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Primary low-grade B-cell lymphomas of the skin are separated into marginal zone and follicle center lymphomas according to the recent World Health Organization-European Organization for Research and Treatment of Cancer classification, with distinct histologic and immunohistochemical profiles. Some cases remain difficult to distinguish. The degree of relationship with their extracutaneous counterparts is currently being investigated on clinical, histologic and molecular grounds. Cytogenetic analysis using fluorescence in situ hybridization was performed on 12 frozen samples of infiltrated skin that had been classified as marginal zone lymphoma (MZL). Chromosomal changes known to be recurrently observed in systemic MZL of the mucosa-associated lymphoid tissue type, and in follicular center lymphoma were analyzed. These included trisomy for chromosomes 3, 7, 12, and 18 as well as t(14;18) IGH/BCL2, t(14;18) IGH/MLT1, and t(11;18) API2/MLT1 translocations. Complementary molecular search of IGH/BCL2 rearrangement using a polymerase chain reaction technique and of API2/MLT1 mRNA expression by reverse transcriptase-polymerase chain reaction were performed. Two cases showed evidence of trisomy 3 at levels varying from 14% to 20% of the analyzed cells. No other chromosomal abnormalities were found with those techniques in the remaining cases. These results demonstrate that known recurrent chromosomal abnormalities rarely occur in primary cutaneous MZLs and suggest the possibility of a variety of initial oncogenic events leading to a common downstream pathway. These data also underline that fluorescence in situ analysis on routine skin punch biopsies represents a reliable tool for the detection of chromosomal changes, but requires consistent dermal infiltration.

We report a case of lymphomatoid papulosis (LyP) localized to the face. Only ten cases of localized LyP have been reported, and those involved the extremities. Three of those cases finally evolved to classic LyP with a widespread distribution of the lesions, the other cases remain limited. The evolution into lymphoma for the localized form appears to be equivalent to that reported with the generalized LyP (20 %), underlining the need for long-term followup of these patients. This is the first reported case of LyP localized to the face.

Background: Non-Langerhans cell histiocytoses form a heterogeneous group defined by the proliferation of cells with macrophage characteristics. Diagnosis is easy in typical cases but becomes more complex in unusual forms., Case Report: We report the case of a 53-year-old patient who presented multiple brown-to-yellowish papules and nodules of the trunk, neck, and head evolving for 6 months. No visceral involvement was found. Histopathological examination revealed histiocytic proliferation with features of secondary xanthomization with the presence of giant foamy multinucleated Touton cells. One year later, all lesions cleared spontaneously. Based on the clinical presentation and evolution and on the immunohistologic data, we retain the diagnosis of adult xanthogranuloma (AXG) in a diffuse shape., Discussion: Multiple AXG is a rare entity (15 cases reported since 1963) with a stereotypic presentation. It is important to recognize because of its good prognosis and the absence of visceral involvement therefore requiring no investigations or aggressive treatments. This case is interesting because of the complete and spontaneous healing of all the lesions within 20 months., (Copyright (c) 2006 S. Karger AG, Basel.)

Atopic dermatitis (AD) is not regarded as a predisposing condition for cutaneous T cell lymphoma. Cyclosporine (CsA) is an efficient therapy in AD, and its side effects, including lymphocytic proliferation, are rare at the low dose used in such cases. So far 3 cases of patients who developed cutaneous T cell lymphoma under CsA treatment for atopy have been described. An adult patient with a history of AD received CsA therapy because of an atypical flare-up of his eruption. He rapidly developed papular atypical lesions, then followed by the onset of a voluminous cutaneous tumor leading to the diagnosis of mycosis fungoides (MF) transformed in CD30+ large cell lymphoma with nodal and blood involvement. No improvement occurred after withdrawing of CsA, and he required a polychemotherapy and an allograft. This case illustrates the dramatic evolution of an MF under CsA given in the hypothesis of AD. It stresses the fact that skin biopsies should be taken in case of atypical AD to rule out MF and avoid such an aggressive evolution under immunosuppressive therapy.

