[Two cases of cutaneous erythema following spinal cord stimulation]. / Erythème cutané provoqué par une neurostimulation cordonale postérieure: deux observations.

INTRODUCTION: Cutaneous complications following implantation of an electric system have been reported mainly after the implantation of pacemakers, cardiac defibrillators and morphine pumps. However, cutaneous complications following implantation of spinal cord stimulators are rarely described in the literature. Here we report two cases of cutaneous eruption at the spinal cord stimulation site, one involving foreign-body reaction to silicone in the neurostimulator electrodes or connector block and the other comprising contact dermatitis to silicone. CASE REPORTS: Case 1: A 43-year-old woman had been implanted with a spinal cord stimulator. Four months after implantation, burning sensation and cutaneous inflammatory erythema were observed in the dorsolumbar region, with reduced efficiency of stimulation. Removal of the neurostimulator led to resolution of the cutaneous symptoms. Histopathological examination of the biopsy sample showed foreign-body granuloma formation. The same symptoms subsequently recurred on the surgical scar and histopathology showed granuloma formation in response to particles of silicone present in the connector. The cutaneous eruption subsided rapidly after excision. Case 2: In a 60-year-old man implanted with a spinal cord stimulator, pruriginous reticular erythema was rapidly observed on the skin just above the neurostimulator, with papules around the erythema. Histopathology of two cutaneous biopsies showed evidence of contact dermatitis. Skin-patch tests performed with components of the neurostimulator leads proved positive to two types of silicone after 72 hours. The eruption was successfully controlled using topical corticosteroid therapy. DISCUSSION: Reports of cutaneous eruptions after spinal cord stimulation are extremely rare. Here we report two different cutaneous reactions with two different pathophysiological mechanisms, both involving silicone. The first case is original because it is the first documented case of a foreign-body reaction involving granuloma formation in response to particles of silicone present in the components of a neurostimulator. The second case concerns contact dermatitis to silicone (present in the connector block and electrode sheaths).

[Hyperkeratotic lesion of the nipple revealing cutaneous leiomyoma]. / Hyperkératose mamelonnaire révélant un léiomyome cutané

BACKGROUND: Leiomyoma of the nipple and areola is a rare benign neoplasm. We report the case of a patient with leiomyoma of the nipple presenting as a hyperkeratotic plaque. OBSERVATION: A 23-year-old patient presented with a five year history of a papillomatous, hyperkeratotic, painful plaque originating in her right nipple. Histological examination of a punch biopsy showed hyperkeratosis of the epidermis with dilatation of the lymphatic vessels within the dermis. Surgical excision revealed a proliferation of smooth muscle fibres, leading to diagnosis of leiomyoma. DISCUSSION: The clinical and histological features were initially consistent with idiopathic naevoid hyperkeratosis of areola. However, associated pain is uncommon in idiopathic lesions. This unusual feature led us to surgical excision enabling the diagnosis of leiomyoma. A hyperkeratotic lesion of the nipple may be associated with benign or malignant neoplasms, hamartoma or chronic dermatoses, or it may be idiopathic. In the present case, the hyperkeratotic lesion revealed subareolar leiomyoma. This is an uncommon clinical presentation not previously seen in medical observations, since leiomyoma usually presents as a firm, painful lump in the subareolar region.

Histological skin changes in heterozygote carriers of mutations in ABCC6, the gene causing pseudoxanthoma elasticum.

BACKGROUND: Pseudoxanthoma elasticum (PXE) is related to mutations in the ABCC6 gene and characterized pathologically by dystrophic and mineralized elastic fibres. Heterozygote carriers of ABCC6 mutations may have a limited PXE phenotype. OBJECTIVE: To compare histological changes in the skin of genotyped siblings from two PXE pedigrees. METHODS: Mutation analysis of ABCC6 was performed. Skin biopsy samples were stained (orcein) and immunolabelled for elastin, and for vitronectin and bone sialoprotein, which are partially responsible for the mineralization within the elastorrhexic fibres. Results In all individuals mutation analysis of ABCC6 allowed definition of the genotype status, i.e. PXE (n = 2), heterozygote (n = 7) or wild type (n = 2). The study identified three histological phenotypes related to the ABCC6 genotype in siblings from both families. Heterozygote carriers had changes in dermal elastic fibre organization, morphology and labelling midway between those seen in PXE skin and normal skin. CONCLUSION: Even though the number of individuals studied here is small and precludes any hasty generalization, having a single mutation in the ABCC6 gene seems enough to modify dermal elastic fibres. The relevance of performing a skin biopsy to identify heterozygote carriers in the family of a PXE patient remains to be determined.

Endocrine tumors of the duodenum. A study of 55 cases relative to clinicopathological features and hormone content.

