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We report the case of a solitary ulcerating lesion on the elbow of a 32-year-old man. Routine histopathological examination strongly suggested a histiocytic malignancy. However, electron-microscopical, enzyme-cytochemical, and immunological studies revealed that the "tumor" cells were T lymphocytes with an unusual (Leu 1+, Leu 3a+, Leu 4+, Leu 5b+, OKT4+, HLA-DR+, Ki-1+, Leu MI+) immunological phenotype and an even more uncommon morphology characterized by the development of giant multivesicular bodies giving some cells a signet-ring cell appearance, and autophagocytosis. The lesion healed spontaneously, notwithstanding its malignant histology.

We report a case of primary cutaneous lymphoma, of the lymphoplasmacytoid type (immunocytoma), in which a small neoplastic component was obscured by a dominating reaction exhibiting characteristic features of lymphadenosis benigna cutis. This abnormal cell population was identified because of the unusual cytomorphology of the tumor cells, which showed deeply indented nuclei in combination with a distinctly plasmacytic cytoplasm. Monoclonality was revealed by the cytoplasmic positively of the tumor cells for lambda chains only. This case strongly suggests that in at least a number of cases of lymphadenosis benigna cutis, a low-grade malignant lymphoma may be present.

Bullous lesions on the extremities of people with diabetes are known as bullosis diabeticorum. A new case is described with lesions restricted to the hands, and the literature is reviewed.

The histologic and immunophenotypical features of 20 primary cutaneous large-cell lymphomas of T-cell origin were investigated and correlated with clinical data to obtain prognostically relevant criteria. Histologic evaluation, using the updated Kiel classification, showed that these large-cell lymphomas represent a morphologic spectrum, often making classification rather arbitrary. It is therefore concluded that the clinical relevance of histologic subtyping is limited for this group of lymphomas. Immunophenotypical studies revealed significant differences between CD30-positive and CD30-negative lymphomas. CD30-positive lymphomas generally presented with localized skin disease, and had a favorable prognosis (9 of 10 patients alive and in complete remission; median survival, 37 months). In contrast, CD30-negative lymphomas often presented with or rapidly developed generalized disease; all patients died of lymphoma (median survival, 17 months). These findings suggest that CD30 expression is an important prognostic parameter for this group of primary cutaneous large-cell lymphomas.

The abnormal cell population in lymphomatoid papulosis was studied by immunohistochemistry, light and electron microscopy in five cases. It resulted in a comprehensive description of all the main variants within the abnormal cell population. In one of the cases an irreversible intracytoplasmic process in the abnormal lymphocytes made it possible to demonstrate the derivation of lymphomatoid cells with cerebriform nuclei resembling the mycosis cells in mycosis fungoides from large histiocyte-like cells resembling the Reed-Sternberg cells in Hodgkin's disease. Variable numbers of the abnormal cells expressed Ki-1 reactivity in all four cases tested and T-cell associated antigens in two cases.

Two patients with clinical manifestations of Zézary syndrome are reported. In both cases from an early stage of the disease in addition to characteristic Sézary cells large numbers of immunoblasts were present in skin lesions and peripheral lymph nodes and in one case also in the blood. Their relationship to the characteristic Sézary cells was shown by morphological, cytochemical and immunological methods. The infiltrates in the skin were epidermotropic in one case and nonepidermotropic in the other. Lymph node structure was effaced by diffuse infiltration of abnormal lymphoid cells. These were found to proliferate in the skin as well as in lymph nodes. Cytogenetical studies of blood lymphocytes indicated an abnormal hypodiploid clone in both cases. Immunologically the tumour cells had properties of peripheral T-lymphocytes but whereas all abnormal cells exhibited inducer/helper cell characteristics in one case, only a minority of the lymphocytes revealed these characteristics in the other case. In this case the tumour cell population changed into a more pleomorphic type. The classification of the cases is discussed.

Skin sections of clinically involved and clinically normal-looking skin from patients with atopic dermatitis were incubated with anti-human IgE antibodies using the indirect immunoperoxidase technique. Apart from positive dermal anti-IgE staining, positive epidermal anti-IgE staining was also observed. The morphology of the epidermal staining cells suggested the involvement of dendritic cells. This was confirmed by positive immuno-double labelling with OKT6 and anti-IgE. This phenomenon seemed to be specific for atopic dermatitis since skin sections from normal non-atopic controls, patients with allergic asthma, contact dermatitis, and schistosomiasis showed no epidermal anti-IgE staining. To further elucidate the nature of the epidermal anti-IgE staining cells, epidermal cell suspensions were prepared from clinically involved skin from patients with atopic dermatitis. These cell suspensions also showed positive anti-IgE staining cells and positive immuno-double labelling with OKT6 and anti-IgE. Immunogold electron microscopy with anti-IgE on epidermal cell suspensions from patients with atopic dermatitis showed gold particles on the cell membranes of cells containing Birbeck granules, being Langerhans' cells. Epidermal cell suspensions from normal non-atopic controls were negative. The presence of IgE molecules on epidermal Langerhans' cells, which seems to be specific for patients with atopic dermatitis, provides an explanation for the high frequency of positive patch test reactions to inhalant allergens.

