Rituximab is an effective treatment in patients with pemphigus vulgaris and demonstrates a steroid-sparing effect.

BACKGROUND: Corticosteroids (CS) with or without adjuvant immunosuppressant agents are standard treatment for pemphigus vulgaris (PV). The efficacy of adjuvant therapies in minimizing steroid-related adverse events (AEs) is unproven. OBJECTIVES: To utilize data collected in a French investigator-initiated, phase III, open-label, randomized controlled trial to demonstrate the efficacy and safety of rituximab and seek approval for its use in PV. METHODS: This was an independently conducted post hoc analysis of the moderate-to-severe PV subset enrolled in the Ritux 3 study. Patients were randomized to rituximab plus 0·5 or 1·0 mg kg-1 per day prednisone tapered over 3 or 6 months, or 1·0 or 1·5 mg kg-1 per day prednisone alone tapered over 12 or 18 months, respectively (according to disease severity). The primary end point was complete remission at month 24 without CS (CRoff) for ≥ 2 months, and 24-month efficacy and safety results were also reported. RESULTS: At month 24, 34 of 38 patients (90%) on rituximab plus prednisone achieved CRoff ≥ 2 months vs. 10 of 36 patients (28%) on prednisone alone. Median total cumulative prednisone dose was 5800 mg in the rituximab plus prednisone arm vs. 20 520 mg for prednisone alone. Eight of 36 patients (22%) who received prednisone alone withdrew from treatment owing to AEs; one rituximab-plus-prednisone patient withdrew due to pregnancy. Overall, 24 of 36 patients (67%) on prednisone alone experienced a grade 3/4 CS-related AE vs. 13 of 38 patients (34%) on rituximab plus prednisone. CONCLUSIONS: In patients with moderate-to-severe PV, rituximab plus short-term prednisone was more effective than prednisone alone. Patients treated with rituximab had less CS exposure and were less likely to experience severe or life-threatening CS-related AEs. What's already known about this topic? Pemphigus vulgaris (PV) is the most common type of pemphigus. Corticosteroids, a standard first-line treatment for PV, have significant side-effects. Although their effects are unproven, adjuvant corticosteroid-sparing agents are routinely used to minimize steroid exposure and corticosteroid-related side-effects. There is evidence that the anti-CD20 antibody rituximab is effective in the treatment of patients with severe recalcitrant pemphigus and in patients with newly diagnosed pemphigus. What does this study add? This study provides a more detailed analysis of patients with PV enrolled in an investigator-initiated trial. Rituximab plus prednisone had a steroid-sparing effect and more patients achieved complete remission off prednisone. Fewer patients experienced grade 3 or grade 4 steroid-related adverse events than those on prednisone alone. This collaboration between academia and industry, utilizing independent post hoc analyses, led to regulatory authority approvals of rituximab in moderate-to-severe PV.

Idiopathic linear IgA bullous dermatosis: prognostic factors based on a case series of 72 adults.

BACKGROUND: Linear IgA bullous dermatosis (LABD) is a clinically and immunologically heterogeneous, subepidermal, autoimmune bullous disease (AIBD), for which the long-term evolution is poorly described. OBJECTIVES: To investigate the clinical and immunological characteristics, follow-up and prognostic factors of adult idiopathic LABD. METHODS: This retrospective study, conducted in our AIBD referral centre, included adults, diagnosed between 1995 and 2012, with idiopathic LABD, defined as pure or predominant IgA deposits by direct immunofluorescence. Clinical, histological and immunological findings were collected from charts. Standard histology was systematically reviewed, and indirect immunofluorescence (IIF) on salt-split skin (SSS) and immunoblots (IBs) on amniotic membrane extracts using anti-IgA secondary antibodies were performed, when biopsies and sera obtained at diagnosis were available. Prognostic factors for complete remission (CR) were identified using univariate and multivariate analyses. RESULTS: Of the 72 patients included (median age 54 years), 60% had mucous membrane (MM) involvement. IgA IIF on SSS was positive for 21 of 35 patients tested; 15 had epidermal and dermal labellings. Immunoelectron microscopy performed on the biopsies of 31 patients labelled lamina lucida (LL) (26%), lamina densa (23%), anchoring-fibril zone (AFz) (19%) and LL+AFz (23%). Of the 34 IgA IBs, 22 were positive, mostly for LAD-1/LABD97 (44%) and full-length BP180 (33%). The median follow-up was 39 months. Overall, 24 patients (36%) achieved sustained CR, 19 (29%) relapsed and 35% had chronic disease. CR was significantly associated with age > 70 years or no MM involvement. No prognostic immunological factor was identified. CONCLUSIONS: Patients with LABD who are < 70 years old and have MM involvement are at risk for chronic evolution.

