Atypical fibroxanthoma. An unusual variant with osteoclast-like giant cells.

We report three cases of an unusual variant of atypical fibroxanthoma with numerous multinucleated osteoclast-like giant cells. Histologically, the lesions were composed of uniformly dispersed multinucleated osteoclast-like giant cells within a pleomorphic cellular proliferation with microscopic and histochemical features of atypical fibroxanthoma. Multinucleated osteoclast-like giant cells, however, stained with KP-1, a macrophage-associated antigen only. These cells are not necessarily an integral part of the tumor and may represent a reactive histiocytic component.

Aggressive digital papillary adenocarcinoma (aggressive digital papillary adenoma and adenocarcinoma revisited).

In 1987 a clinicopathologic study by the Armed Forces Institute of Pathology (AFIP) of rare sweat gland tumors, termed aggressive digital papillary adenoma and adenocarcinoma, was published. Since that time, the AFIP has continued to collect these tumors for study. Based on additional follow-up data, we think the original classification of these tumors requires revision. Sixty-seven cases of aggressive digital papillary adenoma and adenocarcinoma were studied according to their clinical characteristics and histologic features. Fifty of these were originally diagnosed as adenoma and 17 as adenocarcinoma. Follow up on 45 (67%) of the patients was obtained. None of the clinical or histologic parameters studied were found to be predictive of recurrence or metastasis, indicating that the originally proposed criteria for distinguishing between benign (adenoma) and malignant (adenocarcinoma) do not predict biologic behavior. When primary tumors were treated by subsequent reexcision or amputation, only one recurred (5%), when not so treated, 11 recurred (50%) regardless of the original diagnosis (p <0.05). Metastasis occurred in six (14%) cases and in three cases led to the death of the patient. Three of these metastatic cases had met the earlier criteria for adenoma. Pulmonary metastases were observed in five cases. No effective treatment for widespread metastatic disease has yet been developed. Because histologic features with prognostic significance could not be demonstrated in this retrospective review, we propose that all aggressive digital papillary tumors be designated aggressive digital papillary adenocarcinoma.

Spindle cell and epithelioid cell nevi with atypia and metastasis (malignant Spitz nevus).

We report on the clinical and pathologic features of 32 lesions diagnosed as malignant spindle cell and epithelioid cell nevus (S&E nevus). Because of the clinical or initial histopathologic diagnosis of malignant melanoma, six patients had lymph node dissection. Three of these patients also had an enlarged lymph node. In all six cases, metastatic spindle or epithelioid cells were found in at least one of the resected lymph nodes. Of the 30 patients with follow-up information, including all six patients with lymph node metastases, all are alive and well. No recurrences or further metastases have been found. On histopathologic reevaluation, all the lesions had features of S&E nevi. Study of these cases suggests that although some lesions with features of S&E nevi may involve local lymph nodes, widespread metastases do not result.

Acquired dyskeratotic leukoplakia.

A 36-year-old woman developed progressive dyskeratotic leukoplakia of the palate, lips, attached gingiva, and labial surfaces of the genitalia. On microscopic examination, clusters of dyskeratotic cells were found throughout the epithelium, except in the basal cell layer. The clinical and histologic differential diagnosis included candidiasis, oral hairy leukoplakia, lichen planus, condyloma acuminatum, Darier's disease, hereditary benign intraepithelial dyskeratosis, and white sponge nevus. Her clinical and histologic findings do not permit classification into any of these well-recognized entities.

Microcystic adnexal carcinoma. Report of a case with 30-year follow-up.

Microcystic adnexal carcinoma is a recently described neoplasm characterized by a locally aggressive growth pattern. The tumor usually affects the upper lip of middle-aged women, but occurs in other facial areas and in men. Histologically, a distinctive combination of keratin-filled cysts, islands and strands of basaloid and squamous cells, and ducts and glandlike structures is seen. A dense, hyalinized stroma is also a feature as well as frequent perineural invasion by tumor cells. While microcystic adnexal carcinoma is deeply infiltrating in its growth, metastasis has not been reported. However, recurrences with extensive local tumor spread are often observed. We describe a middle-aged man who initially had a lesion of microcystic adnexal carcinoma excised 30 years prior to definitive treatment for a recurrence at the original site. To our knowledge, this is the longest follow-up of a patient with microcystic adnexal carcinoma and confirms its locally infiltrating, indolent biologic behavior.

Localized eosinophilic fasciitis.

