To the editor: Cutaneous leukocytoclastic vasculitis is a clinical and pathologic entity characterized by fibrinoid necrosis of vessel walls associated with vascular and perivascular infiltration by polymorphonuclear leukocytes with leukocytoclasia. The clinical manifestations are variable: palpable purpura (50 % of cases), urticaria, vesiculobullous lesions, erythematous papules, nodules, livedo reticularis, ulcerations ... (1, 2). Collagen vascular diseases, infections, and allergic reactions are the most common causes. This vasculitis can also be associated with malignant disease, most often a lymphoor myeloproliferative disorder, or, rarely, a solid tumor (3). Among the hemopathies, it is a rare finding in Hodgkin’s disease. We report 2 cases of vasculitis that revealed Hodgkin’s disease. Case n”1: A 46-year-old man, was hospitalized in December 1982 with a 3-month history of erythema multifornie of the proximal areas of the limbs and trunk associated with urticaria. Clinical examination revealed enlarged, nontender, bilateral cervical and axillary lymph nodes without hepatosplenomegaly. Laboratory studies disclosed the following: sedimentation rate 35/65, alpha 2 globulin 10 g/l, fibrinogen 4 g/l; white blood cell count 8.4. 109/1 with 82% neutrophils, hemoglobin 148 g/l, platelets 324. 109/l; no antinuclear antibody, circulating immune complexes or cryoglobulin were found; CH50 was normal (205 Vargue units) as were C3 (1400 mg/l) and C4 (470 mg/l); the test for hepatitis B surface antigen was negative. Chest radiographs showed widening of the upper mediastinum. Computed tomography of the abdomen and a bone marrow biopsy were normal. The biopsy of a supraclavicular lymph node revealed Hodgkin’s disease (type I according to the Lukes-Rye classification). A skin biopsy showed marked perivascular neutrophilic infiltration in the dermis and fibrinoid necrosis of the vascular wall, suggesting leukocytoclastic vasculitis. The first course of chemotherapy (ABVD: adriamycin 35 mg/ m2 d l and d8, bleomycin 10 mg/m2 d l and d8, vinblastine 6 mg/m2 d l and d8, DTIC 375 mg/m2 d l and d8) resulted in the disappearance of the skin lesions and lymphadenopathy. Six courses of ABVD associated with radiotherapy to the upper body (40 Grays) achieved complete remission that has lasted for 9 yr since the diagnosis without any recurrence of the vasculitis. Case n”2: In December 198 1, a 69-yr-old man was hospitalized for generalized pruritus and a widespread papular rash. The medical history revealed no ingestion of medication. The physical examination was normal. Blood tests disclosed no acute phase reactants. The white blood cell count was 7.5.109/1 with 75% neutrophils; hemoglobin 121 g/ 1, and platelets 264. 109/l. The skin biopsy demonstrated a leukocytoclastic vasculitis. No antinuclear antibody, circulating immune complexes, or cryoglobulin were found; complement levels were normal (CH50: 300Vargueunits, C3: 1150 mg/l, C4: 750 mg/ 1); the test for hepatitis B surface antigen was negative. Dental extraction, with the administration of antibiotics, brought about no improvement in the vasculitis. Nine months later, the pruritus and skin lesions still persisted. Steroids were instituted (0.5 mg/kg/d) without success. Twelve months after the appearance of the vasculitis, left paratracheal lymphadenopathy was discovered. Mediastinoscopy with lymph node biopsy established the diagnosis of Hodgkin’s disease (type I1 according to the LukesRye classification). A computed tomographic scan of the abdomen and a bone marrow biopsy were normal. The first course of chemotherapy (MOPP: methylchlorethamine 6 mg/m2 d l and d8, vincristine 1.4 mg/m2 d l and d8, procarbazine 100 mg/m2 d 1 to d14, prednisone 40 mg/m2 d l to d14) was followed by the disappearance of the skin lesions. After 4 courses of MOPP chemotherapy and radiotherapy of the upper body (40 Grays), the patient has remained free of Hodgkin’s disease for the last 10 yr, without recurrence of the vasculitis. The finding of specific cutaneous lesions in Hodgkin’s disease is rare, occurring in 3.4% to 7.6% in older series, and is currently found in only 0.5% (4). The trunk and limbs are most frequently involved by nodular lesions that can undergo ulceration; papules or generalized erythema can also be encountered. In such cases, there is cellular infiltration of the superficial and medium dermis by abnormal histiocytes, lymphocytes, neutrophils and eosinophils, as well as Reed-Sternberg’s cells, which are required to establish the diagnosis of skin involvement by Hodgkin’s disease (4). The prognostic value