Calculation of cut-off values based on the Autoimmune Bullous Skin Disorder Intensity Score (ABSIS) and Pemphigus Disease Area Index (PDAI) pemphigus scoring systems for defining moderate, significant and extensive types of pemphigus.

BACKGROUND: Two pemphigus severity scores, Autoimmune Bullous Skin Disorder Intensity Score (ABSIS) and Pemphigus Disease Area Index (PDAI), have been proposed to provide an objective measure of disease activity. However, the use of these scores in clinical practice is limited by the absence of cut-off values that allow differentiation between moderate, significant and extensive types of pemphigus. OBJECTIVES: To calculate cut-off values defining moderate, significant and extensive pemphigus based on the ABSIS and PDAI scores. METHODS: In 31 dermatology departments in six countries, consecutive patients with newly diagnosed pemphigus were assessed for pemphigus severity, using ABSIS, PDAI, Physician's Global Assessment (PGA) and Dermatology Life Quality Index (DLQI) scores. Cut-off values defining moderate, significant and extensive subgroups were calculated based on the 25th and 75th percentiles of the ABSIS and PDAI scores. The median ABSIS, PDAI, PGA and DLQI scores of the three severity subgroups were compared in order to validate these subgroups. RESULTS: Ninety-six patients with pemphigus vulgaris (n = 77) or pemphigus foliaceus (n = 19) were included. The median PDAI activity and ABSIS total scores were 27·5 (range 3-84) and 34·8 points (range 0·5-90·5), respectively. The respective cut-off values corresponding to the first and third quartiles of the scores were 15 and 45 for the PDAI, and 17 and 53 for ABSIS. The moderate, significant and extensive subgroups were thus defined, and had distinguishing median ABSIS (P < 0·001), PDAI (P < 0·001), PGA (P < 0·001) and DLQI (P = 0·03) scores. CONCLUSIONS: This study suggests cut-off values of 15 and 45 for PDAI and 17 and 53 for ABSIS, to distinguish moderate, significant and extensive pemphigus forms. Identifying these pemphigus activity subgroups should help physicians to classify and manage patients with pemphigus.

Place of human amniotic membrane immunoblotting in the diagnosis of autoimmune bullous dermatoses.

BACKGROUND: Fine analysis of antiskin autoantibodies can contribute to the differential diagnosis of autoimmune bullous dermatoses. OBJECTIVES: To develop a high-performance immunoblotting method using human amniotic membrane as the antigen source, and to compare it with current laboratory methods. METHODS: Sera from 113 patients were tested by immunoblotting (IB), rat and monkey oesophagus and salt-split skin indirect immunofluorescence (IIF), and enzyme-linked immunosorbent assay (ELISA) quantification of anti-BP180-NC16a and anti-BP230, or antidesmoglein (Dsg) 1 and 3 antibodies. There were 56 cases of bullous pemphigoid (BP), 22 cases of mucous membrane pemphigoid (MMP), eight cases of epidermolysis bullosa acquisita (EBA), two cases of bullous systemic lupus erythematosus (BSLE), 17 cases of pemphigus vulgaris (PV), and four cases each of pemphigus foliaceus (PF) and paraneoplastic pemphigus (PNP). RESULTS: In BP, the three methods had similar sensitivity (84-89%) for both anti-BP180-NC16a and anti-BP230 antibody detection. In MMP, autoantibodies (mainly directed against BP180 or laminin 332 subunits) were detected in 77% of patients by IB, compared with only 9% by IIF on rat and monkey oesophagus and 36% on salt-split skin, and 14% by anti-BP180-NC16a and anti-BP230 ELISA. In patients with pemphigus, ELISA had 92% sensitivity for anti-Dsg1 and 3, but IB and rat bladder IIF were necessary to confirm PNP by revealing specific and rare patterns (antidesmoplakin I/II, antienvoplakin and antiperiplakin antibodies). IB also revealed anticollagen VII antibodies in 60% of patients with EBA and BSLE, and antibodies to BP180, BP230 and Dsg3 in a few patients who were negative using the other two techniques. CONCLUSION: Amniotic membrane immunoblotting is an interesting diagnostic tool for bullous diseases, as the entire panel of autoantibodies can be detected with a single extract. This method improves the identification of complex and heterogeneous autoimmune processes in conjunction with IIF and ELISA, and is particularly useful for MMP characterization.