Two patients developed an erythematous, rock-hard induration of their forearms after strenuous physical activity. Peripheral eosinophilia was noted in one patient, but hypergammaglobulinemia was absent. Systemic corticosteroid treatment resulted in remarkable involution of the process in one patient, while the other experienced spontaneous involution. The histologic changes were those of eosinophilic fasciitis.

Pigmented breast carcinoma. A clinical and histopathologic simulator of malignant melanoma.

A case of infiltrating breast carcinoma presenting as a pigmented lesion of the areola and nipple is described. The pigmentation was found to be primarily due to interspersed melanocytes and melanophages within the tumor. The histologic criteria and the differential immunohistochemical staining characteristics of melanoma and breast carcinoma are presented. Also emphasized is the difficulty in differentiating pagetoid malignant melanoma and epidermotrophic breast carcinoma.

Rheumatoid neutrophilic dermatitis.

We recently examined a patient with severe rheumatoid arthritis who presented clinically with fixed urticaria-like plaques. Histologic examination revealed a dense neutrophilic dermal infiltrate without associated vasculitis. On clinical and histologic grounds, this case differs from other noninfectious neutrophilic inflammatory disorders. We present a case of rheumatoid neutrophilic dermatitis and review the salient features of this unique disorder.

Oral hairy leukoplakia. A distinctive marker of human T-cell lymphotropic virus type III (HTLV-III) infection.

Oral hairy leukoplakia (HL) is a newly described lesion occurring principally on the lateral borders of the tongue in immunosuppressed homosexual men infected with human T-cell lymphotropic virus type III (HTLV-III). Clinically, HL appears as a slightly raised, poorly demarcated lesion with a corrugated or "hairy" surface. Histologically, the lesion is characterized by keratin projections on the surface (which often resemble hairs), parakeratosis, and acanthosis. In addition, large pale-staining cells with pyknotic nuclei are seen in the upper stratum malpighii, which appear similar to the koilocytes described in uterine condylomata. Candida organisms are frequently observed on the lesion surface. Little, if any, subepithelial inflammation is present. Human papillomavirus and Epstein-Barr virus have been identified in biopsy specimens from lesions of oral HL. The association of this lesion in patients with HTLV-III infection has been established. We saw a patient with HTLV-III infection and HL, in whom the immunochemical and ultrastructural findings revealed the presence of a mixed viral infection. Because oral HL may be of diagnostic value as an early indicator of HTLV-III infection, awareness of its characteristic clinical, histologic, immunochemical, and ultrastructural features is important.

Parapoxvirus infections acquired after exposure to wildlife.

The histopathologic and electron microscopic findings in two patients with skin lesions that developed after exposure to deer and other wildlife were consistent with a parapoxviral infection. Human infections that were morphologically similar to Parapoxvirus infection have been previously described concerning exposure to cervids (deer and related animals). Ours are the first reported cases in which viral particles were demonstrated by electron microscopy.

Erythema multiforme and urticaria. Eruptions induced by chemically related ophthalmic anticholinergic agents.

Erythema multiforme developed in an 80-year-old man following the use of scopolamine hydrobromide ophthalmic drops. The erythema multiforme cleared when the medication was discontinued and recurred on challenge. Later, he was given tropicamide, an anticholinergic ophthalmic preparation that, like scopolamine, has a tropic acid residue. Within 15 minutes an immediate hypersensitivity reaction with generalized urticaria developed in the patient.

Cutaneous histopathologic findings in 'antiphospholipid syndrome'. Correlation with disease, including human immunodeficiency virus disease.

The term antiphospholipid syndrome is used to characterize a complex of clinical and pathologic findings mediated by a group of antibodies formed against a family of antiphospholipids. These antiphospholipid antibodies were originally found in patients with lupus erythematosus in whom the partial thromboplastin time was prolonged and in patients with other autoimmune diseases; subsequently, they have been observed in association with a variety of other conditions, including infections, reactions to drugs, malignant neoplasms, human immunodeficiency virus disease, and as an isolated finding. In recent years, there has been some clarification of the significance of the various tests for antiphospholipid antibodies, including the lupus anticoagulant test and the anticardiolipin antibody tests, in predicting the antiphospholipid syndrome. The mechanism of disease, however, has not been well defined. The most common cutaneous lesion seen in seven patients with lupus anticoagulant and anticardiolipin antibody who have the antiphospholipid syndrome was ulceration due to thrombosis of dermal veins and arteries. Often there is a reactive vascular proliferation around the thrombosed vessels. The presence of primary thrombosis of both veins and arteries in thrombotic disorders is unusual and may provide insight into the mechanism of thrombosis in antiphospholipid syndrome.