This immunohistochemical study war performed on temporal artery biospies from eight patients with giant cell (temporal) arteritis: three before treatment four after a short period of corticosteroid therapy (from 1 day to 7 days) and one during a relapse occuring after a treatment of 9 years; from four subjects with clinical symptoms but without histological features of giant cell arteritis and from five negative controls Before treatment, biopsies of patients with temporal arteritis showed on inflammatory infiltrate with macrophages and T cells, essentially CD4+ and memory T cells (CD45 RO+) expressing the markers of activation IL2 receptor and HLA DR. Few B and NK cells were also detected. Adhesion molecules, LFA1 and I-CAM1, were strongly expressed by T cells and macrophages. In contrast the ligand to the CD2 the CD58 marker, was rarely detected. These immunohistochemical features were also observed after a short corticosteroid treatment CD4+ intravenous methylprednisolone or oral prednisone), with presence of activated T cells, memory T cells, macrophages and I-CAM1 and LFA1 expressing cells in the infiltrate. A temporal biopsy, performed after a long time of corticosteroid therapy, showed activated T cells, macrophages and memorycells in o,ne arteriole. In controls, this study showed some mononuclear cells dispersed in intima and adventia, but without activated or memory T cells. Our results support the presence of immune local respose in temporal arteritis incompletly improved by a short corticosteroid treatment.

Cette étude immunohistochimique porte sur les biopsies temporales, provenant de huit malades ayant une maladie de Horton (MH): trois avant traitement, quatre après une corticothérapie brève (de 1 à 7 jours) et un lors d'une récidive après 9 ans de traitement. Elles sont comparées à celles de cinq témoins négatifs et de cinq maladies de Horton, sans lésion histopathologique classique. Avant traitement, les biopsies de MH révèlent que l'infiltrat inflammatoire est essentiellement composé de macrophages et de lymphocytes T, principalement CD4+; un grand nombre de ces cellules O sont des lymphocytes O mémoires (CD45RO+), elles expriment des marqueurs d'activation (récepteur de l'IL2 et HLA-DR). Il y a peu de lymphocytes T et très peu de cellules NK. Les molécules d'adhésion LFA1 et I-CAM1 sont fortement exprimées par les lymphocytes et, à un degré variable, par les macrophages. En revanche, peu de cellules expriment CD58 dont CD2 est le ligand. Une corticothérapie brève (bolus de méthylprednisolone pendant 1 jour ou prednisone per os pendant 5 à 7 jours) modifie peu ces aspects (présence de cellules O activées, de cellules O mémoires, de macrophages et expression d'I-CAMl et de LFA1). Une biopsie temporale pratiquée pour une récidive après 9 ans de corticothérapie montre la présence d'un processus immunologique actif. Chez les témoins, il existe seulement quelques cellules inflammatoires dispersées dans l'intima et l'adventice, mais sans cellules O activées et sans cellules-mémoires. Ces données confortent l'existence d'une réponse immunitaire locale dans l'artérite temporale, imparfaitement contrôlée par une corticothérapie brève.