Metastasis is a significant event in cancer progression and continues to pose the greatest challenge for a cancer cure. Defining genes that control metastasis in vivo may provide new targets for intervening in this process with profound therapeutic implications. Melanoma differentiation associated gene-9 (mda-9) was initially identified by subtraction hybridization as a novel gene displaying biphasic expression during terminal differentiation in human melanoma cells. Mda-9, also known as syntenin, is a PDZ-domain protein overexpressed in many types of human cancers, where it is believed to function in tumor progression. However, a functional role of mda-9/syntenin in tumor growth and metastasis and the signaling pathways involved in mediating these biological activities remain to be defined. Evidence is now provided, using weakly and highly metastatic isogenic melanoma variants, that mda-9/syntenin regulates metastasis. Expression of mda-9/syntenin correlates with advanced stages of melanoma progression. Regulating mda-9/syntenin expression using a replication-incompetent adenovirus expressing either sense or antisense mda-9/syntenin modifies the transformed phenotype and alters metastatic ability in immortal human melanocytes and metastatic melanoma cells in vitro and in vivo in newborn rats. A direct relationship is observed between mda-9/syntenin expression and increased phosphorylation of focal adhesion kinase, c-Jun-NH2-kinase, and p38. This study provides the first direct link between mda-9/syntenin expression and tumor cell dissemination in vivo and indicates that mda-9/syntenin expression activates specific signal transduction pathways, which may regulate melanoma tumor progression. Based on its ability to directly alter metastasis, mda-9/syntenin provides a promising new focus for melanoma cancer research with potential therapeutic applications for metastatic diseases.

Aims: histological and clinical relationship study of 44 cases of primary cutaneous B cell lymphoma, classified according to WHO classification., Materials and Methods: histological, immunological and molecular analysis was correlated with clinical data., Results: 33 cases (75%) were marginal zone B-cell lymphomas (MZL), with head and trunk predominance (median age = 54 years). Relapses in other mucosa-related sites occurred in 3 patients. Histological transformation implied a more aggressive treatment in 3 cases. A favorable outcome was observed in most cases. 9 (20%) diffuse large B-cell lymphomas (DLBCL) appeared as a unique nodule, with female predominance (median age = 74 years) and 4 lymphoma-related deaths which seemed age-related (> 70 years) with only one lower limb localization. Histological aggressive features were present in 3 patients with a fatal outcome. 2 (5%) WHO grade 3 follicular lymphomas (FL) had an heterogeneous phenotype, with head localization, cutaneous relapses and good outcome., Conclusions: MZL, the predominant type, has a good prognosis, although transformation can occur, needing a more aggressive treatment. DLBCL, observed in older patients, has clinical and histological prognostic factors identical to extra-cutaneous lymphomas. FL appears rare and has an heterogeneous phenotype. Relationships with its nodal counterpart remains unclear.

Background: Elastosis perforans serpiginosa (EPS) is a reactive perforating dermatosis characterized by the elimination of abnormal elastic fibers from the upper dermis through the epidermis. In a few cases, it occurs as a side effect of treatment by D-penicillamine (DPA). The first case of EPS induced by DPA was described in 1972 in a patient treated for Wilson's disease. Subsequently, cutaneous changes resembling pseudoxanthoma elasticum (PXE) were observed in patients treated with DPA and were reported as pseudo-PXE., Case Report: We report herein the clinical, pathological and ultrastructural study of 2 new cases of DPA-induced EPS and pseudo-PXE. These patients had been treated for Wilson's disease since 14 and 16 years, respectively. Characteristic abnormal elastic fibers were found on histopathological examination of both EPS and pseudo-PXE skin and confirmed by an ultrastructural study. There was no ABCC6 mutation., Discussion: Penicillamine is able to induce widespread, cutaneous and systemic, elastic fiber damage. Our patients present typical features of DPA-induced elastosis, presenting as EPS and pseudo-PXE. ABCC6 mutation is associated with PXE and, as expected, it was absent in our cases of pseudo-PXE. This elastopathy has been related to morphologic changes in elastic fibers secondary to prolonged therapy in most cases. DPA may interfere with elastin cross-linking through inhibition of the enzyme lysyl oxidase, or by formation of complexes with the cross-linked precursors, impairing a normal maturation of elastic fibers. However, no fatal complication of DPA-induced elastopathy has been reported so far. An improvement of the cutaneous lesions is expected after the drug discontinuation.

