[Mucinous nevus of late onset]. / Nævus mucineux de révélation tardive.

INTRODUCTION: Mucinous nevus is a rare disease characterized by mucin deposits in the dermis, with only 23 cases reported to date. It belongs to the connective tissue nevus family and is characterized by dermal mucin deposits. Two histological subtypes have been described: pure mucinous dermal nevus (connective tissue nevus of the proteoglycan [CTNP]) and combined mucinous nevus (combined CTNP) associating epidermal nevus with dermal mucin deposits. Lesions generally appear in childhood or early adulthood. We report herein the case of a 59-year-old man with a symptomatic lesion. OBSERVATION: A 59-year-old man consulted for unilateral lesions occurring linearly along the Blaschko line on the right lower limb, composed of brown verrucous papules, which started at the age of 50. The patient complained of itching caused by friction from clothes. Histological examination of a papule highlighted an acanthotic epidermis, with elongation of rete ridges and mucin deposits in the dermis showing uptake of alcian blue stain. The clinicopathological analysis enabled diagnosis of combined CTNP. We treated this nevus by surgical dermabrasion and observed a slight and cosmetically acceptable scar. DISCUSSION: To our knowledge, our patient is the oldest reported to date and the only one to have complained of symptoms. Our review of the literature highlights the importance of skin biopsy to guide aesthetic or symptomatic treatment. CO2 laser and dermabrasion are recommended for combined CTNP as they treat the epidermal component with low risk of secondary scarring. However, these techniques should be avoided in pure dermal CTNP, where surgical excision alone is acceptable in order to achieve optimal cosmetic results.

[Livedo-like cutaneous sarcoidosis]. / Sarcoïdose cutanée à type de livédo.

BACKGROUND: The cutaneous signs of sarcoidosis are extremely polymorphous and may be classified under several different headings. PATIENTS AND METHODS: Herein, we report the case of a 51-year-old female patient presenting bilateral livedo of the knees accompanied by systemic signs with polyarthralgia, impaired general state of health, weight loss, and a sensation of dyspnoea. Skin biopsy revealed giant-cell granuloma around the dermal vessels, with no caseous necrosis. Histopathological examination of the patient's blood vessels revealed no abnormalities. Laboratory tests showed high levels of angiotensin converting enzyme (1.5×ULN), bilateral mediastinal adenomegaly and incipient pulmonary fibrosis. Systemic sarcoidosis was diagnosed on the basis of the overall clinical and laboratory picture. The patient subsequently presented hepatic involvement and hypercalcaemia associated with the sarcoidosis; systemic corticosteroid therapy (prednisone) was initiated, with slow dose-reduction. The outcome was favourable with a return to normal laboratory values, regression of the adenomegaly and subsidence of the livedo. DISCUSSION: The literature contains reports of a number of cases of livedo heralding sarcoidosis. The majority of such cases involved young or middle-aged female patients of Asian origin presenting systemic sarcoidosis, with a high frequency of ocular and neural involvement. Livedo could be accounted for by the specific disposition of granulomas around the arterioles, resulting in disturbance of local blood flow, which was probably associated with the mechanical compression of vessels and with the micro-thrombi noted at histology. CONCLUSION: Livedo may be considered a clinical sign of cutaneous sarcoidosis. Laboratory tests to screen for sarcoidosis may be proposed in the exploration of atypical livedo.

[Epidermolysa bullosa acquisita and graft-versus-host disease]. / Épidermolyse bulleuse acquise et réaction du greffon contre l'hôte.

