Cutaneous lymphomas other than mycosis fungoides: follow-up study of 52 patients.

Cutaneous lymphomas other than mycosis fungoides (MF) represent a rare and heterogeneous group of lymphomas. Their clinical behavior remains largely unknown. In this study, the clinical and immunohistologic characteristics and follow-up data of 52 well-documented cases of cutaneous lymphomas other than MF, presenting with initial cutaneous lesions, were reviewed. Twenty-seven patients presented with skin disease alone (stage IE), and 25 patients had concurrent cutaneous and extracutaneous disease (stage IV). The tumors were grouped into high-grade lymphomas (HGLs; 21%), intermediate-grade lymphomas (IGLs; 58%), and low-grade lymphomas (LGLs; 21%). A B-cell phenotype was most often expressed by cutaneous lymphomas (73%), particularly by stage IE lymphomas (85%). Among 13 cases of T-cell lymphomas, loss of one of the pan-T-cell antigens was detected in all cases but one. The clinical course of cutaneous lymphoma was closely dependent on stage and histologic subtype but not on T-cell or B-cell phenotype. Of 20 patients with stage IV HGL or IGL, 13 were treated by polychemotherapy with curative potential. Their median survival was 37 months. Fourteen patients with stage IE HGL or IGL were treated by radiotherapy alone. Nine patients (69%) relapsed within 2 years posttreatment. Seven of them relapsed in the skin outside the initial site involved, suggesting that radiotherapy alone is not an adequate treatment for these patients. Preliminary results concerning seven other patients with stage IE IGL or HGL treated by an initial third-generation polychemotherapy regimen are presented.

Vestibular papillae of the vulva. Lack of evidence for human papillomavirus etiology.

Vestibular papillae of the vulva are usually considered as anatomical variants of the vestibular mucosa. Clinically, however, they are quite often interpreted as condylomata acuminata and recent studies have suggested that they could be related to human papillomavirus infection. This prompted us to search for human papillomavirus DNA using the Southern blot hybridization technique, by analyzing biopsy specimens taken from 29 patients who presented with papillae of the vestibular mucosa. Human papillomavirus sequences were detected only in two (6.9%) cases. By the same technique, human papillomavirus sequences were detected in 24 (96%) of 25 vulvar warts used as the control specimens. Thus, it appears unlikely that vestibular papillae are related to human papillomavirus infection. They are usually distinguishable from condylomata acuminata by clinical examination alone.

Latent hepatitis B virus (HBV) infection in systemic necrotizing vasculitis.

We have investigated hepatitis B virus (HBV) infection in systemic necrotizing vasculitis (SNV). Our approach included the detection of the viral surface antigen (HBsAg) with a radioimmunoassay employing monoclonal anti-HBs (m-RIA); in addition, HBV DNA was looked for in serum and peripheral mononuclear blood cells. Among 28 subjects with SNV, 12 were found to be positive for HBsAg with the conventional test (p-RIA) and 7 additional subjects had anti-HBc and/or anti-HBs. From the 16 HBsAg negative individuals, 9 had HBsAg epitopes identified in serum with the m-RIA test and 1 had a low amount of circulating viral DNA. In contrast, only 1 among 6 subjects with other systemic vasculitis showed a positive test for m-RIA and HBV DNA assays; this individual had acquired HIV infection through transfusions which were also probably the source of his HBV infection. HBV DNA sequences were identified in peripheral mononuclear blood cells of 9 from the 37 tested, including 2 individuals who were HBsAg positive only with m-RIA. Therefore, our study indicates a much higher rate of HBV infection in patients with polyarteritis nodosa than previously suspected.

Dermatitis due to orthopaedic implants. A review of the literature and report of three cases.

The cases of three patients who had dermatosis that was caused by an orthopaedic implant are reported. The main clinical pattern was localized or generalized eczema or urticaria. The diagnostic criteria that have been proposed by various authors are reviewed. Removal of the implant did not always result in rapid disappearance of the dermatosis, presumably because a few particles of metal remained in the area of the implant.

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.

Failure of isotretinoin and interferon-alpha combination therapy for HPV-linked severe vulvar dysplasia. A report of two cases.

BACKGROUND: Retinoids (RA) and interferon (IFN) have been reported to be active against a variety of tumors and human papillomavirus (HPV)-related lesions. Because chronic and recurrent HPV-linked vulvar intraepithelial neoplasia 3 (VIN 3) have a high risk of invasion, we evaluated combined therapy of IFN-alpha with 13-cis-retinoic acid (13 cRA) in the treatment of two VIN 3 cases of this type. CASE: Two patients with chronic and recurrent VIN 3 were treated with combined therapy of IFN-alpha (4.5 x 10(6) five times a week) and 13 cRA (1 mg/kg/d) for six months. Clinical regression was observed at the end of treatment in both cases, but histologic features of VIN 3 were still present. CONCLUSION: These data demonstrate the ineffectiveness of the combined regimen of IFN-alpha and 13 cRA with this schedule for a period of six months in recurrent and chronic VIN 3.

