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To determine the magnetic resonance (MR) imaging features of subungual glomus tumors.Thirty-one patients with a clinical suspicion of glomus tumor and 10 control subjects underwent MR imaging at 1.5 T. MR images of normal glomus bodies of a cadaver finger were correlated with histologic slices. With a local surface gradient coil, the pixel size reached 117 microns in one direction. Relaxation times were measured. Gadoterate meglumine was injected in 19 patients.Normal glomus bodies were visualized in the reticular dermis of the nail bed. Twenty-seven of 28 pathologically confirmed glomus tumors were detected with MR imaging. A peripheral capsule was present in most tumors. The nail matrix was compressed in 13 cases. The authors were able to differentiate three subtypes of glomus tumors (vascular, solid, and myxoid) on the basis of relaxation times and enhancement characteristics. Four patients had mucoid cysts or angioma in the nail bed.MR imaging can help accurately define the location and limits of glomus tumors before excision.

Angiotropic large cell lymphoma (ALCL), the so-called malignant angioendotheliomatosis, is characterised by proliferation of tumorous cells within small vessels. Manifestations in the CNS and cutaneous lesions prevail in the clinical presentation, although any organ can be involved. The recent classification of this lymphoma as part of the large cell lymphomas has modified the therapeutic approaches employed. This should improve the prognosis of this usually fatal disease. An unusual case presenting with fever, mononeuritis multiplex, and cutaneous lesions is reported. Peripheral neuropathy without other neurological symptoms is uncommon, and, to our knowledge, such isolated mononeuritis multiplex with nerve lesions has not been previously reported in ALCL. The clinical diagnosis was a systemic necrotising vasculitis and it is considered that its differential diagnosis must include angiotropic large cell lymphoma.

A case of a cutaneous metastasis as a first sign of a linitis plastica is reported.A 57 year-old man presented for a cervical infiltrated skin plaque. Histological examination and immunohistochemical staining gave the diagnosis of metastasis probably of gastrointestinal origin. Gastric endoscopy and biopsy confirmed the diagnosis of a linitis plastica.Cutaneous metastases from gastric carcinoma are uncommon and exceptionally the first sign of the disease. Their clinical and histological aspects are reviewed.

The aim of this retrospective study of 136 patients was to specify the natural history and the systemic prognosis of chronic cutaneous lupus erythematosus. It has been stated that in most of the cases, the disease only affects the skin.From 10 October 1980 to 31 December 1990, 136 patients with the following criteria were included in this retrospective study: clinical signs suggestive of chronic cutaneous lupus erythematosus, characteristic histology, insufficient evidence for the diagnosis of systemic lupus erythematosus.The prevalence of systemic clinical involvement in the population under study was nearly the same as in the general population. The following biologic or immunologic abnormalities were quite common: leukopenia, lymphopenia, thrombopenia (significantly more frequent among patients having widespread chronic cutaneous lupus erythematosus), low titers of complement levels, positive antinuclear antibodies (usually at a low titer). Eleven out of the 136 patients developed systemic lupus erythematosus, most often over 5 years after the onset of the cutaneous lesions. Four cases out of these 11 had poor prognosis: renal and/or neurologic involvement.This data suggests that patients with chronic cutaneous lupus erythematosus could benefit from long-term follow up, since the course to systemic disease occurs in only a few. Usually, antimalarials used singly or in combination with topical steroids may lead to the clearing of the lesions. Thalidomide will occasionally be useful whenever the disease is unresponsive to the preceding measures.

Two new cases of mid-dermal elastolysis are reported. In the first case, a 32-year-old woman, the clinical picture included the typical perifollicular protrusions of the trunk and wrinkled skin of the members. There was no prodrome and no other signs were associated. In the second case, a 39-year-old woman, after a 14-year clinical course, the skin manifestations involved the entire body with the exception of the face. Over the years, the initial follicular papules progressively formed plaques of wrinkled skin.

Epithelioid hemangioendothelioma is a rare, low-grade vascular malignancy reported for the first time in 1982 by Weiss and Enzinger. It involves one or, more rarely, several organs. We report a case involving the lungs and liver, in which the first manifestation was symptomatic hypertrophic osteoarthropathy. Findings four years after the diagnosis included very slow tumor spread, resolution of symptoms, and stabilization of radiological changes.

Lupus mastitis is an infrequent dermatologic disease. We describe a case of bilateral lupus mastitis heralding a severe systemic lupus erythematosus with the antiphospholipid syndrome.