Background: Hereditary subtotal leuconychia is a rare nail disease. The gene(s) underlying this phenotype is (are) not known. Immunohistochemical and ultrastructural studies of nails are performed infrequently., Objectives: To perform genetic linkage analysis and to assess ultrastructure and soft/hard keratin expression in hereditary white nails., Methods: We have analysed microscopically and ultrastructurally the white nails of a patient from a family in which the trait is inherited in an autosomal dominant manner as an isolated symptom. No skin lesions or hair abnormalities could be detected. Genetic linkage studies were performed on DNA samples obtained from several members of the affected family. A longitudinal surgical biopsy of the nail from a great toe was split in two parts. One part was fixed in formalin and processed for histopathology. Another part was further subdivided and embedded either in Epon, following fixation in 2% glutaraldehyde, or in Lowicryl K4M, after fixation in 3% paraformaldehyde. Dewaxed nail sections and Lowicryl ultrathin sections were also stained with various antikeratin antibodies., Results: Genetic linkage studies of the family pointed to the disease gene mapping to the chromosomal 12q13 region. Genes mapping within this chromosomal region include the genes coding for type II (basic) cytokeratins and hard keratins. The nail matrix presented an abnormal hypergranulosis. The upper part of the nail plate, originating from the proximal nail matrix, had a nonhomogeneous lamellar appearance, with numerous intracellular 'lipidic' vacuoles and 'empty' spaces separating keratin filament bundles. These cells were progressively shed at the nail surface. The cell loss was compensated by hyperproliferation of the distal matrix and of the nail bed keratinocytes, with persistent marked parakeratosis and loose arrangement of keratin bundles. The distal matrix and the nail bed contributed equally to formation of the lower plate. This presented the characteristics of a tissue composed of soft keratins. Accordingly, there was virtually no labelling with the Hb1 antibody to a basic hard keratin in the white nail, whereas the labelling with AE3 antibody to all type II keratins and with KL1 recognizing suprabasal soft keratins was normal or even enhanced., Conclusions: Genetic linkage indicates that the gene defect underlying the leuconychia in the family studied resides on chromosome 12q13. As the type II keratins map within this chromosomal interval, it is possible that a mutation in one of these keratin genes may be a cause of the hereditary leuconychia. The white appearance of nails in this disease seems to be due to an abnormal keratinization of cells originating from the proximal nail matrix, leading to the presence of abundant intracellular vacuoles and to a lesser compactness of keratins.

Due to the technical evolution of radiation therapy of breast carcinoma, new manifestations occur as side effects. Newly described unexpected cutaneous events are morphea of the breast and pseudosclerodermatous panniculitis after irradiation (PPAI). They appear as specific consequences of megavoltage radiation. Radiotherapy-induced bronchiolitis obliterans organizing pneumonia (R-BOOP) is one of the pulmonary manifestations due to recent advances in radiotherapy of breast carcinoma. We report a case of PPAI and R-BOOP of simultaneous onset in a 60-year-old woman 6 months after megavoltage radiation therapy for breast carcinoma. We also noted an improvement of both unexpected reactions with systemic corticosteroid therapy and a common relapse when tapering the treatment. Only eight cases of PPAI have previously been reported. We believe that this entity is often underdiagnosed. The symmetrical onset and parallel evolution suggest a common origin for PPAI and BOOP in our case: the megavoltage radiation therapy. The pathogeny of R-BOOP is not direct toxicity of irradiation because untreated areas can also be affected. Pathogenesis of PPAI remains unknown, but a simultaneous onset with R-BOOP suggests a similar mechanism. While a cumulative dose of irradiation does not seem to be determinant an immune reaction to neoantigens induced by radiation could be an hypothesis.