The immunohistochemical study was performed on temporal artery biopsies from eight patients with giant cell (temporal) arteritis: three before treatment, four after a short period of corticosteroid therapy (from 1 day to 7 days) and one during relapse occurring after a treatment of 9 years; from four subjects with clinical symptoms but without histological features of giant cell arteritis and from five negative controls. Before treatment, biopsies of patients with temporal arteritis showed an inflammatory infiltrate with macrophages and T cells, essentially CD4+ and memory T cells (CD45 RO+), expressing the markers of activation IL2 receptor and HLA DR. Few B and NK cells were also detected. Adhesion molecules, LFA1 and I-CAM1, were strongly expressed by T cells and macrophages. In contrast, the ligand to the CD2, the CD58 marker, was rarely detected. These immunohistochemical features were also observed after a short corticosteroid treatment (by intravenous methylprednisolone or oral prednisone), with presence of activated T cells, memory T cells, macrophages and I-CAM1 and LFA1 expressing cells in the infiltrate. A temporal biopsy, performed after a long time of corticosteroid therapy, showed activated T cells, macrophages and memory cells in o,ne arteriole. In controls, this study showed some mononuclear cells dispersed in intima and adventia, but without activated or memory T cells. Our results support the presence of immune local response in temporal arteritis, incompletely improved by a short corticosteroid treatment.

IL-6 serum levels are increased in patients suffering from GCA, and this cytokine may play a role in the systemic symptoms associated with this disease. We analyzed by in situ hybridization and immunohistochemistry whether IL-6 production originates from arterial granulomas in GCA. Seven arterial biopsy specimens taken from patients with histologically proved GCA were studied. IL-6 production was detected in all cases at both the mRNA and the protein levels. IL-6-producing cells were distributed in the intima, in the media, and in the adventitia. Positive cells were clearly enriched in the media, however, and particularly in contact with the internal elastic lamina. The morphology of the IL-6-producing cells showed that they belonged to several cell populations. In the media, most IL-6-producing cells were macrophages, whereas fibroblasts also produced IL-6 in the intima. Neither endothelial cells nor giant cells were found to express the IL-6 gene. Increased IL-6 serum concentrations returned to normal levels in most patients after administration of corticosteroids, indicating that inhibition of IL-6 production by GCA granuloma cells may be one of the mechanisms of action of corticosteroids in this condition. Production of IL-6 in abnormal arteries may thus participate to the systemic manifestations of GCA.

Fourteen monoclonal antibodies were used to immunohistochemically label 22 temporal artery biopsy specimens taken from Patients with temporal arteritis before treatment (n = 10), after 1-3 days of corticotherapy, (n = 6) and after 12-30 days of steroids (n = 6). Histological sections from untreated patients revealed an inflammatory infiltrate comprised of approximately equal proportions of macrophages and T lymphocytes ; the majority of the latter belonged to the CD4+ subset (the CD4+/CD8+ ratio varied from 2/1 to 4/1, depending upon the biopsy). These cells expressed high levels of HIA DR and low levels of interleukin-2 (IL2) receptor. A few B lymphocytes and plasmocytes were seen, mainly in the adventitia. Antigen-presenting cells (APC) were always found in the damaged media and natural killer cells (few in number) were sometimes present. Some macrophages were positively immunolabeled for IL6. A short, 1-to-3-day course of corticosteroids did not appreciably modify the lesions : cells remained highly activated, APC were seen' in half the biopsies and IL6 immunolabeling persisted. The findings were essentially the same in treated but poorly controlled patients. Biopsies from 2 Patients in clinical and biological remission revealed the persistance of an active immunological process. These observations indicate that the immunological process is poorly controlled by corticosteroid therapy.

We report the case of a 77-year-old woman who presented an acute form of dermatopolymyositis. Six months later, during oral corticotherapy with high efficiency we found a mediastinal mass with suspicion of liver metastases. A neuroendocrine tumor was proven by mediastinoscopy and a chemotherapy was given in outpatient with complete thoracic and hepatic response and without related toxicity.