Smooth muscle hamartoma is an uncommon cutaneous dysembryoplasia usually diagnosed in infancy. Among the 61 cases published since 1923, 56 were congenital and 3 appeared in young adults. We report a case in which the lesions started at the age of 15 years as a papular plaque in the right mammary region of a young woman. A review of the literature showed that the usual clinical presentation is a frequently pigmented plaque made of often follicular papules and measuring 1 to 10 centimeters on average. Excessive hairiness is the most frequent sign, being observed in more than two-thirds of the cases, and Darier's pseudo-sign is present in about 53 p. 100 of the patients. The disease is electively located on the lumbar region, the back and the root of the limbs. In 3 cases the lesions were generalized and the patients looked like fatty "Michelin-Tire Babies". The course of the disease is always favourable, and associated pathologies remain exceptional: urticaria pigmentosa and psychomotor retardation have been reported in two cases of the generalized form. Histology is characterized by the presence of numerous smooth muscle fibres disseminated in the dermis and diversely oriented, sometimes in contact with hair follicles which retain their normal morphology. The differential clinical diagnosis is with naevocytic naevus, café-au-lait spots, mastocytosis and connective tissue hamartoma. Belatedly revealed forms of the disease must be distinguished from Becker's hamartoma, but it must be known that in certain cases the classification is so difficult that some authors have suggested that smooth muscle hamartoma and Becker's hamartoma are only two poles of a single spectrum of dysembryoplastic lesions involving to varying degrees the epidermic and hair structures. Finally, the distinction between the localized forms of late onset smooth muscle hamartoma and multiple leiomyomas "en plaques" remains difficult both anatomico-clinically and nosologically.

We report a case of ciliated cutaneous cyst located on the dorsal side of the right shoulder in a 22-years old woman. The anatomoclinical features of this rare variety of cyst are described and compared with those of the 17 published cases. The differential diagnosis with other glandular cysts is presented. The mullerian duct origin of ciliated cutaneous cysts is controverted, and the starting point of this lesion remains mysterious.

Traditionally, the prognosis of pemphigus erythematosus is thought to be more favourable than that of pemphigus vulgaris. A retrospective study of the records of 10 patients with pemphigus erythematosus and 13 patients with pemphigus vulgaris was set up to compare the courses of the two diseases. This comparison, carried out in populations with similar age, sex ratio, pretreatment duration of the disease and treatment received, showed that relapses were more frequent in the course of pemphigus erythematosus, whereas remissions, mean duration of the disease and iatrogenic complications were the same in both groups. This study, therefore, throws some doubts on the dogma of relative mildness of pemphigus erythematosus, which goes back to a period long before systemic corticosteroid therapy was known. Mortality studies performed since the event of this treatment have shown that the prognosis had improved and tended to be the same in both diseases. The other data concerning the course of treated pemphigus erythematosus are little known and were never compared with those concerning pemphigus vulgaris. Our study shows that treatments similar to those of pemphigus vulgaris are necessary to obtain remissions in pemphigus erythematosus and that these diseases share the same evolutive profile. However, the question of the best therapeutic strategy to be used has not yet been answered.

Bilateral symmetrical polyarthritis occurred in three patients (2 males and 1 female), with no previous history of inflammatory rheumatologic disease, given alpha-interferon for 1 1/2, 7, and 10 months as treatment of chronic non A-non B hepatitis, myelofibrosis, and thrombocytopenia with myeloproliferative disorder, respectively. Joint manifestations developed 1 1/2, 3, and 10 months after initiation of alpha-interferon in a dosage of 3.10(6) U three times a week, 4.5.10(6) U per day, and 8.10(6) U three times a week. Polyarthritis persisted following withdrawal of alpha-interferon in the two last patients of whom one had rheumatoid nodules and positive rheumatoid serology and the other had scleritis, exanthema, and negative rheumatoid serology. Erosive rheumatoid arthritis was diagnosed after 28 months and 12 months, respectively, in two patients who required systemic corticosteroids with antimalarials (1 case) or azathioprine after failure of methotrexate (one case). Follow-up in the third case (12 months) is too short to allow differentiation of systemic lupus erythematosus (ANA: 1/1500 H with anti-DNA antibodies 58 U/ml) and chronic autoimmune hepatitis. Reports of chronic inflammatory rheumatologic disease during alpha interferon therapy are exceedingly few in number. In the cases reported herein, alpha-interferon may have either triggered or revealed the joint disease. To prevent occurrence of this complication, exclusion from alpha-interferon therapy of patients with autoantibodies or a positive history for clinical evidence of immune dysfunction may be considered.

