Cytokine-release syndrome related to rituximab limited to lesions and excision scars of lesions of primary cutaneous lymphoma.
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Biomedical subjects
Publications and source records attributed to Javier Fraga.
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Herpes zoster (HZ) occurs with an increased incidence in immunosuppressed patients, in whom it frequently displays atypical clinical presentations. Herpetic syringitis, the involvement of the eccrine epithelium by herpes virus infection, is an infrequently described histologic pattern that has been rarely and almost exclusively reported in HIV-infected patients. We report the case of a woman with Burkitt lymphoma who developed 2 nodular, asymptomatic lesions while receiving treatment with chemotherapy and radiotherapy for her hematological disease. Histology showed viropathic changes in the epithelium of eccrine glands not in the epidermis. PCR was positive for varicella-zoster virus (VZV). Nodular herpes zoster seems to be an exceptional clinical presentation. We report another such case which is, as far as we know, the first report of herpetic syringitis with no concomitant epidermal involvement.
UNLABELLED: Leukemia cutis used to be a late manifestation of leukemia. On the other hand, Grover's disease has been described in the setting of cancer. CASE REPORT: A patient diagnosed as having a chronic myelomonocytic leukemia presented with lichenoid, slightly infiltrated plaques on the anterior aspect of his thorax and abdomen and papulovesicles on his back. A skin biopsy showed the co-existence of leukemia cutis, Grover's disease, and syringoma-like features. DISCUSSION: We discuss and comment upon this unusual association.
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OBJECTIVE: To describe a series of 41 patients with fresh lesions of Sweet syndrome in which the histopathologic study demonstrated an inflammatory infiltrate mostly composed of histiocytoid mononuclear cells. DESIGN: Histopathologic, immunohistochemical, and cytogenetic studies of the inflammatory infiltrate in a case series of histiocytoid Sweet syndrome. SETTING: University departments of dermatology and a private laboratory of dermatopathology. METHODS: Conventional histopathologic study as well as immunohistochemical investigations were performed using the alkaline phosphatase antialkaline phosphatase technique with a large panel of antibodies. In some cases, fluorescent in situ hybridization studies were performed to investigate the presence of the bcr/abl gene fusion. RESULTS: Immunohistochemical studies demonstrated that most cells of the infiltrate showed immunoreactivity for CD15, CD43, CD45, CD68, MAC-386, HAM56, and lysozyme, which is consistent with a monocytic-histiocytic immunoprofile. However, intense myeloperoxidase reactivity was detected in most of the cells with histiocytic appearance, which raised the possibility of specific cutaneous involvement by myelogenous leukemia. Nevertheless, cytologic peripheral blood examinations, fluorescent in situ hybridization studies to investigate the bcr/abl gene fusion, and follow-up of the patients, taken all together, ruled out this possibility. CONCLUSIONS: This case series demonstrates that some fresh cutaneous lesions of Sweet syndrome are histopathologically characterized by an infiltrate mostly composed of cells that may be misinterpreted as histiocytes, when in fact they are immature myeloid cells. We named this histopathologic variant histiocytoid Sweet syndrome, which should not be mistaken with leukemia cutis or other inflammatory dermatoses that are histopathologically characterized by histiocytes interstitially arranged between collagen bundles of the dermis.
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Follicular mycosis fungoides is an infrequent variant of mycosis fungoides. It has classically been defined by the presence of an atypical lymphoid infiltrate around and in the follicular epithelium with little or no epidermotropism, and no follicular mucin deposits. The fact that there are cases with epidermal involvement and/or follicular mucinosis means that some uniform diagnostic criteria are necessary. We describe two cases of follicular mycosis fungoides with follicular mucinosis and with varying degrees of associated epidermotropism.
Large B-cell lymphoma of the leg (LBCLL) accounts for 2 % of primary cutaneous lymphomas, and the EORTC considers it to be of intermediate prognosis. It usually presents in elderly patients, and manifests clinically as erythematous nodules or tumors, often unilateral, on the lower third of the legs. On rare occasions, it presents with extracutaneous dissemination. We present the case of a 77-year-old male who consulted his physician because of an asymptomatic indurated erythematous papule which had appeared one month earlier on his left leg and had quickly and progressively increased in size. Concurrently, other similar lesions had been appearing on the thigh and back of the same leg. Histological analysis detected a large cell lymphoid infiltrate positive for CD20 and bcl-2 throughout the dermis. The extension study was normal. The patient was treated with an intravenous infusion of rituximab for four consecutive weeks, with full remission of the lesions.
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Marginal zone B-cell lymphoma (MZL) is probably the most frequent of the primary cutaneous B-cell lymphomas, which are entities with indolent behavior. Clinically, it appears in middle-aged patients as papules, nodules or erythematous plaques, solitary or multiple, on the trunk and proximal part of the limbs. The prognosis is excellent despite frequent cutaneous recurrences. We present the case of a 40-year-old male who, after having several recurrences of MZL over a ten-year period, was treated with rituximab for multiple skin lesions. The patient showed full remission after four weeks of treatment, and developed cytokine-release syndrome after the first infusion of the drug.
Lupus miliaris disseminatus faciei (LMDF) is a chronic granulomatous disease, which tends to be located on the central part of the face. Its typical histological image is one of epithelioid granulomas with caseous necrosis. Its association with epidermal cysts has been described on few occasions in literature. We present a case of LMDF associated with epidermal cysts in a female Caucasian patient. This association suggests that the rupture of the epidermal cysts may be related to the appearance of LMDF. However, there are probably other factors that may also contribute to its pathogenesis.
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Takayasu's arteritis (TA) is a chronic inflammatory and fibrosing arteriopathy that can also involve cutaneous vessels. The disease typically presents with a prepulseless phase that overlaps or is followed by the characteristic pulseless stage. In both phases of TA, cutaneous manifestations may be present. Lesions considered to be 'specifically' associated with TA have been described most frequently simulating erythema nodosum, erythema induratum and pyoderma gangrenosum. We report 2 Caucasian patients with TA and nodular cutaneous lesions. Nine skin biopsies from these patients were studied. A necrotizing vasculitis was present in 5 biopsies. We review those patients with TA and well-documented cutaneous manifestations in the English literature, with special interest in nodular lesions, the most frequent cutaneous manifestation of TA in Caucasian patients. Biopsies from lesions with similar morphology frequently show different histological findings.
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