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Some research on parapsoriasis and lymphomas.

Thirty-five cases of benign parapsoriasis en plaques, 24 cases of prereticulotic poikiloderma (3 of which were in evolution towards polymorphous lymphomas), 15 cases of lymphoma and 10 cases of other various skin proliferative disorders were studied. For various reasons the first two conditions are preferably indicated as type 1 and type 2 parapsoriasis. Attention is drawn to the possibility of finding a dermal fibro-histiocytary proliferative condition, more often in type 2 parapsoriasis than in type 1. Dysprotidemia, signs of a reactive bone marrow condition, and changes of the tryptophan leads to niacin pathway, as signs of various degrees of damage of connective tissue, were found in type 2 parapsoriasis and lymphomas.

Adult↗

Parapsoriasis and mycosis fungoides: the Northwestern University experience, 1970 to 1985.

One hundred sixty skin biopsy specimens from 89 patients with the clinical diagnosis of large plaque parapsoriasis and 240 specimens from 106 patients with mycosis fungoides were reviewed. Through the use of chart reviews and a retrospective questionnaire, various factors (sex, age, history of eczema/atopy, occupation) were examined in these two patient groups. Mycosis fungoides developed in 30% of the patients in the parapsoriasis group. Nineteen percent of patients in the mycosis fungoides group had worked in industry. Once the clinical diagnosis of mycosis fungoides was considered, an average of four biopsy specimens were needed to establish the diagnosis. The average interval from the initial visit to the diagnosis of mycosis fungoides from examination of biopsy specimens was 22 months. These findings support further the view that large plaque parapsoriasis represents an important precursor of mycosis fungoides. A designation of premycosis fungoides would emphasize this relation more than the term parapsoriasis.

Adolescent↗

Comparative clinicopathological study on pityriasis lichenoides chronica and small plaque parapsoriasis.

The term parapsoriasis refers to a group of chronic asymptomatic scaly dermatoses of unknown etiology about which there is still controversy over the nosology and nomenclature of the different conditions that comprise the group, particularly pityriasis lichenoides chronica (PLC) and small plaque parapsoriasis (SPP). In an attempt to establish the distinctive clinicopathologic features of these two dermatosis, we prospectively studied 44 patients who presented with the typical clinical and histologic picture of either of these two diseases. SPP was clinically characterized by scaly oval plaques on the trunk and proximal aspect of extremities. Spongiosis was the salient histopathologic feature, with absence of fibrosis or melanophages. PLC presented with a scaly papular eruption over the trunk and extremities and histologically was characterized by an interface dermatitis. We conclude that sufficient clinical and histologic features differentiate these two entities and we propose that the term parapsoriasis be used only to designate SPP and large plaque parapsoriasis.

Adolescent↗

T cells and T-cell subsets in mycosis fungoides and parapsoriasis. A study of 18 cases with anti-human T-cell monoclonal antibodies and histochemical techniques.

Skin lesions from 15 patients with mycosis fungoides (MF) and from three with parapsoriasis were studied immunohistochemically with monoclonal antibodies against T cells (Leu 1) and against T-cell subsets (Leu 2a, Leu 3a). Lymphoid cell reactivity was diverse among these sampled cases. In two cases of parapsoriasis and nine of MF, there was a predominance of helper/inducer (Leu-3a-reactive) cells over suppressor/cytotoxic (Leu-2a-reactive) cells. In one case of parapsoriasis and one (advanced tumor stage) of MF, there was suppressor/cytotoxic cell predominance. One case of MF showed strong reactivity for both T-cell subset markers. Four cases of MF (two plaque-stage and two tumor-stage) featured a predominant cell type in the dermis which was nonreactive for all three antibodies. The intraepidermal lymphoid cellularity was Leu-1-reactive in ten cases of MF and two of parapsoriasis. Among these 12 cases, the intraepidermal cellularity was Leu-2a-reactive in four and Leu-3a-reactive in three. The use of such studies of T-cell subsets on in situ cutaneous lymphoid infiltrates may demonstrate a correlation with cytomorphology, clinical stage, and disease prognosis.

