[Eosinophilic angiocentric fibrosis and granuloma facial with extra facial presentation, the same pathology?]. / Fibrose angiocentrique à éosinophiles et granulome facial à forme extrafaciale, une même entité ?

Eosinophilic angiocentric fibrosis is a rare fibro inflammatory lesion of unknown etiology which occurs usually in the upper respiratory tract mucosa of middle-aged adults. The histologic features show an eosinophilic vasculitis and an angiocentric fibrosis with onion-skin pattern. Firstly described as a mucosal variant of the granuloma facial, which is a rare cutaneous vasculitis with eosinophils, it is considerated by some authors as separated entities. Four cases have been described in the orbit and three of them were in fact an extension of a sinusal lesion. We report the first case affecting a 69-years-old male patient who showed an isolated orbital involvement in association with granuloma facial, extra facial. This observation illustrates the relationship between these two pathologies and consolidates the first hypothesis of a single disease with cutaneous or mucosal involvement.

Cutaneous collagenous vasculopathy: ultrastructural and immunohistochemical study of a new case.

Cutaneous collagenous vasculopathy is a very rare entity first described in 2000, manifesting clinically with acquired, progressively diffuse, cutaneous telangiectases with a histologically distinct aspect, characterized by a thick hyaline collagenous wall. Its cause is unknown. Differential diagnosis includes mainly generalized essential telangiectasia and telangiectatic mastocytosis. We report a 65-year-old Caucasian patient who presented with progressive telangiectasia starting over the lower limbs that spread progressively upwards. Histopathologic (light and electron microscopic) examinations were typical of cutaneous collagenous vasculopathy. We review the salient clinicopathologic features of this poorly known condition.

Porokeratoma (porokeratotic acanthoma): immunohistological study of a new case.

Porokeratoma (porokeratotic acanthoma) is a very recently described tumor-like acanthoma with features of porokeratosis (cornoid lamellation). We report herein a new case of this poorly known lesion that was studied immunohistochemically and discuss briefly its relationship with porokeratosis.

Conversion from calcineurin inhibitors to sirolimus reduces vascularization and thickness of post-transplant cutaneous squamous cell carcinomas.

BACKGROUND: Immunosuppression favors the development of skin cancer. Experimental data suggest that sirolimus (SRL) has antitumoral and antiangiogenic properties. An investigation was undertaken into the effects of SRL on squamous cell carcinoma (SCC) developing in organ transplant recipients (OTR) receiving immunosuppressive treatments, with special emphasis on vascularization. MATERIALS AND METHODS: SCC that developed in eight OTR before and after conversion from calcineurin inhibitors (CNI) to SRL were compared for thickness, differentiation, ulceration, perineural invasion, density of peritumoral infiltrate, peritumoral vascularization, density of T-regulatory cells and of intratumoral Langerhans cells and growth fraction. RESULTS: SCC developing under SRL showed lower peritumoral vascularization and thickness, and higher growth fraction and density of peritumoral T-regulatory cells. CONCLUSION: Conversion from CNI to SRL at clinically relevant doses is associated in vivo with a reduced vascularization and thickness of post-transplant human cutaneous SCC. This effect could account for the beneficial effect of SRL on immunosuppression-induced skin carcinogenesis in humans.

Multinucleate cell angiohistiocytoma: report of three new cases and literature review.

Multinucleate cell angiohistiocytoma (MCA) is a rare benign vascular proliferation of the skin of unknown cause. About 75 cases have been reported previously. We present herein three new cases of MCA studied immunohistologically and present a review the relevant literature that delineates the salient clinicopathological features of this unusual entity.

Granular parakeratosis in a patient treated with liposomal doxorubicin for ovarian carcinoma.

Granular parakeratosis is a rare benign dermatosis caused by an acquired disorder of keratinization that usually manifests with reddish-brown keratotic papules and plaques in intertriginous areas. It has specific histologic features but its pathogenesis remains unclear. Its frequency is probably underestimated because the condition is usually misdiagnosed as simple intertrigo. We report herein a new case of granular parakeratosis in a woman treated with liposomal doxorubicin for ovarian carcinoma that showed complete remission after discontinuation of chemotherapy. The relationship between granular parakeratosis and chemotherapy is discussed.

Prospective multicenter study of pegylated liposomal doxorubicin treatment in patients with advanced or refractory mycosis fungoides or Sézary syndrome.

OBJECTIVE: To assess the rate of objective response to pegylated liposomal doxorubicin hydrochloride (Caelyx) in patients with advanced or refractory cutaneous T-cell lymphoma (CTCL). DESIGN: Prospective, open, multicenter study. SETTING: Thirteen dermatology departments in France. PATIENTS: Twenty-five patients with either (1) stage II to stage IV CTCL previously unsuccessfully treated with at least 2 lines of treatments or (2) histologically transformed epidermotropic CTCL requiring chemotherapy. INTERVENTION: Administration of Caelyx intravenously once every 4 weeks at a dose of 40 mg/m(2). MAIN OUTCOME MEASURES: The response to treatment was evaluated by clinical evaluation. RESULTS: At the end of treatment, we observed an objective response (primary end point) in 56% of the patients (14 of 25): 5 complete responses and 9 partial responses. The median overall survival time was 43.7 months. For the 14 patients who experienced an objective response, the median progression-free survival time after the end of treatment was 5 months. CONCLUSIONS: This prospective study demonstrates the effectiveness of Caelyx in treating CTCL, with an overall response rate of 56% in spite of the high proportion of patients with advanced-stage disease. Responses were observed in 2 subpopulations of patients in which the prognosis is known to be poorer: Sézary syndrome (overall response rate, 60%) and transformed CTCL (overall response rate, 50%). Moreover, this study shows that dose escalation to 40 mg/m(2) does not seem to improve the effectiveness but increases toxic effects (especially hematologic toxic effects) compared with the dose previously tested of 20 mg/m(2).

