[Macular lymphocytic arteritis and periarteritis nodosa: A case report showing diagnostic and nosological challenges posed by these two entities]. / Artérite maculeuse lymphocytaire et périartérite noueuse : un cas illustrant les difficultés diagnostiques et nosologiques posées par ces deux entités.

BACKGROUND: Macular lymphocytic arteritis is a recently described type of cutaneous vasculitis involving vessels of medium size. Authors consider it as a form of polyarteritis nodosa. Herein we report a case of macular lymphocytic arteritis during the course of which periarteritis nodosa appeared. PATIENTS AND METHODS: A 50-year-old man, with no history other than chronic venous insufficiency of the lower limbs, presented with an asymptomatic eruption involving all four limbs, mainly the lower limbs, and appearing in episodes. Physical examination revealed brown macules, in some cases outlining the configuration of livedo reticularis. Laboratory findings were normal except for the presence of low levels of anticardiolipin activity at diagnosis, which had subsided three months later. Histological examination of the skin biopsy showed lymphocytic arteritis with some histiocytes and neutrophils, as well as an eosinophilic ring of parietal necrosis. Six months later, the patient developed multineuritis, leading to the diagnosis of polyarteritis nodosa. A diagnosis of cutaneous polyarteritis nodosa could also have been made based on the association of cutaneous livedo and locoregional polyneuritis without systemic involvement. DISCUSSION: The diagnosis of macular lymphocytic arteritis is based upon clinical and histological findings and upon disease progression. This entity seems to belong to the same spectrum as periarteritis nodosa, especially in the cutaneous form. Given the lack of knowledge concerning progression from macular lymphocytic arteritis to nodosa periarteritis, close patient monitoring is called for, as illustrated by our case report.

Cutaneous and mucosal aphthosis during temsirolimus therapy for advanced renal cell carcinoma: review of cutaneous and mucosal side effects of mTOR inhibitors.

Temsirolimus, an inhibitor of the mammalian target of rapamycin (mTOR), is a new targeted therapy used in advanced renal cell carcinoma and mantle cell lymphoma and is currently tested in several other human tumors. It induces several cutaneous and mucosal side effects, including painful, dose-limiting stomatitis. We report the unusual case of a 77-year-old man who developed severe mucosal, scrotal and perianal cutaneous aphthous-like ulcerations, 6 weeks after introduction of temsirolimus therapy for advanced-stage renal cell carcinoma. Other causes of aphthous-like ulcerations were ruled out. Topical corticosteroids remained ineffective. It led to the interruption of the treatment. Introduction of colchicine resulted in a dramatic improvement within 1 month. Reintroduction of temsirolimus with concomitant colchicine therapy was followed by a delayed recurrence of the lesions. We provide here a review of the potential cutaneous and mucosal side effects of mTOR inhibitors.

Lack of evidence of HTLV-I/II infection in T CD8 malignant or reactive lymphoproliferative disorders in France: a serological and/or molecular study of 169 cases.

Human T lymphotropic virus type II (HTLV-II), originally isolated in 1982 from a patient with a "T hairy cell leukemia", has not yet been proven to be the causative agent of any specific hematological disease. In order to screen for such an event, and because HTLV-II has a preferential tropism for OKT8 (CD8) T cells (both in vivo and in vitro), we searched for the presence of HTLV-II in lymphoproliferative diseases (LP) of CD8+ T cells. We report a serological and/or molecular study of 169 patients with a T CD8 LP, including 76 patients with malignant or reactive T CD8 LP (34 lymphomas, 27 large granular leukemias, three prolymphocytic leukemias, one hairy cell leukemia, 11 reactive T CD8 LP) and 93 HIV-1+ patients with a T CD8 peripheral lymphocytosis ( > 1500/mm3) from a prospective HIV cohort involving 1264 individuals. In the first series, the 40 sera available were all HTLV-I/II negative, except a 67-year-old French Guyanan man, with a cutaneous large T CD8 cell lymphoma, HTLV-I+. Furthermore, the molecular analysis of the 69 available DNA samples by PCR failed to detect any proviral HTLV-I/II sequences, except for the HTLV-I+ patient. The serological study of the 93 HIV-1+ individuals with CD8 lymphocytosis, showed that three patients were HTLV-I+, but none was HTLV-II+. Thus, in contrast to HTLV-I, whose etiological role in adult T cell leukemia is now well established, there is neither epidemiological nor molecular evidence that prototypic HTLV-II may be etiologically associated specifically with any of the CD8+ T cell LP investigated in this report.

