Hair casts and cutaneous spicules in multiple myeloma.
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Biomedical subjects
Publications and source records attributed to Michael D Ioffreda.
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A 55-year-old white female with a complex medical history including mixed connective tissue disease and peripheral vascular disease developed a group of red-purple papules on her proximal medial thigh that was followed, five months later, by the development of a large violaceous patch. She reported a history of radiation to this site (for melanoma) during her childhood. She was admitted to the hospital with a presumptive diagnosis of cellulitis, but failed to respond to antibiotics. A biopsy was performed and demonstrated a well-differentiated angiosarcoma arising in conjunction with reactive angioendotheliomatosis. Excision of the lesion was performed, and fifteen months of follow-up have shown no recurrence or metastasis.
BACKGROUND: The CD30-positive lymphoproliferative disorders lymphomatoid papulosis (LyP), primary cutaneous anaplastic large cell lymphoma (C-ALCL), and systemic anaplastic large cell lymphoma (S-ALCL) are lesions that overlap clinically, histopathologically, and immunophenotypically. Their biologic behaviors, however, vary considerably. In particular, lesions of LyP regress spontaneously while those of S-ALCL persist and often progress. Apoptosis has been suggested as the mechanism by which the lesions of LyP regress, but the underlying signaling pathways remain unclear. In this study, we used newly developed activation state-specific antibodies to demonstrate apoptosis signaling through the death receptor-mediated pathway regulated by FADD and caspase 3. METHODS: Dual immunohistochemistry for CD30 and activated forms of FADD and caspase 3 was performed on cutaneous biopsy specimens from 27 patients with CD30-positive lymphoproliferative disorders involving the skin. The patients included 18 with primary cutaneous CD30-positive LPDs (15 with LyP and 3 with C-ALCL) and 9 with S-ALCL. RESULTS: The proportion of CD30-positive cells expressing activated FADD was significantly different between primary cutaneous CD30-positive lymphoproliferative disorders and S-ALCL (36.4% vs. 14.5%, P = 0.0083). Expression of cleaved caspase 3 was also significantly different between primary cutaneous lesions and S-ALCL (9.2% vs. 1.9%, P = 0.048). CONCLUSIONS: Although a larger number of cases should be studied to validate these results, these data provide evidence that differences in signaling through the death-receptor apoptosis pathway mediated by FADD may be responsible for the varying biologic behaviors of CD30-positive lymphoproliferative disorders involving the skin.
BACKGROUND: Primary cutaneous LELC is a cutaneous neoplasm with histopathologic features identical to those seen in the undifferentiated subtype of nasopharyngeal carcinoma. It is extremely rare, with only approximately 30 cases reported in the literature. METHODS: We report a case of primary cutaneous LELC arising on the forehead of a 72 year-old male in which a proportion of the neoplastic cells demonstrated distinctive spindle cell morphology. RESULTS: Microscopic examination showed a dense lymphoplasmacytic infiltrate admixed with large spindle-shaped cells with vesicular nuclei, prominent nucleoli, and frequent mitotic figures. These cells were negative for an extensive panel of immunohistochemical markers and positive only for broad-spectrum cytokeratins and epithelial membrane antigen. There was no connection between the tumor and the epidermis and no epidermal dysplasia. In situ hybridization for Epstein-Barr virus was negative. CONCLUSIONS: The spindle cell differentiation in this case is unusual and suggests that in some cases the differential diagnosis of cutaneous spindle cell neoplasms might include primary cutaneous LELC.
Neutrophilic eccrine hidradenitis (NEH) is an acute, self-limited, inflammatory neutrophilic dermatosis. It is most commonly described in patients with acute myelogenous leukemia (AML) following chemotherapy. NEH generally presents as erythematous papules and plaques on the head, trunk, and extremities. Histologically, NEH is characterized by a neutrophilic infiltrate surrounding eccrine ducts and coils with occasional necrosis. We describe a case of NEH with an unusual presentation of annular plaques. A search of the literature revealed only one other case report of NEH presenting as an annular eruption.
Basal cell carcinoma (BCC) has many histologic variants. These variants may show differentiation toward benign and malignant tumors of cutaneous appendages, especially hair follicles. Herein we describe a lesion that was clinically thought to be a BCC, but a superficial biopsy showed histologic features suggestive of an apocrine hidrocystoma. Because of some cytologic atypia, complete excision was recommended. The excision specimen revealed a cystic BCC. The importance of examining the entire neoplasm before reaching a final pathologic diagnosis is emphasized.
