[Metastatic cervical fasciitis revealing invasive lobular breast carcinoma]. / Fasciite cervicale métastatique révélant un carcinome lobulaire infiltrant du sein.

BACKGROUND: We describe the case of a 71-year-old woman presenting cervical metastatic fasciitis with invasive lobular carcinoma (ILC) of the breast. PATIENTS AND METHODS: The patient consulted for a deep and painless skin infiltration of the neck associated with dysphagia and restricted cervical mobility. Skin and muscle biopsies were normal. Muscle fascia biopsy showed a linear infiltration of metastatic cells in "single file", revealing ILC of the right breast. DISCUSSION: ILCs have a particular metastatic pattern. They can permeate through tissue planes, infiltrate solid organs and spread on serous membranes in an insidious fashion. CONCLUSION: Our case shows that ILC can metastasise into muscular fascia, causing "fasciitis-like" symptoms. Dermatologists should be aware of this particular pattern of dissemination.

[Multiple primary cutaneous plasmacytoma]. / Plasmocytomes cutanés primitifs multiples.

BACKGROUND: Primary cutaneous plasmacytoma is a rare form of cutaneous B-cell lymphoma. PATIENTS AND METHODS: A 51 year-old male with an unremarkable history gradually presented erythematous papulonodular lesions that had appeared gradually over the whole body throughout a two-year period and showing histologic and immunohistochemical features of cutaneous plasmacytoma. Staging investigations confirmed the primary character of the disease, and because of this and the absence of functional impairment, we opted for therapeutic abstention. No progression was noted after 4 years of regular monitoring. DISCUSSION: Primary cutaneous plasmacytoma (PCP) is characterized by clonal proliferation of plasma cells in skin. Multiple PCPs are extremely rare and to date have been treated in most cases by chemotherapy, either with or without radiotherapy. The prognosis is poor, with 2-year survival of only 25%. The present case is original, being the only one to our knowledge in which therapeutic abstention was followed by a lack of progression after 4 years of regular follow-up. Consequently, certain indolent forms of PCP do not warrant automatic institution of chemotherapy.

Solitary subungual keratoacanthoma arising in an MSH2 germline mutation carrier: confirmation of a relationship by immunohistochemical analysis.

BACKGROUND: Muir-Torre syndrome (MTS) is an autosomal dominant disorder characterized by the concurrent or sequential development of at least 1 sebaceous gland tumor or keratoacanthoma and 1 or more internal malignancies. It is actually considered as a variant of hereditary nonpolyposis colorectal cancer (HNPCC) as both MTS and HNPCC are more often associated with germline mutations in the DNA mismatch repair (MMR) gene. OBJECTIVE AND METHODS: We report the case of MTS diagnosed after the occurrence of a solitary subungual keratoacanthoma (SKA) in a man with a well-known family history of HNPCC and who is carrying a constitutional 1-7 deletion in the MSH2 MMR gene. RESULTS: The link between the germline mutation and the skin tumor was reinforced by immunohistochemical staining. MSH2 immunoreactivity was decreased in SKA tumoral cells when compared to normal adjacent epidermis and to 5 cases of sporadic KA used as controls. CONCLUSION: This observation indicates that a solitary SKA may be the first clinical manifestation of MTS and brings up the relevance for regular dermatological screening for MTS-associated skin lesions among gene carriers (and symptomatic individuals) for HNPCC syndrome.

[Tumoral and pilotropic mycosis fongoides without mucin: evolution towards a pustulous erythroderma]. / Mycosis fongoïde tumoral et pilotrope sans mucine: évolution vers une érythrodermie pustuleuse.

INTRODUCTION: Pilotropic mycosis fongoides is a particular form of the disease, because of its clinical and histological aspects, its poor prognosis and its resistance to treatment. We report a case of pilotropic mycosis fongoides without mucinosis, immediately tumoral, the fatal progression of which was marked by the occurrence of pustular erythroderma. OBSERVATION: In 1998 a 69 year-old man presented with infiltrated erythro-squamatous plaques and nodules on the limbs associated with follicular lesions predominating on the cervical-cephalic area. Histological explorations revealed a pilotropic infiltrate with atypical CD4+ CD8 CD30 T-cells, without epidermotrophism or mucinosis. Study of genetic rearrangements found a clone lymphocyte T-cell in the skin. Diagnosis of pilotropic mycosis fongoides at the tumoral stage was made and, despite various treatments, the disease developed towards fatal pustular erythroderma. DISCUSSION: At the onset of its progression, pilotropic mycosis fongoides is sometimes difficult to distinguish from classical mycosis fongoides, during which follicular involvement is often seen. However it is important to differentiate these entities because of the poor prognosis of pilotropic mycosis fongoides. Development of tissue micro dissection techniques and lymphocyte T-cell clones from human skin would help to separate these cutaneous T-cell lymphoma sub-groups. Our case report is original because of the absence of dermal mucinosis combined with an immediately tumoral form and the progression towards generalized pustulosis. It also underlines the poor prognosis and resistance to treatment of pilotropic mycosis fongoides.

