Immunophenotype analysis of dendritic cells and lymphocytes associated with cutaneous malignant melanomas.

The purpose of this study was to investigate the relationship between changes in density and distribution of dendritic cells, both in epidermis and in peritumoral infiltrate, and lymphocyte subset variations in malignant melanomas (MM) of patients belonging to different risk groups. The collective immunoreactive expression of six markers (S100 protein, CD1-a, HLA-DR, CD4, CD8 and CD25) was analyzed in 13 cutaneous malignant melanomas. Changes were observed in density and distribution of Langerhans cells (LC) (S100+, CD1-a+) in the epidermis overlying the tumor, as well as in peritumoral and intratumoral locations, independently of the tumor-invasion level. A decrease was recorded in LC (S100+, CD1-a+) in the epidermis overlying six tumors, whereas most of the MM studied showed an increase of LC (S100+, CD1-a+) in peritumoral infiltrate. The expression of HLA-DR in tumor cells was controversial; it was observed in three moderate-risk MM, but it was negative in high-risk tumors. The percentage of CD4+ cells was in most cases greater than that of CD8+ in the peritumoral infiltrate, irrespective of the degree of histopathological malignancy. The concomitant expression of the lymphocytic activation marker CD25 (receptor for interleukin 2) in lymphocytic infiltrate was variable. Peritumoral infiltrate in three high risk MM contained few CD25+ cells, and a concomitant decrease was recorded in LC. This preliminary report shows that alterations in the density and distribution of LC may be responsible for determining the degree or T lymphocyte activation, and this may be critical for the development of effective tumor-directed immunity. Further studies are required to demonstrate these hypothetical interrelations.

[Proteus syndrome. 8 cases]. / Syndrome Protée: huit cas.

INTRODUCTION: Proteus syndrome most often includes: corporal hemi-hypertrophia, gigantism of the extremities with hyperplasia of the palms and soles, vascular abnormalities and varied types of other hamartomatous tumours. The dysmorphic growth generates functional orthopaedic and orthognatic disabilities that increase with age. OBSERVATIONS: We report, on eight cases, our experience in management of Proteus syndrome abnormalities and the benefits of new techniques in imaging and interventional vascular radiology. The correction of limb orthopaedic abnormalities is complicated with elephantiasic enlargement of soft tissues. In some patients the absence of a deep venous network contra-indicates transcutaneous occlusion of dysplatic vessels. Facial asymmetry and orthognatic anomalies are better managed after permanent teeth have appeared. COMMENTARIES: The management of these disabilities is carried out by staff of different specialities but should be organized by a practitioner who regularly follows the child and detects early the functional disturbances. Aesthetic corrections are performed later.

Nonepidermotropic cutaneous lymphomas. A histopathological and immunohistological study of 52 cases.

We report the detailed histological and immunohistological findings in 52 cases of nonepidermotropic cutaneous lymphoma, as revealed by cutaneous lesions. The patients presented mainly with cutaneous nodules and, more rarely, with infiltrated plaques, annular erythema, or erythroderma. The staging procedure following the diagnosis revealed lymph node and/or bone marrow involvement in half of the cases. Nearly 60% of the nonepidermotropic cutaneous lymphomas were of the large-cell type. The reticular dermis was involved in all of the cases, and the papillary dermis was involved in only 11 of them. Nonepidermotropic cutaneous lymphomas were of the B-cell, T-cell, and non-B-, non-T-cell type in 38, 13, and 1 case(s), respectively. A monotypic immunoglobulin light chain expression was detected in 33 of 35 tested cases of the B-cell lymphomas, and a loss of one or several pan-T-cell antigens was observed in all of the cases of the T-cell lymphomas. In seven cases (13%), the diagnosis of malignancy was based only on these immunohistological criteria. This study shows that nonepidermotropic cutaneous lymphomas are B-cell lymphomas in 75% of the cases, most often of the large-cell type. It also emphasizes the value of immunohistochemistry to firmly establish malignancy when routine light microscopical findings are questionable.

[Bowen's disease and bowenoid papulosis: comparative clinical, viral, and disease progression aspects]. / Maladie de Bowen et papulose bowénoïde: données cliniques, virologiques et évolutives comparatives.