Anetoderma of Jadassohn-Pellizzari.

Anetoderma (derived from the Greek anetos, meaning slack) is a term used to describe localized increased laxity of the skin with herniation or outpouching, resulting from abnormal dermal elastic tissue. Primary anetoderma is distinctly rare. We describe a case where we suspect an auto-immune aetiology.

[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.

[Cutaneous ulcerations in sarcoidosis]. / Ulcérations cutanées au cours d'une sarcoïdose.

We report the case of a 23 year-old Caribbean woman with sarcoidosis who developed specific skin ulcerations. Ulcerative lesions in sarcoidosis are distinctly unusual, generally multiple, painless, with preponderant location on the lower limbs. The diagnosis is difficult. The pathogenesis is discussed. The most successful therapy is hydrochloroquine with corticosteroids.

[Histopathology of cutaneous lesions]. / Histopathologie des lésions cutanées.

Kaposi's sarcoma in AIDS syndrome differs in many ways from the typical nodular lesions observed in older patients. Clinical lesions are usually multiple with no predilection for the lower extremities; they usually present as small macules or patches with little or no infiltration, pink rather than deeply violaceous; indeed their clinical recognition may be difficult. Histological features of early patches of Kaposi are subtle: they are not as obvious as in well-developed nodular lesions: however certain precise clues exist which permit a correct diagnosis to be made.

[Kala-azar in immunosuppressed patients. Apropos of a case disclosed by skin lesions]. / Kala-azar chez l'immunodéprimé. A propos d'un cas révélé par des signes cutanés.

Kala-azar was revealed by skin lesions in a patient treated for recurrent Hodgkin's disease. These lesions, extremely discreet, consisted of papules on the forehead and in the peribuccal area. Histological examinations initially showed nodular infiltration of the dermis with very few Leishman-Donovan bodies; subsequently, foamy, Virchow-type histiocytes appeared, and Leishman-Donovan bodies became numerous. This clinical and histological similarity with leprosy has already been noted for post-kala-azar dermal leishmaniasis which is frequent in India and in Ethiopia. Skin lesions in kala-azar appear to be exceptional since they were not observed in the 7 cases of kala-azar in immunodepressed patients previously published. The fact that they are discreet may account for their apparent rareness. Easily accessible, they make it possible to diagnose visceral leishmaniasis--an often difficult diagnosis in these patients owing to the underlying disease. Immunodepression has little influence on the clinical and biological manifestations of kala-azar, but reserves must be made concerning the usual criteria for cure.

[Congenital cutaneous Langerhans histiocytosis. Apropos of 7 cases]. / Histiocytoses langerhansiennes congénitales cutanées. A propos de 7 cas.

Seven cases of congenital Langerhans' cell histiocytosis (LH) are reported, with emphasis on clinical and immunohistochemical features. This is a polymorphic disease at birth. In 4/7 cases, the diffuse, generalized rash could be classified as cutaneous Letterer-Siwe disease (LSD); 3/4 remained purely cutaneous and healed in less than 3 months; whereas the fourth-one persisted, pulmonary lesions appeared, and the infant died on his 40th day. In 3/7 cases, the clinical diagnosis at birth was either a Blueberry Muffin Baby (BMB) or Hashimoto-Pritzker type LH (HPLH); the lesions healed rapidly, although one cas was contradictory: typical BMB at birth, histology mimicking a monoblastic cutaneous leukemia, no T.O.R.C.H. syndrome, normal bone marrow, immunophenotyping of LH, auto-involution; 2/3 were MZ twins, both with few lesions. We would like to stress the fact that the clinical spectrum of LH should include BMB, which, however, in most cases must be considered a differential diagnosis. Regarding cutaneous congenital LH, an eponymic classification (LSD, HPLH) is difficult to follow strictly, because overlapping pictures are observed. There is a wide spectrum of cutaneous congenital LH. The main problem at birth is the lack of prognostic criteria. Neither the presence of the rash at birth, nor its type and extension, is necessarily evidence of risk of systemic disease. Cases of HPLH involute, as also do cases of cutaneous LSD, and the "Blueberry Muffin" type of LH; overlapping clinical aspects exist. Histopathological data, electron microscopy or immunohistochemistry, define LH, but they do not enable the outcome to be predicted.(ABSTRACT TRUNCATED AT 250 WORDS)

[Multifocal epithelioid hemangioendothelioma with partial remission after interferon alfa-2a treatment]. / Hémangioendothéliome épithélioïde multifocal en rémission partielle sous traitement par interféron alpha 2a.

