R. K. Winkelmann

Atypical lipoma, atypical intramuscular lipoma, and well differentiated retroperitoneal liposarcoma. A reappraisal of 30 cases formerly classified as well differentiated liposarcoma

Thirty cases formerly diagnosed as Grade I lipogenic liposarcoma (well differentiated liposarcoma) were reviewed. The basic histologic pattern in all was that of adult fat modified by the presence of cells with enlarged, hyperchromatic nuclei; in most cases there was also a component of myxoid and/or fibrous tissue. The length of follow‐up ranged from two to 30 years. Nine of the tumors were located in the subcutaneous layer. None of these recurred after excision, not even those which were simply “shelled out,” and none metastasized. The term “atypical lipoma” is proposed for this group. Thirteen were located within or between muscles of the limbs, limb girdles, and head and neck. Nine of these recurred at least once, but there were no metastases and no deaths due to tumor. These were designated “atypical intramuscular lipoma.” The remaining eight originated in the retroperitoneum. Although none of these patients developed metastases, five suffered inoperable recurrence and three died as a result of the neoplasm. It is suggested that the term “well differentiated retroperitoneal liposarcoma” be retained for cases of this type. Cancer 43:574–584, 1979.

Necrobiotic xanthogranuloma with paraproteinemia

Eight patients had multiple xanthomatous plaques and subcutaneous nodules that had a predilection for the periorbital area, flexures, and trunk and that tended to ulcerate. Skin biopsy specimens showed a combination of xanthogranulomatous nodules with necrobiosis. All patients had an accompanying dysproteinemia, which was a monoclonal IgG paraprotein in six. Hyperlipidemia, low serum complement, and cryoglobulinemia were variable features. Five patients had leukopenia. Bone marrow examination showed myeloma in two patients, a lymphoproliferative process in one, and some atypical plasma cells in two. Cutaneous necrobiotic xanthogranuloma is a distinctive histologic pattern most frequently related to plasma cell dyscrasias, and it should be distinguished from normolipemic plane xanthoma and other necrobiotic granulomas.

Diseases Caused by Bacteria

Gonorrhea is an infectious bacterial disease, which predominantly affects the urogenital mucous membranes, caused by Neisseria gonorrhoeae (gonococcus). Gonorrhea attacks the urogenital organs in men and women. Disease of the sexual organs is considerably dangerous, because during this process, through adhesion and scarring of the epididymis or Fallopian tubes caused by inflammation, permanent sterility may occur.

Differential diagnosis of malignant and benign cutaneous lymphoid infiltrates. A study of 57 cases in which malignant lymphoma had been diagnosed or suspected in the skin

Clinical and histopathologic findings were reviewed in 57 cases in which there had been a diagnosis or suspicion of malignant lymphoma in the skin other than mycosis fungoides. Patients were included only if 1) extracutaneous tissue satisfactory for lymphoma classification was available, or 2) there was no evidence of extracutaneous involvement after a minimum of five years. Thirty‐five patients had systemic lymphoma; in eight of these that disease first became manifest in the skin. There were two patients with typical lymphomatoid papulosis and one with a somewhat similar but solitary lesion which did not recur after excision; we have termed the latter a “solitary lymphomatoid papule.” The remaining 19 cases without extracutaneous involvement were regarded as benign lymphoid hyperplasia. Clinically, the cutaneous lesions of malignant lymphoma were difficult to distinguish from those of lymphoid hyperplasia; only lymphomatoid papulosiswas distinctive. Histologically, it was found that a definite diagnosis of malignant lymphoma (other than mycosis fungoides) could be made in the skin when three criteria were fulfilled: 1) exclusive presence or marked preponderance of medium‐sized or larger lymphocytes, 2) absence of preferential involvement of the upper dermis, and 3) absence of extensive epidermal infiltration. These criteria were met in 21 cases of lymphoma. In both cases of lymphomatoid papulosis, the infiltrate was predominantly composed of large lymphocytes but was primarily limited to the upper dermis. The “solitary lymphomatoid papule” contained a similar cell population and involved the full thickness of the dermis; however, it displayed a prominent intraepidermal component. Fourteen cases of malignant lymphoma and all cases of lymphoid hyperplasia were characterized by a mixture of lymphocytes of varying size or a predominance of small lymphocytes. Nine cases of lymphoid hyperplasia exhibited at least one of three findings not observed in lymphoma; these findings were 1) germinal centers, 2) marked paucity of medium‐sized lymphocytes in comparison to both small and large lymphocytes, and 3) sharp margination between adjacent groups of small and large lymphocytes. Aside from one case of small lymphocytic lymphoma which showed deep subcutaneous extension, the remaining cases (13 malignant and 10 benign) could not be reliably differentiated from each other by skin biopsy findings alone and were thus considered histologically indeterminate. The 13 lymphoma cases in this group were all of the small lymphocytic or follicular types; the failure of follicular lymphoma to exhibit a follicular pattern in the skin was a major impediment to its recognition in that location. The cutaneous infiltrates in all cases of large lymphocytic, Burkitts, and lymphoblastic lymphoma were diagnostic of malignancy. Cancer 44:699‐717, 1979.