Cutaneous T cell lymphomas: mycosis fungoides, Sezary syndrome and HTLV-I-associated adult T cell leukemia (ATL) in Mali, West Africa: a clinical, pathological and immunovirological study of 14 cases and a review of the African ATL cases.

Cutaneous T cell lymphomas (CTCL) are rare lymphoproliferative diseases, which are frequently suspected to be of viral origin. As very few data were available concerning cutaneous T cell lymphomas in tropical Africa, we undertook a clinical, histopathological, immunological and viro-molecular study of patients with a clinical diagnosis of cutaneous lymphoma, in Bamako, Mali. While prior to this study, no case of CTCL had been reported in this country, 14 patients (five women, nine men; mean age 58 years) with a diagnosis of cutaneous lymphoma were seen over a period of 30 months (1992-1994) in the only dermatological department in Mali. Clinically, the most frequent pattern was an infiltrated erythrodermia similar to Sezary syndrome. Nodular lesions and/or plaques were rarely observed. All these cutaneous tumors were T cell lymphoproliferations, only one expressing the CD8+ antigen. A comprehensive analysis of all the available data permitted characterization of three cases of adult T cell leukemia/lymphoma (ATL) associated with HTLV-I (one definitive case, of leukemic type, with demonstration of clonal integration of HTLV-I proviral genome and two probable ATL cases), three cases of Sezary syndrome (SS), two cases of mycosis fungoides (MF) and five cases of pleomorphic cutaneous lymphoma. In one case, the differentiation between MF and pleomorphic cutaneous lymphoma could not be established. HTLV-I serological and/or molecular markers were restricted to the three ATL cases. From the unique definitive ATL case, a T cell line was established from culture of peripheral blood mononuclear cells and sequence analysis of the env gene and the U3-LTR region demonstrated that the virus present in this patient belonged to the cosmopolitan subtype A. Thus, we report here the first evidence of HTLV-I infection and associated ATL in Mali. This is the second ATL case described for the whole Sahelian region (one ATL of the lymphoma type was reported previously in a Mauritanian patient). Furthermore, we demonstrate that the main types of CTCL described in Europe and North America are also present in this African area and that the prevalence of these diseases is greatly underestimated in such regions. Furthermore, no association was observed between HTLV-I/II infection and SS, MF or pleomorphic cutaneous lymphoma in Mali in contrast to other studies.

Human herpes virus 8 (Kaposi's sarcoma herpes virus) and malignant lymphoproliferations in France: a molecular study of 250 cases including two AIDS-associated body cavity based lymphomas.