High plasma proteasome levels are detected in patients with metastatic malignant melanoma.

BACKGROUND: Proteasomes, nonlysosomal proteolytic structures, are implicated in cell growth and differentiation. An abnormal expression has been described in haematopoietic malignancies and in some solid tumours. OBJECTIVES: To study the plasma proteasome levels in patients with malignant melanoma (MM) using an enzyme-linked immunosorbent assay (ELISA) technique, and to compare them with the values obtained in a normal population and in patients with severe psoriasis or chronic idiopathic urticaria (CIU). METHODS: Plasma proteasome level was measured using a sandwich ELISA test in normal donors (n = 14), and in patients with stage I/II (n = 13), stage III (n = 6) and stage IV (n = 10) MM, severe psoriasis (n = 13) and CIU (n = 6). Tissue proteasome expression was also detected by immunohistology using a monoclonal antibody in paraffin-embedded samples of normal tissue, psoriasis skin and MM. RESULTS: In normal donors, mean +/- SEM plasma proteasome concentration was 2138 +/- 221 ng mL(-1). Patients with stages III and IV MM exhibited a significantly higher value (3373 +/- 470 ng mL(-1) and 8931 +/- 1232 ng mL(-1), respectively). Values in patients with stage I/II MM and CIU were not significantly different from those in normal volunteers. Patients with severe psoriasis also exhibited increased values (3398 +/- 374 ng mL(-1)) but to a lesser extent than in patients with stage IV MM. There was a significant correlation of proteasome levels with serum lactate dehydrogenase in the MM group. Tissue expression as demonstrated by immunohistochemistry paralleled these findings. The strongest expression was seen on MM slides and to a lesser extent in psoriasis samples, the weakest expression being observed in normal skin. CONCLUSIONS: Proteasomes are strongly expressed in cutaneous MM; high levels of circulating proteasomes are detected in patients with metastatic MM with a high melanoma burden, and at a lesser extent in psoriatic patients, which suggests proteasomes represent a marker more of nonspecific inflammation than of early cancer.

[Cutaneous angiosarcoma of the breast after treatment with conservative surgery and radiotherapy of breast carcinoma]. / Angiosarcome cutané du sein après traitement par chirurgie conservatrice et radiothérapie d'un carcinome mammaire.

Secondary cutaneous angiosarcoma of the breast is uncommon. This tumor develops after conservative treatment of breast cancer. We present a case that developed seven years after conservative treatment with surgery and radiotherapy for breast cancer. Several factors may be implicated in the pathogenesis of this tumor (carcinogenic effect of radiotherapy, HHV8 infection), but the essential factor appears to be chronic edema. Prognosis is poor but initial wide surgical resection can achieve prolonged remission. Early diagnosis and treatment are the main prognostic factors.

An association between autosomal dominant cerebral cavernomas and a distinctive hyperkeratotic cutaneous vascular malformation in 4 families.

Cerebral cavernomas (CCMs) are vascular malformations that may be inherited as an autosomal dominant condition for which a gene, CCM1, was mapped to chromosome 7. Poorly defined cutaneous malformations were sometimes described in association with CCMs. During a national survey, 57 French CCM families were studied. Co-occurrence of CCMs and a distinctive cutaneous vascular malformation was observed in 4 families. Ten individuals belonging to these families showed similar hyperkeratotic cutaneous capillary venous malformations (HCCVMs). In 3 families, the histology showed orthokeratosis and hyperkeratosis as well as dilated capillaries in the dermis extending to the hypodermis and confirmed the diagnosis of HCCVM. Genetic analysis strongly supports linkage of these families to the CCM1 locus on chromosome 7. The HCCVM seems to be a peculiar cutaneous vascular malformation associated with CCMs. These data strongly suggest that HCCVMs and CCMs in these families are due to the same genetic abnormality.

