[Ulceration of lymphomatous skin lesions in a patient with Sezary syndrome treated with brentuximab-vedotin]. / Ulcérations de lésions cutanées lymphomateuses sous brentuximab-védotin chez un patient atteint de syndrome de Sézary.

BACKGROUND: Brentuximab-vedotin (BV), an anti-CD30 agent combined with an anti-neoplastic agent, has recently yielded promising results in the treatment of cutaneous T-lymphomas such as Sezary syndrome. Surprisingly, these results are observed regardless of levels of CD30 expression by tumour cells. PATIENTS AND METHODS: Herein, we report the case of a 52-year-old man with Sézary syndrome (SS) with skin and lymph node involvement, and whose tumour cells were not expressing CD30 at the time of disease progression. His disease was refractory to several lines of treatment, and a partial response was obtained using BV therapy, with secondary ulceration of SS-specific skin lesions. DISCUSSION: Ulceration of our patient's skin lesions was concomitant with clinical improvement and the semiology was identical to cases of skin ulceration under methotrexate in patients with psoriasis or mycosis fungoides. The mechanism of action appears to be related to sudden interruption of the cell cycle in a context of rapid cell renewal, although the way in which the antibody acts on keratinocytes has not yet been fully elucidated.

[Acute generalized pustular bacterid followed by Sweet's syndrome and erythema nodosum]. / Pustulose aiguë généralisée post-streptococcique suivie d'un syndrome de Sweet et d'un érythème noueux.

INTRODUCTION: Streptococcal infections can cause various skin manifestations related to the direct action of the offending organism itself or to a reactional mechanism. Reactional manifestations are less well known and understood, and they include generalized acute pustulosis belonging to the spectrum of neutrophilic dermatoses. We report a case of generalized acute pustulosis followed by Sweet syndrome and erythema nodosum occurring after a streptococcal infection. PATIENTS AND METHODS: A 60-year-old woman was consulting for a diffuse pustular rash after a throat infection, with high levels of anti-streptolysin (337 U/L) and anti-streptodornase (2560 U/L). The biopsy showed folliculitis and a neutrophilic infiltrator of the dermis, and bacteriological and mycological cultures were sterile. The patient then developed papules evoking Sweet syndrome followed by nodules typical of erythema nodosum after 20 days. A favourable outcome was achieved under colchicine. DISCUSSION: Generalized acute pustulosis is a form of neutrophilic dermatosis whose mechanisms, area predilection and treatment are poorly known. The clinical presentation of this patient was initially typical and the secondary progression to lesions like those in Sweet syndrome is consistent with the pathophysiological continuity and overlap of these entities.

[Tattoo hypersensitivity reaction in a patient receiving combined BRAF and MEK inhibitors]. / Hypersensibilité retardée aux tatouages induite par un traitement combiné anti-BRAF/anti-MEK.

INTRODUCTION: For inflammation of a tattoo occurring decades after its creation, sarcoidosis should be considered first of all. Two case of extremely delayed hypersensitivity tattoo reaction have been recently reported in patients treated with BRAF and MEK inhibitors. We report a similar new case strongly suggesting a specific effect of this drug combination. PATIENTS AND METHODS: A 58-year-old man bearing 20-year-old tattoos was treated with dabrafenib and trametinib for advanced melanoma. A painful erythematous swelling appeared on all the patient's tattoos two months later, while his general tolerance of the treatment was poor. Skin biopsy demonstrated perivascular lympho-histiocytic infiltrate without granuloma, but with prominent pigment-loaded macrophages. Inflammatory signs quickly regressed after drug discontinuation. DISCUSSION: Great similarity exists between this new case and the first reported case, in which a female patient presented painful infiltration of old tattoos following repeated reintroduction of dabrafenib and trametinib in a setting of advanced melanoma. The immunomodulatory properties BRAF/MEK inhibition may enhance loss of tolerance to tattoo ink, accounting for the extremely long time to reaction. This third case militates in favour of a specific drug-induced reaction, of which patients with tattoos should be informed when anti-BRAF/MEK therapy is being considered.

[Suprapubic Nicolau syndrome following subcutaneous injection of glatiramer acetate]. / Dermite livédoïde de Nicolau sus-pubienne après injections sous-cutanées d'acétate de glatiramère.