Cutaneous histopathologic, immunohistochemical, and clinical manifestations in patients with hemophagocytic syndrome. Military Medical Consortium for Applied Retroviral Research (MMCARR).

BACKGROUND AND DESIGN: The hemophagocytic syndrome (HPS) is characterized by fever, wasting, generalized lymphadenopathy, hepatosplenomegaly, and pancytopenia, often with associated coagulopathy. The most common cutaneous manifestations are panniculitis and purpura. Cytophagic histiocytic panniculitis fits within the spectrum of HPS, and the most consistent histopathologic feature in HPS is a proliferation of mature histiocytes that exhibit prominent erythrophagocytosis and cytophagocytosis. The clinical spectrum, the underlying causes, and the histopathologic features found in HPS are broad. The characteristic phagocytic histiocytes seen in HPS have been confused with malignant histiocytes in the past, but are now known to be reactive. The clinical findings, histologic, and immunohistochemical features of 10 cases of HPS with cutaneous lesions were reviewed. Immunohistochemical markers included KP-1, beta F-1, UCHL-1, L-26, MAC-387, factor XIIIa, and S100 protein. RESULTS: The HPS was associated with T-cell lymphoma and/or viral infection. Most biopsy specimens showed edema and hemorrhage with a lymphohistiocytic infiltrate and prominent histiocytic cells showing erythrophagocytosis and, in some cases, cytophagocytosis. The histiocytic cells showed positive reactions for KP-1 and negative reactions for the lymphoid markers. In all cases the lymphoid cells showed a mixed pattern with most cells positive for beta F-1 and UCHL-1, and a small percentage positive for L-26. CONCLUSION: In HPS, the prominent phagocytic histiocytes are reactive and are stimulated by T-cell lymphocytes, either neoplastic or in response to viral infection. Many of the findings in the HPS may also be due directly or indirectly to cytokines produced by proliferating T-cell lymphocytes and/or reactive phagocytic histiocytes.

The recognition of recently described and potentially problematic melanocytic lesions of the skin.

Our attempt has been to examine unusual or problematic melanocytic lesions to enable the reader to recognize them histologically and to enhance the understanding of their biologic significance. An awareness of these lesions by the clinical dermatologist will, it is hoped, result in optimal patient management. The proper identification of melanocytic neoplasms is, and will continue to be, of prime importance to all concerned: the clinician, the dermatopathologist, and especially the patient.

Cutaneous angiosarcomas with a starry-sky pattern.

OBJECTIVE: A subgroup of epithelioid angiosarcomas, which we have identified, has a prominent starry-sky histologic pattern. All these neoplasms were evaluated with routine histopathology and with immunohistochemical stains. Clinical follow-up was also obtained to determine whether these angiosarcomas' had any other distinctive properties. DESIGN: Histopathologic features and immunohistochemical stains, including cytokeratin, epithelial membrane antigen, CD34, LCA, factor VIII-related antigen, KP-1, vimentin, HMB-45, and S100 protein were performed on all four neoplasms. PATIENTS: These angiosarcomas occurred on the extremities of patients with chronic edema as well as on the scalp of elderly patients. MAIN OUTCOME: All patients were dead within 5 years of diagnosis. Of the three patients where angiosarcoma was listed as the cause of death, all were dead within 30 months. RESULTS: All tumors showed a similar immunohistochemical staining pattern. Vimentin, factor VIII-related antigen, and CD34 were positive in all tumors. The remaining immunohistochemical stains including cytokeratin, which may be positive in other epithelioid angiosarcomas, were negative. CONCLUSIONS: We present a distinctive starry-sky histologic pattern in epithelioid angiosarcomas. Knowledge of this pattern may be important in the early diagnosis of this rapidly fatal neoplasm.

Microcystic adnexal carcinoma (sclerosing sweat duct carcinoma).

Microcystic adnexal carcinoma (MAC) is a recently described malignant neoplasm of the adnexal structures of skin that shows a marked propensity for early infiltrative and locally aggressive growth. Its banal clinical appearance may lead to a delay in diagnosing the tumor by biopsy, and its multifaceted histologic features may lead to an incorrect tissue diagnosis. Confusion with benign tumors and less aggressive malignancies can lead to inadequate initial treatment and extensive recurrences. We describe three cases of microcystic adnexal carcinoma and review the clinical and histologic features, treatment, and prognosis of this neoplasm.