[Multiple congenital smooth-muscle hamartomas]. / Hamartomes musculaires lisses congénitaux multiples.

BACKGROUND: Smooth muscle hamartoma is an uncommon lesion. Diagnosis is usually made at birth in infants presenting a plaque with minimal or no infiltration and covered with long dark hairs. Congenital forms with multiple plaques are rarely reported. CASE REPORT: A 5-day-old infant (normal pregnancy and delivery) had plaques localized on the buttocks, the left thigh, leg and shoulder and the right ankle. The plaques were minimally infiltrative and covered with long black hairs. Histology examination showed hyperplastic smooth muscle bundles with varying orientation. The diagnosis was smooth muscle hamartoma. The rest of the clinical examination was normal. CONCLUSION: This case of congenital smooth muscle hamartoma showed a particular form with partially regressive multiple plaques.

First report of mycetoma caused by Arthrographis kalrae: successful treatment with itraconazole.

We report the first case of eumycetoma of the hand caused by Arthrographis kalrae. Cure was obtained with a 4-month course of itraconazole.

[Pneumatosis cystoides of the colon. Apropos of 7 cases]. / Pneumatose kystique du côlon. A propos de 7 observations.

Seven cases of pneumatosis cystoides intestinalis affecting the colon (PKC) revealed 5 times by a nonspecific symtomatology and 2 times by an occlusion were reported. In three of the patients the disease was found as a primary idiopathic form free of clinical antecedents. In 4 of the patients the pneumatosis was found to be secondary to a pulmonary disease, a gastric ulcer, a connectivite of a corticotherapy. The PKC was generally diagnosed either by barium enema or by computed tomography and less frequently by colonoscopy with deep biopsy allowing differential diagnosis with colonic polyposis. The mechanism and etiology of the PKC were not fully understood. The illness is a benign condition that often responds to a conservative management--i.e. abstention, oxygenotherapy, diet or antibiotherapy- or exceptionally to surgical colonic resection in case of acute complication.

Primary sinusoidal lymphoma of the liver revealed by autoimmune hemolytic anemia.

Primary liver lymphomas usually present with the clinical picture of a liver tumor, and are characterized by a predominantly portal invasion by lymphoid cells of the B-cell phenotype. We report a case of primary sinusoidal lymphoma of the liver, in a 36 year-old male patient, revealed by homogeneous hepatosplenomegaly and infiltration of liver sinusoids by morphologically normal lymphocytes, without destruction of the parenchyma. Immunohistochemistry in paraffin-embedded tissue sections was positive for the pan T-cell marker MTI, weakly positive for UCHLI, and negative for CD3, and B-cell markers were negative; these findings were consistent with the diagnosis of T-cell lymphoma. The clinical, histological and immunological presentation of this lymphoma was similar to that of hepatosplenic gamma delta T-cell lymphoma. Autoimmune hemolytic anaemia preceded the lymphoma. Despite chemotherapy, the patient died 24 months after the initial presentation in the leukemic phase. A better understanding of this exceptional but characteristic entity is required for an accurate and early diagnosis.

[Acne induced by amineptin: adnexal toxiderma]. / L'acné induite par l'amineptine: une toxidermie annexielle.

INTRODUCTION: Amineptine-induced acne is a classical side effect often encountered after prolonged and excessive use of the antidepressor. CASE REPORT: We report the case of a young female drug addict (benzodiazepines and alcohol) who denied overuse of amineptine. The initial inflammatory aspect of the lesion was unusual as was the histology report. The sebaceous glands were the site of cystic dilatation as would be expected, but in addition the sudoriparous glands showed keratinizing syringometaplasia with areas of neutrophilic eccrine hidradenitis. DISCUSSION: This is the second report of such a case similar to other due to chemotherapy, benaxoprofene or toxic agents for example, and corresponds to direct toxicity to the sudoriparous glands which accumulate amineptine. We therefore propose the term of adnexal toxic drug eruption.

Tubal sterilization by selective catheterization in an animal model.