[Pyoderma gangrenosum with aseptic spleen abscess]. / Pyoderma gangrenosum et abcès splénique aseptique.

BACKGROUND: Pyoderma gangrenosum is a neutrophilic dermatosis in which systemic involvement is rare. It may be associated with systemic disease. We report a case of pyoderma gangrenosum in the spleen. CASE REPORT: A 68-year-old man presenting pyoderma gangrenosum with pustules and stage I multiple myeloma was admitted for asthenia and abdominal pain. There were no skin lesions. Laboratory tests showed inflammatory syndrome with polynuclear leucocytes of 25,000/mm(3). CAT scans and abdominal ultrasound revealed a splenic abscess. A spleen biopsy was performed and histological examination showed polynuclear leukocyte infiltration, while cultures were negatives. Diagnosis of pyoderma gangrenosum with splenic involvement was made. Increased systemic corticosteroid therapy produced a successful outcome. Haematological findings remained unchanged. DISCUSSION: Spleen involvement in pyoderma gangrenosum is very rare and can mimic an infectious process. In such cases, routine screening is essential for associated diseases, particularly haematological malignancies.

[Multiple aortic aneurysms in chronic atrophic polychondritis]. / Anévrysmes aortiques multiples au cours d'une polychondrite atrophiante.

BACKGROUND: Although rare, cardiovascular involvement is the second most frequent cause of mortality in chronic relapsing polychondritis behind tracheobronchial tree chondritis. The most frequent cardiovascular complications are valvulopathy and aortic aneurysm. CASE REPORT: We report a case of chronic relapsing polychondritis with multiple aortic aneurysms that were clinically silent but continued to progress despite systemic corticosteroids and immunosuppressive therapy. DISCUSSION: Progression of aortic aneurysms and extravascular disease do not appear to be correlated. Although the disease may appear to be in remission, vascular lesions can continue to progress independently. This case shows that medical treatment has little effect on the progression of these aneurysms. Consequently, it is necessary to opt for surgical therapy at the opportune moment.

[Haemolytic uremic syndrome and acute mesenteric ischemia caused by interferon-alpha-2b in the treatment of Kaposi's sarcoma in an AIDS patient]. / Syndrome hémolytique et urémique et ischémie mésentérique aiguë lors d'un traitement par interféron alpha-2b pour une maladie de Kaposi associée au VIH.

BACKGROUND: Interferon alpha is approved for the treatment of Kaposi's sarcoma in HIV infected patients. Hemolytic and uremic syndrome (HUS) is a rare side-effect of interferon alpha and has been reported primarily in chronic myelogenous leukemia. CASE REPORT: A 44-year-old HIV-infected woman from Cameroon was admitted for treatment of cutaneous Kaposi's sarcoma. Two days later, she presented severe HUS. Abdominal pains subsequently revealed non-occlusive mesenteric ischemia. The patient rapidly improved after interferon withdrawal. DISCUSSION: To our knowledge this is the first case of HUS induced by interferon alpha given for Kaposi's sarcoma. Further, no cases of acute mesenteric ischemia have been reported with interferon alpha. It is possible that the condition may have been induced or aggravated by HUS or a low infusion rate. Interferon can exert vascular toxicity on both the mesenteric vessels and the renal vessels in a setting of microangiopathy.

[Correlation between the presence of type-2 anti-pemphigoid antibodies and dementia in elderly subjects with no clinical signs of pemphigoid]. / Corrélation entre présence d'anticorps anti-antigène de type 2 de la pemphigoïde et démence chez les sujets âgés sans manifestation clinique de pemphigoïde.

