Cutaneous sarcoidosis occurring during anti-TNF-alpha treatment: report of two cases.

We report two cases of cutaneous granuloma induced by anti-TNF-alpha therapy: a 47-year-old man suffering from psoriatic arthritis treated with infliximab and a 56-year-old woman treated with adalimumab for polyarticular juvenile rheumatoid arthritis. The biospies confirmed the diagnosis of a 'sarcoidosis-like' reaction. No systemic involvement was observed. Such cases of noninfectious granulomatous diseases occurring during anti-TNF-alpha therapy are becoming increasingly frequent.

[Bortezomib-induced eruption: Sweet syndrome? Two case reports]. / Toxidermie au bortézomib : syndrome de Sweet? Deux cas.

BACKGROUND: Bortezomib (Velcade) is a proteasome inhibitor used in the treatment of myeloma and other blood dyscrasias. We report the cases of two patients who developed a peculiar toxic rash suggestive of Sweet's syndrome while receiving bortezomib; one patient also presented giant mucous membrane ulcerations. PATIENTS AND METHODS: Case 1: bortezomib treatment was started in a 62-year-old man for mantle cell lymphoma. Ten days after the first treatment cycle, giant, painful oral ulcerations were noted but they resolved spontaneously. One week after the second cycle, further oral ulceration appeared, this time with a papulonodular skin rash. Histology showed neutrophilic dermal infiltrates in the skin with predominantly lymphocytic inflammation of the oral mucosa. Bortezomib was stopped and all lesions resolved with colchicine treatment. Case 2: a 46-year-old woman was receiving bortezomib treatment for plasma cell leukemia. A febrile skin rash appeared two days after the first treatment cycle but resolved spontaneously. After the first bortezomib injection during the next cycle, painful papules and nodules appeared on the trunk. The skin biopsy results were consistent with Sweet's syndrome. The lesions disappeared spontaneously. Dexamethasone was administered concomitantly with bortezomib in the ensuing cycles and there was no relapse of the skin lesions. DISCUSSION: Bortezomib-induced skin lesions are common and usually do not justify treatment withdrawal. Published observations of bortezomib-induced eruption occasionally show clinical and histological features of Sweet's syndrome, but there has been no mention of oral mucosal ulcerations. In our cases, these could be related to bortezomib-induced neutrophilic dermatosis.

Medallion-like dermal dendrocyte hamartoma: the main diagnostic pitfall is congenital atrophic dermatofibrosarcoma.

Medallion-like dermal dendrocyte hamartoma is a newly described and rare clinical and pathological entity. This congenital, round, erythematous and atrophic lesion in the thoracic area is histologically characterized by a CD34+ dermal and hypodermal spindle-cell infiltration. We describe the clinical, histopathological, cytological and molecular features of three cases of dermal dendrocyte hamartoma. In all the cases, atrophic congenital dermatofibrosarcoma protuberans (DFSP) was the first histological diagnosis. In one case, wide surgery had been performed on the basis of the clinical and histological presentation. The histological pattern was similar in all the cases: epidermal atrophy and a spindle to ovoid cell proliferation in the dermis and in the subcutaneous fat. Immunochemical staining for CD34 and factor XIIIa was positive. Cytogenetic and molecular studies were performed; no chromosomal abnormality nor translocation t(17;22)(q22;q13) was observed. Fluorescence in situ hybridization analysis did not reveal the DFSP fusion gene COL1A1-PDGFB. We observed that the main diagnostic pitfall of medallion-like dermal dendrocyte hamartoma is atrophic congenital DFSP due to clinical and histological similarities. We emphasize that molecular studies to eliminate the t(17;22)(q22;q13) translocation of DFSP may provide determinant elements for diagnosis in order to avoid unnecessary mutilating surgery.

[Drug reaction with eosinophilia and systemic symptoms (DRESS) following imatinib therapy]. / DRESS (Drug reaction with eosinophilia and systemic symptoms) après traitement par imatinib (Glivec).

BACKGROUND: Imatinib (Gleevec) is a tyrosine kinase inhibitor used to treat chronic myeloid leukemia. We describe a case of drug reaction with eosinophilia and systemic symptoms (DRESS) after institution of treatment with imatinib. PATIENT AND METHODS: A 78-year-old woman was treated with low-dose imatinib for chronic myeloid leukemia since November 2003. A macular and pruritic eruption appeared on the patient's trunk after 7 weeks of treatment and gradually worsened. After 1 month, she was admitted for generalized skin eruption with fever and diffuse lymphadenopathy. Laboratory data showed hypereosinophilia and blood cultures positive for Staphylococcus aureus. Imatinib was stopped and replaced with hydroxyurea (Hydrea). Improved clinical and laboratory results were seen with antibiotics and topical steroids. DISCUSSION: To our knowledge, this is the first case of DRESS following treatment with imatinib. Cutaneous reactions to imatinib are frequent and are usually mild, comprising maculopapular eruption, pruritus and facial edema. Few cases of serious skin reactions have been reported until now. Several authors suggest that the prevalence and severity of cutaneous manifestations are related to a pharmacologic effect of imatinib. Our observation cannot rule out an underlying immunologic mechanism. Septicemia may also play a part in the development of DRESS.