Adult↗

Quantitative analysis of lymphoid cells in myocosis fungoides, Sézary's syndrome and parapsoriasis en plaques.

Semi-thin sections are superior to paraffin sections for differentiating lymphoid cells. To test their possible significance in the diagnosis of cutaneous T-cell lymphomas, the following investigation was made: A quantitative analysis of lymphoid cells in the cellular infiltrate of cutaneous lesions of mycosis fungoides (11 cases), Sézary's syndrome (9 cases), and parapsoriasis en plaques (14 cases) was made using semi-thin sections of epon-embedded biopsies. As controls biopsies of patch test reactions, contact dermatitis, initial psoriasis, and erythema chronicum migrans were studied. A high percentage of Lutzner (Sézary) cells was consistently found in Sézary's syndrome and occasionally in parapsoriasis en plaques only. In mycosis fungoides lymphocytoid cells with the nuclei less dense than in normal lymphocytes were predominant. In most tumorous lesions of mycosis fungoides and of Sézary's syndrome an increased percentage of immunoblasts was noted. Normal lymphocytes were the most frequent cell type in parapsoriasis en plaques as well as in the controls. These findings may prove helpful in the diagnosis of problem cases, for which the use of semi-thin sections is recommended.

Cell Nucleus↗

Treatment of parapsoriasis and mycosis fungoides: the role of psoralen and long-wave ultraviolet light A (PUVA).

Psoralen and long-wave ultraviolet light A (PUVA) was used to treat 12 patients with parapsoriasis and 19 patients with mycosis fungoides. Excellent results were obtained in all 12 patients with parapsoriasis: 8 patients had complete clearing, 3 had more than 80% improvement, and 1 had considerable improvement. The response to PUVA in patients with mycosis fungoides varied according to the stage of the disease. Of 10 patients with early disease (stage I or II), 7 had complete clearing and the 3 others had more than 80% improvement. The nine patients with more advanced disease generally did not respond well to PUVA, with the exception of one patient with stage IV disease and one with stage V disease. PUVA may be the treatment of choice for parapsoriasis and is highly effective in the early stages of mycosis fungoides.

Female↗

HLA-DR-positive cells in large plaque (atrophic) parapsoriasis.

The development of a monoclonal antibody directed against HLA DR (Ia-like) antigens of B cells and monocytes but not against normal peripheral human T cells suggested that this antibody might be used as a marker of B cells and monocytes in tissue sections. The T cell nature of large plaque (atrophic) parapsoriasis has recently been demonstrated by the immunoperoxidase technic. Immunoperoxidase examination of serial sections of tissues from two cases of large plaque parapsoriasis with one T cell antiserum, two monoclonal T cell antibodies, and one monoclonal reagent directed against HLA DR indicated that T cells in the cutaneous infiltrates were also HLA DR-positive. Evidence is accumulating that HLA DR positivity may be expressed by activated T cells. The findings here therefore suggest that many of the T lymphoid cells in two cases of large plaque (atrophic) parapsoriasis examined were activated in nature, and that HLA DR may not be a specific marker for B cells and monocytes in certain pathologic conditions. Caution should therefore presently be exercised in attempting to use this marker for the specific identification of B cells and monocytes in pathologic specimens, without simultaneous testing for T cell markers.

Atrophy↗

In situ demonstration of T cell subsets in atrophic parapsoriasis.

