[Post-radiation atypical vascular lesions and angiosarcoma: 11 cases]. / Lésions vasculaires atypiques et angiosarcomes post-radiothérapie : 11 cas.

BACKGROUND: Post-radiation atypical vascular lesions of the skin display clinical and morphological overlap with well-differentiated angiosarcomas, and correct diagnosis may be difficult. PATIENTS AND METHODS: We studied clinical, histological and immuno-histochemical aspects (CD31, CD34, D2-40 and VEGFR-3) of eight post-radiation atypical vascular lesions comparatively with three post-radiation angiosarcomas. RESULTS: All patients were female and received radiation therapy for breast carcinoma. On average, atypical vascular lesions occurred 4.3 years after radiation therapy and presented as small papulonodules or erythematous plaques. The clinical course after simple excision was benign. Histologically, they were relatively circumscribed lesions and showed slit-like vessels dissecting dermal collagen in all cases. On average, angiosarcomas occurred 5 years after radiation therapy and presented as more extensive lesions with a more aggressive clinical course. The lesions showed histological overlap with atypical vascular lesions, but were poorly circumscribed, with deeper invasion, cytological atypia and mitosis. Although the immuno-histochemical profiles were similar, expression of VEGFR-3 was greater in two cases of angiosarcoma. CONCLUSION: Post-radiation atypical vascular lesions are benign lesions that display clinical, histological and immuno-phenotypic overlap with well-differentiated angiosarcoma, and diagnosis requires good clinicopathological correlation. VEGFR-3 may be useful for differential diagnosis, as well as amplification of the MYC gene.

[Disseminated perinaevic Meyerson phenomenon revealing melanoma]. / Phénomène de Meyerson périnævique disséminé révélateur d'un mélanome.

BACKGROUND: Meyerson phenomenon (MP) consists of an eczematous reaction occurring around a pre-existing dermatologic lesion that is usually melanocytic and generally benign, and which is known as a Meyerson naevus. We report a case of multiple Meyerson naevi revealing melanoma, which itself was surrounded by a halo of eczema. PATIENTS AND METHODS: A 55-year-old man of phototype III with atopic eczema presented for pruritic eczema present for a fortnight, found solely on and around the naevi on his trunk and at roots of his limbs. One of the melanocytic lesions affected by these Meyerson phenomena was clinically atypical and had been active for several years. Excision confirmed the diagnosis of level II extensive superficial melanoma measuring 0.75 mm in thickness and associated with lesional and perilesional eczematous remodelling. After surgery involving a 1-cm excision margin and local corticosteroid therapy of the eczema, the Meyerson phenomenon subsided with complete remission of the melanoma at 1 year. DISCUSSION: Meyerson phenomenon can affect one or more naevi at the same time; it is generally transient, may recur on occasion, and has a favourable outcome either spontaneously or with corticosteroid treatment. When not removed for histological verification, the melanocytic lesion regains its initial appearance following resolution of the phenomenon. MP differs from Sutton phenomenon (SP), which is a perinaevic vitiligo reaction leading to complete or partial regression of the melanocytic lesion, which may be either benign or malignant. CONCLUSION: This case of Meyerson phenomenon revealing melanoma shows that the melanocytic lesions targeted by MP are not necessarily benign.

Asymmetrical transmission of human herpesvirus 8 among spouses of patients with Kaposi sarcoma.

BACKGROUND: Among heterosexuals, the sexual transmission of human herpesvirus 8 (HHV8) has not been established. OBJECTIVES: To assess HHV8 seroprevalence in spouses of patients with classic and endemic Kaposi sarcoma (KS) and to suggest possible routes of transmission. METHODS: A case-control study was carried out in a teaching hospital among spouses of human immunodeficiency virus-negative patients with KS (cases - exposed subjects) and controls who did not have KS nor were related to patients with KS (nonexposed subjects). HHV8 seroprevalence in spouses of patients with KS was compared with HHV8 seroprevalence in controls matched for age, gender and place of birth. Other serology tests were compared between cases and controls. Among heterosexual couples, HHV8-seropositive and HHV8-seronegative spouses were compared for possible risk factors for virus transmission. RESULTS: HHV8 seroprevalence was significantly higher among spouses of patients with KS (13 of 22; 59%) than among matched controls (19 of 58; 33%; P = 0.043). Among heterosexual couples, five of five (100%) male spouses were HHV8 positive vs. six of 15 (40%) female spouses (P = 0.04). There was no significant difference between HHV8-seropositive and HHV8-seronegative spouses for all other factors screened for among heterosexual couples. CONCLUSIONS: Being a spouse of a patient with KS is a risk factor for HHV8 seropositivity. Our results suggest that female-to-male HHV8 transmission could be more efficient than male-to-female transmission among couples including a patient with KS. Transmission could involve distinctive behaviours, or currently unknown biological properties of HHV8.