European Journal of HaematologyVolume 50, Issue 1 p. 53-54 Hodgkin's disease revealed by cutaneous vasculitis: Two cases M. Cransac, Corresponding Author M. Cransac Clinical Hematology, Pr Bordessoule, Dupuytren Hospital, 2 Avenue Alexis Carrel, 87042 Limoges, CEDEX, France Laboratory of Anatomopathology, Pr Catanzano, Dupuytren Hospital, 2 Avenue Alexis Carrel, 87042 Limoges, CEDEX, FranceSearch for more papers by this authorE. Vidal, Corresponding Author E. Vidal Clinical Hematology, Pr Bordessoule, Dupuytren Hospital, 2 Avenue Alexis Carrel, 87042 Limoges, CEDEX, France Laboratory of Anatomopathology, Pr Catanzano, Dupuytren Hospital, 2 Avenue Alexis Carrel, 87042 Limoges, CEDEX, FranceSearch for more papers by this authorE. Liozon, Corresponding Author E. Liozon Clinical Hematology, Pr Bordessoule, Dupuytren Hospital, 2 Avenue Alexis Carrel, 87042 Limoges, CEDEX, France Laboratory of Anatomopathology, Pr Catanzano, Dupuytren Hospital, 2 Avenue Alexis Carrel, 87042 Limoges, CEDEX, FranceSearch for more papers by this authorC. Lavignac, Corresponding Author C. Lavignac Clinical Hematology, Pr Bordessoule, Dupuytren Hospital, 2 Avenue Alexis Carrel, 87042 Limoges, CEDEX, France Laboratory of Anatomopathology, Pr Catanzano, Dupuytren Hospital, 2 Avenue Alexis Carrel, 87042 Limoges, CEDEX, FranceSearch for more papers by this authorL. Réméniéras, Corresponding Author L. Réméniéras Clinical Hematology, Pr Bordessoule, Dupuytren Hospital, 2 Avenue Alexis Carrel, 87042 Limoges, CEDEX, France Laboratory of Anatomopathology, Pr Catanzano, Dupuytren Hospital, 2 Avenue Alexis Carrel, 87042 Limoges, CEDEX, FranceSearch for more papers by this authorD. Bordessoule, Corresponding Author D. Bordessoule Clinical Hematology, Pr Bordessoule, Dupuytren Hospital, 2 Avenue Alexis Carrel, 87042 Limoges, CEDEX, France Laboratory of Anatomopathology, Pr Catanzano, Dupuytren Hospital, 2 Avenue Alexis Carrel, 87042 Limoges, CEDEX, FranceSearch for more papers by this author M. Cransac, Corresponding Author M. Cransac Clinical Hematology, Pr Bordessoule, Dupuytren Hospital, 2 Avenue Alexis Carrel, 87042 Limoges, CEDEX, France Laboratory of Anatomopathology, Pr Catanzano, Dupuytren Hospital, 2 Avenue Alexis Carrel, 87042 Limoges, CEDEX, FranceSearch for more papers by this authorE. Vidal, Corresponding Author E. Vidal Clinical Hematology, Pr Bordessoule, Dupuytren Hospital, 2 Avenue Alexis Carrel, 87042 Limoges, CEDEX, France Laboratory of Anatomopathology, Pr Catanzano, Dupuytren Hospital, 2 Avenue Alexis Carrel, 87042 Limoges, CEDEX, FranceSearch for more papers by this authorE. Liozon, Corresponding Author E. Liozon Clinical Hematology, Pr Bordessoule, Dupuytren Hospital, 2 Avenue Alexis Carrel, 87042 Limoges, CEDEX, France Laboratory of Anatomopathology, Pr Catanzano, Dupuytren Hospital, 2 Avenue Alexis Carrel, 87042 Limoges, CEDEX, FranceSearch for more papers by this authorC. Lavignac, Corresponding Author C. Lavignac Clinical Hematology, Pr Bordessoule, Dupuytren Hospital, 2 Avenue Alexis Carrel, 87042 Limoges, CEDEX, France Laboratory of Anatomopathology, Pr Catanzano, Dupuytren Hospital, 2 Avenue Alexis Carrel, 87042 Limoges, CEDEX, FranceSearch for more papers by this authorL. Réméniéras, Corresponding Author L. Réméniéras Clinical Hematology, Pr Bordessoule, Dupuytren Hospital, 2 Avenue Alexis Carrel, 87042 Limoges, CEDEX, France Laboratory of Anatomopathology, Pr Catanzano, Dupuytren Hospital, 2 Avenue Alexis Carrel, 87042 Limoges, CEDEX, FranceSearch for more papers by this authorD. Bordessoule, Corresponding Author D. Bordessoule Clinical Hematology, Pr Bordessoule, Dupuytren Hospital, 2 Avenue Alexis Carrel, 87042 Limoges, CEDEX, France Laboratory of Anatomopathology, Pr Catanzano, Dupuytren Hospital, 2 Avenue Alexis Carrel, 87042 Limoges, CEDEX, FranceSearch for more papers by this author First published: January 1993 https://doi.org/10.1111/j.1600-0609.1993.tb00074.xCitations: 4AboutPDF ToolsRequest permissionExport citationAdd to favoritesTrack citation ShareShare Give accessShare full text accessShare full-text accessPlease review our Terms and Conditions of Use and check box below to share full-text version of article.I have read and accept the Wiley Online Library Terms and Conditions of UseShareable LinkUse the link below to share a full-text version of this article with your friends and colleagues. Learn more.Copy URL Share a linkShare onFacebookTwitterLinked InRedditWechat No abstract is available for this article.Citing Literature Volume50, Issue1January 1993Pages 53-54 RelatedInformation