It has been demonstrated recently in mycosis fungoides and lichen planus that T lymphocyte subsets may be identified in cutaneous lymphocytic infiltrates using the immunoperoxidase technic in conjunction with monoclonal antibodies produced by the technic of Kohler and Milstein. This communication describes the application of this technic to cutaneous lymphoid infiltrates of parapsoriasis in which T cell predominance has been demonstrated previously. The lymphoid infiltrates of six patients with atrophic parapsoriasis were examined by the indirect immunoperoxidase technic using monoclonal antibodies (from two commercial sources) directed against "helper" and "suppressor" T cell subsets. Both "helper" and "suppressor" cells (as defined by a positive reaction with monoclonal antibodies) could be identified in cutaneous infiltrates. "Helper" cells predominated, but in varying degrees among patients. The relevance of these findings in relation to the possible development of clinical mycosis fungoides from atrophic parapsoriasis is discussed. In addition, factors causing difficulty in the consistent identification of cell subtypes are discussed. These factors suggest that in the present state of imperfection, difficulty will be experienced in using this technic for the accurate quantification of percentages of lymphocyte subsets in tissue sections.U

Antibodies, Monoclonal↗

Guttate parapsoriasis/digitate dermatosis (small plaque parapsoriasis) is mycosis fungoides.

Authors of most textbooks of dermatology and dermatopathology consider guttate parapsoriasis and digitate dermatosis to be variants of small plaque parapsoriasis which, they aver, is not related to mycosis fungoides. On the basis of a study of clinical and histopathologic findings in guttate parapsoriasis and digitate dermatosis, we conclude that those conditions actually represent two of the many clinical faces of mycosis fungoides.

Adolescent↗

Parapsoriasis with hyper-IgE.

A 56-year-old black man had generalized chronic dermatitis for two years before parapsoriasis en plaque (large type) with hyper-IgE (12,000-22,000 ng/ml) was diagnosed. Lymphocyte cultures showed decreased response to mitogens. Direct immunofluorescence of involved skin showed epidermal and dermal mast cells with bound IgE. Parapsoriasis with hyper-IgE may represent a form of "hypersensitive immunosurveillance" allowing a patient to reject a clone of malignant cells and retard transformation from a benign state (parapsoriasis) to a malignant state (mycosis fungoides).

Fluorescent Antibody Technique↗

[Behavior of delayed-type hypersensitivity in parapsoriasis].

The authors present a study of behaviour of the late hypersensitivity in fourteen patients carrying parapsoriasis with age varying from 36 to 64 years being nine men and five women with time of the illness development from one to eleven years. There were thirteen white sick people and a yellow one. The parapsoriasis diagnosis was confirmed in all of them clinica and histopathologica. The patients were not getting any therapeutic with corticosteroids or immunosuppressive drugs at the time of study. They were studied immunological through methods in vivo. 1. Methods in vivo. a) Intradermoreactions with the infectious antigens: PPD, trichophytin, levedurin, E. coli. It was used as a control group of fourty normal fellows. b) Sensibility to DNCB. The results obtained allow the following thesis: 1. Low table of positivity to the intradermal reactions to the late reading only one of the antigens tested on the patients (E. coli) related to the others. According to these elements we cannot get at a conclusion for depression of the inespecific cellular immunity in our sick patients. 2. Significant statistics difference on the sensibility test to DNCB on the sick patients related to the controls, a fact which shows a certain degree of the depression of the inespecific cellular immunity. 3. The sick patients with parapsoriasis before taking some treatment should go through the study of a late hypersensitivity.

Adult↗

[Immunoglobulin E in cutaneous lymphomas and in parapsoriasis en plaques (Brocq) (author's transl)].

Immunoglobulin E was determined in 13 patients with cutaneous lymphoma and in 30 patients with parapsoriasis en plaques. 11 out of the patients with cutaneous lymphomas showed mycosis fungoides. In 8 patients with parapsoriasis en plaques serum IgE was elevated. These patients revealed signs of the premalignant form of parapsoriasis en plaques. In mycosis fungoides elevated IgE is common, but normal levels are found, too.

Humans↗

Cutaneous T-cell lymphoma (parapsoriasis en plaque). An association with pityriasis lichenoides et varioliformis acuta in young children.