The new human herpes virus 8 (HHV8) was recently detected in cases of body cavity based lymphoma (BCBL), a rare B cell lymphoma, mostly AIDS-associated. We investigated for HHV8 DNA sequences a series of 250 B or T cell lymphoproliferative malignancies, as seen in France, including 126 leukemias and 124 lymphomas (232 non-AIDS-associated and 18 AIDS-associated tumors). HHV8 sequences were detected in only three patients. The first two were homosexual males, HIV-infected since 1985 who suffered from a BCBL initially characterized in one case by a pleural lymphomatous effusion and a peritoneal one in the other case. A high level of HHV8 copies was detected in the tumoral cells of these two BCBL. In contrast, in the third positive patient who had an AIDS-associated immunoblastic lymphoma, the HHV8 sequences level was quite low. In the two BCBL patients, the HHV8-infected clonal B cells had a large immunoblastic feature with an indeterminate phenotype and were also infected by Epstein-Barr virus. In one BCBL case, a semiquantitative PCR analysis revealed that the HHV8 sequences were much more abundant in the effusion tumor cells than in the cutaneous Kaposi's biopsy while no HHV8 sequence was detectable in the peripheral blood lymphocytes. This study reports HHV8-associated BCBL in European AIDS patients and confirms that HHV8 is present at a high copy number in the tumoral B cells of this malignancy. Furthermore, HHV8 does not seem to play a pathogenic role in any of the other T or B malignant lymphoid neoplasias studied so far. This study also stresses the necessity for quantification studies in interpretation of a positive PCR analysis for HHV8 sequences, especially in patients at risk for HIV infection or Kaposi's sarcoma.

SCORTEN: a severity-of-illness score for toxic epidermal necrolysis.

The mortality of toxic epidermal necrolysis is about 30%. Our purpose was to develop and validate a specific severity-of-illness score for cases of toxic epidermal necrolysis admitted to a specialized unit and to compare it with the Simplified Acute Physiology Score and a burn scoring system. A sample of 165 patients was used to develop the toxic epidermal necrolysis-specific severity-of-illness score and evaluate the other scores, a sample of 75 for validation. Model development used logistic regression equations that were translated into probability of hospital mortality; validation used measures of calibration and discrimination. We identified seven independent risk factors for death and constituted the toxic epidermal necrolysis-specific severity-of-illness score: age above 40 y, malignancy, tachycardia above 120 per min, initial percentage of epidermal detachment above 10%, serum urea above 10 mmol per liter, serum glucose above 14 mmol per liter, and bicarbonate below 20 mmol per liter. For each toxic epidermal necrolysis-specific severity-of-illness score point the odds ratio was 3.45 (confidence interval 2.26-5.25). Probability of death was: P(death) = elogit/1 + elogit with logit = -4.448 + 1.237 (toxic epidermal nec-rolysis-specific severity-of-illness score). Calibration demonstrated excellent agreement between expected (19. 6%) and actual (20%) mortality; discrimination was also excellent with a receiver operating characteristic area of 82%. The Simplified Acute Physiology Score and the burn score were also associated with mortality. The discriminatory powers were poorer (receiver operating characteristic area: 72 and 75%) and calibration of the Simplified Acute Physiology Score indicated a poor agreement between expected (9.1%) and actual (26.7%) mortality. This study demonstrates that the risk of death of toxic epidermal necrolysis patients can be accurately predicted by the toxic epidermal necrolysis-specific severity-of-illness score. The Simplified Acute Physiology Score and burn score appear to be less adequate.

Magnetic resonance imaging in adults presenting with severe acute infectious cellulitis.

BACKGROUND AND DESIGN: Early categorization of some acute soft-tissue infections, such as severe infectious cellulitis (IC) without or with secondary abscess formation, necrotizing fasciitis (NF) or pyomyositis, is frequently difficult. The first one requires only medical treatment, the remaining ones require either surgery or closed drainage. To determine the presence and the extent of these infections early, we have prospectively studied the value of magnetic resonance imaging in patients admitted for IC with local or general criteria of severity. Images were analyzed on a blind basis. Definite diagnosis was obtained by reviewing clinical records and, in most patients, the results of an invasive procedure. RESULTS: Twenty-six patients (56 +/- 23 years old) were included in this study. Among them, 13 received gadolinium-diethylene-triaminepenta-acetic acid intravenously. The final diagnosis was pyomyositis (five patients), NF (three patients), or IC with (seven patients) or without (11 patients) subcutaneous abscess. Images specific for these diseases were best outlined with T2-weighted sequences. In patients with pyomyositis or subcutaneous abscess(es), we observed spindle-shaped or round, well-defined areas of high signal intensity within the muscles or subcutis, respectively. Patients with NF exhibited numerous homogeneous, well-defined dome-shaped areas of hypersignal in the deep hypodermis. In patients with uncomplicated IC, these dome-shaped areas of hypersignal appeared ill-defined, heterogeneous, smaller, thinner, and less numerous than those in patients with NF. CONCLUSIONS: In patients presenting severe IC, magnetic resonance imaging provided an early clue in the diagnosis of pyomyositis, NF, and abscess-complicated IC. By precisely defining the extent of these infections, it helped to plan surgical treatment.