BACKGROUND: Pemphigoid is a form of auto-immune bullous dermatosis characterised by the production of antibodies directed against components of hemidesmosomes in the basal membrane. The physiopathological process responsible for unmasking of these antigens is unknown. Pemphigoid is more common in elderly subjects and is most often seen in debilitated subjects. The prevalence of pemphigoid anti-pemphigoid antibodies (anti-PB) is not known in the elderly population presenting no dermatological signs evocative of the disease. We studied the prevalence of anti-PBAg2 antibodies in elderly subjects with no signs of pemphigoid as well as in the correlation between the presence of these antibodies and diagnosis of dementia. PATIENTS AND METHODS: Elderly subjects (aged over 69 years) with no signs of pemphigoid were recruited consecutively in dermatology and geriatrics departments (138 subjects). Details of concomitant medication were recorded for all subjects and clinical examination was performed with calculation of MMS (Mini Mental Score). The subjects were then divided into two groups based on MMS score. The first group comprised subjects without dementia (MMS > 24) while the second comprised subjects with dementia. Serum anti-PBAg2 antibodies were determined by ELISA and indirect immunofluorescence with confirmation by Western blot. Antinuclear antibodies, used as a control for non-specific immune response, were assayed in all serum samples. The prevalence of these antibodies was compared between the two groups. RESULTS: The two groups were comparable in terms of age, sex and presence of dermatological diseases (ulcers, bedsores, erysipelas). Each group comprised 69 subjects. The overall presence of anti-PBAg2 antibodies in subjects with no signs are suggestive of pemphigoid was 3.6%. Presence of anti-PBAg2 antibodies was associated with diagnosis of dementia (p=0.04; 0% and 7% in groups 1 and 2, respectively). No correlation was seen between the presence of anti-PBAg2 antibodies and concomitant medication or dermatological disease. The overall prevalence of antinuclear antibodies was 14.5% and the figure was similar between the two groups. DISCUSSION: The presence of anti-PBAg2 could be associated with the diagnosis of dementia in elderly subjects.

Docetaxel in anthracycline-pretreated AIDS-related Kaposi's sarcoma: a retrospective study.

BACKGROUND: Kaposi's sarcoma (KS) is a potentially life-threatening multifocal neoplasm. Despite the significant decline in the incidence of acquired immune deficiency syndrome (AIDS)-related KS with the use of highly active antiretroviral therapy (HAART), some patients, even those with a good immune restoration, still have aggressive disease. Liposomal anthracyclines or combination chemotherapy are widely used but adverse effects limit their utilization. OBJECTIVES: We studied the efficacy and tolerance of docetaxel in the treatment of AIDS-related KS after pretreatment with anthracycline. PATIENTS/METHODS AND MAIN OUTCOME MEASURE: A retrospective cohort study was done. Nine human immunodeficiency virus (HIV)-infected patients were treated from 1997 to 2002 with docetaxel. Tumour response was evaluated using the AIDS Clinical Trial Group (ACTG) staging criteria. Clinical and biological toxicity was evaluated. AIDS status with HIV viral load and CD4 T-cell count were measured at the beginning and at the end of the treatment. RESULTS: A major (complete or partial) response and a stabilization of the disease were demonstrated in seven and two patients, respectively. Grade 4 neutropenia and thrombocytopenia were observed in four of nine and one of nine patients, respectively. One patient died after sepsis. CONCLUSIONS: Docetaxel has a good and rapid efficacy in anthracycline-pretreated patients with severe AIDS-related KS. Phase II/III trials should be done to compare docetaxel with liposomal anthracyclines as a first-line treatment.

[Bullous pemphigoid in young patients: a retrospective study of 74 cases]. / Pemphigoïde du sujet jeune.

INTRODUCTION: Bullous pemphigoid usually affects elderly people. Only a few isolated cases among people younger than 65 years have been reported. OBJECTIVES: Describe the clinical and biological characteristics of patients younger than 60 years suffering from bullous pemphigoid, compare them with the usual characteristics known among elderly people and search for potential pathological associations. PATIENTS AND METHODS: Retrospective, national, multicenter study. Clinical, biological and histological characteristics were recorded with a standardised questionnaire as well as treatments and associated pathologies. RESULTS: Seventy-four cases of bullous pemphigoid diagnosed between June 1970 and March 2002 were analyzed. Mean age at the beginning of the disease was 46 +/- 11.6 years. Further explorations by indirect immunofluorescence of separated skin and/or immuno-electron microscopy and/or immunoblotting were performed for 42 patients (56.8 p. 100). Clinical characteristics among this restricted population were comparable to those found among the 32 other cases. Compared to usual data on bullous pemphigoid in elderly people, we observed a greater proportion of extensive form of disease (75 p. 100), a more frequent head and neck involvement (39.2 p. 100) and an overexpression of anti-BP180 autoantibodies (48 p. 100). Neoplasm was notified for 7 patients (9.5 p. 100), 18 (24.3 p. 100) suffered from a pathology of the basement membrane zone (6 psoriasis, 6 atopic dermatitis and 6 lichen) and 13 from neurological disease, among which 4 were bedridden. Fourty-six patients (62.2 p. 100) received drugs for the long term (mean 2.12 +/- 2.43), 4 patients were treated by PUVAtherapy and 2 by radiotherapy. DISCUSSION: Our results suggest that bullous pemphigoid among young people is more severe and more active than the usual form in the elderly. This particular form could be the result of a higher expression of anti-BP180 autoantibodies, which are considered as a marker of poor prognosis in this disease. We also found a high frequency of pathological associations and physical treatment, all responsible for damage to the basement membrane zone, which can involve auto-immunization against hemidesmosome components.

