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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.
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.
The clinical history of a patient affected by a chronic relapsing eruption is presented. Successive papulopustular, necrotic and ulcerous lesions with fever and general distress appeared. There was no visceral affectation. The case corresponded to the necrotic and hyperthermic parapsoriasis "en gouttes" or acute pityriasis lichenoides as described by Degos and col., 1966. The presence of vasculitis with lymphohistiocytic inflammation invading the epidermis is described. The aspect was similar to the parapsoriasis "en gouttes" pattern even though a biopsy showed the presence of necrotizing angeitis of small vessels, as previously reported. The patient also presented oral and nasopharyngeal ulcerous lesions similar to those found in malignant midline granuloma and Wegener granulomatosis, even though histopathologically different. It is interesting to note the absence of any visceral involvement in a 12 years evolution.
Selective UV-Phototherapy with lambda 300-320 nm (SUP) as well as oral photochemotherapy with 8-methoxy-psoralene plus UVA-radiation (PUVA intern) are very effective in clearing the lesions of th generalized psoriasis and those of the chronic forms of parapsoriasis. Being treated with 4 suberythemal doses per week psoriasis patients are free or nearly free of symptoms after averaging 6.3 weeks of SUP-therapy or after 5.3 weeks of PUVA orally. The PUVA-therapy is mainly indicated in pustular, inverse and erythrodermic psoriasis as well as in parapsoriasis in plaques and variegata. In all other forms of psoriasis and in pityriasis lichenoides chronica, we prefer the SUP-therapy because of less acute or chronic side effects, and because of its better practicability. X-rays are indicated in psoriasis of nails, grenz-rays in superficial psoriatic lesions of the face, the armpits, the genitals and the anal region.
The paper deals with the origin of parapsoriasis nodularis, which in its micronodular variant belongs to chronic dermatosis and has a clear-cut clinical and histological appearance. The disease arises most frequently after stress and occurs for the most part in middle-aged males. The leading role in pathogenesis of parapsoriasis nodularis is played by cellular immunity as indicated by reduced count of T-lymphocytes, active T-lymphocytes as well as the emergence of delayed hypersensitivity.
A 59-year-old female, clinically diagnosed as having parapsoriasis en plaque for ten years, was referred on June 1991 to the Department of Oral Surgery in the Dental School of showa University with a complaint of painless swelling of the left malar area. The swelling was found to have been caused by a tumor. Since the excised tumor was suspected to be a malignant lymphoma, she was admitted to our hospital. The specimen was subsequently confirmed to be a malignant lymphoma of the diffuse large cell type according to the LSG classification, and was immunologically confirmed to be a B cell lymphoma. In addition, the excised skin was suspected of incipient mycosis fungoides. She was classified as having stage IE disease according to her bone marrow biopsy and other examinations. she was treated with combination of chemotherapy (CHOP) and radiation therapy. This subject was interesting because her tumor probably resulted from a parapsoriasis en plaque skin lesion.
We report on a 78-year-old Japanese woman with a 50-year history of large-plaque parapsoriasis that had evolved into cutaneous T-cell lymphoma. Her large-plaque parapsoriasis had been treated with psoralen plus ultraviolet A for 10 years. Subsequently an isolated nodule appeared on her right lower leg. Prior or concurrent patches or plaques were absent. Histology revealed a diffuse nonepidermotropic infiltrate of large lymphocytes in the dermis, which had enlarged nuclei and prominent nucleoli. A diagnosis of CD30- cutaneous large T-cell lymphoma was made. Following systemic chemotherapy, there was clinical improvement. No evidence of recurrence or systemic lymphoma has subsequently been found.
We report on a 65-year old woman suffering from parapsoriasis with large plaques. The cellular infiltrate was analysed by means of monoclonal antibodies and an immunoperoxidase technique. Our findings proved that the majority of the cellular infiltrate in the upper dermis is composed of helper-inducer T-cells (Leu 4+, Leu 3a+). A notable number of Leu-2a-reactive suppressor-cytotoxic T-cells associated with hydropic degeneration of the basal cell layer were found within the dermal infiltrate and the dermoepidermal interface. Large numbers of Leu-6-reactive dendritic Langerhans' cells were noted in the lower layers of the epidermis, in particular in areas where the epidermis showed focal exocytosis of lymphoid cells. Langerhans' cells, in contrast, were present in the upper portions of epidermis lacking exocytic T-cells. HLA-DR antigens were expressed on Langerhans' cells as well as on nearly all T-lymphocytes. Positive intercellular staining for HLA-DR antigens were only found in localized areas of the epidermis. Small numbers of both Leu-11B-reactive natural killer cells and cells expressing interleukin-2 receptors were seen within the basal cell layer and at the dermo-epidermal junction. Our findings suggest that a cell-mediated immune response which is directed against antigens expressed by keratinocytes may play an important role in the pathogenesis of parapsoriasis en plaques.
