[Pachydermatodactyly associated with plantar pachydermy]. / Pachydermodactylie associée à une pachydermie plantaire.

INTRODUCTION: Pachydermodactyly is a superficial fibromatosis located on the proximal portion of fingers'phalanges and interphalangeal joints. Several types of this disease have been described depending on topography, etiology and pathological associations. We report a typical observation of pachydermodactyly associated with a plantar pachydermy. CASE-REPORT: A 19 year-old man was followed for a psychotic disease, associated with mental retardation. On clinical examination, he showed a typical pachydermodactyly, predominantly located on the second, third and fourth fingers of both hands, associated with recent acrocyanosis. A pachydermic aspect was also observed on the external part of the feet sole. Histopathological analysis was identical on digital and plantar lesions with collagen swelling. These lesions extended into the subcutaneous fat only in the feet. DISCUSSION: This observation is compatible with the most classical form of pachydermodactyly which involves several fingers of both hands, usually in men. Furthermore, the association with a psychiatric disorder and the notion of repeated traumatisms have been frequently reported in the literature. However, acrocyanosis and the important disability observed in our case are unusual. Association with plantar pachydermy has never been reported. The identical histological aspect on finger and feet sole suggests that pachydermatodactyly should be integrated in framework of acral pachydermy.

[Sub-total hereditary leukonychia, histopathological and electron microscopy study of "milky" nails]. / Leuconychie héréditaire subtotale. Etude histopathologique et ultrastructure des ongles "laiteux".

INTRODUCTION: Leuconychia is the most common of ungueal discoloration or dyschromia. The hereditary form, sub-total or total, is very rare. We report the observation of a family in which thirteen members, distributed over four generations, presented a partial or subtotal leukonychia of all nails. On the basis of this observation and histopathological analysis of the propositus nail, we discuss the different hypotheses proposed to account for white nails. OBSERVATION: A 45 year-old man without previous medical history, showed partial or sub-total leukonychia of his twenty nails. Clinical examination revealed soft nails with slow growth. Discoloration or dyschromia was associated with koilonychia on the fingers without other cutaneous or visceral abnormalities. A longitudinal biopsy of the whole ungueal apparatus of the large toe was performed. Histopathological analysis showed parakeratosis and an abnormal granular layer thickened on the proximal and ventral womb. These abnormalities were responsible for heterogeneous HES coloration, lamellar and dissociated aspect of the nail plates. Electron microscopy revealed dissociated keratin bundles and the existence of intracytoplasmic clear vacuoles probably of lipid origin. This aspect was observed on the proximal part of the dorsal tablet and disappeared in the distal portion of the nail. DISCUSSION: According to Newton's theorem, a surface appears white when it reflects all the radiation of visible light. This mechanism can be proposed to explain leukonychia. On histological level, parakeratosis and dissociation of the keratin bundles may play a role in the modification of the solar light reflection by ungueal plates. In our case, parakeratosis and disorganization of keratin bundles were present in the white part of the nail, but also in the distal, pinkish crescent (distal dorsal plate). Electron microscopy analysis showed clear vacuoles located in the white part of the nail, whereas they were not seen in the distal part. These observations confirm the probable participation of parakeratosis and keratin abnormalities in this pathology but also suggest an important role of lipid vacuoles. Therefore, some white nails may in fact be "milky" nails. Finally, disorganization of the keratin bundles observed, was also reported in other genodermatosis e.g.: epidermolysis bullosa simplex. In this latter pathology, some hard keratin genes mutations (K5 and K14) have been demonstrated. According to these results, a genetic study is on going in this family in order to search for a mutation in one of the hard keratin genes.

Guess what! SALT - related B-cell lymphoma presenting as a xanthomatous infiltration of the neck.

A 37-year-old man without previous medical history working as a lock keeper was seen in our unit for a progressive painless subcutaneous flesh coloured infiltration of the lower anterior area of the neck growing slowly over 5 months. Clinical cutaneous findings showed a non pruriginous yellowish papulonodular eruption mimicking xanthomas (Figs. 1 and 2). No other clinical abnormalities were found. A cutaneous biopsy specimen was performed. Histopathological examination revealed, under a normal epidermis, a dense lymphoplasmocytoid infiltrate involving the dermis with periadnexal and perivascular reinforcement (Figs. 3 and 4).

Bronchial carcinoid tumor: demonstration by dynamic inversion recovery turbo-flash MR imaging.

A 38-year-old woman was referred to our hospital following the discovery of a right hilar mass on chest radiograph. Retrospectively, a hilar mass could be seen on a chest X-ray which had been obtained 5 years earlier. A coronal dynamic inversion recovery turbo-FLASH gadolinium-enhanced sequence was performed, demonstrating the right lesion which enhanced during the systemic arterial phase indicating an arterial supply from the bronchial arterial circulation. The surgical and pathological findings were a bronchial carcinoid tumor, with foci of bone formation.