A retrospective study in 9 patients with Zoon's balanoposthitis was done to determine the immunoglobulin class distribution in the plasma cellular infiltrate. This was carried out on paraffin-embedded specimens using an unlabelled antibody peroxidase-antiperoxidase method. In all lesions IgG-producing plasma cells predominated. IgA- and IgD-positive cells were also present in about equal quantities but fewer than IgG. IgM-positive cells were either absent or present in very low numbers. Especially in erosive lesions a high number of plasma cells was noticed. The kappa/lambda ratio was variable with a preference for lambda in 5 of 8 cases. These findings are suggestive of a nonspecific polyclonal stimulation of B cells, which might be caused by a persistent infection. The above-mentioned findings were compared with premalignant and malignant skin lesions in which a major amount of plasma cells was present in the inflammatory infiltrate. Comparable results were obtained with slight variations. The role of plasma cells in these infiltrates is discussed.

A possibly new type of T cell pseudolymphoma is described in three cases. It presented as a small, solitary lesion on the trunk of 3 to 5 months' duration. It was slightly elevated, round, and erythematous, with a diameter of 1 to 2 cm. It showed a tendency to regress spontaneously. In sharp contrast to its benign clinical appearance and biologic behavior, a distinct malignant histopathologic picture was observed, however. It was characterized by a nonepidermotropic bandlike subepidermal infiltrate dominated by large T lymphocytes with cerebriform nuclei identical in morphologic and immunologic characteristics to the abnormal cellular component of cutaneous T-cell lymphomas.

A report is given on two male patients who showed all the main characteristics of Sézary syndrome (SS). When phototested, however, they proved to be extremely photosensitive, which suggested a diagnosis of actinic reticuloid (AR). This was supported by the predominance in blood and skin specimens of lymphoid cells with a suppressor/cytotoxic phenotype, the absence of clonal cell proliferation and a benign clinical course. Differential diagnostic problems of SS and erythrodermic AR are discussed.

This report describes the clinical, histologic, and immunologic characteristics of 16 diffuse large cell lymphomas of follicular center cell origin with only skin lesions at presentation. These patients presented with nodular and tumorous skin lesions, which in 10 of 16 cases were confined to a circumscribed area on the trunk. Four patients, all elderly women, presented with skin tumors on the lower legs. Histologically, these 16 lymphomas showed nonepidermotropic diffuse dermal infiltrates, mainly consisting of large follicular center cells, with a variable admixture of small cleaved cells, immunoblasts, T-lymphocytes, and macrophages. The relative numbers of large cleaved and large noncleaved cells, respectively, varied considerably in these lymphomas. Immunophenotypically, almost all lymphomas expressed monotypic surface immunoglobulins and HLA-DR antigens, whereas all lymphomas were reactive with B-cell-associated monoclonal antisera B1, Leu-14, and/or To15. Three of four elderly female patients presenting with disease on the lower legs died. Of the 12 other patients, 11 are currently alive and in complete remission, which suggests a favorable prognosis for this type of cutaneous large cell lymphoma.

A male patient with classical neurofibromatosis is reported. Examination of the other family members revealed 2 patients with solitary cutaneous neurofibromas and 2 with one or more Lisch nodules without other cutaneous or systemic signs of neurofibromatosis. This suggests that Lisch nodules can be a marker for the neurofibromatosis gene in otherwise unaffected family members.

2 middle-aged men are described with slowly progressive papular and nodular lesions, limited to the skin of the back. Clinically and histologically both resembled Crosti's reticulosis ('réticulo-histiocytome du dos de l'adulte'). It was determined by electron microscopic, enzyme cytochemical and immunological studies that the 'histiocyte' component, which was derived from T lymphocytes, consisted of blast-like cells with characteristic multilobated nuclei with nucleoli in marginal position. For this type of lymphoma, which may have been described previously as lymphocytoma cutis or reticulum cell sarcoma, the term 'multilobated cutaneous T cell lymphoma' is proposed. According to its slowly progressive clinical course it may be classified as a malignant lymphoma of low grade malignancy.

We report a detailed clinical, histopathologic, and photobiologic study of 16 Dutch patients with actinic reticuloid. All were middle-aged or elderly men who had persistent plaques on light-exposed skin only (two patients), extension of lesions to nonexposed areas (four patients), or prolonged or persistent episodes of erythroderma (10 patients). They were sensitive to UVB, UVA, and visible light. In 10 of 13 patients tested, the dermal infiltrate contained predominantly suppressor T cells. Many also had a reversed helper-suppressor T cell ratio. Circulating lymphocytes with deeply indented nuclei were present in all but were most pronounced in the most photosensitive erythrodermic patients. Tolerance induction therapy with UVB irradiation produced an excellent or good response in 13 of 15 patients. One patient responded to cyclosporine therapy.