BACKGROUND: Subcutaneous glatiramer acetate, commercialized under the name of Copaxone®, is licensed for the treatment of relapsing multiple sclerosis. Its major adverse effects are skin reactions at the injection site. Nicolau syndrome is a rare but serious iatrogenic accident. Herein we report a case seen in a setting of change of dosage and administration rate of Copaxone®. PATIENTS AND METHODS: A 64-year-old woman, treated since 2010 with daily sub-cutaneous injections of Copaxone® 20mg/L, reported the appearance of a painful, indurated and erythematous plaque in the suprapubic area following changeover to 40mg/mL injections three times weekly. The suprapubic injections were continued and ugly greyish spots with stellate purpuric borders appeared. Fournier gangrene was ruled out by means of a soft tissue scan. DISCUSSION: We report this latest case of Nicolau syndrome to alert readers to the non-exceptional nature of this complication associated with use of glatiramer acetate, particularly at a dosage of 40mg/L injections three times weekly. In our case, onset of Nicolau syndrome appears to have been favored by continued injection in areas already showing inflammation. Re-injection of the drug in these areas should thus be proscribed.

Amicrobial pustulosis of the folds: Where have we gone 25years after its original description?

BACKGROUND: Twenty-five years ago at the Journées Dermatologiques de Paris, Prof. Béatrice Crickx described a new association, "antimicrobial pustulosis and systemic lupus erythematosus", a condition now known as amicrobial pustulosis of the folds (APF). The aim of this study is to analyse the clinical and laboratory characteristics of APF and to outline the gradual advancement of knowledge regarding this disease. MATERIALS AND METHODS: Based on a case of APF seen in our department, we carried out a review of the literature since 1991 by searching the Medline database for scientific articles using the following keywords: "Amicrobial Pustulosis" or "Pustular Dermatosis" and "Folds". RESULTS: We collated 63 cases of APF. In total, 90% involved women and the mean age was 30 years. Cutaneous lesions consisted of pustules in an erythematous setting, frequently erosive and affecting the skinfolds, anogenital region, scalp, external auditory meatus and umbilicus. Histological examination revealed spongiform subcorneal pustulosis associated with mixed inflammatory infiltrate in the dermis. DISCUSSION: APF belongs to the spectrum of neutrophilic dermatoses. Its complex physiopathology involves an auto-inflammatory mechanism. It is associated with a variety of autoimmune diseases, in most cases systemic lupus erythematosus. Diagnosis of the condition is difficult and is based on histological correlation and negative microbiological culture. The most effective treatment is still systemic corticosteroids, although new therapeutic alternatives are emerging such as anakinra and anti-TNF-alpha drugs.

[A new case of Rowell's syndrome]. / Un nouveau cas de syndrome de Rowell.

INTRODUCTION: This article introduces a new case of Rowell's syndrome, a controversial entity defined by the association of lupus erythematosus and erythema multiforme. OBSERVATION: A 43-year-old woman was diagnosed with lupus erythematosus induced by esomeprazole. Because her eruption did not improve after withdrawal of the drug, hydroxychloroquine was administered. Two weeks later, the patient described new annular lesions on her chest and arms, both erosive and crusted, and some had a target-like appearance. The oral mucosa was also affected. Histology revealed sub-epidermal blistering with keratinocytic necrosis, strongly suggesting erythema multiforme. Screening for other causes of erythema multiforme proved negative. A positive outcome was achieved with corticosteroids and hydroxychloroquine. One year later, the patient was in complete remission for both lupus erythematosus and erythema multiforme. DISCUSSION: The association of lupus erythematosus and erythema multiforme first described in 1963 is known as Rowell's syndrome. While diagnostic criteria have been established in the literature, the reality of this entity is still contested. The annular lesions of subacute lupus erythematosus may be confused with the lesions of erythema multiforme. As suggested in the above section, other authors consider Rowell's syndrome to be a singular entity. Indeed, our patient developed lesions distinct from those initially suggesting subacute lupus erythematosus, in particular: the target-like aspect of the elementary lesions, mucosal involvement, a distinct histological aspect, and dissociated outcomes. Ultimately, the definition of Rowell's syndrome remains highly debated.