Up to one fourth of patients with rheumatoid arthritis (RA) may have extraarticular findings such as subcutaneous nodules. These are discrete subcutaneous granulomatous nodules located on extensor surfaces, especially of the elbows. Over the past 10 to 15 years, there have been reports of accelerated cutaneous nodulosis in patients receiving methotrexate therapy. Recently, antitumor necrosis factor alpha (anti-TNFalpha) biologic therapy has become commonplace in the management of RA, especially in methotrexate-resistant or toxic patients. There have been recent reports of accelerated nodulosis in patients with RA on etanercept. We describe what we believe is the first case of accelerated cutaneous nodulosis resulting from infliximab anti-TNFalpha therapy in a patient with RA. One year after the initiation of infliximab, with RA in remission, our patient noted the rapid development of rheumatoid nodules of both hands. A biopsy was characteristic of a rheumatoid nodule, revealing palisading granulomas and fibrinoid necrosis.
BACKGROUND: Angiolymphoid hyperplasia with eosinophilia is a benign vascular proliferation that typically presents on the head and neck. Multiple treatment modalities have been proposed for angiolymphoid hyperplasia with eosinophilia, each with limited success or undesirable side effects. At this time, standard surgical excision is considered the treatment of choice but carries recurrence rates of 33% to 50%. OBJECTIVE: The objective was to present a case of angiolymphoid hyperplasia with eosinophilia successfully extirpated using Mohs micrographic surgery. METHODS: A 52-year-old woman presented with an ill-defined solitary plaque of angiolymphoid hyperplasia with eosinophilia within her left conchal bowl that enlarged despite conservative therapy with intralesional and topical corticosteroids. Mohs micrographic surgery using the fresh tissue technique and standard hematoxylin and eosin staining was performed. The characteristic histologic features of angiolymphoid hyperplasia with eosinophilia were readily identifiable on frozen sections and complete extirpation required two stages of micrographically controlled resection. RESULTS: Resection of angiolymphoid hyperplasia with eosinophilia of the conchal bowl with complete resolution of symptoms and no evidence of clinical recurrence 8 months after surgery. CONCLUSION: Given the high recurrence rates reported for standard excision, Mohs micrographic surgery with complete margin examination should be considered as a treatment option for angiolymphoid hyperplasia with eosinophilia, particularly for lesions with ill-defined margins or in locations where tissue sparing is desirable.
BACKGROUND: Nevus sebaceus has a well-documented potential to develop a wide variety of neoplasms of both epidermal and adnexal origins. It is highly unusual for more than three tumors to arise simultaneously within a single nevus sebaceus. Sebaceous carcinoma arising within a nevus sebaceus is a rare occurrence. OBJECTIVE: The objective was to report the case of a patient with a nevus sebaceus that simultaneously developed five distinct neoplasms of epidermal and various adnexal origins and to report the fourth case of sebaceous carcinoma arising within a nevus sebaceus. METHODS: A 45-year-old woman presented with a nevus sebaceus that contained five separate neoplasms, including sebaceous carcinoma, basal cell carcinoma, trichoadenoma, trichoblastoma, and syringocystadenoma papilliferum. RESULTS: Complete excision of the nevus sebaceus and the five tumors was performed. Systemic work-up showed no evidence of metastatic disease or association with Muir-Torre syndrome. CONCLUSION: This case report highlights the diverse neoplastic potential of nevus sebaceus and demonstrates the capacity of this hamartoma to develop aggressive tumors, such as sebaceous carcinoma. Prophylactic excision or at least close clinical surveillance for sudden development of new growths is warranted in all cases of nevus sebaceus.
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In 1999, we noted the development of inflammation and/or abscesses at the site of radial access in a group of patients. Over a 3-year period, we noted this inflammation in 33 patients out of 2,038 (1.6%) who had catheterization via the radial approach. The radial abscesses occurred in 30 patients out of 1,063 (2.8%) in whom we could confirm the use of a hydrophilic-coated sheath, but in no patient for whom we can document that an uncoated sheath was used. No infectious agent could be implicated, and the time course for the development of the abscess, typically 2 to 3 weeks, seemed long for a bacterial infection. Later patients had biopsies, and granulomatous reactions were seen in most. Additionally, a few of the biopsies showed an amorphous extravascular substance consistent with the catheter coating. All patients had good long-term outcomes.
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