[Pseudoneoplastic lesion of erythema elevatum diutinum]. / Forme pseudotumorale d'erythema elevatum diutinum.

BACKGROUND: Erythema elevatum diutinum is a rare, chronic and recurrent dermatosis affecting adults. The disease is characterized by symmetrical, red, brownish-purple, and yellow papules, plaques, and nodules distributed mainly over the extensor surfaces of the extremities. The aetiology is unknown. The condition can occur in association with haematological malignancies (30%), and most frequently with IgA monoclonal gammapathy. Histological diagnosis is sometimes difficult, especially in the late stages of the disease. We describe an unusual case of erythema elevatum diutinum with clinical and histopathological pseudoneoplastic features. CASE REPORT: A 60-year-old man with no significant medical history consulted for a large nodule of the left knee measuring 2.5cm and red-purple papules symmetrically distributed on the extensor surfaces. Surgical removal of the nodule was performed. The histologic findings were characterized by a predominant concentric fibrosis forming well-circumscribed dermal nodules, composed of small aggregates of spindle cells with palisading and lamellar patterns. Within the nodules, pycnotic polymorphonuclear leucocytes were observed. The nodules were surrounded by a lymphoplasmocytic and histiocytic infiltrate. The diagnosis of erythema elevatum diutinum was thus made and dapsone given, with partial improvement of the lesions. DISCUSSION: This case illustrates the nodular pseudoneoplastic presentation and the difficulty of histological diagnosis, since long-standing lesions can mimic connective tissue tumours. Clues for histopathological diagnosis are neutrophils scattered throughout the lesion, sometimes associated with vasculitis. Extensive investigation should be made for polymorphonuclear leucocytes that are always present, even in late fibrotic lesions.

[Excision skin margin and recurrence rate of skin cancer: a prospective study of 844 cases]. / Marges d'exérèse chirurgicale et taux de récidive des carcinomes cutanés: etude prospective de 844 cas.

OBJECTIVES: In order to evaluate recurrence rates after a mean follow-up period of five years, the aim of this unicentric prospective study was to collect data of the surgical procedure concerning both skin basal cell carcinomas (BCC) and squamous cell carcinomas (SCC). PATIENTS AND METHOD: Eight hundred and fourty-four patients were included and data were retrospectively analyzed. During the surgical procedure, the peripheral clinical clearance margin was, respectively, 4mm for the skin basal cell carcinomas, 8 mm for the sclerodermiform type and 10mm for the squamous cell carcinomas. RESULTS: Basal cell carcinomas (BCC) were represented in 80% and the most frequent locations were the nasal area (30%). On average, the BCC measured 13.2mm. The peripheral histological clearance margin average was 4.1mm. The rate of recurrence was 3.8% after follow-up at an average of 36 months. Squamous cell carcinomas were represented in 20% cases and their locations were mainly located on the lips, the cheeks, the genital region and the extremities. On average, the SCC measured 22.45 mm. The peripheral histological clearance margin average was 10.26 mm. The rate of recurrence was 5% after follow-up at an average of 33 months. CONCLUSION: In our study, the rate of recurrence was less than 5% after follow-up of five years. To avoid repetitive operations and the risk of recurrence in anatomically sensitive areas, these tumors should be treated with standard wide margins chosen between 4 and 10 mm, function of the histological type.

Skin involvement in scleroderma--where histological and clinical scores meet.

OBJECTIVES: A clinico-pathological study in diffuse systemic sclerosis (SSc) patients was performed to analyse whether the skin histological organization and the pro-fibrotic signals elicited by TGF-beta in fibroblasts vary according to the modified Rodnan skin score (mRSS). METHODS: Twenty-seven SSc patients underwent 45 skin biopsies with simultaneous measure of mRSS before or after treatment by immunosuppressive drugs, with or without autologous peripheral haematopoietic stem cell transplantation (HSCT). RESULTS: Double-blind optic microscopy analysis of the biopsies standard extracellular matrix stains allowed to define three histological subgroups: 6 with grade 1 weak fibrosis, 30 with grade 2 moderate fibrosis and 9 with grade 3 severe fibrosis. A significant (P < 0.0001) was identified between the grades of fibrosis and the mRSS. In skin fibroblast cultures, Smad3 phosphorylation levels, as well as mRNA steady-state levels of two transforming growth factor (TGF)-beta/Smad3 targets, COL1A2 and PAI-1, increased in parallel with the mRSS. When compared with pre-transplant values the degree of fibrosis observed after HSCT in the papillary and in the reticular dermis decreased in parallel with the fall in mRSS (n = 5 consecutive patients with repeated biopsies). CONCLUSIONS: The histological extent of skin fibrosis correlates closely with the mRSS. Both parameters appeared to regress after HSCT. The extent of TGF-beta signalling activation in SSc skin fibroblasts appears to parallel the severity of disease.

