[Sézary syndrome and Leser-Trélat sign: a chance association?]. / Syndrome de Sézary et syndrome de Leser-Trélat: association fortuite?

BACKGROUND: Leser-Trélat sign involves the combined sudden onset of seborrheic keratosis and cancer. However, some doubt surrounds the existence of this syndrome. We report a case of Leser-Trélat sign that led to the discovery of Sézary syndrome. PATIENTS AND METHODS: A 59-year-old woman presented generalized pruritus with secondary appearance of multiple seborrheic keratosis. Leser-Trélat sign was diagnosed and 20 months later, Sézary syndrome was discovered. Extracorporeal photopheresis was initiated, after which there was a marked reduction in the patient's pruritus, erythroderma and numbers of seborrheic keratoses and Sézary cells. DISCUSSION: Leser-Trélat sign is often associated with gastric carcinoma or lymphoproliferative tumours. Rampen and Schwengle [J Am Acad Dermatol 21 (1989) 50-5] have thrown doubt on this entity because of the "subjective" definition, the frequent dissociation between the course of the tumour and that of the seborrheic keratosis, the disparity between the frequency with which rapid onset seborrheic keratosis is seen and the rarity of cases in which this phenomenon reveals a tumour and the absence of association with any specific type of malignancy. The time between diagnosis of Sézary syndrome and cutaneous symptoms of Leser-Trélat sign appears very long in the present case. In the absence of any established physiopathology, it is impossible to prove any direct link between these two syndromes. Leser-Trélat sign remains controversial. Knowledge of its pathogenesis could help determine whether Leser-Trélat sign should or should not be considered a paraneoplastic syndrome.

[Palmar fasciitis associated with polyarthralgia: rare paraneoplastic syndrome]. / Fasciite palmaire associée à des polyarthralgies: un syndrome paranéoplasique rare.

BACKGROUND: Palmar fasciitis associated with polyarthralgia is a rare paraneoplastic syndrome, frequently observed in women, and in most cases associated with ovarian carcinoma. CASE REPORT: A 51 year-old man complained of extensive palmar dermatological lesions associated with polyarthralgia of the legs. A few months earlier, cholangiocarcinoma had been diagnosed due to cholestatic icterus. The cutaneous biopsy confirmed the diagnosis of palmar fasciitis associated with polyarthralgia. DISCUSSION: This paraneoplastic syndrome is rarely described in digestive carcinoma and never in cholangiocarcinoma. The diagnosis is clinical, confirmed by certain laboratory exams. The evolution is similar to that of the carcinoma. Only curative treatment can achieve regression of the symptoms.

[Unusual cutaneous lesions of familial Mediterranean fever]. / Manifestations cutanées rares de la maladie périodique.

BACKGROUND: Familial mediterranean fever belongs to the periodic fever syndromes. During the attacks, fever is associated with abdominal pain, arthralgia, or both. Cutaneous involvement occurs in 7 to 46 p. 100 of cases and mainly consists in erysipelas-like erythema. We report on three patients treated for familial Mediterranean fever who developed unusual cutaneous lesions. OBSERVATIONS: All the patients had long past history of familial mediterranean fever without cutaneous involvement except, for the third patient who had pseudo-erysipela. The first patient had diffuse Sweet's syndrome-like lesions, the second developed long lasting panniculitis of the thigh and the third had a persistent and lichenified erysipela-like plaque. In two patients, skin histology revealed an inflammatory infiltrate with neutrophils. In all cases, an increase in the colchicine dose led to the rapid resolution of the lesions. DISCUSSION: In our 3 case reports, the lesions were particular because of their atypical clinical appearance, their long duration, and they differed from the usual pseudo-erysipela aspect. Histopathologically, the lesions were similar to pseudo-erysipela, which has led some authors to hypothesize that cutaneous lesions of familial mediterranean fever belong to neutrophilic dermatoses. This hypothesis is supported by the response to the increase in colchicine doses.