[Myxofibrosarcoma associated with anetoderma]. / Myxofibrosarcome dans une forme anétodermique.

BACKGROUND: Association of malignant cutaneous tumor and secondary anetoderma is rare. Secondary anetoderma in myxofibrosarcoma has not been described to date. We report a case below. CASE REPORT: A 80-year-old woman presented with a 40 x 40 mm, round, flesh-colored lesion on her left buttock. Physical examination showed a soft, protuberant lesion, without firm underlying subcutaneous mass. Pathologic examination of the surgical specimen revealed a myxofibrosarcoma, with focal loss of elastic fibers in the overlying dermis. There was no evidence of systemic involvement. One year later, she developed a recurrent tumor at the same site, with similar clinical presentation, which was treated by broad excision. DISCUSSION: Secondary anetoderma is usually seen in association with cutaneous infections and benign skin tumors. An anetodermic presentation of myxofibrosarcoma has not been reported to our knowledge. Myxofibrosarcoma (formerly referred to as myxoid malignant fibrous histiocytoma) is characterized by an abundant myxoid background in at least one half of the tumor. The tumor recurs in almost two-thirds of cases and metastasizes in one-fourth. Our case confirms that a unique, acquired anetodermic lesion can reveal a malignant tumor. A large deep biopsy should be performed systematically when this variety of anetoderma is observed.

[Venous ulcer on the hand due to a treatment-related arteriovenous fistula]. / Ulcère veineux de la main secondaire à une fistule artérioveineuse thérapeutique.

BACKGROUND: An arteriovenous fistula of the arm for hemodialysis needs to last long and provide easy access for puncture. CASE-REPORT: A 50 year-old woman, with type 2 diabetes complicated by chronic renal failure, presented with an ulcer on the dorsum of the right hand that had developed over the past year. Humeral artery to basilica vein, side-to-side, arteriovenous fistula in the right arm was created in 1996. In 1999, she received a renal transplant. In 2002, she developed a deep ulcer on the dorsum of the right hand that progressed over one year, without improvement good local treatment. Doppler echography and a fistulography revealed proximal stenosis of the basilica vein, and a less restricted distal stenosis before the shunt. A venous ulcer on the dorsum of the hand due excessive venous pressure in the draining area. DISCUSSION: The complications of arteriovenous fistulas can be severe. Arterial stenosis is frequent and is the consequence of intimal hypertrophy. Chronic ischemia symptoms can be observed, but the hemodynamic loss is usually asymptomatic. When clinical signs are noisy emergency surgery is required. Venous stenosis is responsible for ischemia through venous overload, leading to edema of the arm, and rarely to venous-ulcer type trophic disorders as seen in this patient. Screening for stenosis on the vascular vein network must be systematic before creating an arteriovenous fistula.

[Perforating granuloma annulare with transfollicular perforation]. / Granulome annulaire perforant unique avec phénomène d'élimination transfolliculaire.

INTRODUCTION: The perforating granuloma annulare is a rare form of granuloma annulare. The clinical diagnosis is difficult and the confirmation is histological. The localisation is unique in less than 10% of all cases. We report a documented case with a histological suspicion of transfollicular perforation. OBSERVATION: A 36 year-old woman, without any particular antecedent, presented on her upper arm a single ulcerated nodular lesion that had evolved for one year. The clinical examination and biological investigations were normal. The histological examination led to the diagnosis of perforating granuloma annulare with a large epidermic ulceration. Treatment with a topical corticosteroid was disappointing and the removal was decided. DISCUSSION: In our observation, the single localization of this lesion raises the problem of differential diagnosis such as cutaneous tuberculosis, atypical mycobacteriosis, skin sarcoidosis, foreign body granuloma, epidermoid carcinoma or perforating dermatitis. The histological examination permitted diagnosis of a perforating granuloma annulare with large epidermic ulceration. The infiltration and destruction of a hair follicle evoked the possible transfollicular elimination of the necrotic material. Other pathologies with the histological aspect of a palisading granuloma were excluded from this context. No associated pathology such as diabetes nor any other favouring factors such as ultraviolet light or insect bites or traumas were identified. The physiopathology of perforating granuloma annulare and the process of perforation remain unknown. Numerous therapies have been proposed with variable results.

Post-transplant cutaneous T-cell lymphomas.

Post-transplant cutaneous lymphomas are rare. Cutaneous T-cell lymphomas account for 30% of these lymphomas. The clinical appearance of the skin lesions is identical to cutaneous lymphomas observed in non-immunosuppressed patients, with infiltrated plaques, nodular and ulcerated tumors, but with an increased frequency of erythroderma. Standard histology and immunohistochemistry are also consistent with the features of mycosis fungoides and CD30+ cutaneous lymphomas observed in the general population. However, the pronostic differs from the usually favourable outcome of cutaneous T-cell lymphomas, as 8 out of the 13 patients of our series died, in less than 1 year for 6 of them. This unfavourable course appears to be the same as that observed for systemic T-cell lymphoma in transplant recipients. In contrast to post-transplant B-cell lymphomas (systemic and primary cutaneous), the link to a virus has not been demonstrated. The prognosis is also less favourable for post-transplant cutaneous T-cell lymphomas than for post-transplant cutaneous B-cell lymphomas.