[Feasibility and technical problems of sentinel node analysis in melanoma]. / Analyse du ganglion sentinelle au cours du mélanome. Etude de faisabilité et problèmes techniques rencontrés: à propos de 97 cas.

BACKGROUND: Development of the sentinel lymph node (SLN) biopsy the last 10 years has changed surgical approach of solid tumor treatment and particularly of melanoma. The aim of our study was to analyze in our hospital, the feasibility of the SLN biopsy technique in order to define a better prognostic classification of melanomas. PATIENTS AND METHODS: Between July 1999 and October 2003, 97 patients were included in this study in our center. Criteria for inclusion were cutaneo-mucosal melanoma of Breslow >or=1,5 mm, and/or Clarck >or=IV, and/or ulceration, and/or signs of regression, before any surgical margins. RESULTS: Lymphoscintigraphy (LS) identified at least 1 SLN in 94 cases/97 (97%), thus permitting intraoperative SLN mapping and sentinel node biopsy of at least 1 lymph node in 88 cases/94 (94%). Failure of the SLN procedure was noted in 9 cases: in 3 cases, no lymph node was individualized by LS, in 1 patient, intraoperative SLN mapping failed to find the previously identified SLN and in 5 cases, a SLN was identified by LS and intraoperative mapping but could not be removed because of its deep location and difficulty of dissection. In 17 patients, removal of one or two "non sentinel lymph node(s)" was (were) made by the surgeon because of its (their) suspected aspect (black or large). Among the 88 patients who had dissection of at least 1 SLN, a micrometastasis was detected by standard histological evaluation and/or immunohistochemical stains in 14 cases (16%) and into a "non SLN" in 2 cases (2,3%). The median follow up of patients was 16 months (1- 48 months). Among the 14 patients with positive SLN, 6 (43%) relapsed. The other eight were in complete remission of their melanoma with a mean follow up of 11,44 months . Among the 74 patients with negative SLN, 7 (9,5%) developed a recurrence. Among the 9 patients in whom any sentinel lymph node have been removed, 3 had a relapse (one in transit than on lymph nodes, and two on lymph nodes). CONCLUSION: Our results are in accordance with the literature, and confirm the feasibility of SLN mapping and of SLN histological analysis in our center. We described in this study technical problems we encountered. Our study also show the prognostic value of this technique. However, advantage in global survey of sentinel node dissection and regional lymph node dissection in cases of micrometastases has still to be demonstrated.

[Analysis of lymph mode recurrence in patients with melanoma and negative sentinel lymph node negative. Retrospective study at the Hospital Saint-Louis, Paris]. / Evaluation du taux de récidive ganglionnaire après ganglion sentinelle négatif chez les patients porteurs de mélanome, et analyse des résultats. L'expérience de l'hôpital Saint-Louis, Paris.

Melanoma is a malignant tumor, with dominant lymphatic extension. Sentinel lymph node is the first lymph node touched by melanoma. Our retrospective and monocentric study is about 87 patients, between July 1999 and July 2003. The inclusion criteria were malignant melanoma with Breslow level superior or equal 1.5 mm, and/or Clark level superior or equal IV, and/or ulcerated, and/or in regression. Sentinel lymph node has been negative on histological analysis in 75 patients (86.2%). About these 75 patients, we found five metastatic lymph node recurrence (6.66%) in a short notice (median 10.2 months). For the five patients with recurrence, the original slides and tissue blocks were available for reexamination. Then, we found micrometastasis in two patients (40% of occult metastasis). Our rate of lymph node recurrence in patients with sentinel lymph node negative is about 6.66%. Our analysis make us believe that early recurrence are essentially linked to histological analysis limits, and maybe to skip metastasis existence.

High-dose therapy and autologous stem cell transplantation in relapsing cutaneous lymphoma.