BACKGROUND: Broadband UV-B phototherapy has been used for many years in the treatment of small plaque parapsoriasis (SPP) and early-stage mycosis fungoides (MF). Our purpose was to investigate the effect on these diseases of narrowband (311-nm) UV-B therapy, which was recently established for the treatment of psoriasis and found to be more effective than broadband UV-B therapy. OBSERVATIONS: Twenty patients (5 women, 15 men; age range, 39-85 years) with histologically confirmed SPP or early-stage MF were enrolled. Six patients had early-stage MF (patch stage), and 14 had SPP. Treatment with 311-nm UV-B was given 3 to 4 times a week for 5 to 10 weeks. In 19 patients, lesions completely cleared after a mean number of 20 treatments (range, 14-29 treatments) and a mean cumulative UV-B dose of 16.3J/cm2 (range, 7.4-36.4 J/cm2) within a mean time of 6 weeks (range, 5-10 weeks). Biopsy specimens taken immediately after the end of phototherapy showed only sparse inflammatory infiltrates but no signs of SPP or MF. Relapses at cutaneous sites occurred in all patients within a mean time of 6 months (range, 2-15 months). CONCLUSIONS: Narrowband UV-B therapy is an effective short-term treatment modality for clearing SPP and early-stage MF. However, the treatment response did not sustain long-term remission. Further studies are necessary to examine how the clinical response to and follow-up after narrowband UV-B therapy compares with that of established phototherapy modalities in these diseases.
Parapsoriasis is a group of uncommon but not rare disorders that was created in 1902 as part of a now long forgotten scheme to classify all inflammatory dermatoses. This artificial grouping has led to an enormously confused nosology of these disorders, that are, for the most part, otherwise unrelated. The use of a number of different terms at different institutions and by different physicians to denote the same diseases, together with the use of particular, single terms to denote different diseases, has caused much unnecessary confusion. In this review these terms are examined and an attempt is made to propose a new, unambiguous classification. Using this system, physicians with different views regarding which of the parapsoriases constitute distinct entities should have not difficulty communicating, and should have clearer frame of reference within which to work. An attempt is made to evaluate critically which of the parapsoriases are distinct entities and whether one or more of them should be considered an early form of cutaneous lymphoma in light of currently available data.
A monoclonal antibody (OKT10), which was developed recently, reacts with pro-thymocytes, T cell acute lymphoblastic leukemia (ALL) cells, cells in normal bone marrow (including plasma cells), and activated T cells. Tissues from patients with cutaneous T cell lymphoma were studied for the presence of OKT10-reactive cells with the use of an indirect immunoperoxidase technic. OKT10-reactive cells were identified in three of eight cases of mycosis fungoides, one of two cases of Sézary syndrome, with an equivocal reaction in one of ten cases of large-plaque parapsoriasis and in one of seven positive patch tests (allergic contact dermatitis). The biologic and possible clinical implications are discussed.
The surface contours of lesional skin of certain skin diseases, such as parapsoriasis en plaque (PEP) and extramammary Paget's disease (EMPD), in which there is a massive infiltration by non-epidermal cells, looks somewhat different from that of the adjacent normal skin, needless to state that they are apparently different from that of ordinary chronic inflammatory dermatoses where we found acanthotic epidermis accompanied by hyperkeratosis. We attempted to objectively characterize these unique skin surface changes qualitatively and quantitatively using non-invasive methods. Negative impression replicas were taken from the lesional skin of patients with EMPD or PEP as well as from the adjacent uninvolved skin. The findings were confirmed histologically. The replicas were examined by using computerized image analysis. Several parameters were analyzed that correlate with the changes in the anisotropy of the skin furrows (VC1), average skin roughness (KSD), average length of skin furrows (LEN), and number of skin furrows (NUM). There were significant decreases in KSD and NUM in EMPD, indicating a smoother skin surface in the lesional skin than in the adjacent normal skin. In contrast, the PEP lesion had an increase in VC1 and LEN and a decrease in NUM, which suggests larger skin ridges in the lesional skin than in the uninvolved skin. Thus the unique skin surface of the cutaneous disorders accompanied by epidermal invasion by non-epidermal cells, such as EMPD and PEP, was characterized both qualitatively and quantitatively using computerized image analysis of negative impression replicas.
Twelve patients with large plaque parapsoriasis (LPP) were investigated for the presence of predominant T-cell clones, analyzing the T-cell receptor (TCR) gamma-chain gene. The diagnostic and prognostic significance of TCR gene rearrangement status was assessed by a correlation with the long-term clinical follow-up. Six out of 12 patients showed a clonal T-cell population. Clinically, among the patients with clonal disease one developed clearcut mycosis fungoides (MF) after a follow-up of 8 years, in the other 5 patients no such diagnosis could be made after follow-up of 2-21 years (median: 9 years). In patients with polyclonal infiltrates the lesions remained virtually unchanged. These findings indicate that in LPP TCR gene rearrangement status has no prognostic significance and does not allow distinction of LPP and early MF. Both conditions show a clonal T-cell infiltrate with similar frequency, are very similar in clinical and histologic presentation and according to recent studies share the same low risk to develop overt MF. Therefore both terms refer to the identical clinical situation. This should be designated as early MF and efforts should concentrate on identifying those patients that are at risk to develop aggressive disease.