BACKGROUND: Epidermolysis bullosa acquisita (EBA) is a rare acquired blistering disorder caused by production of auto-antibody directed against type-VII collagen. Autoimmune disorders can occur after allogenic bone marrow transplantation as manifestations associated with chronic graft-versus-host disease (GVHD). To date, there have been 10 cases reported in the literature concerning autoimmune blistering diseases following allogenic stem-cell transplants. Herein we describe a new case involving EBA. OBSERVATION: A 46-year-old woman developed EBA 4 years after allogenic cord blood transplantation for non-Hodgkin T-cell lymphoma complicated by acute digestive and cutaneous GVHD. At physical examination, she had some cutaneous blisters on the abdomen, arms and face, as well as numerous erosions in the buccal cavity. Direct immunofluorescence microscopy revealed linear IgG and C3 deposits along the dermal-epidermal basement membrane zone. Indirect immunofluorescence showed weak IgG G4 anti-basement membrane zone antibodies, which reacted with the dermal side of 1M NaCl-split skin; the autoantibodies were directed against type-VII collagen. This second case of EBA was evocative of a GVHD blistering disease. After the therapeutic failure of dapsone and of combined mycophenolate-prednisone, treatment with rituximab proved effective. DISCUSSION: EBA may form part of the autoimmune signs associated with chronic GVH. The destruction of basement membrane and of epidermal basal cells that occurs in GVH could give rise to autoimmune bullous disease. However, in our patient, in whom manifestation of chronic GVH was restricted to the lungs, it is difficult to rule out the fortuitous onset of EBA, which presented at a sizeable interval after acute GVH.

[An uncommon cutaneous presentation of cat scratch disease]. / Présentation cutanée atypique d'une maladie des griffes du chat.

BACKGROUND: Herein we report a case of cat scratch disease on account of its atypical presentation. PATIENTS AND METHODS: A 21-year-old woman presented erythema nodosum associated with painful bilateral inguinal adenopathy, odynophagia, joint pain and evening urticaria in a setting of impaired general condition. Initial serological testing for Bartonella henselae was negative. PCR for Bartonella henselae performed on an adenectomy fragment was positive. A favourable outcome was achieved with azithromycin. COMMENTS: This case shows an atypical and severe presentation of cat scratch disease and raises the problem of sensitivity of serotyping.

[Long-term evolution of achalasia of the lower esophageal sphincter and intestinal paraneoplasic pseudo-obstruction revealing small cell lung carcinoma]. / Evolution à long terme d'une achalasie du sphincter inférieur de l'esophage et d'une pseudo-obstruction intestinale paranéoplasiques révélatrices d'un carcinome pulmonaire à petites cellules.

We report a case of a small cell carcinoma of the lung revealed by chronic intestinal pseudo-obstruction associated with achalasia of the lower esophageal sphincter. Tumoral remission was achieved for more than 21 months after chemoradiotherapy but this did not prevent the paraneoplasic syndrome from persisting and medical treatment was not successful in treating the intestinal pseudo-obstruction or the dysphagia, which was not improved by esophageal dilation.

Cutaneous T cell lymphoma of signet ring cell type: a specific clinico-pathologic entity.

We describe a new case of signet ring cell peripheral T cell lymphoma in a 45-year-old man. This lymphoma had a very indolent course, since--without treatment--the clinical staging has shown no evidence of disease progression 11 years after initial symptoms. Immunophenotype indicated pan T antigens (Leu 4 CD3, Leu 1 CD5) and T suppressor cytotoxic antigen (IOT8 CD8) expression. Several T antigens (Leu 5b CD2, Leu 9 CD7, Leu 3a CD4) were not expressed. The proliferation index was less than 5% with Ki 67 monoclonal antibodies. The ultrastructural study showed characteristic cytoplasmic vacuoles containing microvesicles. Five cases of signet ring T cell lymphoma, which were very similar to our case, have been previously described. Their characteristics were primary cutaneous presentation, indolent course, good response to current therapies and a long survival period. The indolent course of these signet ring cell lymphomas may indicate that this type of lymphoma is a low grade malignant lymphoma and not only a morphological pattern.

A primary cutaneous multi-lobed B-cell lymphoma.

Multi-lobed non-Hodgkin's lymphoma (NHL) has recently been recognized as a NHL variant. A patient presented with a scalp nodule which, upon skull X-Ray, was seen to be associated with a bone defect. Immunophenotyping clearly demonstrated that this was a B-cell proliferation. Histologically the B-lymphocytes were closely related to centroblasts. There were no other extra cutaneous localizations. The present report emphasizes the importance of this clinico anatomical entity which shows prominent extra-nodal involvement, large lymphoid cells with multi-lobed nuclei and a good response to chemotherapy. Multi-lobed NHL may be a T-cell lymphoma, or a B-cell lymphoma closely related to centroblastic NHL. Although multi-lobed lymphomas have a predilection for cutaneous localizations, our case is the first primary cutaneous multi-lobed B-NHL, proven by immunophenotyping.