BACKGROUND/AIMS: Study of prognosis of duodenal endocrine tumors. METHODOLOGY: Retrospective study concerned 55 duodenal endocrine tumors discovered in biopsy or surgical specimens. Follow-up records available for 49 patients indicated that inconspicuous associated clinical manifestations were often found subsequently. Seven patients were classified as Zollinger-Ellison syndrome and seven as multiple endocrine neoplasia (6 MEN I and 1 MEN II). RESULTS: Tumors were small (mean 1.28cm) and located preferentially in the first and second part of the duodenum. Fifty-four were well-differentiated and one poorly differentiated. Immunochemistry revealed 30 G-cell tumors (54.6%), 15 D-cell (27.3%), two plurihormonal (EC cell and G cell), and one GRH-cell, whereas seven could not be classified. Fifteen patients died (five in relation to their disease). Twenty-one had metastases (liver, nodes, lung), eight of whom are still alive. CONCLUSIONS: Eighty-eight percent of duodenal endocrine tumors were gastrinomas, small plurifocal tumors and somatostatinomas preferentially located in the ampullar region and diagnosed because of hematemesis or icterus. Size is an important prognostic factor in determining whether surgery is required. The prognosis is better for D- and G-cell tumors than pancreatic endocrine tumors. Duodenal endocrine tumors in multiple endocrine neoplasia have a good prognosis, but can be associated with pancreatic plurihormonal tumors and metastases.

[Vascular purpura in a patient with severe sarcoptic acariasis]. / Purpura vasculaire au cours d'une gale sévère.

INTRODUCTION: The common cutaneous manifestations of human sarcoptic acariasis are erythematous and pruriginous papules. Vascular purpura in patients with scabies has rarely been reported. We report a case of vascular purpura in the presence of a transient elevation of anticardiolipin antibodies. CASE REPORT: A 79 year-old woman was treated with prednisone and chlorambucil for a lymphoma. She was referred to our institution for diffuse sarcoptic acariasis. Clinical examination showed a petechial purpura on her legs. Histology of two biopsies of purpuric papules showed non inflammatory thrombosis of the upper dermis vessels. Investigations revealed a transient elevation of anticardiolipin antibodies, moderate hyperhomocysteinaemia and blood eosinophilia. Treatment consisted in ivermectin and repeated applications of benzyl benzoate lotion. The purpura healed within one month, without specific treatment. DISCUSSION: Histological findings of sarcoptic acariasis with vascular purpura correspond to leukocytoclasic vasculitis. Focal glomerulonephritis has been reported. In our case, histopathology showed a non inflammatory thrombosis of the dermal vessels. We hypothesize that the anticardiolipin antibodies, hyperhomocysteinaemia and blood eosinophilia may have been the cause of vascular thrombosis.

[Duodenal somatostatinomas associated with von Recklinghausen's neurofibromatosis. Apropos of 2 cases]. / Somatostatinomes duodénaux et neurofibromatose de von Recklinghausen.

Somatostatinomas are rare neuroendocrine tumors; they are essentially located in the pancreas and in the duodenum. The association with a neurofibromatosis type I is especially observed when the tumor is located in the ampulla of Vater. These tumors are not associated with a "somatostatin syndrome", but often present with gastrointestinal bleeding, abdominal pain and obstructive jaundice. The diagnosis is confirmed by immunohistochemical studies. The aim of this study is to report 2 cases of metastazing duodenal periampullary somatostatinomas associated with von Recklinghausen's disease and to discuss the prognosis of these tumors. Future genetic research are necessary as point out the familial feature of this association in one of our cases.

[Primary cutaneous neuroendocrine carcinoma (Merkel tumor). Clinical and therapeutic update apropos of 4 cases]. / Carcinome neuroendocrine cutané primitif (tumeur de Merkel). Actualités cliniques et thérapeutiques à propos de quatre cas.

The clinical and pathological features of 4 cases of cutaneous neuroendocrine carcinoma are reported. There are unusual malignancies mostly observed in elderly, the prognosis is poor by loco-regional aggressivity and disseminated metastases. They are diagnosed by a pathologist: ultrastructural studies and immunohistochemistry support the diagnosis. A combination of a wild local excision, node dissection and post-operative radiation is recommended.

[Metastasis of breast cancer to a uterine leiomyoma]. / Métastase d'un cancer du sein dans un léiomyome utérin.

The authors report a case of metastasis of cancer from the breast in a uterine leiomyoma. The metastases into the body of the uterus from extragenital cancers are rare. If they occur they are usually in the myometrium where they are asymptomatic and where the diagnosis is difficult, or in the endometrium where the diagnosis can be made by biopsy after curettage. A few rare cases of metastases in uterine leiomyoma have been reported in the literature. They can be the cause of very sudden increase in size of fibroids with compression of the pelvic organs.