Pityriasis lichenoides et varioliformis acuta (PLEVA) and pityriasis lichenoides chronica (PLC) are related benign disorders without recognized association with cutaneous T-cell lymphoma (CTCL). We report the cases of two children with documented PLEVA evolving into CTCL over several years. One child had the clinical lesions of PLC but the dermatopathologic findings of PLEVA at age 2 years. At age 12 years, he had skin changes of poikiloderma atrophicans vasculare and dermatopathologic findings consistent with parapsoriasis en plaque. The second child presented at age 7 years with scaling dermatitis and dermatopathologic findings of PLEVA. At age 12 years, the histologic diagnosis was parapsoriasis. Monoclonal antibody studies performed on biopsy specimens from both patients revealed 70% to 100% cells staining with CD5, 80% to 90% staining with CD4, 30% to 50% staining with CD8, and an increase in CD1-staining cells in the papillary dermis, indicating a predominantly helper T-cell infiltrate. We believe that PLC and PLEVA may be part of the spectrum of CTCL. Furthermore, CTCL may be more common in young children than once thought.

Child↗

OKT 9 reactivity in mycosis fungoides and large plaque (atrophic) parapsoriasis.

A monoclonal antibody OKT 9 which detects a determinant expressed by a variety of proliferating cell types has been recently developed. This antibody was used in conjunction with the immunoperoxidase technique to study the cutaneous lymphoid infiltrates of nine patients with mycosis fungoides, one patient with lymphomatoid papulosis, two patients with Sézary syndrome, and ten patients with large plaque atrophic parapsoriasis (a condition which may terminate in overt mycosis fungoides.) OKT 9 reactive cells were identified in all cases of mycosis fungoides examined, in one case of lymphomatoid papulosis, one of two cases of Sézary syndrome, and one of ten cases of large plaque atrophic parapsoriasis. These results suggest that further studies using OKT 9 should be performed to assess whether OKT 9 reactivity may be used as a prognostic marker in cutaneous lymphomas and prelymphomas.

Antibodies, Monoclonal↗

Photochemotherapy in cutaneous T cell lymphoma and parapsoriasis en plaques. Long-term follow-up in forty-three patients.

Forty-three patients with clinical plaque- and tumor-stage mycosis fungoides, the erythrodermic/Sézary syndrome variant of mycosis fungoides, and parapsoriasis en plaques were treated with oral psoralens and ultraviolet A (PUVA). Pretreatment skin biopsies, evaluated by light microscopy, revealed seventeen diagnostic, seventeen suggestive, and nine nonspecific specimens. Clinical and histologic parameters were followed for an average of 38.4 months (range, 4-67 months). Twenty-five patients had complete clearing, and fourteen did not respond. Most patients in the complete-response group had either plaque lesions of mycosis fungoides or parapsoriasis en plaques prior to PUVA. Most patients in the no-response group had either tumor lesions or the erythrodermic/Sézary mycosis fungoides at the start of PUVA. In the no-response group the treatment modalities used prior to PUVA were twice the number used in the complete-response group. Patients in the complete-response group had clearing of their lesions after an average PUVA dose of 117 joules/cm2. Relapse occurred in seventeen patients after an average remission time of 6.3 months and responded to additional PUVA. Patients whose skin remained clear after the first course of PUVA continued to have clear skin for up to 58 months, with an average complete remission of 29.5 months by the end of the study period. Histologic evaluation before PUVA and at clearing revealed a definite trend toward a normal microscopic picture, but at least a mild inflammatory infiltrate usually persisted. At the end of the study period, the lesions of ten patients had entirely cleared for an average of 44 months, the lesions of five had cleared during a second course of PUVA, five had stable limited-plaque disease while receiving maintenance PUVA, eleven were undergoing electron beam radiation therapy or chemotherapy for progressive disease, ten had died, and two patients were lost to follow-up. Therefore, in the early stage of mycosis fungoides, PUVA may induce significant disease-free intervals. Prior treatment with a variety of modalities, the patient's age, and/or the duration of disease may affect response to PUVA.

Adult↗

Shortened telomere length is demonstrated in T-cell subsets together with a pronounced increased telomerase activity in CD4 positive T cells from blood of patients with mycosis fungoides and parapsoriasis.