Among the VIN (vulvar intraepithelial neoplasia), it is necessary to distinguish the classical Bowen's disease (CBD) and bowenoid papulosis (BP). This retrospective study concerning 75 VIN (36 CBD and 39 BP) demonstrated that the most efficient criteria are the clinical aspect and the age of the onset (CBD = 55.6 years old; BP = 33.2 years old). BP is clinically related to genital warts (41 cases). They share the same age of onset, the fact to be sexually transmitted diseases and the benign evolution. Although virological results between CBD and BP are very similar (human papilloma virus type 16, 18, 31, 33), the development of an invasive carcinoma is mostly related to the CBD (27.8%) and is exceptional in the BP (2.6%).

[Demyelinating nervous lesions in myalgia-eosinophilia syndrome induced by L Tryptophan]. / Lésions nerveuses démyélinisantes au cours du syndrome myalgies-éosinophilie secondaire à la prise de L-tryptophane.

A case of severe peripheral nerve lesions in a patient with eosinophilia-myalgia syndrome induced by L-Tryptophan is reported. Both superficial radial nerves were very tender on palpation. Biopsy of the radial nerve showed myelin abnormalities of the type observed in experimental chronic nerve entrapment. Nerve lesions included asymmetrical distortion of the myelin sheath, segmental demyelination, axonal degeneration and regeneration. Such abnormalities suggest that mechanical factors, presumably due to proliferation of connective tissue, play a role in the nerve lesions observed in this patient.

[Paget disease of the vulva. 36 cases]. / Maladie de Paget vulvaire: 36 cas.

Thirty six patients with Paget's disease of the vulva were reviewed. The median age of the patients at diagnosis was 67 years (range: 45-91 years). One patient had a history of previous mammary adenocarcinoma. Screening for malignancy revealed two colonic tumours. Two patients with negative screening at presentation developed, 12 and 18 months respectively after vulvectomy, an ovarian carcinoma stage IIc and a cervical and urethral adenocarcinoma. All patients were treated by surgery based on extent of the disease. The operations performed included total vulvectomy (n = 11), partial vulvectomy (n = 14) and wide local excision (n = 4). Out of the 36 patients, 29 were available to follow-up. The median follow-up period was 74 months (range 2-204 months). Three patients died of metastatic disease due to vulval adenocarcinoma and breast carcinoma, or of liver metastases from an unknown adenocarcinoma. Eighteen of the 29 patients followed up remained free of disease. Five out of the 16 patients with positive margins recurred, as did 5 out of 9 patients with negative margins. Treatment of Paget's disease of the vulva is surgical. In order to prevent recurrence, some authors have proposed surgical excision extending beyond the visible clinical lesions with intraoperative frozen sections. The data we recorded show that free margins do not seem to correlate with recurrence, so that large excision beyond the clinical lesion is not useful.

[Recurrent nodular panniculitis associated with pancreas divisum]. / Panniculite nodulaire récidivante associée à un pancreas divisum.

We report the case of a 46-year old woman who, over a 12-year period, had 6 episodes of nodular panniculitis. Each lesion regressed and disappeared within four to six weeks. Fistulization before formation of a cupula-shaped scar was observed on two occasions. These episodes of hypodermitis seemed to be isolated when they began, but histological examination of the skin showed cytosteatonecrosis of the hypodermis pointing to a pancreatic origin. Signs of pancreatic abnormality appeared progressively: first an increase of amylasaemia concomitant with the cutaneous flares, then morphological abnormalities of the pancreatic ducts suggestive of pancreas divisum, and finally dilatation of the main pancreatic duct associated with upstream pancreatitis. No radical treatment of the pancreatic abnormality was carried out.

An atypical case of penile carcinoma in situ associated with human papillomavirus DNA type 18.

We report an unusual case of penile carcinoma in situ in a heterosexual man. The histologic study showed a severe dysplasia, and the typing of human papillomavirus DNA, performed at the Pasteur Institute, disclosed a type 18, which is usually associated with penile and cervical carcinomas. The identification of an oncogenic human papillomavirus type in the penile lesions, such as type 18 found in this case, should lead to a search for cervical carcinoma in the sexual partner.