Altogether, 209 skin biopsies from 103 patients with mycosis fungoides (MF), large plaque parapsoriasis (LPP), and benign chronic dermatoses (BCD) have been examined immunohistologically with the use of a panel of 21 monoclonal antibodies against lymphoid cells and their subsets. All the infiltrates contained a mixture of T-lymphocytes, Langerhans cells, and other types of HLA-DR-positive dermal macrophages. The neoplastic T-cells in MF lesions expressed proliferation-(transferrin receptor) and activation-(the OKT10 antigen) associated markers more frequently than the T-cells in LPP and BCD. In other respects, the neoplastic T-cells in plaque lesions of MF resembled those seen in LPP and BCD; and most of these cases demonstrated a clear predominance of T-cells of helper/inducer type. The neoplastic T-cells in tumor lesions of MF were much more heterogeneous in phenotype. Only eight of these cases could be classified as T-helper neoplasms. In the remaining ten tumor cases, the neoplastic cells expressed either suppressor/cytotoxic or aberrant T-cell phenotypes. There were no phenotypic differences between the "classical" tumor stages and MF d'emblee cases. The data indicate that the early lesions of MF show an immunohistologic reaction pattern common to many immune responses of the skin and that the neoplastic cells in the advanced stages are more heterogeneous in phenotype than previously recognized.
Thirteen patients with stage I or II mycosis fungoides (MF) and 10 patients with large-plaque parapsoriasis en plaques (PEP) were examined for immunologic and cytogenetic disturbances. Total lymphocyte counts and immunoglobulin concentrations in the blood were normal. In vitro lymphocyte responses to polyclonal activators and various antigens in standard concentrations were normal. However, titration of phytohemagglutinin and concanavalin A (ConA) disclosed significantly lowered responses to suboptimal concentrations in the patient group, most pronounced in patients with MF II. ConA-induced leukocyte migration inhibitory factor (LIF) production, tested in an indirect leukocyte migration inhibitory assay, was low in the patient group. Furthermore spontaneous LIF production in vitro and small amounts of serum LIF were demonstrated in a few patients. The chromosomal banding pattern, sister chromatid exchange, and break frequency were within normal limits except for 3 translocations in the MF group. It is concluded that even in early-stage MF a pathologic function of blood lymphocytes can be demonstrated, when sensitive methods are applied. The findings might be important for monitoring disease activity and effect of treatment.
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.
Narrow band (311 nm) ultraviolet B (NB-UVB) has been shown to be a safe and effective treatment for psoriasis and other inflammatory skin diseases. We have therefore employed NB-UVB in the treatment of small plaque parapsoriasis (SPP) since 1996. All patients (16/16) responded with complete remission of the disease after a mean number of 32.8 exposures and a mean total dose of 35.4 J/cm2. Unwanted side-effects were rare (3.3%) and always mild. Relapse of the disease occurred after an average of 29 weeks in those patients who came for follow-up visits. Therefore, NB-UVB is an effective, comparably safe and convenient alternative to psoralen and ultraviolet A therapy or other treatment modalities in the suppression of SPP.
BACKGROUND: Narrowband UVB (NB-UVB) phototherapy has been shown to be effective for the treatment of various dermatoses. OBJECTIVE: To analyze the effects of NB-UVB phototherapy for small plaque parapsoriasis (SPP). METHODS: The response of 45 patients (24 females, 21 males, age range 20-58 years) with histologically confirmed SPP were assessed. NB-UVB therapy was given 3-4 times weekly. The initial treatment dose was 70% of the minimal erythema dose. The doses were increased gradually with a standard increment of 20/10/0. Clinical response was determined as follows: complete response (CR), at least 90% clearing of skin lesions; partial response (PR), at least 50% but less than 90% clearing and no response (NR), less than 50% clearing. The follow-up period was 6-24 months after the treatment. RESULTS: NB-UVB treatment led to CR in 33 of 45 patients (73.3%) with a mean cumulative dose of 14.3 J/cm(2) (range 3.2-24.1 J/cm(2)) after a mean number of 29 exposures (range 16-51 sessions); PR in 12 of 45 (26.6%) with a cumulative dose of 15.6 J/cm(2) (range 10.4-23.3 J/cm(2)) after a mean number of 29.4 exposures (range 25-50 sessions). Nineteen patients with CR had skin phototype II, 13 had type III and 1 had type I. Among the patients with PR, 7 had skin phototype II and 5 had type III. Postinflammatory hyperpigmentation was observed in 51% of the patients. Relapses occurred in six patients within a mean time of 7.5 months (2-12 months). CONCLUSION: NB-UVB phototherapy has several advantages over treatment with broadband UVB and PUVA. NB-UVB therapy for patients with SPP is an effective, safe and practical alternative treatment modality. Further larger studies with longer follow-up periods are necessary to determine the proper clinical response and long-term complications of NB-UVB therapy in this disease.