Background: We report the second case of enteric-type villous adenoma of the vulva. The differential diagnosis, histogenesis, and pathogenesis are discussed., Case: A 66-year-old woman had a tumor resected from the posterior aspect of the vulva. The tumor was characterized by its villous architecture, with columnar epithelium and goblet cells. This vulvar tubulovillous adenoma was identical to a tumor resected 6 months before from this patient's rectal wall., Conclusion: These two tumors behaved benignly, and the vulvar adenoma possibly originated with the müllerian vestige.

Background: Hypomelanosis of Ito was first described by Ito in 1952 as incontinentia pigmenti achromians. The consistent feature of the disease is a characteristic cutaneous hypopigmentation area following the lines of Blaschko, but the clinical manifestations are varied and hypomelanosis of Ito is regarded as a neurocutaneous syndrome. Hypomelanosis of Ito is sporadic but is probably a non-specific expression of chromosomal mosaicism; we report a case with four clones., Case Report: We report the case of a 26 year-old woman with neurocutaneous hypomelanosis of Ito and Trisomy 13 mosaicism. She also exhibited skeletal and ophthalmologic disorders. Immunohistology revealed a PS100 and Melan A decrease in hypopigmented skin. Cytogenetic study of normal and hypopigmented skin fibroblasts showed mosaicism with four clones., Discussion: This is the third case of Trisomy 13 mosaicism associated with hypomelanosis of Ito, although other anomalies on chromosome 13 have been described. Happle published "phylloid" pigmented cases, which are mainly associated with Trisomy 13. This is the first observation of four-clone mosaicism and can be explained by successive mutations during embryogenesis. Anomalies on chromosomes 5,6 and 21 have never been described. The definition of hypomelanosis of Ito is not well established and the disease is presently included in a group of "pigmentary dysplasia" with genetic mosaicism.

Apocrine skin carcinoma (ASC) is a rare aggressive cutaneous tumor. We describe a case of ASC occurring on a nevus sebaceus of Jadassohn (NSJ) in a 66-year-old man. Malignant degeneration of NSJ is a rare complication usually found in elderly patients. Most of these tumors are basal cell carcinomas or squamous cell carcinomas. Only five cases of ASC on NSJ have been previously reported. This report recalls the potential of NSJ for degeneration.

Junctional or compound melanocytic nevus and melanoma may involve the nail apparatus and are usually discussed in the differential diagnosis of nail pigmentation. Blue nevus has been very rarely reported in this location. We report the second clinicopathologic study of a subungual blue nevus.

Introduction: Pachydermodactyly is a superficial fibromatosis located on the proximal portion of fingers'phalanges and interphalangeal joints. Several types of this disease have been described depending on topography, etiology and pathological associations. We report a typical observation of pachydermodactyly associated with a plantar pachydermy., Case-Report: A 19 year-old man was followed for a psychotic disease, associated with mental retardation. On clinical examination, he showed a typical pachydermodactyly, predominantly located on the second, third and fourth fingers of both hands, associated with recent acrocyanosis. A pachydermic aspect was also observed on the external part of the feet sole. Histopathological analysis was identical on digital and plantar lesions with collagen swelling. These lesions extended into the subcutaneous fat only in the feet., Discussion: This observation is compatible with the most classical form of pachydermodactyly which involves several fingers of both hands, usually in men. Furthermore, the association with a psychiatric disorder and the notion of repeated traumatisms have been frequently reported in the literature. However, acrocyanosis and the important disability observed in our case are unusual. Association with plantar pachydermy has never been reported. The identical histological aspect on finger and feet sole suggests that pachydermatodactyly should be integrated in framework of acral pachydermy.

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Background: Lichen sclerosus remains an elusive disease with an uncertain relationship to morphea and scleroderma. The disorder has been difficult to treat, with no consistent and reproducible efficacious therapy. Recently, a beneficial effect of treatment with oral calcitriol (1-25 dihydroxyvitamin D3) in patients with scleroderma or morphea was described. This fact could be ascribed to the immunomodulatory effects of calcitriol observed in vitro and to inhibition of fibroblastic growth. Because of the success of calcitriol in localized scleroderma, we attempted this therapy in a patient with LS., Observation: One patient with cutaneous generalized LS resistant to different therapeutics was treated with calcitriol in an oral daily dose of 0.5 mcg. After 6 months of treatment, the skin extensibility increased, and the lesions improved. The improvement persisted after discontinuation of therapy during a follow-up period of one year. The only side effect was hypercalciuria, which resolved with dose reduction., Conclusion: Calcitriol has shown a beneficial effect in scleroderma and morphea during open studies. A case is reported of a patient with LS who had a dramatic response to calcitriol. Double blind, placebo-controlled trials are needed to assess the therapeutic value of calcitriol in patients with LS.

