[Thoracic radiodermatitis in interventional cardiology. Apropos of 6 cases]. / Radiodermite thoracique en cardiologie interventionnelle. A propos de 6 cas.

The aim of this study is to describe thoracic radiodermatitis, a rare but not to be forgotten complication of interventional cardiology. The appearances are variable, from often oval-shaped erythema to cutaneous necrosis, with risk of chronic ulceration and malignant degeneration. The authors report 6 cases observed in 1997 after coronary angioplasty. Complex and long procedures are the main causes of this complication. Prevention requires a contribution from all the medical cardiological team, for the diagnosis, determining the indication of the type of revascularisation and for limiting the dose of X-radiation administered.

[Atrial myxoma: cutaneous manifestations]. / Myxome de l'oreillette: manifestations cutanées.

INTRODUCTION: Atrial myxomas may have variable misleading symptoms, explaining frequent delays in diagnosis. Multiple embolisms with irreversible consequences may occur. The dermatologist has a prominent role to play, as the clinical picture includes cutaneous signs. CASE REPORT: A 60 year old woman, with previous history of joint pain and Raynaud's phenomenon suffered a sudden neurological transitory ischemic event. The cutaneous manifestations associated left forearm erythematous macules, distal ischemia of the left fourth and fifth fingers and livedo of both lower limbs. Cardiac echography confirmed the clinical diagnosis of atrial myxoma. Surgical excision was followed by rapid and complete cure. DISCUSSION: Atrial myxoma is a rare benign tumor usually revealed by prominent cardiac and embolic manifestations. Systemic and cutaneous signs may however be the sole symptoms, warranting the dermatologist's attention.

[CREST syndrome presenting as pulmonary hypertension]. / Hypertension artérielle pulmonaire révélatrice d'un CREST syndrome.

The authors report the case of a 62-year-old patient complaining of recent onset of disabling breathlessness on exertion, and presenting clinical signs of previously undiagnosed scleroderma. Echocardiography revealed a diagnosis of precapillary pulmonary hypertension (74/14 mmHg) (PHT), with no pulmonary cause revealed by pulmonary ventilation-perfusion scintigraphy or by thoracic fine section computed tomography. The diagnosis of PHT in the context of circumscribed scleroderma was confirmed by x-rays of the hands, capillaroscopy, oesophageal investigations and positive anticentromere antinuclear antibodies. The clinical course was marked by rapid deterioration of the symptoms, requiring treatment with prostacyclin by continuous intravenous infusion. The appearance of PHT in a context of circumscribed scleroderma, usually a relatively benign disease, is a rare, late event, exceptionally revealing the disease, as in this case, and indicating a very unfavourable prognosis.

Unusual erythema elevatum diutinum with fibrohistiocytic proliferation.

A 78-year-old woman with a history of symmetrical erythematous plaques on the arms, and a monoclonal gammopathy, developed a strange striped reticulate papular dermatosis with central atrophy. Histological examination was compatible with a very late stage of erythema elevatum diutinum (EED), showing a fibrohistiocytic proliferation with areas of granulation tissue. This fibrosis may result from the chronic dermal injury of leucocytoclastic vasculitis and is sometimes the predominant histology of EED. Investigations for underlying haematological anomalies, such as paraproteinaemia and infection with human immunodeficiency virus, must be performed. Dapsone is ineffective once the fibrous nodules have appeared.

[Follicular basaloid hamartoma localized in the sacral region with papular plaque-type]. / Hamartome basaloide folliculaire localisé en plaque papuleuse de la région sacrée.

INTRODUCTION: Basaloid follicular hamartoma is a benign tumor with superficial dermal proliferation of thin basaloid cords in a fibrous stroma. CASE REPORT: A congenital case with sacral plaque-like papules is described. DISCUSSION: Three different forms have been reported: a solitary form (usually an alopecic scalp plaque), a generalized acquired form associated with myasthenia, and a linear form. Histological diagnosis may be difficult and require clinico-pathological evaluation.

[Osteomalacia cured by resection of cutaneous neurinoma]. / Ostéomalacie guérie par résection d'un neurinome cutané.

INTRODUCTION: Regression of osteomalacia after exeresis of a skin tumor is unusual. CASE REPORT: A 62-year-old man had suffered from bone and joint symptoms for several years due to osteomalacia which was confirmed both biologically and histologically. The patient also had a plantar neurinoma. After exeresis of the tumor the biological results returned to normal levels within one week followed by regression of the clinical signs of osteomalacia. DISCUSSION: The neurinoma in this patient was apparently the cause of osteomalacia, since signs of the disease disappeared after exeresis. To date, three cases of neurinoma associated with osteomalacia have been published, including a single case with skin localization. The tumor would secrete a substance which inhibits the synthesis of vitamin D and enhances phosphorus excretion.

Hydroxyurea-induced dermatomyositis-like eruption.

Hydroxyurea is frequently used to treat myeloproliferative syndromes. Cutaneous lesions resembling those seen in dermatomyositis have rarely been reported in the course of treatment with hydroxyurea. We report six additional patients with this unusual adverse effect. All of the patients had a very typical and similar cutaneous eruption, with scaly, linear erythema on the dorsa of the hands. Leg ulceration occurred in two cases. Muscle involvement was never observed. One patient had unexplained lung disease. In all the others the disorder pursued a benign course, even when hydroxyurea was not withdrawn. Dermatomyositis-like lesions seem to be a not infrequent and characteristic adverse reaction to hydroxyurea. Investigations are not required, and the course is usually benign.

Hyperkeratotic spicules and monoclonal gammopathy.

Hyperkeratotic spicules are a rare disorder that has been associated with different types of malignancies. An association with multiple myeloma raises the question of a relation between the monoclonal component and skin manifestations. We describe hyperkeratotic spicules in a 61-year-old man with a monoclonal gammopathy of undetermined significance. Immunofluorescence and immunoelectron microscopic findings demonstrated the deposition of the monoclonal immunoglobulin at the dermoepidermal junction. Our findings support previous reports that the association between hyperkeratotic spicules and monoclonal gammopathy is not fortuitous and may be related to particular properties of the monoclonal immunoglobulin.

[Viral type particles in the germinal centers during a lymphadenopathic syndrome related to AIDS]. / Mise en évidence de particules de type viral dans les centres germinatifs au cours d'une syndrome lymphadénopathique relié au SIDA.

The identification of virus-like particles in the germinal centers of an AIDS-related lymph node with histological findings of group IA is reported. A 23 year-old man, intravenous drug user, presents an inversion of T helper/T suppressor cell ratio and a peripheral generalized lymphadenopathy, suggesting a diagnosis of ARC. Lymph node biopsy shows follicular hyperplasia and features consistent with group IA of AIDS-related lymphadenopathy. Immunohistochemical study reveals an increase of intrafollicular T8+ lymphocytes. Electronmicroscopic examination of follicles demonstrates the presence of particles showing features of viral nucleocapsids; these particles are seen between the cytoplasmic processes of follicular dendritic cells. Pathologic findings associated with lymphoid follicular hyperplasia in AIDS prodromal period are examined. The importance of lymph node biopsy with histological, immunohistochemical and ultrastructural studies, in patients at high risk for AIDS, is demonstrated. The detection of type D viral particles suggestive of retrovirus is in accordance with results of virologic studies which indicate the involvement of a retrovirus (called LAV or HTLV III) in the etiology of the disease.