroids and rituximab in the treatment of recalcitrant epidermolysis bullosa acquisita]. / Succès de l'association rituximab-corticoïdes généraux dans le traitement d'une épidermolyse bulleuse acquise récalcitrante.

BACKGROUND: Epidermolysis bullosa acquisita (EBA) is a rare autoimmune subepidermal blistering disease; it is potentially serious and is often refractory to conventional treatments, including corticosteroids. We report a new case of successful treatment of EBA using rituximab (anti-CD20 antibody) without relapse after 1 year of follow-up. CASE REPORT: A 76-year-old man was seen for blisters of the skin and mucosa, atrophic scars and milia on areas of friction. The diagnosis of EBA was made on the basis of histological and immunohistochemical criteria. The patient was unsuccessfully treated with topical steroids, dapsone, topical tacrolimus, systemic steroids, mycophenolate mofetil, doxycycline and methotrexate. Four weekly infusions of rituximab of 375 mg/m(2) body area were performed, combined with systemic steroids: they proved beneficial within 3 weeks, with a noticeable improvement and no further blisters at 7 months. After 1 year of follow-up, the skin disease is still stable with 5 mg/day of prednisone alone being given. DISCUSSION: This is the eighth reported case of treatment of EBA with rituximab and the sixth successful therapeutic outcome, with good steroid sparing effect and undeniable improvement in quality of life within several months and good tolerability at 12 months of follow-up. This treatment may be proposed early in cases of EBA refractory to conventional treatments. However, clinical observation is necessary to study potential long-term adverse effects.

Morphoea-like plaques revealing an eosinophilic (Shulman) fasciitis.

Eosinophilic fasciitis (EF) is a connective-tissue disease characterized by thickened fascia. Involvement of the dermis may occur during the progression of the disease, characterized clinically by morphoea-like plaques (MLPs). This more superficial feature of EF carries a poor prognosis, suggestive of refractory EF that requires intensive therapy. We report a case in which morphoea-like plaques occurred before the EF. This case and previous cases of MLPs show some differences between MLPs and classic morphoea. Clinically, MLPs present with ill-defined brown plaques, with no lilac ring or ivory colour. Histologically, there is a more marked inflammatory infiltrate and an increase in the number of eosinophils. The discovery of MLPs at an early stage should prompt examination of the fascia with magnetic resonance imaging or surgical biopsy, and aggressive treatment.

[Diffuse systemic sclerosis after occupational exposure to trichloroethylene and perchloroethylene]. / Sclérodermie systémique diffuse liée à une exposition professionnelle au trichloroéthylène et perchloréthylène.

BACKGROUND: Diffuse systemic sclerosis (DSS) is an autoimmune disease that is most often endogenous but which can also be induced by exogenous substances of occupational origin. PATIENTS AND METHODS: We report a case of DSS involving prolonged intermittent occupational exposure to solvents (trichloroethylene [TCE] and perchloroethylene [PCE]). The disease was rapidly fatal with cardiac arrest secondary to myocardial fibrosis. DISCUSSION: In the event of exposure to TCE/PCE, we suggest more systematic prevention and diagnosis of DSS.

[Occurrence of psoriasis in a patient presenting Shulman fasciitis treated with infliximab]. / Survenue d'un psoriasis au cours d'un traitement par infliximab pour fasciite de Shulman.

BACKGROUND: Anti-TNFalpha drugs are used in certain rheumatologic and gastrointestinal inflammatory diseases and are also effective in cutaneous psoriasis. Several case reports have recently been published concerning induction of paradoxical psoriasis during the course of anti-TNF therapy. We report a new case involving infliximab used to treat Shulman fasciitis. CASE REPORT: A 39-year-old woman was treated with infliximab for corticoid-dependent Shulman fasciitis. No personal or familial cutaneous psoriasis was noted in her history. Two months after the third infusion, she developed psoriasis vulgaris and pustular palmoplantar psoriasis which improved under topical corticosteroids. Her psoriatic lesions worsened one month after the first maintenance infusion. Since the Shulman fasciitis was not under control, infliximab was withdrawn and replaced with azathioprine. Six months later, her psoriasis was in remission and the Shulman fasciitis was under control. DISCUSSION: TNFalpha plays an important role in the physiopathology of psoriasis through its action on inflammatory infiltrate, angiogenesis and keratinocyte proliferation. Several studies have reported the efficiency of TNFalpha inhibitors in moderate to severe cutaneous psoriasis. However, fourteen cases of induction or worsening of psoriasis have been reported with these drugs, suggesting a class effect. We report a new case of cutaneous psoriasis induced by infliximab in a patient presenting corticoid-dependent Shulman fasciitis, and we discuss the possible immunological mechanisms responsible for this paradoxical side effect. Other cutaneous lesions have been reported during treatment with TNFalpha inhibitors. The benefits of this treatment on the underlying inflammatory disease must be balanced against the potential cutaneous side effects when deciding whether to continue with anti-TNFalpha treatment.