Treatment of cutaneous T-cell and B-cell lymphomas is difficult and relapses are frequent. To evaluate the efficiency of high-dose therapy (HDT) and autologous stem cell transplantation (ASCT) on relapsing cutaneous lymphomas, we conducted a retrospective study of 14 patients. We investigated the clinical and histological parameters of the lymphoma, previous treatments to ASCT, short-term complications of ASCT, and occurrence of a relapse. There were 11 males and three females, with a median age of 42 years. Most often, the skin disease was disseminated without extracutaneous involvement. Four patients had a B-cell lymphoma and 10 a T-cell lymphoma. CD30 was negative in 8/10 T-cell lymphomas. Before ASCT, 13 patients had chemosensitive disease; one had refractory disease. The conditioning regimen included TBI in nine cases. No toxic death occurred. Relapse of the lymphoma occurred in eight cases (T-cell lymphoma in seven cases), within 4 months after ASCT in six cases. Relapses were treated with local treatment, interferon or classical chemotherapy. At the end of the study, 11 patients were alive and three patients had died. HDT and ASCT do not benefit patients with T-cell lymphomas. For patients with disseminated relapsing cutaneous B-cell lymphomas, this procedure should be considered.

[Hypertrophic eyelid conjunctival scar. A tarsal keloid]. / Cicatrice hypertrophique de la conjonctive palpébrale ou chéloïde de la conjonctive tarsale. A propos d'une observation anatomo-clinique.

The clinicopathologic case of a 53-year-old female patient with an abnormal tumor growing on the mucous part of the superior right eyelid is reported. The patient was operated on for ten years ago and a whitish mass slowly developed on the conjunctival face of the eyelid disturbing the use of corneal lenses. It was hard, painless and had the shape of a flat mushroom. The removal was performed under local anesthesia and allowed us to resect a hard and fibrous lesion. Histopathology showed that the lesion was made of a fibrous tissue organized like a hypertrophic scar. Surgical treatment of chalazion is frequent and rarely gives rise to abnormal scarring.

[Painful bruising syndrome mimicking cellulitis of the leg]. / Syndrome des ecchymoses douloureuses simulant une dermohypodermite bactérienne de jambe.

BACKGROUND: Autoerythrocyte sensitization syndrome (painful bruising syndrome) is marked by spontaneous and painful ecchymotic bruising, without any biological abnormality, occurring in women with pathological mental profile. Sometimes, when the inflammation is severe, an infectious cellulitis or muscular compression may be suspected. CASE REPORT: A 21-year-old woman was referred for the rapid occurrence of a diffuse and painful inflammation of the right leg, with fever. The leg was red, warm, with diffuse bruising. The foot was fixed in varus equin. The biological parameters were normal. Magnetic resonance imaging showed normal muscles but modifications in the subcutaneous fat, similar to an acute cellulitis. A cutaneous biopsy showed a moderate oedema of the dermis and erythrocyte extravasation. Antibiotics were inefficient on the pain and the aspect of the lesions. Because of the efficacy of saline serum injections on the symptoms and a pathological psychological profile, an autoerythrocyte sensitization syndrome was suspected. The skin test was negative. Psychiatric evaluation revealed a severe personality disorder associated with depression. The ecchymotic lesions disappeared with adapted psychiatric treatment. DISCUSSION: Painful bruising syndrome occurs most often in young women with various psychiatric problems. The lesions are characterized by recurrent, painful, swollen and bleeding bruises in any part of the body. Fever, abdominal pain and external bleeding are possible. Haematological and immunological findings are usually normal. The histology of early lesions reveals oedema of the dermis and modest perivascular infiltrate, whereas extravasated erythrocytes appear later. The magnetic resonance imaging aspect of the lesions has never been described. Abnormalities of the subcutaneous fat are similar to those of acute cellulitis, expressing the inflammation process secondary to the ecchymoses. The syndrome results from complex somatic and psychological mechanisms. Many drugs were tried for the treatment of the disease, without any significant improvement.

Use of voriconazole to successfully treat disseminated Trichosporon asahii infection in a patient with acute myeloid leukaemia.

Trichosporon spp. is an emerging fungal pathogen in immunocompromised hosts, and disseminated infection is often fatal in neutropenic patients. Reported here is a case of disseminated infection in a neutropenic patient with acute leukaemia. After failure of amphotericin B and fluconazole therapy, the course of infection dramatically improved with voriconazole treatment. A literature search revealed 69 additional cases of disseminated Trichosporon spp. infections in neutropenic patients, and these are also reviewed. Clinical symptoms that suggest infection include fever, disseminated papulopustular cutaneous lesions and pulmonary involvement. Despite treatment with antifungal agents (amphotericin B, fluconazole), 78% of patients died. Voriconazole may represent a promising therapy for this life-threatening infection.