The clinicopathologic spectrum of lymphomatoid papulosis: study of 31 cases.

Herein we review the Mayo Clinic experience with thirty-one cases of lymphomatoid papulosis seen since 1965. All patients had chronic, recurrent, and self-healing erythematous papulonodular lesions, which often became pustular, ulcerated, and resolved with scarring. The clinical features often corresponded to those seen in Mucha-Habermann disease; however, the predominant histopathologic feature was an infiltrate composed primarily of atypical lymphoid cells suggestive of malignant lymphoma. In six patients, a lymphoproliferative disorder was eventually diagnosed. There were two cases of mycosis fungoides (stage I), one case of nodular sclerosing Hodgkins disease, and three cases of malignant lymphoma--one diffuse mixed large and small cell type with features of T-immunoblastic type, one diffuse large cell type, and one follicular small cleaved cell type. The clinical course of the lymphomatoid papulosis was unaffected by chemotherapy for the lymphoproliferative disorder. Our data indicate that, with sufficient duration of follow-up, malignant lymphoma may develop in some patients with lymphomatoid papulosis.

Skin lesions associated with circulating lupus anticoagulant

This retrospective study of 295 patients extends the earlier findings of an association between lupus anticoagulation and thrombosis by demonstrating the occurrence of cutaneous lesions related to the coagulation defect in 70 patients. The most frequent cutaneous associations were thrombophlebitis, skin ulcer, gangrene, haemorrhage, and cutaneous necrosis. Of the patients with skin lesions, 41% had the skin lesion as the first sign of the disease. It is important to recognize this association because nearly 40% of the patients with skin lesions have multisystem thrombotic phenomena in the course of the disease.

The clinical and histopathologic spectrums of urticarial vasculitis: Study of forty cases

Urticarial skin lesions may occur in patients as a manifestation of necrotizing vasculitis. We describe a series of forty patients with idiopathic chronic urticaria and histologic features of necrotizing vasculitis. On the basis of clinical evaluation, we have classified urticarial vasculitis into two major groups: (1) hypocomplementemic (sixteen patients, ten of whom had evidence of renal disease) and (2) normocomplementemic (twelve patients with systemic disease and twelve with only cutaneous involvement). Most patients with hypocomplementemia presented with arthritis, and some had abdominal pain or airway compromise. Although patients with normocomplementemia and systemic disease had a less severe clinical course, four exhibited renal disease that was characterized by microhematuria and proteinuria. Direct immunofluorescence microscopy of the skin aids in assessing renal involvement in some cases of hypocomplementemic urticarial vasculitis, particularly when IgG and IgM are deposited at the basement membrane. There seems to be a spectrum of disease in urticarial vasculitis, ranging from benign cutaneous lesions to systemic disease.

Angiolymphoid hyperplasia with eosinophilia in the skin

Angiolymphoid hyperplasia may present varied proliferative and inflammatory histologic patterns, which explains much of the previously confusing nomenclature. Ultrastructural and histochemical studies revealed the histiocytic qualities of the endothelial cell that constitutes much of the tumor. A similar transitional histiocytic endothelial cell with surrounding basal lamina, which contained massed cytoplasmic filaments suggestive of myofilaments, was observed alone or in groups. We believe that both the vascular and the histiocytic qualities of this cell population are characteristic and are helpful in diagnosis.

Circulating Sézary cells in hospitalized dermatology patients

Sézary cells in the circulation were counted daily in 14 hospitalized dermatology patients: six with contact dermatitis, five with atopic dermatitis, and three with exfoliative psoriasis. All had circulating Sézary cells, but absolute counts of Sézary cells of more than 1000/mm3 appeared to correlate with a severe refractory course in two patients. It is probable that low, intermittent levels of circulating Sézary cells accompany many inflammatory dermatoses, and it is reasonable that monitoring this cell population may aid in anticipating more consequential skin disease.

Histopathology of necrobiotic xanthogranuloma with paraproteinemia

Fifty‐three cutaneous biopsies from 22 patients with necrobiotic xanthogranuloma (NXG) were reviewed. One or more biopsies from each patient displayed a typical pattern of palisading histiocytic xanthogranuloma with bands of hyaline necrobiosis. Multiple foam cells and Touton giant cells were present, and atypical, bizarre‐appearing foreign body giant cells were characteristic. Cholesterol clefts were found in 18 specimens, and lymphoid nodules were found in 24, 8 of which had germinal center formation. Twenty‐one specimens contained foci of plasma cells, and these were located perivascularly, at the periphery of lymphoid nodules, and, as infiltrates, between dermal collagen bundles. Unique features were xanthogranulomatous panniculitis, often appearing as Touton cell panniculitis, and a rare but distinctive palisading cholesterol cleft granuloma. The cytoplasm of giant cells and histiocytes contained PAS‐positive, diastase‐resistant polysaccharide. Examination of 3 cases by electron microscopy revealed dendritic cells in the epidermis and vacuolated dermal histiocytes. S‐100 antibody was negative in 3 cases. Leukocyte monoclonal antibody studies in 6 patients showed predominantly T‐helper lymphocytes within the granuloma. NXG is a T‐helper cell, non‐X histiocytic granuloma with a distinctive histopathology.