Introduction: Among the multiple forms of clinically atypical cutaneous sarcoidosis, lesions limited to light-exposed areas are rare. We describe a fourth observation., Case Report: A phototype V 51-year-old woman, born in Tunisia presented with papular erythema on the face. Treatment with topical steroids did not result in any improvement. Biopsy specimen revealed a non caseating granuloma according with sarcoidosis. Assessment for systemic sarcoidosis was negative. Phototesting showed no abnormalities. The patient was successfully treated with chloroquine., Discussion: Only three similar observations are reported in the literature. The role of light exposure despite the negative phototesting seems to be real. The tallying of these four observations permits one to isolate photo-induced sarcoidosis as a single entity.

Introduction: Leuconychia is the most common of ungueal discoloration or dyschromia. The hereditary form, sub-total or total, is very rare. We report the observation of a family in which thirteen members, distributed over four generations, presented a partial or subtotal leukonychia of all nails. On the basis of this observation and histopathological analysis of the propositus nail, we discuss the different hypotheses proposed to account for white nails., Observation: A 45 year-old man without previous medical history, showed partial or sub-total leukonychia of his twenty nails. Clinical examination revealed soft nails with slow growth. Discoloration or dyschromia was associated with koilonychia on the fingers without other cutaneous or visceral abnormalities. A longitudinal biopsy of the whole ungueal apparatus of the large toe was performed. Histopathological analysis showed parakeratosis and an abnormal granular layer thickened on the proximal and ventral womb. These abnormalities were responsible for heterogeneous HES coloration, lamellar and dissociated aspect of the nail plates. Electron microscopy revealed dissociated keratin bundles and the existence of intracytoplasmic clear vacuoles probably of lipid origin. This aspect was observed on the proximal part of the dorsal tablet and disappeared in the distal portion of the nail., Discussion: According to Newton's theorem, a surface appears white when it reflects all the radiation of visible light. This mechanism can be proposed to explain leukonychia. On histological level, parakeratosis and dissociation of the keratin bundles may play a role in the modification of the solar light reflection by ungueal plates. In our case, parakeratosis and disorganization of keratin bundles were present in the white part of the nail, but also in the distal, pinkish crescent (distal dorsal plate). Electron microscopy analysis showed clear vacuoles located in the white part of the nail, whereas they were not seen in the distal part. These observations confirm the probable participation of parakeratosis and keratin abnormalities in this pathology but also suggest an important role of lipid vacuoles. Therefore, some white nails may in fact be "milky" nails. Finally, disorganization of the keratin bundles observed, was also reported in other genodermatosis e.g.: epidermolysis bullosa simplex. In this latter pathology, some hard keratin genes mutations (K5 and K14) have been demonstrated. According to these results, a genetic study is on going in this family in order to search for a mutation in one of the hard keratin genes.