The occurrence of elastic tissue abnormalities in the digestive tract is not common. There are sometimes observed in genetically transmitted connective tissue diseases in which numerous systemic complications are associated as in Ehlers-Danlos and in Grönblad-Strandberg syndromes. We report 7 cases of an unusual lesion localized in the colon in which abnormally increased amounts of elastic tissue were observed. This elastosis raises many nosological and diagnostic difficulties. We propose the term "colonic elastoma" to describe this lesion. The review of the literature allows to consider several etiopathogenetic possibilities.

We report three cases of systemic lupus erythematosus (SLE) associated with necrotizing histiocytic lymphadenitis (Kikuchi's disease) and immunologically proven human parvovirus B19 infection. Simultaneous occurrence of SLE and Kikuchi's disease was a characteristic of the three cases. Kikuchi's disease is an uncommon disease that usually affects young women and is characterized by painless unilateral cervical lymph-node enlargement. T-cell regions of affected lymph nodes are exclusively involved with patchy paracortical necrosis surrounded by a polymorphous cell population of histiocytes and macrophages. However, lymphadenopathy in patients with SLE may be histologically indistinguishable from Kikuchi's necrotizing lymphadenitis. The cause of Kikuchi's remains uncertain, although infectious agents have been proposed. A positive IgM-specific anti-human parvovirus B19 antibody test in our three cases suggests that B19 can induce a necrotizing histiocytic lymphadenitis and possibly a clinical SLE flare. High-dose (1 mg/kg/day) and medium-dose (0.5 mg/kg/day) oral prednisone was an effective treatment for constitutional and visceral symptoms of Kikuchi's and SLE diseases.

Florid necrotizing lymphadenitis, characterized by segmental infarction and lymphoid hyperplasia, is an uncommon feature of systemic lupus erythematosus (SLE). Kikuchi's disease is a well-defined clinicopathological entity, with a strong preference for the cervical lymph nodes of young women. The etiology of histiocytic necrotizing lymphadenitis (HNL) remains unknown, although viral agents have been proposed. HNL may reflect a self-limited SLE-like autoimmune disease but full-blown SLE associated with this condition has not, to the best of our knowledge, been reported. Thus, ours is the first description of the coexistence of SLE and HNL in 3 patients with immunologically proven parvovirus B19 infections. SLE and HNL were diagnosed simultaneously in 2 patients, but was retrospective in the third, in whom anti-tuberculous therapy was ineffective. Patients 1 and 2 were treated with prednisone (1 mg/kg/d) and responded rapidly. These data suggest that both HNL and SLE flares can be caused by parvovirus B19 infection.

One case of Muir-Torre Syndrome (MTS) is reported. It is characterized by an unusual association of an unique cutaneous sebaceous epithelioma and multiple visceral adenocarcinomas. The patient was a 67 year-old white woman, from a family with inherited cancer syndrome, who developed previously four colic and one endometrial carcinomas. The morphologic spectrum of the cutaneous tumors observed in the MTS is represented by kerato-acanthoma and/or sebaceous tumors as in the original case described by Torre. The cutaneous tumors are usually multiple and follow after numerous visceral cancers such as colic, endometrial, urinary, or gastric carcinomas. The visceral cancers are remarkable because of the familial character, the multiplicity of tumors and the low-grade malignancy. Our case highlights the difficulty to make an accurate diagnosis for rare histological variants of cutaneous tumors in the MTS.

The clinical and histological findings of a lymphoepithelial tumor of the skin are presented and compared to the 11 cases already published in the literature. This review permits to point out the main characteristics of this recently described tumor. In 10 of the 11 cases the tumor is located on the face. The diagnosis of basal cell carcinoma is most often evoked. The mean age is 40 and 9 of the 11 cases are women. The tumor histologically presented as an epithelial proliferation of basaloid cells with peculiar features: cyst-like cavities infiltrated by mononuclear cells, histiocytic and lymphocytic cells; marks of pilosebaceous differentiation: curling of epithelial cells, areas of keratinisation, large clear cells of sebaceous aspect. The origin of this tumor is discussed and the hypothesis are oriented towards a rare histological form of basal cells carcinoma or a hair adnexial tumor. New cases would provide more informations to specify the nosological place of this tumor.