The authors report a case of eosinophilic fasciitis associated with several autoimmune diseases, such a Graves' disease, pernicious anaemia and vitilgo. These associations suggest an autoimmune etiology for the eosinophilic fasciitis.

Fourteen monoclonal antibodies were used to immunohistochemically label 22 temporal artery biopsy specimens taken from patients with temporal arteritis before treatment (n = 10), after 1.3 days of corticotherapy (n = 6) and after 12-30 days of steroids (n = 6). Histological sections from untreated patients revealed an inflammatory infiltrate comprised of approximately equal proportions of macrophages and T lymphocytes; the majority of the latter belonged to the CD4+ subset (the CD4+/CD8+ ratio varied from 2/1 to 4/1, depending upon the biopsy). These cells expressed high levels of HLA DR and low levels of interleukin-2 (IL2) receptors. A few B lymphocytes and plasmocytes were seen, mainly in the adventitia. Antigen-presenting cells (APC) were always found in the damaged media and natural killer cells (few in number) were sometimes present. Some macrophages were positively immunolabeled for IL6. A short, 1-to-3-day course of corticosteroids did not appreciably modify the lesions: cells remained highly activated, APC were seen in half the biopsies and IL6 immunolabeling persisted. The findings were essentially the same in treated but poorly controlled patients. Biopsies from 2 patients in clinical and biological remission revealed the persistence of an active immunological process. These observations indicate that the immunological process is poorly controlled by corticosteroid therapy.

A 64 year-old man was found to have a vascular purpura with histologic features of cutaneous necrotizing vasculitis, fifteen days after influenza vaccination. No other cause (neoplasic, autoimmune, infectious or drug-induced) was apparent, and the reponsability of vaccination is retained while it is a very rare complication.

The authors report two unusual cases of primary Sjögren's syndrome which occured in young north africans patients. The two patients showed a multisystemic disorder, with, in the first case, renal tubular acidosis (associated with nephrocalcinosis), pulmonary involvement and haemotologic features, and in the second case, severe pulmonary fibrosis and cutaneous vasculitis.

Cutaneous involvement by Hodgkin's disease (HD) is rarely the presenting feature. However, non specific lesions occur in 15–50 % of cases and result often from pruritus. The association vasculitis and hematological disease is uncommon. We report two cases of leukocytoclastic vasculitis preceeding and revealing Hodgkin's disease.