Background: Diagnosis of longitudinal melanonychia is usually difficult, and neither a single clinical criterion nor a combination of symptoms currently can be used to clearly distinguish malignant from benign bandlike pigmented nail lesions. Biopsy is painful and often leaves definitive dystrophic scars., Objectives: To describe and evaluate dermoscopic patterns associated with longitudinal nail pigmentation., Patients and Methods: A total of 148 unselected consecutive cases of longitudinal melanonychia were included over a period of 4 years (20 melanoma, 37 nevi, 16 drug-induced nail pigmentation, 45 nail apparatus lentigo of various types, 8 ethnic-type nail pigmentation, and 22 subungual hemorrhages). All patients were recruited from the dermatology unit outpatient clinic of the Hôtel Dieu de Lyon. All cases were photographed in vivo under oil immersion (dermoscopy). Patterns were recorded prior to final pathologic diagnosis. An independent biostatistics unit performed statistical evaluation using 7 semiologic patterns., Results: Melanoma cases were significantly associated with a brown coloration of the background and the presence of irregular longitudinal lines (P =.001). Blood spots were mostly observed in subungual hemorrhages (P =.001); however, their presence could not rule out melanoma. Micro-Hutchinson sign was observed only in melanoma, but its rare occurrence did not allow any statistical evaluation of its specificity. Nail apparatus nevi were significantly associated with a brown coloration of the background and the presence of regular lines (P =.001). Nail apparatus lentigo, ethnic-type pigmentation, and drug-induced pigmentation were significantly associated with homogeneous longitudinal thin gray lines and gray coloration of the background (P =.001). Microscopic longitudinal grooves were unspecific, occurred in several conditions, and were associated with any type of ungual discoloration., Conclusions: We believe that dermoscopic examination of the nail plate in cases of longitudinal melanonychia provides useful information that could help clinicians to more accurately decide if a nail apparatus biopsy should be performed; however, histopathologic diagnosis remains the gold standard in doubtful cases.

Introduction: Dermal melanocytosis is characterized by the presence of ectopic melanocytes in the dermis. There are several clinical forms. This report describes an unusual case of acquired dermal melanocytosis probably associated with leptomeningeal melanosis. An ultrastructural study was performed., Case Report: A 52-year-old Caucasian woman presented with a fifteen-year history of slowly spreading gray-blue macular pigmentation, associated with multiple blue nevi. Histologic examination revealed dermal melanocytosis. An extracellular sheath surrounded the melanocyte and their cytoplasmic processes. Medullar magnetic resonance findings were compatible with leptomeningeal melanosis., Discussion: Dermal melanocytosis is thought to represent a disorder of neural crest migration. Melanocytes are thought to be aberrant and become arrested during migration, remaining in the dermis. Reactivation of latent dermal melanocytes should explain the pathogenesis of acquired forms. Ultrastructural examinations show an extracellular sheath enclosing dermal melanocytes in all types of dermal melanocytosis.

We report a localized form of lymphomatoid papulosis (LyP) presenting as pustular papules of the hands. The histopathology revealed a moderate inflammatory infiltrate composed of atypical pleomorphic large lymphocytes with atypical mitosis and large nuclei. Epidermotropism could be observed. These atypical cells expressed CD4 and CD30. Laboratory examinations and bone marrow explorations remained negative. The clinical presentation of this case of LyP is unusual. Only histopathological features allowed to diagnose LyP. The knowledge that LyP may be associated with neoplasia or lymphoma underlines the need for a long-term follow-up of these patients., (Copyright 2002 S. Karger AG, Basel)

We report a 53-year-old woman who presented with multiple painful red cutaneous papules that had been growing slowly for 13 years. Histopathology showed typical features of neuroma. Biological, morphological and genetic investigations were negative and excluded the diagnosis of multiple endocrine neoplasia type 2b. After reviewing the literature, we concluded that our patient has an extremely unusual acquired disease, which must be considered as a distinct entity in the spectrum of cutaneous neurological disorders.

