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[The difference forms of "parapsoriasis en plaques". A report of 90 cases (author's transl)].

The term parapsoriasis was used by Brocq (1902) to group a number of conditions previously described under different names. This group has since then been modified, the same conditions being described under separate names and these have led to a great confusion especially between countries. In this study of 90 cases, three types of parapsoriasis en plaques are distinguished. The "parapsoriasis digitiforme" (40 cases) or benign type, or xanthoerythrodermia perstans, or chronic superficial dermatitis is characterized by small, oval or finger-like, yellow or pink patches. The histology is frequently not characteristic, but in a few cases, there is an exocytosis localized "en flammèches" in the epidermis. The condition is usually permanent but none of these cases has progressed to mycosis fongoides. The parapsoriasis "en grandes plaques simples" (25 cases) is characterized by few pink patches (3 to 5), larger than in benign type. In our cases the transformation to poikiloderma atrophicans vasculare is not observed. One of these cases progressed to mycosis fongoides. The "parapsoriasis en grandes plaques poïkilodermiques" (25 cas), or poikiloderma atrophicans vasculare, prereticulotic poikiloderma, atrophic parapsoriasis, parapsoriasis lichenoides, is characterized by large patches, in limited number, showing a reticulated pigmentation and slight atrophy with telangiectasia. Five cases changed into mycosis fongoides and 4 cases showed some symptoms of malignancy; The histologic features are the same in the two last types: sometimes they are non-specific, in numerous cases the picture is characteristic with micro-abscesses or "flammèches"; in some cases there is a dense infiltrate with a clearly defined lower limit: this histologic appearance can be seen in cases without transformation into mycosis fongoides.

Adult

[Phototherapy of parapsoriasis].

The effectiveness of photo(chemo)therapy was tested on 69 patients with different forms of parapsoriasis (parapsoriasis guttata, n = 14; pityriasis lichenoides et varioliformis acuta, n = 6; and parapsoriasis en plaques, n = 49). The forms of parapsoriasis differed in their response to phototherapy and in their relapse rates. Whereas parapsoriasis guttata can be regarded as a definite indication for UVA therapy, much higher UVA doses are required for pityriasis lichenoides et varioliformis and the probability of relapses is higher. It was not possible to interrupt the relapsing course of parapsoriasis en plaqes in the majority of patients. Relapses occurred within 7 months in 63.2% of the cases. A healing effect that lasted for more than one year was achieved only in 18 patients.

Adolescent

Parapsoriasis and related conditions.

Classification of parapsoriases is revised into a simple practical table. Pityriasis lichenoides (guttate parapsoriasis) is not a type of parapsoriasis. The clinical features of small patch and large plaque parapsoriasis are described in detail. Six clinical varieties of large plaque parapsoriasis and three clinical varieties of exfoliative dermatitis including Sézary syndrome have been clearly recognized as distinctive categories. Histopathology is useful for the diagnosis of parapsoriasis and mycosis fungoides. Sézary cell count is not significant for the diagnosis of Sézary syndrome.

Dermatitis, Exfoliative

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

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

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

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

History of psoriasis and parapsoriasis.

Parapsoriasis is not a disease entity. It is an auxiliary term introduced in 1902 to group several dermatoses with a faint similarity to psoriasis. The historical development leading to the creation of the term parapsoriasis is outlined and the history of psoriasis is briefly reviewed.

Europe

[Poikilodermic parapsoriasis. Case report and discussion of nomenclature].

A case of a 59 year-old patient with premycotic parapsoriasis lichenoides of a special poikilodermic type is presented. In addition, the formation of multiple bowenoid actinic keratoses and even squamous cell carcinomas on both forearms was apparently favored by the poikilodermic change. An attempt is made to summarize the nomenclature of the parapsoriasis-poikiloderma group of dermatoses.

Carcinoma, Squamous Cell

Treatment of a case of mycosis fungoides and one of parapsoriasis en plaque with topical PUVA using a monofunctional furocoumarin derivative, 4,6,4'-trimethylangelicin.

A case of plaque stage mycosis fungoides and one of parapsoriasis en plaque were treated with topical PUVA therapy using a monofunctional furocoumarin derivative, 4,6,4'-trimethylangelicin (TMA). Both patients showed complete clearance of eruptions within 16 treatments. The therapeutic effectiveness of TMA was confirmed by the fact that those eruptions exposed to UVA alone, without TMA application, showed slower and less significant improvement. Histologically, dermal infiltrates of mycosis cells and associated epidermotrophism disappeared almost completely in response to TMA PUVA. No side effects or changes in values in laboratory examinations were observed during treatment.

Administration, Cutaneous

[Acute parapsoriasis in a 5-year-old girl].

A case is reported of rarely observed skin changes in a girl aged 5 years. The changes resembled those observed in acute parapsoriasis (p. lichenoides et varioliformis of Mucha-Habermann). The diagnosis was established after finding characteristic polymorphic lesions in the form of papulae, necrotizing vesicles, ulcerations, desquamation of certain papulae typical of p. guttata, long-term persistence of the lesions and good general condition of the child. The lesions were situated on the trunk, and in a lower degree on the face and extremities. Before the disease the girl hand contact with insecticides (Ovadofox) and detergents.

Acute Disease

Treatment of parapsoriasis en plaques, mycosis fungoides, and Sézary's syndrome with trioxsalen baths followed by ultraviolet light.

Three patients with parapsoriasis en plaques (PEP), fifteen with mycosis fungoides (MF), and one with Sézary's syndrome (SS) were given a bath to which a small amount of trioxsalen solution had been added, and then exposed to ultraviolet (UV) radiation from a bank of dysprosium lamps. Within 2--6 months of this treatment the skin lesions healed completely or almost completely in all 3 patients with PEP, in all 7 with MF stage II and in 4 of 5 with MF stage III. Two patients with MF stages IV--V showed a fair degree of improvement. One with erythrodermic form of MF responded, but poorly. The patient with SS and the one with erythrodermic MF responded with severe phototoxic reactions.

Adult