An unusual annular erythema occurred in a 6-month-old girl. Recurrent annular lesions appeared on the face and back, but annular lesions persisted for ten months on the distal extremities. Eleven months after the eruption appeared, it resolved completely without recurrence. This seemingly unique condition is compared with other cases of chronic annular erythemas, especially those previously described as erythema gyratum perstans.

The case of a male diabetic patient with recurrent bullous lesions restricted to the hands is reported. Areas of spongiosis in the epidermis, as well as subepidermal blister formation, were observed in repeated biopsies. On electron microscopy, separation was seen at the level of the lamina lucida. No immunopathology was observed in involved skin. A review of forty-four reported cases of bullosis diabeticorum is given.

Monoclonal antibodies to human T-cell membrane antigens were used to evaluate the immunological phenotype of the tumour cells in two cases of a recently described cutaneous T-cell lymphoma of 'multilobated' type. In one case the distinctive abnormal blast cells expressed positivity for OKT6, anti-HTAI, OKT4 and Leu-3a, and showed binding capacity for peanut agglutinin (PNA). These are properties normally displayed by immature cortical thymocytes. The antigen expression of the abnormal blast cells in the other case was OKT3, Leu-I, OKT4, Leu-3a in the absence of PNA-binding; these properties bear more resemblance to more mature T-inducer/helper cells. When combined with consideration of the morphological aspects we suggest that in both cases the abnormal cells represented precursor T-lymphocytes.

B cells formed a distinctive component (greater than 5%) of the infiltrates in specimens of 28% (n = 12) of the 43 cases of mycosis fungoides studied. A large majority expressed surface immunoglobulin (Ig) D and/or IgM and a small minority IgA, similar to the proportions in inflammatory infiltrates. There was a prevalence of lambda light-chain expression. In about half of these cases, specimens revealed the formation of B cell follicles at various stages of development. Plasma cells formed a substantial number (greater than 25/mm2) in specimens from 21% (n = 9) of the patients. In six of these cases, including all four cases with advanced follicle formation, the same specimens were also rich in B lymphocytes. In most of the specimens the varying majorities of IgA or IgG producing cells and the polytypical expression of kappa and lambda light chains were similar to the patterns in inflammatory disorders. Two cases showed a striking prevalence of IgG, kappa-positive plasma cells, which was present in multiple specimens of one case and was associated with atypical plasmacytoid cells in a tumor of the other case. The pertinence of these observations for a special relationship between the abnormal T cells and the B cell component is discussed.

Three cases of a new type of lymphoma of the skin are described. Clinical manifestations were the development of papules, nodules and tumours which slowly progressed in size and extent in one region of the skin of elderly men. Dissemination to a regional lymph node occurred in only one. Histopathologically the lesions demonstrated a variably perivascular or diffuse dermal infiltrate often with a nodular configuration and sparing the epidermis and a clear subepidermal zone. The infiltrates were dominated by abnormal lymphoid cells with irregular nuclei and blast cells exhibiting a characteristic combination of multilobated nuclei with marginal nucleoli. By immunological and enzyme cytochemical methods the cells were identified as T lymphocytes. It is suggested that the cases described represent different parts of spectrum running from lymphocytoma cutis-like conditions to disseminated malignant lymphoma.

Skin biopsy specimens from 40 patients with mycosis fungoides (MF) were examined by a selective procedure for the assessment of immunophenotypic deviation. In 19 cases (48%), aberrant phenotypes were detected. Type and degree of aberration differed greatly without apparent clinicopathologic correlation. Different phenotypes in the same patient occurring either simultaneously or during the course of the diseases were observed. Phenotypic aberration showed a statistically significant correlation with tumor stage MF, the large cerebriform tumor cell type, blast cell transformation, and diffuse dermal infiltration. In combination with longer-existing disease, more lymph node and visceral involvement and a higher mortality rate of MF in the aberrant group indicated an association with advanced disease. Its prognostic relevance for the individual patient, however, seemed limited because of the association of aberration and a long-lasting indolent course in many other patients. The assessment of phenotypic aberration formed a valuable contribution to the diagnosis of MF, especially in cases with little cellular atypia.

Jessner's lymphocytic infiltration of the skin is a well-known but poorly understood disorder. Some doubt still exists about whether it is a single entity or a heterogeneous group that can pass into polymorphous light eruption, discoid lupus erythematosus, or even malignant lymphoma. Therefore, a large number of patients with lymphocytic infiltration of the skin (N = 100; 46 male, 54 female) were examined to elucidate these questions. We conclude that lymphocytic infiltration of the skin is a single entity. Progression into polymorphous light eruption, discoid lupus erythematosus, or lymphoma was not observed. However, this study shows that lymphocytic infiltration of the skin and polymorphous light eruption cases occur simultaneously in 1 patient. In this study the cases of 10 patients with this combination are reported. An effective but harmless therapy is yet unknown. Intermittent use of topical steroids can be useful but is not effective in many patients.

A case of a congenital 'bronchogenic' cyst of the skin over the right scapula of a 4-year-old boy is reported. The continuity of its epithelial lining with the epidermis and its appendages is stressed and the consequences of this relationship for current theories about the pathogenesis of cutaneous ciliated cysts are discussed.