Emergence of the invasive phenotype is a key event in the progression of human melanoma from benign proliferative lesions to malignant lesions. Recently we successfully selected in vivo from a poorly metastatic M4Beu. human melanoma cell line two variants (7GP and T1P26) that generate a higher frequency of spontaneous metastases to the lungs into immune-suppressed neonatal rats. Both cell lines showed no significant differences in the integrin profile of the subunits analyzed except for beta3, which was reduced to a background level in metastatic variants. To investigate how these variant sublines of human melanomas manage to sustain growth in the absence of alpha(v)beta3, a subtractive immunization approach was used to elicit host antibody response against cell surface proteins expressed on metastatic variants. In this study, a new monoclonal antibody (MoAb), LY1, that is highly specific for the 7GP and T1P26 variants, was isolated. LY1 identifies a membrane protein of Mr 55,000 on melanoma variants with epitopes that were resistant to sugar-cleaving enzymes. Immunostaining cells from variants by LY1 showed that staining is distributed to the cell periphery with high labeling intensity at the cell-to-cell contact points. This MoAb significantly inhibited invasion of metastatic variants through a reconstituted basement membrane (Matrigel) in vitro. Moreover, tumor growth of melanoma variants was dramatically affected in vivo with this MoAb. In vitro studies indicate that the LY1 MoAb does not inhibit chemotactic migration of the metastatic variants, the adhesion of tumor cells to vitronectin, collagen IV, fibronectin, and laminin, or cell proliferation. Expression of this antigen is high in human striated muscle, heart, spleen, brain, and lung and absent in kidney, liver, and pancreas. Using 59 fixed, paraffin-embedded archival tissues of human melanomas and nevi, LY1-reactive cells were not observed in melanocytes, nevi, or radial growth phase primary melanomas. In sharp contrast, LY1 selectively stained melanocytes derived from the vertical growth phase of many primary melanomas and metastatic melanomas. These results provide evidence that the Mr 55,000 protein expressed by selected variants with increased metastatic properties in vivo plays a functionally important role in determining metastasis. This molecule may represent a new metastatic risk marker in human melanoma and may be of biological importance in the identification of fatal metastatic subpopulations that have acquired competence for metastasis production.

A 37-year-old man without previous medical history working as a lock keeper was seen in our unit for a progressive painless subcutaneous flesh coloured infiltration of the lower anterior area of the neck growing slowly over 5 months. Clinical cutaneous findings showed a non pruriginous yellowish papulonodular eruption mimicking xanthomas (Figs. 1 and 2). No other clinical abnormalities were found. A cutaneous biopsy specimen was performed. Histopathological examination revealed, under a normal epidermis, a dense lymphoplasmocytoid infiltrate involving the dermis with periadnexal and perivascular reinforcement (Figs. 3 and 4).

The clinical and pathological characteristics of cutaneous melanomas occurring in Camargue-type gray-skinned horses are reported. Examination of 83 tumor-bearing horses revealed that the tumors occurred most frequently underneath the tail (93.9%) and at high rates in the peri-anal region (43.0%), the lips (33.0%), and the eyelids (24.0%), but rarely in the vulva (3.8%). Tumorous lesions were characterized by the presence of either hemispheric nodules or large infiltrated plaques, or their combinations. Microscopic examination indicated that tumorous lesions were composed mostly of melanocytes and numerous melanophages and that component cells manifested a remarkable cellular atypia with anisocytosis and anisokarinosis. Pathological examination of lesions corresponding to earlier stages of the tumors disclosed that tumor formation and its related melanogenesis occurred in close topographical association with apocrine sweat glands, but not at the dermal-epidermal junction. Larger nodules of the tumors were often composed of a concentric deposition of cell layers, each being separated by dermal components from the epidermis. Because of the absence of their tropism toward the epidermis and their multiloculation, horse melanomas are considered to be different in their pathological characteristics from human melanomas.

We report the case of a 69-year-old woman who presented a papular eruption on the eyelids. Histological features revealed a tuberculoid granuloma with a central caseating necrosis. Laboratory and radiological investigations revealed no tuberculosis and no systemic granulomatosis. Absence of vascular symptoms, inefficiency of cyclines and histopathological findings excluded granulomatous-type rosacea. Lupus miliaris disseminatus faciei (LMDF) was our final diagnosis. On the basis of our findings and a literature review, we believe that LMDF is an entity distinct from either skin tuberculosis or granulomatous-type rosacea. However, its name is confusing, and we propose to change it to 'facial idiopathic granulomas with regressive evolution (FIGURE)'., (Copyright 2000 S. Karger AG, Basel)

Epithelioid hemangioendothelioma described first by Weiss and Enzinger in 1982 is an uncommon vascular tumor usually involving soft tissue, less frequently the lung and the liver and exceptionally the skin. We herein report a 52-year-old woman who presented an isolated moderately painful persistant ulceration of the concha of her left ear. Histopathological findings showed strands and nests of epithelioid endothelial cells typical of cutaneous epithelioid hemangioendothelioma. Immunohistochemical stainings confirmed the vascular nature of the tumor. Surgical excision by ear amputation was performed. In a review of the literature, to our knowledge, this clinical presentation as ulceration has never previously been reported.