RATIONAL AND OBJECTIVES: The feasibility of tubal occlusion by selective salpingography was tested in an animal model; three novel occluding materials also were tested for this application. METHODS: Unilateral selective salpingography was performed in three groups of six rabbits; fallopian tubes were embolized with ethanol (group 1), a hydrogel (group 2), or an occluding emulsion (Ethibloc, Laboratoire Princeps, Neuilly sur Seine, France) (group 3). Animals were killed 2 days or 30 days after the procedure, according to randomization; tubal patency and histologic modifications were evaluated. RESULTS: Selective tubal catheterization was obtained in 100% of the cases. In group 1, no satisfactory occlusion was obtained; in group 2, 65% of the tubes were occluded with little histologic damage; in group 3, 80% of the tubes were occluded, but significant inflammation and tissue necrosis were noted. CONCLUSION: Selective salpingography proved a suitable method for gaining access to the fallopian tube and allowed selective injection of occluding materials. More research is needed to determine a suitable occluding material, focusing on biocompatibility and on long-term efficacy.

PIP: Unilateral selective salpingography was performed in 3 groups of 6 rabbits. 4-6 month old, virgin New Zealand White female rabbits were used with a mean weight of 4.4 kg. The fallopian tubes were embolized with ethanol (group 1); a viscous radiopaque solution which solidifies rapidly after injection to produce a biocompatible and inert hydrogel (group 2); or an occluding emulsion (a radiopaque heterogeneous alcoholic solution of zein, poppy seed oil, propylene glycol, and sodium amidotrizoate from Ethibloc, Laboratoire Princeps, Neuilly sur Seine, France) (group 3). Animals were killed 2 days or 30 days after the procedure, according to randomization; and tubal patency and histologic modifications were evaluated. Selective tubal catheterization was possible in all 18 cases, in 12 cases on the right side (66%), in 6 cases on the left side (33%); in 11 cases with a 5F catheter (61%), in 7 cases with a 2.5F catheter (39%). In group 1, no satisfactory occlusion was obtained; in group 2, 65% of the tubes were occluded with little histologic damage; and in group 3, 80% of the tubes were occluded, but significant inflammation and tissue necrosis were noted. The fallopian tubes were selectively catheterized over variable lengths: over 10 mm in 5 rabbits (28%), between 5 and 10 mm in 4 rabbits (22%), and between 1 and 5 mm in 9 rabbits (50%). Before injection of the occluding materials, all the catheterized tubes were patent. Mean volume of occluding material injected was 0.36 mL in group 1, 0.30 mL in group 2, and 0.83 mL in group 3. The ethanol injected reached the peritoneum in all 6 rabbits. The gel was injected an average length of 11 mm in the tube, while the emulsion opacified all the volume of the tubes with a peritoneal spill of emulsion in 3 of 6 cases. Reflux of occluding material into the uterus was noted in 1 of 6 rabbits in group 1, in 4 of 6 rabbits in group 2, in 3 of 6 rabbits in group 3. Selective salpingography proved a suitable method and allowed selective injection of occluding materials.

[Vulvar involvement in Crohn disease]. / Atteinte vulvaire au cours d'une maladie de Crohn.

INTRODUCTION: There are many skin manifestations of Crohn's disease including specific lesions occurring late after digestive signs. These manifestations are also termed metastatic and are rarely located on the vulva. CASE REPORT: We observed a metastatic localization in the vulva of Crohn's disease in a 34-year-old woman with predominantly rectosigmoid disease. The right labia major was greatly enlarged with oedema involving the inguinal cural area. Pustulization and erosive lesions were seen. The course of these lesions was independent of the colonic disease and responded temporarily only to a triple drug combination of metronidazol, prednisolone and minocyclin. CONCLUSION: This case emphasizes the frequency of colonic or rectal manifestations associated with vulval involvement in Crohn's disease. The metastatic lesions follow a course independent of the digestive disease raising therapeutic problems in this localization.

[Cystic dystrophy in an aberrant pancreas. Contribution of computerized tomography and MRI]. / Dystrophie kystique sur pancréas aberrant. Apport de la TDM et de l'IRM.

Case report of a cystic dystrophia appearing in an ectopic pancreas. The clinical diagnosis was preoperatively suspected by the findings of CT scan revealing a tumor located in the wall of the duodenum and by the data of MRI indicating its cystic and fibrotic structure. A focus of reacting pancreatitis was found in the vicinity of the cystic dystrophia while the remaining pancreas was normal. The surgical treatment was a duodenopancreatectomy, justified by the potential risks of complications.