The clinical and histological spectrum of lymphomatoid papulosis

A review of sixty-four skin biopsies from sixteen patients with lymphomatoid papulosis revealed two characteristic histological types (type A and type B), which also had a different clinical behaviour. In lymphomatoid papulosis type A lesions, four histological patterns corresponding with the age of the lesion could be distinguished. Such a relationship was not found in type B lesions. The finding of transitional forms in some biopsy specimens, showing histological features of both type A and type B, and the presence of both types in different but concurrent lesions, suggests that these two types are not different entities but rather represent the ends of a spectrum. At least two different populations of atypical cells can be distinguished in lymphomatoid papulosis. Apart from the atypical cerebriform mononuclear cells, which are T-lymphocytic in origin and predominant in type B lesions, large atypical cells with vesicular nuclei, prominent nucleoti and abundant cytoplasm are found, particularly in type A lesions. Preliminary immunohistochemical and cytochemical investigations suggest that these cells are not lymphoid in origin, but are related to the Langerhans cell series.     

Familial Jessner's lymphocytic infiltration of the skin, occurring in a father and daughter

Familial occurrence of Jessner's lymphocytic infiltration of the skin is reported in a father and daughter. A definitive diagnosis of Jessner's disease was greatly facilitated by the presence of large numbers of plasmacytoid monocytes within the dermal infiltrates in both patients. A role for skin-directed lymphocyte migration is suggested to account for the presence of this peculiar cell type.     

Sézary''s syndrome: a clinical evaluation of eight patients

Clinical and laboratory investigations of 8 patients with Sézary's syndrome are presented. Sézary cells were found in peripheral blood as well as in bone marrow and lymph nodes. The overall follow-up period was 8 years. Different therapeutic regimens were given. The best results were obtained with chlorambucil and prednisone orally, combined with topical application of nitrogen mustard. Three patients died of pneumonia and septicaemia.     

Immunophenotyping on simultaneously occurring plaques and tumours in mycosis fungoides and Sézary syndrome

Skin biopsy specimens from 15 patients with mycosis fungoides and Sézary syndrome, with simultaneously occurring plaques and tumours, were examined to assess phenotypic deviation. We focused on immunophenotypic differences between the two types of lesions with respect to the T-cell markers CD2, CD3, CD4, CD5 and CD8. In six patients (40%) loss of one or more T-cell markers occurred in at least one of the lesions. Three of the patients studied (20%) showed a difference in immunophenotype between plaques and tumours, with an additional loss of one of the T-cell markers in the tumours (respectively, CD5, CD2 and CD4). All three of these patients showed a larger number of blast cells in the tumour compared with the plaque. No correlation between this loss of antigenicity and the prognosis was observed. The results of this study show that different immunophenotypes can occur simultaneously in an individual patient. Furthermore, we were able to confirm a relationship between the number of intraepidermal CD1⁺ cells in plaque lesions and the prognosis.     

Cytodiagnostic use of Feulgen-DNA measurements in cell imprints from the skin of patients with mycosis fungoides

In the present immunofluorescence study we report the occurrence of fibrin or related substances and of IgM in the upper part of the dermis in all forty lichen planus lesions examined. Different amounts of various complement components and fragments were located in the epidermal basement zone, and in the colloid bodies. IgG, IgM, IgA and fibrin were also found in the colloid bodies, sometimes together with albumin. The earliest pathological change, which was seen in perilesional skin and in clinically normal skin from sites of predilection, was the presence of colloid bodies and pigment containing macrophages in the upper dermis. Deposition of fibrin appeared to be another early sign, as it was discernible in areas devoid of inflammatory infiltrate. The possible significance of these findings is discussed.     

Actinic reticuloid: immunohistochemical analysis of the cutaneous infiltrate in 13 patients

An immunohistological study on cryostat sections of skin biopsies in 13 patients with actinic reticuloid has been performed using a panel of 21 monoclonal antibodies against lymphoid and non-lymphoid infiltrate cells. The infiltrate consisted of activated T cells, numerous histiocytes and macrophages, and small numbers of B cells. In 10 out of 13 patients the majority of the lymphocytes expressed the phenotype of suppressor cells. The number of Leu 8+ cells was inversely proportional to HLA-DR expression by the dermal infiltrate, which suggested a negative correlation between a state of activation and the concentration of Leu 8+ cells. There was a striking number of IgE bearing dendritic cells in the dermis associated with elevated serum IgE levels.     

Cutaneous T-cell lymphoma: clinicopathological relationships, therapy and survival in ninety-two patients

Clinicopathological findings, methods of treatment and survival in ninety-two patients with cutaneous T-cell lymphoma (CTCL) (eighty-five mycosis fungoides and seven Sezary syndrome), seen in the Leiden University Hospital between 1974 and 1980, are reported. All patients were staged and treatment was given accordingly. Patients without signs of extracutaneous disease (74%) were treated with either total-skin electron-beam irradiation (E beam), topical mechlorethamine (HN₂) or PUVA. Topical HN₂ and E beam had an equal effect on survival. Electron-beam induced a higher number of initial complete remissions than HN₂, but fewer patients relapsed while on maintenance treatment with HN₂. In the patients with lymph node and/or visceral involvement, E beam followed by systemic chemotherapy—cyclophosphamide, vincristin (Oncovin) and prednisone (COP)—gave better results than topical therapy or COP alone. Survival was influenced most by the type of skin lesion, the presence or absence of lymph node and/or visceral involvement, and the ability to induce a complete remission initially.     

Total skin electron beam irradiation for cutaneous T-cell lymphoma (mycosis fungoides)

Forty patients with the cutaneous T-cell lymphoma (mycosis fungoides) were treated with total skin electron beam irradiation. The total follow-up period was up to 116 months (median 57.5 months). The patients were irradiated with a total dose of 35 Gy over 10 weeks, using the six-field technique. Ten of these patients had lymph node involvement and were subsequently treated with chemotherapy. After the initial electron irradiation complete remission of the skin lesions was obtained in 87.5% of the patients. Relapse of skin lesions occurred in 52% of the patients after 2-72 months (median 4 months). Second line therapy consisted primarily of topical nitrogen mustard. The overall survival rate at 5 years was 70%. Despite the side-effects this treatment was tolerated well by all patients.     

Non-HIV-related Kaposi's sarcoma suppressed by dapsone

Summary Two patients with a history of slowly progressive non-HIV-related Kaposi's sarcoma were successfully treated with dapsone.