Background: Primary cutaneous B-cell lymphoma is a heterogeneous group among which marginal zone B-cell lymphoma (MZL) appears to be the most common subtype., Objective: We analyze clinical presentation, histologic aspects, and outcome of patients with primary cutaneous MZL., Methods: All samples classified as primary cutaneous lymphoma over the past 10 years were reviewed, and cases of primary MZL were identified., Results: Nine cases of MZL were analyzed, all from the upper body region, with a predominance in elderly women. Histologic aspects included a dense, nodular, deep-seated infiltrate containing various proportions of small cells displaying a centrocyte-like, plasmacytoid or monocytoid appearance. Surface expression of CD5, CD10, and CD23 was negative. Long survival was noted but relapses in the skin, nodes, orbit, salivary glands, and breast were observed., Conclusion: MZL is the predominant primary cutaneous lymphoma of our study. It has distinctive histologic and clinical features as well as outcome.

We report 2 cases of acquired lymphangiectasias after breast radiosurgical treatment. This well known, rarely reported complication is probably due to a mechanical obstruction of the lymphatic network, and is generally preceded by lymphedema. Our 2 cases, however, did not have previous lymphedema. After a review of the literature, we discuss the role of reparative surgery and other treatment options.

Colony-stimulating factors are widely used for bone marrow recovery after chemotherapy. Various cutaneous side-effects have been described in most cases involving neutrophils. We report the first case of lichenoid reaction at injection sites of granulocyte colony-stimulating factor (G-CSF) in a 40-year-old patient treated for breast cancer. The eruption cleared after drug withdrawal, no recurrence was observed after drug replacement by granulocyte-macrophage colony-stimulating factor. Mainly lymphocyte-mediated lichenoid eruption to G-CSF was shown. Cutaneous side-effects to G-CSF do not share unequivocal pathogeny based on stimulation of neutrophils.

We report on a 32-year-old female with a 3-year history of an asymptomatic erythema of the forehead. The lesion had a linear distribution following the lines of Blaschko. Histopathological findings and direct immunofluorescence allowed to establish the diagnosis of cutaneous lupus erythematosus. Treatment with local corticosteroid and antimalarial agents given for 2 months resulted in a complete remission.

Introduction: Solitary schwannoma is usually located in the head area and the limbs. We report a case of benign solitary schwannoma of the retroglandular sulcus of the glans penis., Case Report: A 64-year-old man had developed over the past year an asymptomatic nodule arising from the retroglandular sulcus of the glans penis. The nodule was removed surgically and histopathological study showed benign schwannoma. There was no association with neurofibromatosis or schwannomatosis., Discussion: Benign solitary schwannoma of the penis is rare. It is most often located on the dorsal shaft of the penis in a young adult. Surgical excision is required. Malignant schwannoma of the penis is very rarely reported.

Introduction: Membranous lipodystrophy represents a peculiar dermatopathologic type of cystic forming adipose tissue necrosis. In skin pathology two distinct entities are currently known: a primary idiopathic type and a secondary type found in association with various cutaneous or systemic diseases (lupus erythematosus, scleroderma, dermatomyositis, venous disorders, trauma, diabetes mellitus...). This secondary type is more common., Case Report: We report the case of a female patient treated with cytostatic regimen for metastatic adenocarcinoma of the breast. She was seen with cyclic painful attacks of hypodermitis of lower limbs following drug infusions. Attacks finally vanished after treatment withdrawal. Membranous lipodystrophy was observed microscopically., Discussion: This is, to the best of our knowledge, the first case of membranous lipodystrophy occurring during cytostatic treatment. The origin of such a phenomenon remains nuclear but cystic formation occurs after adipocyte necrosis and membranes are thought to be formed from dead cells plasma membrane remnants. Primary cell injury could be due to ischemia to which adipose tissue is especially susceptible. In our case, role of circulatory troubles or cytostatic chemotherapy could be equally discussed. However cyclic attack of painful hypodermatitis following drug administration was strongly in favour of treatment responsibility. Moreover clearing of lesions after treatment discontinuation brought more arguments for this last hypothesis.