[Treatment of pemphigus with intravenous immunoglobulin]. / Immunoglobulines intraveineuses dans le traitement du pemphigus.

INTRODUCTION: The interest of intravenous immunoglobulins (Igi.v.) in the treatment of pemphigus is discussed. Pemphigus is not a recognized indication for this treatment by the CEDIT (French Committee for the assessment and diffusion of technological innovations). The aim of this study was to assess the efficacy of Igi.v. in the treatment of severe corticosteroid-dependent or resistant pemphigus. MATERIAL AND METHODS: A retrospective study using a standardized questionnaire was conducted in the various departments of dermatology among the "Groupe Bulles" of the French Society of Dermatology. The study collected the following information from 12 patients suffering from pemphigus and treated with Igi.v.: 1) general demographical data; 2) characteristics of the pemphigus; 3) different treatments applied, and 4) efficacy and side effects of treatments. RESULTS: Among the 12 patients studied at the end of treatment with Igi.v., 8 were in complete remission (1 fleetingly for 2 months and 1 preceding initiation of Igi.v.), and 2 were improved (1 temporarily for 4 months). A reduction in corticosteroid therapy was possible in 75 p. 100 of cases (9 patients). During treatment with Igi.v., immunosuppressors were combined with oral corticosteroids in 3 cases. It was possible to reduce their dose 1 one case and to stop them in another case. No major side effect related to treatment with Igi.v. was observed. Six months and one year after the treatment, complete remission rates were respectively 6/10 and 5/8. One patient relapsed more than one year after the end of Igi.v. treatment. CONCLUSIONS: Although very expensive, treatment with Igi.v. appears of interest in the treatment of severe corticosteroid-dependent or resistant pemphigus. Moreover tolerance is excellent. The results of our study warrant confirmation in a prospective study.

[Vinblastine treatment for extensive non-X histiocytosis (xanthoma disseminatum)]. / Histiocytose non langerhansienne (xanthoma disseminatum) extensive traitée par vinblastine.

INTRODUCTION: Xanthoma disseminatum is a non-Langerhans histiocyte proliferation, described by Montgomery in 1938. This rare entity is characterized by skin and mucous membrane xanthomatosis, associated with diabetes insipidus and normal lipid metabolism. In this case report, vinblastine produced the regression of the lesions. OBSERVATION: A 51 year-old man presented in 1999 with a four-year history of progressive xanthomatous papulonodular lesions of his trunk, axillary and inguinal folds, neck and face (leonin facies). Treatment with thalidomide for 6 months was ineffective. A rapid extension of the lesions to the pharynx, larynx and trachea with dyspnea occurred and required a tracheotomy. Histopathological study showed a dense histiocytic infiltrate within the upper and mid dermis with Touton giant cells and inflammatory cells. The histiocyte cells were positive for CD68. Neither lungs nor hypophysis were involved. A treatment with 6 cures of cyclophosphamide was insufficient. Vinblastine therapy (32 cycles: 0.1 mg/kg/cycle) produced a spectacular regression of the mucous-cutaneous papulonodular lesions leaving cheloid scars. The patient required a transitory tracheotomy and a bilateral commissuroplasty. DISCUSSION: Prognosis of xanthoma disseminatum is in related to the mucous membrane manifestations (50 p. 100 of cases) and involvement of the upper respiratory tract. The response to any form of therapy in xanthoma disseminatum is unsatisfactory. Surgical excision or laser therapy can improve physical and functional aspects but the evolution is characterized by very frequent relapses. Treatment with antimitotic drugs seems to be ineffective in many cases. In our patient, vinblastine induced a spectacular regression of mucocutaneous lesions without neurologic toxicity. To our knowledge, this is the first report of vinblastine efficacy in this rare and severe disorder.