We have recently demonstrated that telomerase activity is increased and telomere length shortened in lymphocytes from peripheral blood of patients with cutaneous T-cell lymphoma. In order to determine which cell type has increased telomerase activity and shortened telomere length, CD4+, CD8+, CLA+ CD3+ and CLA- CD3+ T cells were isolated from peripheral blood of 25 patients, including 15 patients with mycosis fungoides and 10 patients with parapsoriasis. Eleven healthy individuals were used as controls; CD19+ B cells were separated from each individual as an internal control. The results showed that the increased telomerase activity was significantly predominating in the CD4+ T-cell subset. Significantly shortened telomere length was found in CD4+ and CD8+ T-cell subsets from the patients compared with the same cell subsets obtained from healthy individuals. However, no difference was observed between the subsets; CD19+ B cells collected from patients and healthy control individuals had similar telomerase activity and telomere length which was significantly different from the values found in T cells. The telomere length was significantly shorter in CLA+ CD3+ subset than in CLA- CD3+ subset. Interestingly, increased telomerase activity and shortened telomere length was also detected in CD4+ T cells from patients with parapsoriasis indicating that alteration of telomerase activity and telomere length in CD4+ T cells is an early event in the pathogenesis of cutaneous T-cell lymphoma. Thus, the results indicate that a significant high level of telomerase activity and shortened telomere length frequently occur in T cells of patients with CTCL and may reflect tumorigenesis.

Aged↗

Parapsoriasis in two children: a clinical, immunophenotypic, and immunogenotypic study.

Parapsoriasis has been described rarely in childhood; a few reports mention this condition as preceding early mycosis fungoides. We report a boy age 15 and a girl age 8 years with different clinical features of parapsoriasis who were followed clinically and histologically for about four years. Immunophenotyping of the skin infiltrate and a T cell receptor gene rearrangement analysis were performed on each. The infiltrate was composed mainly of CD4+ and CD45+ lymphocytes in the first patient and of CD8+ and CD45+ in the second. No T cell receptor gene rearrangements were found. The paucity of knowledge about the evolution of this entity in childhood and its relationship to mycosis fungoides makes follow-up critical. The importance of immunophenotyping and immunogenotyping is particularly stressed.

Adolescent↗

[Small-plaque parapsoriasis: case report].

INTRODUCTION: Small plaque parapsoriasis is a relatively rare, chronic, idiopathic dermatosis, most often seen in middle age people. This disease shows a definite male predominance of approximately 3-4: 1. It is characterized by presence of round or oval erythematous, slightly scaly plaques on the limbs and trunk, which histologically reveal mild eczematous changes. CASE REPORT: A male patient, 61 years of age, was admitted to the Clinic of Dermatovenereology in Novi Sad due to long persisting erythematous patches on his upper and lower limbs. Plaques were of oval and round shape, pretty well marginated. They were of light red colour, covered with fine scales with a slightly wrinkled surface. He complained of itching. Laboratory findings showed no abnormalities. Histopathologic examination of the skin specimen revealed epidermal atrophy, focal parakeratosis, perivascular dermal infiltrate of mononuclear cells with exocytosis in the epidermis. This finding was compatible with the clinical diagnosis. After treatment with topical corticosteroid cream combined with whole body exposure to sunlight irradiation, vast majority of skin lesions regressed. DISCUSSION: The clinical course of small plaque parapsoriasis is very long. The plaques are remarkably stubborn, responding to treatment with steroid creams or to natural or artificial sunlight, but usually reappearing promptly when treatment is discontinued. The patches increase in number for a time, and then remain relatively constant for a long time. A small minority of cases clears entirely. Recent studies provided evidence of monoclonality and immunophenotypic abnormalities. Rearrangement of T-cell receptor genes was demonstrated by using PCR method. Detection of monoclonal T-cell populations in skin lesions, as a characteristic of lymphoproliferative diseases, forced some authors to include this dermatosis into a group of abortive cutaneous T-cell lymphomas. CONCLUSION: This case deserves a long and probably life-long clinical and histological assessment, especially due to new knowledge about the possible nature of this disease.

Humans↗