The authors report the case of a 26 year old man, in whom recurrent hematuria revealed the diagnosis of urethral condylomata with bladder extension, and HIV serology positive. This case reminds us that it is essential to search for immunodeficiency in every case of extensive or recurrent condylomata. The analysis of such cases in the literature allows discussion of the various clinical courses according to the Human Papilloma Virus types found in condylomatous tissues.

Four cases of neuroendocrine carcinoma following Bowen's disease are presented. An immunohistochemical study was performed. The four patients, 3 men and a woman, ranging from seventy to eighty-seven years of age, developed a nodular tumor on a preexisting cutaneous lesion. In one of those cases the diagnosis of Bowen's disease was confirmed histologically before the apparition of the nodular tumor. The tumors were localized on the scalp, thorax, dorsum of the hand, and the scrotum. The four tumors were immunohistologically typical of neuro-endocrine carcinoma: there was a positivity for neurofilaments, cytokeratins and neurone-specific enolase. The clinico-pathological characteristics of those 4 neuro-endocrine carcinomas associated with a Bowen's disease, when compared with the 15 similar described in the literature, are identical to the isolated neuroendocrine carcinoma, from a clinical, morphological and evolutional point of view. The majority are seen in patients older than 60 years old and one third of the cases described survived at least 5 years. The coexistence of Bowen's disease and neuroendocrine carcinoma, the association of neuroendocrine and epidermoid cells in other cutaneous tumors, reactivate the controversy concerning the histogenesis of the so-called Merkel cell carcinoma. In fact, the histogenesis of the tumor is still not fully understood.

An immunohistochemical study of 40 primary and secondary malignant melanomas in 27 patients, with routinely-used monoclonal antibodies, demonstrated the antigenic pattern exhibited by these tumors, as well as the frequency of aberrant positivities for epithelial cell markers. The following results were found: 95% of melanomas stained positively for S100 protein and vimentin, which is the characteristic immunohistochemical pattern in melanomas. In most cases, the primary tumor and its metastases in a given patient had the same antigenic phenotype. As for epithelial markers, 10% of melanomas stained positively for KL1 and 27.5% for EMA. Despite this relatively high frequency of KL1 and EMA positivities, in practice, simultaneous positivity for S100 protein and vimentin, confronted with the patient's history and with histologic features, firmly establishes the diagnosis of malignant melanoma in virtually all cases.

Recently, applications of immunohistochemical techniques for the cytoplasmic localization of intermediate filaments has produced advances in tumor diagnosis and characterization. We report a 58-year-old white male with a clinically and histologically typical metastatic malignant melanoma. This case was peculiar because the same neoplastic cells stained for both S-100 protein and keratin on paraffin embedded tissue. These facts illustrate how cautiously we must interpret the positivity of immunohistochemical technics. We thus insist on the importance of the clinical and basic histologic data for the diagnosis, in order to avoid errors.

Disseminated infection by Fusarium is being increasingly reported in immunocompromised patients. We report the first case of Fusarium oxysporum disseminated infection with well-documented fungal myocarditis. Despite antifungal therapy and hematologic recovery, the patient died in cardiogenic shock, myocardial involvement clearly contributing to the fatal outcome.

Resume Les auteurs rapportent l'observation d'un kyste anevrysmal “juxta-cortical” du troisieme metacarpien plus de 3 ans apres son exerese. Aucun signe de recidive n'a ete observe. Une revue de la litterature precise l'originalite de cette localisation et permet de discuter sa nosologie ainsi que les indications therapeutiques.

Resume Les observations de grossesse au cours de la granulomatose de Wegener sont tres rares quatre seulement ont ete decrites. Les auteurs rapportent un cinquieme cas au cours duquel la maladie s'est declaree dans le post-partum, ce qui a ete deja note deux fois dans la litterature. L'interruption de la grossesse ulterieure a ete suivie d'une flambee evolutive qui a emporte la malade. Le role du post-partum et du post-abortum dans le declenchement et l'aggravation de la maladie parait ainsi probable. Les deux malades qui recevaient des immunosuppresseurs, en association avec les corticoides n'ont pas presente de poussees evolutives apres l'accouchement. L'encadrement systematique de l'accouchement ou de l'avortement par une telle association parait licite, d'autant plus que les craintes quant a la toxicite fœtale d'un tel traitement semblent exagerees.

Resume Les auteurs rapportent une experimentation sur le rat adulte avec etude histologique des effets de la revascularisation d'un segment osseux isole. Un pedicule arterio-veineux est implante dans une tete humerale transplantee en souscutane. Il determine une neogenese vasculaire active qui s'accompagne d'une construction osseuse venant rehabiter la necrose constituee.