[Bacterial cellulitis. Forms borderline between medical and surgical (3 cases)]. / Dermohypodermites bactériennes.

BACKGROUND: An acute infectious cellulitis may be managed medically (erysipelas or non-necrotizing infectious cellulitis) or surgically (necrotizing infectious cellulitis, necrotizing fasciitis). We report 3 cases of non-necrotizing infectious cellulitis borderline between medical and surgical forms, complicated by compartment syndrome, the surgical decompression of which permitted patients' cure. CASE REPORTS: Three patients, 27, 52 and 84 years old, were admitted for an acute infectious cellulitis of the leg. At admission, the leg area involved was erythematous, painful, indurated, with one or several bullae, purpura, pustules, hypoesthesia or limited skin necrosis, and no immediate need for surgical exploration. The clinical evolution was characterized by the slow appearance or extension of signs of severity, despite the modification in antibiotic treatment. Magnetic resonance imaging findings were indicative of a non-necrotizing infectious cellulitis in 2 patients. In one patient, necrotizing fasciitis could not be excluded. In all patients, surgical exploration showed an important quantity of non-purulent fluid between muscles and hypodermis, with no evidence of abscess or necrosis. A large incision rapidly cured all patients. DISCUSSION: These three observations were characterized by the initial signs of moderate severity and no response to an appropriate medical treatment, which led to surgical exploration. Surgery showed no abscess or necrosis but an important quantity of sterile fluid; it also permitted rapid cure of patients. These cases present a borderline form of infectious cellulitis, with severe local inflammation caused by a compartment syndrome. Surgical decompression was needed for cure. The potential value of magnetic resonance imaging in this situation should also be stressed.

Detection and genetic polymorphism of human herpes virus type 8 in endemic or epidemic Kaposi's sarcoma from West and Central Africa, and South America.

Kaposi's-sarcoma-associated herpesvirus(KSHV)/human-herpes-virus-8(HHV-8) sequences originally detected in AIDS-associated Kaposi's sarcoma have been found in almost every KS tested, whether endemic, classic, iatrogenic or epidemic. Most of the studies on African KS involved East African patients. We report herewith the study of 17 African or Guyanan KS patients, 3 with epidemic KS (EKS) from Central African Republic, 3 from Senegal (2 EKS and 1 endemic KS), 3 EKS from Cameroon and 8 from French Guiana (3 EKS and 5 endemic KS). Serum-specific antibodies directed against latent and/or lytic HHV-8 antigens were present in 16 of them (94%), detected either by immunofluorescence assay and/or by immunoperoxidase. Polymerase chain reaction (PCR), using specific primers for HHV-8 ORF26 (233 bp) and ORF75 (601 bp), was carried out on DNA extracted from KS cutaneous biopsies, clinically uninvolved skin biopsies and peripheral-blood mononuclear cells (PBMC). HHV-8 DNA was detected in 16 out of 16 (100%) KS biopsies, regardless of their origin or clinico-pathological sub-type, in 7 out of 15 (47%) normal skin samples and 7 out of 16 (44%) PBMC. Comparative PCR, carried out in 7 patients, regularly found a much higher viral load in KS biopsies than in autologous normal skin and PBMC samples. Sequencing of fragments of the ORF26 and of the ORF75 demonstrated that the 16 HHV-8 strains were of the A, B or C sub-type. Furthermore, sequences of the entire ORF K1 of 4 strains showed that these HHV-8 strains of African origin were of the A5 or the B sub-type.