[Association of mycosis fungoides and Jaccoud syndrome]. / Association d'un mycosis fongoïde et d'un syndrome de Jaccoud.

We report here a case of a 49 year-old woman who developed a unilateral Jaccoud's arthropathy 9 years after a mycosis fungoides of the left hand. This rheumatic affection, described in 1866, is a deformation of the hands or of the feet linked to a periarticular disease without any bone lesion. Many causes have been registered but the most frequent etiology is the systemic lupus erythematosus. The mycosis fungoides had never been reported as yet to be responsible to the Jaccoud's arthropathy.

[Pachydermodactyly]. / Pachydermodactylie.

INTRODUCTION: Histologically pachydermodactyly results from fibromatosis. Characteristically, the clinical picture occurs almost exclusively in young men. The lesion is localized on the laterodorsal aspect of the proximal phalanges of both hands. CASE REPORT: We observed a 19-year-old man with deformity of the laterodorsal aspect of the first phalanx of the fingers on both hands which had been present for several years. The clinical course and histology of the deformity as well as the lack of any bone lesions led to the diagnosis of pachydermodactyly. COMMENTS: Pachydermodactyl is a rare disease. Our case was comparable to those reported in the literature. A mechanical cause is accepted by most authors.

[Metastasis of a malignant melanoma simulating a primary mandibular sarcoma]. / Métastase d'un mélanome malin simulant un sarcome mandibulaire primitif.

The rare case reported here was a mandibular metastasis of a malignant melanoma located in the nape of the neck and surgically removed. 4 years later, occurred a fibrosarcomatous mandibular tumour without local recurrence. The metastatic nature of this tumour was difficult to prove by means of immunohistochemical and ultrastructural methods. This case illustrated the eventuality of a desmoplastic change in metastasis of a common malignant melanoma. The histogenesis of this rare type of melanoma was discussed.

[Haber's syndrome. First French family (2 cases)]. / Le syndrome de Haber. Première famille française (deux cas).

Haber's syndrome is a genodermatosis first described by Sanderson and Wilson (17) in 1965. Pursuing Dr. Haber's work, these authors reported three cases from one single family presenting with the dermatosis. A second family was reported by Seiji and Otaki (17) in 1971; Izaka described two cases (pedigree unknown, 13) and two more by Kikuchi (10, 13) in 1981 and 1983. We report here two new cases (brother and sister) discovered in 1984 (7) and representing the first French family. The dermatosis is characterized by clinical, genetic and histological criteria. It is transmitted as an autosomal dominant trait (fig. 1 and 2). Clinically, the face is affected by a rosacea-like dermatitis beginning in childhood and proceeding with pustular flare-ups. The facial lesions are frequently aggravated by exposure to the sun. The patients have pigmented keratotic lesions of the trunk resembling seborrhoeic warts. These lesions are very numerous and begin, on average, during the second decade of life. Some patients present with lesions that are diagnosed as Bowen's disease at histological examination (7, 17). Xerosis cutis is also present. Microscopically, the facial lesions consist of a necklace of basaloid cells around the hair and sebaceous follicles. The keratotic lesions have been reported as either intra-epidermal epitheliomas (17), or seborrheic warts without signs of malignancy (7, 10, 13), or equivalents of the facial lesions (19).

[Pemphigus herpetiformis (eosinophilic spongiosis) (author's transl)]. / Pemphigus herpétiforme (spongiose à éosinophiles).

A 71-year-old male had a clinically atypical pemphigus (pemphigus herpetiformis), associated with histopathologic eosinophilic spongiosis. This case is discussed and was frequently found reported in iatrogenic pemphigus.