INTRODUCTION: Epithelioid hemangioendothelioma (EHE) is a rare vascular tumour of the soft tissue having an intermediate malignancy. Cutaneous presentations have exceptionally been reported. OBSERVATION: A young woman, initially affected by an EHE with cutaneous and bone involvement, then with pulmonary and hepatic localizations, was in partial remission after one year of treatment with alpha 2a interferon. DISCUSSION: In a critical review of the literature, we describe the main features of this tumour and the difficulties to classify this entity in the vascular tumors' spectrum. Histologically, epithelioid hemangioendothelioma has a very characteristic appearance and is clearly different from other vascular tumors as angiolymphoid hyperplasia with eosinophilia and Kimura's disease. The assumption of a multicentric or a metastatic origin when multiple tumor deposits are found, is still not elucidated. Different therapeutic approaches are proposed: surgery, interferon, interleukin-2, retinoids. Randomized studies would be necessary to confirm our results with alpha 2a interferon but are probably difficult to realize because of the lack of cases.

[Male genital dyskeratotic tumor. 2 case reports]. / Tumeur dyskératosique génitale masculine. Deux observations.

Two cases of genital vegetant tumors are described associated with lichen sclerous in male patients. Clinically they were very close to giant condyloma acuminatum. This diagnosis was rejected after histological examination of the tumors. The pathological changes consisted of epithelial hyperplasia, dyskeratosis, epidermal dysplasia and presence of some vacuolated keratinocytes. After surgical excision, the relapses are frequent. Etr etinate or local 5-fluorouracil seemed to prevent these relapses.

[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.

[Cutaneous manifestations of angio-immunoblastic lymphadenopathy]. / Manifestations cutanées des lymphadénopathies angio-immunoblastiques.

The authors report four cases of angio-immunoblastic lymphadenopathy, presenting with cutaneous lesions: these have a tumoral presentation in two cases while in the other two, the polymorphic aspect of the cutaneous lesions is reminiscent of a toxidermic eruption. In contrast to the relative clinical polymorphism, the pathological findings are monomorphic, i.e., in all cases, there was a vascular proliferation together with a more or less dense cellular infiltrate consisting of cells indistinguishable from those involving the abnormal lymph nodes.

[Primary cutaneous lymphoma, with the exception of mycosis fungoides]. / Lymphomes primitivement cutanés en dehors du mycosis fongoïde.

Cutaneous lymphomas other than mycosis fungoides (MF) form a rare and heterogeneous group. Their clinical behavior remains largely unknown. In this study, the clinical, immunohistological characteristics and follow-up data of 27 well-documented cases of primary cutaneous lymphomas other than MF, limited to the skin (stage IE) were reviewed. The tumors were divided into large-cell lymphomas (LCL) (21/27 = 77 p. 100) and small-cell lymphomas (SML) (6/27 = 23 p. 100). A B-cell phenotype was most often expressed by cutaneous lymphomas (23/27 = 85 p. 100). The clinical course of cutaneous lymphoma was closely dependent upon the histological subtype. Fourteen patients with LCL were treated by radiotherapy alone. Nine patients (64 p. 100) relapsed within two years post-treatment. Seven of them relapsed in the skin outside the initial site, suggesting that radiotherapy alone is not an adequate treatment for these patients. The preliminary results concerning 7 other patients with LCL treated with an initial third generation polychemotherapy regimen are presented.

[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.

[Menkes' disease (new skin and hair ultrastructural abnormalities) (author's transl)]. / Maladie de menkes anomalies ultrastructurales cutanéophanériennes nouvelles.

The authors report the sixth case of Menkes' kinky hair disease. This boy has been observed for as long as 16 months, and he his still alive at the time of publication. This genetic, X linked disorder of copper metabolism is always fatal in childhood. Diagnosis is evoked when is noted the conjunction of progressive cerebral degeneration, seizures, with pili torti and monilethrix. It can be asserted with the very low copper and cerulo-plasmin blood levels. Recognition of the disease in utero might be possible. New findings in skin' electron microscopy and hair' scanning electron microscopy are reported here. And two RX scanner of the brain have been performed.

[Lymphomatoid granulomatosis: cyclic nodular lymphomatoid panniculitis with immunologic deficiency]. / Hypodermite nodulaire lymphomatoide ascendante a évolution cyclique avec déficit immunitaire: granulomatose lymphomatoide

The case of a 66 years old woman having presented an unusual cyclic dermatosis of 11 months duration is reported. The cutaneous elements were successively located in a subcutaneous, dermal and epidermal situation, and presented a spontaneous healing with sometimes deep retractive residual scars after an ulceration or not. Pathologically, the lesions consisted in dense mononuclear infiltrates and vascular lesions. This disease was associated with an immuno-deficiency state characterized by extremely low levels of circulating IgM. This deficit was found to be persistant as it was still present two years later. However during this lapse of observation no cutaneous lesions recurred. The possible connexions of such a case with those of lymphomatous granulomatosis are discussed. In the absence of pulmonary lesion in the case reported here, no identification to the syndrome isolated by Liebow seems permitted. The authors offer a new denomination for this unusual entity.