[Cutaneous necrosis is predictive of cancer in adult dermatomyositis]. / Les nécroses cutanées dans les dermatomyosites de l'adulte sont prédictives de l'association à une néoplasie.

INTRODUCTION: Adult dermatomyositis is associated with cancer in 15 p. 100 to 50 p. 100 of cases and, hence, investigations should be systematically performed to search for cancer. A number of predictive factors have been reported. The aim of our study was to search for predictive factors of cancer, among adults with dermatomyositis. METHODS: We prospectively assessed 26 adults presenting with dermatomyositis, hospitalised in our department of dermatology from January 1993 to June 2000. The parameters assessed were: association with a cancer, age, gender, cutaneous necrosis, muscular weakness, electromyographic abnormalities, erythrocyte sedimentation rate, and muscular enzyme levels. RESULTS: Mean age was of 52 years and sex ratio (M/F) was of 0.53. Cancers were diagnosed in eight cases (31 p. 100) (mean age: 59.5 years; sex ratio=1; cancer localization: lung (2), breast (2), ovary, endometrium, bladder, and melanoma). Five patients in the cancer group had cutaneous necrosis and only 2 in the without cancer (p=0.01; PPV=71.4 p.100). Elevation of muscular enzyme was also associated with cancer. CONCLUSION: Our report demonstrates that cutaneous necrosis is closely associated with cancer and it suggests that in selected patients with dermatomyositis and cutaneous necrosis, more exhaustive and repeated investigations should be performed to search for cancer. The interest of elevation in muscular enzyme as a predictive factor of cancer is discussed.

[Seizures during interferon alpha therapy: three cases in dermatology]. / Crises épileptiques généralisées: un effet secondaire de l'interféron alpha: 3 cas.

INTRODUCTION: Interferon alpha has many side effects. Among them the risk of occurrence of seizures is not well known by dermatologists. We report three cases of seizures that occurred in patients treated with interferon alpha in two dermatological diseases: mycosis fungoides and melanoma. OBSERVATIONS: A 68 year-old man, treated for mycosis fungoides, and two men aged 47 and 52 years, treated for melanoma, were under interferon alpha. After 11 months, 3 weeks and 9.5 months, respectively, the three patients had seizures without any past history of epilepsy. Anamnesis and assessment of each patient (brain CT, biological results) suggested the responsibility of interferon alpha. After withdrawal of the treatment, no relapse was observed after 3 months, 6 months and 1 year later, respectively. DISCUSSION: Seizures during treatment with interferon alpha have already been reported. According to the series their prevalence would be of 1 to 4 p. 100. Their pathophysiology is not well known, but apparently interferon alpha lowers the epileptogenic threshold by affecting the central nervous system either directly or through cytokines or neuromediators. The risk of occurrence of seizures must be known by the prescribing physician who must systematically search for past history of epilepsy or risk factors for seizures. This rare but existing side effect raises the problem of information to be supplied to the patient by the prescribing physician.

[Retroperitoneal fibrosis secondary to metastatic neoplasm revealed by a leg lymphedema]. / Lymphoedème du membre inférieur révélant une fibrose rétropéritonéale d'origine métastatique.

INTRODUCTION: Diagnosis of retroperitoneal fibrosis is generally delayed and revealed by various non-specific signs. We report the case of an isolated lymphedema of the lower limb revealing retroperitoneal fibrosis complicating a metastatic squamous cell carcinoma. CASE REPORT: In an 83-year-old women, a lymphedema appeared that remained isolated for several months before being associated with alteration in general health. Morphological examinations showed bilateral compression of the urinary excretory tracts and led to the diagnosis of retroperitoneal fibrosis. Histological examination of a sub-clavicular adenopathy that had evolved over 9 months, confirmed the diagnosis of a metastatic squamous cell carcinoma of pulmonary cancer. DISCUSSION: Retroperitoneal fibrosis is an exceptional etiology that must be recognized in isolated lymphadomas of the lower limbs. In view of the possible tumoral origin of retroperitoneal fibrosis, any evocative sign accompanying the lymphedema must be searched for.