Mild recessive bullous congenital ichthyosiform erythroderma due to a previously unidentified homozygous keratin 10 nonsense mutation.

We have identified a previously unreported homozygous nonsense mutation p.Cys427X in the keratin 10 (K10) gene (KRT10) in a Turkish girl with recessive bullous congenital ichthyosiform erythroderma (BCIE) showing superficial blistering. p.Cys427X is located upstream of the previously reported homozygous truncation mutation within the same exon 6 causing mRNA decay. Immunohistochemical examination showed a complete absence of K10 protein in the patient's epidermis. The findings of this study suggest that K10 knockout patients show unique clinicopathological features of clinically mild BCIE with blisters occurring within the granular layer. In addition, the unaffected, heterozygous carriers of the mutation indicate that the K10 peptide from one normal allele alone is sufficient for keratin network formation.

[Dermatological side effects of epidermal growth factor receptor inhibitors]. / Effets secondaires dermatologiques des inhibiteurs de l'EGFR.

Epidermal growth cell receptors (EGFR) play a key role in tumor proliferation. Their inhibitors (anti-EGFR) are promising treatments for various types of cancers. Papulopustular follicular eruptions are the most frequent dermatological side effect and occur in 45 to 85% of patients receiving this treatment. Paronychias appear after several weeks of treatment. They are painful and impair hand and foot function. A positive correlation seems to exist between the intensity of the papulo-follicular eruption, tumor regression and survival.

Bowen's Disease masquerading clinically as a follicular cyst in a renal transplant recipient.

Lesions developing in organ transplant patients may have a misleading clinical appearance. We report herein a case of Bowen's disease that developed within the epithelial wall of a hair follicle and masqueraded clinically as a banal follicular cyst in a renal transplant recipient. We speculate that this lesion developed from pre-existing Bowen's disease extending to the hair follicles.

Trichilemmal carcinoma of the skin mimicking a keloid in a heart transplant recipient.

Trichilemmal carcinoma is a rare cutaneous adnexal malignant tumor deriving from the outer root sheath of hair follicles. It is only rarely reported in recipients of solid (renal) organ transplants. We describe the first case of trichilemmal carcinoma presenting with a misleading clinical aspect in a heart transplant recipient. We then briefly outline the salient clinicopathologic features of this malignancy.

Keratotic skin lesions and other risk factors are associated with skin cancer in organ-transplant recipients: a case-control study in The Netherlands, United Kingdom, Germany, France, and Italy.

This study examines the association of keratotic skin lesions with the development of skin cancer in 915 solid organ-transplant recipients in five European countries. In a hospital-based case-control study, cases with squamous- and basal-cell carcinoma were compared with controls without skin cancer. Questionnaires, scrutiny of medical charts, and skin examination were delivered according to a standardized protocol. Keratotic skin lesions and viral warts were counted on different body sites. Keratotic skin lesions were strongly associated with an increased risk of squamous-cell carcinoma, with adjusted odds ratios of 4.1 (2.4;7.0) and 12.1 (6.1;24) for 1-49 and 50 and more keratotic skin lesions compared with no lesions, respectively. Keratotic skin lesions were also associated with basal-cell carcinoma with adjusted odds ratios of 2.9 (1.7;4.9) and 4.0 (1.7;9.2) for 1-49 and 50 and more lesions, respectively. Lighter skin types and painful sunburns were also significantly associated with an increased risk of squamous- and basal-cell carcinoma. Keratotic skin lesions are strongly associated with skin cancer and are, thus, an important clinical criterion for identifying those organ-transplant recipients at an increased risk of skin cancers who should be offered more intensive skin surveillance.

Merkel cell carcinoma in organ-transplant recipients: report of two cases with unusual histological features and literature review.

BACKGROUND: Non-melanoma skin cancers are the commonest malignancies after organ transplantation and are often associated with human papillomavirus (HPV). Merkel cell carcinoma is an uncommon neuroendocrine skin tumor, of which 67 cases have been reported up till now, usually briefly, in organ transplant patients. METHODS: Among a cohort of 2340 organ-transplant recipients, two patients (one renal, one heart) developed cutaneous Merkel cell carcinomas 5 and 12 years of post graft, respectively. These were studied histologically and immunohistochemically, as well as virologically for the presence of HPV. A thorough literature review of all reported cases of Merkel cell carcinoma following solid organ transplantation was performed. RESULTS: Despite a typical immunophenotype, the tumors showed unusual histological features: both were epidermotropic, and one was intermingled with a bowenoid squamous cell carcinoma. Search for HPV by immunohistochemistry and PCR proved negative in both cases. CONCLUSION: In the setting of organ transplantation, Merkel cell carcinoma is much rarer than other non melanoma skin cancers but may show unusual histologic features. HPV do not seem to be involved in its pathogenesis.