[Histopathology of the vein of Galen]. / Histopathologie de la veine de Galien.

Histology of cerebral arteries and veins are reviewed to show variances with extracerebral blood vessels. Muscles fibers even in large cerebral veins are easily overlooked. The thick wall of the vein of Galen grossly and microscopically resembled dura mater fed by microscopic blood vessels and capillaries. The authors reported 4 cases of arteriovenous aneurysms of the great vein of Galen. When compared to the rest of the histological reports on this subject published till now, it is obvious that this new cases bring new facts. First is the presence of an irregular muscle coat in the aneurysmal wall suggesting early hemodynamic troubles. The second and most important data is the association of multiple malformations of the pineal gland, of the vascular elements of the tela choroidea of the third ventricle between which the aneurysm of the great cerebral vein is found. These unusual data suggested complex congenital malformation in the early stages of development.

[Primary pulmonary sarcoma with spindle-shaped cells. Apropos of a case]. / Sarcome pulmonaire primitif à cellules fusiformes. A propos d'un cas.

Primary pulmonary sarcoma is a very rare malignant tumour. The authors report a case of a fusiform cell sarcoma simulating a pulmonary metastasis in a woman who previously had a mammary carcinoma. Lymph node invasion is rare. The nature of the tumour was confirmed by histology and aided by immunochemistry or electron microscopy. The primary origin of the tumour can only be confirmed after negative results from careful examination of the marrow, the gastro-intestinal tract, the genito-urinary system and above all the uterus. The prognosis depends on the size of the tumour and the mitotic score. Surgery is the only curative treatment.

[Ultrasonic, arteriographic and anatomopathological correlation in 59 cases of carotid atherosclerosis]. / Corrélations ultrasoniques, artériographiques et anatomopathologiques dans 59 cas d'athérosclérose carotidienne.

This study was carried out on 49 patients who underwent an ultrasound study by continuous Doppler and echotomography B real-time, an angiography of supra-aortic arteries, and a carotid endarterectomy. Thirteen had had transient ischemic attacks and 20 a stroke. A quantification of the carotid stenosis in 4 stages was established for each method. For detection of lesion an excellent correlation was observed between the results of echotomography and pathology. For the quantification of lesion the correlation between echotomography and pathology was excellent also in 79,7 p. 100 of the cases, discordant in 20,3 p. 100. It was similar to the correlation between arteriography and pathology (excellent in 83 p. 100 of the cases). The correlation with pathology enabled to distinguish for stages of atherosclerotic lesions, to clarify their type of complications, and to evaluate their functional significance, particularly those with a high risk of embolism.

[Cirrhosis induced by vitamin A]. / Cirrhose induite par la vitamine A.

The case of a 75-year-old woman who had been treated for 12 years by vitamin A (250,000 UI/day) for psoriasis is presented. During the hospitalisation, hepatic cirrhosis was detected and attributed to hypervitaminosis A based on disease history, clinical hypervitaminosis A features, increased vitamin A blood values and histological patterns, i. e. perisinusoidal fibrosis and spontaneous fluorescence of lipid vacuoles within fat-storing cells. Thus, although rare, vitamin A intoxication can be responsible for cirrhosis.

[Merkel cell tumor]. / Tumeur à cellules de Merkel.

Two cases of skin carcinoma which display endocrine differentiation are reported. In the relevant literature, these neoplasms are considered to be of Merkel cells lineage. These two carcinomas demonstrated salient morphological features. The first tumor contained small aggregates of cells with a deeply indented nucleus. It is postulated that these formations represent foci of Merkel cells maturation. It is suggested that this distinctive focal histological feature may permit recognition of these neoplasms. The second tumor exhibited an admixture of endocrine and epidermoid differentiation. Such observations prompt us to postulate that Merkel cells and keratinocytes originate from the same stem cell.