Parapsoriasis Overview of Parapsoriasis
- Author: Henry K Wong, MD, PhD; Chief Editor: Dirk M Elston, MD more...
Overview of Parapsoriasis
Parapsoriasis describes a group of cutaneous diseases that can be characterized by scaly patches or slightly elevated papules and/or plaques dispersed on the trunk or proximal extremities that have a resemblance to psoriasis—hence the nomenclature. However, this description includes several inflammatory cutaneous diseases that are unrelated with respect to pathogenesis, histopathology, and response to treatment. Because of the variation in clinical presentation and a lack of a specific diagnostic finding on histopathology, a uniformly accepted definition of parapsoriasis remains lacking.
In 1902, Brocq initially described 3 major entities that fit the description:
Pityriasis lichenoides (acuta and chronica)
Small plaque parapsoriasis
Large plaque parapsoriasis (parapsoriasis en plaque)
Pityriasis lichenoides (acuta and chronica)
Pityriasis lichenoides variants describe scaly dermatoses with necrotic papules that are clinically and histologically different from parapsoriasis. These diseases generally are benign and undergo spontaneous resolution but, at times, may have a protracted course (see Pityriasis Lichenoides for further discussion).
Large plaque and small plaque parapsoriasis
Current terminology of parapsoriasis refers to 2 disease processes that are caused by T-cell–predominant infiltrates in the skin. These disease processes are large plaque parapsoriasis and small plaque parapsoriasis.
As the nomenclature and description of the disease spectrum under the descriptive term parapsoriasis evolved, the primary focus has been on the distinction of whether the disorder progresses to mycosis fungoides (MF) or cutaneous T-cell lymphoma (CTCL). Small plaque parapsoriasis is a benign disorder that rarely if ever progresses. Large plaque parapsoriasis is more ominous in that approximately 10% of patients progress to MF/CTCL. Controversy exists currently in the classification of large plaque parapsoriasis because some believe it is equivalent to the earliest stage CTCL, the patch stage.[2, 3, 4]
The duration of parapsoriasis can be variable. Small plaque disease lasts several months to years and can spontaneously resolve. Large plaque disease is chronic, and treatment is recommended because it may prevent progression to CTCL.
El-Darouti et al reported on a 7-year study of a hypopigmented disorder that the researchers believe should be classified as a new variant of parapsoriasis en plaque.
No clear etiology for small plaque or large plaque parapsoriasis is known, and no specific association has been made with contact exposure or infections.
For more information, see the topic Psoriasis.
Pathophysiology of Parapsoriasis
The initiating cause of parapsoriasis is unknown, but the diseases likely represent different stages in a continuum of lymphoproliferative disorders from chronic dermatitis to frank malignancy of cutaneous T-cell lymphoma (CTCL).
Small plaque parapsoriasis
Small plaque parapsoriasis likely is a reactive process of predominantly CD4+ T cells. Genotypic pattern observed in small plaque parapsoriasis is similar to that observed in chronic dermatitis, and the pattern of clonality of T cells is consistent with the response of a specific subset of T cells that have been stimulated by an antigen. Multiple dominant clones can be detected by polymerase chain reaction (PCR) of T-cell receptor gene usage, which supports a reactive process. Lymphocytes do not show histologic atypia to suggest malignant transformation. Southern blot analysis of T-cell receptor genes from parapsoriasis does not identify a dominant clone of T cells.
Some physicians believe that small plaque parapsoriasis is an abortive T-cell lymphoma; however, no clear distinguishing evidence, such as genetic changes (eg, TP53 mutations) observed in other malignancies, exists to support this contention. Nevertheless, a hint to the verity of this hypothesis is the recent identification of increased telomerase activity in T cells from CTCL at low-grade stages, high-grade lymphoma, and parapsoriasis, which is activity not exhibited in normal T cells. A better understanding is likely to develop from further molecular characterization.
Large plaque parapsoriasis
Large plaque parapsoriasis is a chronic inflammatory disorder, and the pathophysiology has been speculated to be long-term antigen stimulation. This disorder is associated with a dominant T-cell clone, one that may represent up to 50% of the T-cell infiltrate. If the histologic appearance is benign, without atypical lymphocytes, classification of large plaque parapsoriasis is made. If atypical lymphocytes are present, many would classify such patients as having patch stage CTCL.
In one study, human herpesvirus type 8 was detected in up to 87% of skin lesions of large plaque parapsoriasis. This is the first association of a specific infectious agent with large plaque parapsoriasis, and the significance is unclear. Further studies are important to determine the significance of this finding.
The close relationship between large plaque parapsoriasis and mycosis fungoides is highlighted by the detection of TOX expression, a new marker that has been described to be frequently detected in the abnormal T cells in mycosis fungoides. In one study, large plaque parapsoriasis has expression of TOX similar to that of mycosis fungoides.
Epidemiology of Parapsoriasis
There are no accurate statistics on the incidence and frequency of parapsoriasis, but digitate dermatoses may be underreported because of the lack of symptoms and subtle presentation. Patients may underreport the frequency of large plaque parapsoriasis when it is asymptomatic and subtle. Thus, the prevalence of large plaque parapsoriasis is not known and may be greater than the reported incidence of mycosis fungoides, which is approximately 3.6 cases per million population per year.
Mortality has not been reported for small plaque parapsoriasis. Morbidity is limited to symptoms, which are minimal. For large plaque parapsoriasis, mortality may be associated with progression to MF (cutaneous T-cell lymphoma [CTCL]). The patch stage of MF represents the early stages of CTCL, and the 5-year survival rate is greater than 90%. Long-term survival is the same as that from a matched controlled population.
Small plaque parapsoriasis is associated with male predominance. The male-to-female ratio is 3:1. A slight asymmetry favoring male dominance for large plaque parapsoriasis may exist.
For both small plaque parapsoriasis and large plaque parapsoriasis, presentation most frequently is in middle age; peak incidence is in the fifth decade of life.
Onset of parapsoriasis is indolent. It develops from a few patches and becomes more visible over a protracted period of time. Additional lesions develop progressively in some individuals.
Small plaque parapsoriasis can last months to several years; the disease often resolves spontaneously.
Large plaque parapsoriasis is a chronic disorder that manifests in an indolent manner and progresses over many years, sometimes decades. It may progress to mycosis fungoides (MF), a cutaneous T-cell lymphoma (CTCL), after an indeterminate number of years.
Large plaque parapsoriasis does not enter remission without treatment.
Lesions of small plaque parapsoriasis are well-circumscribed, slightly scaly, light salmon-colored patches that measure less than 5 cm in diameter and are scattered over the trunk and extremities. Digitate pattern is a distinctive form of small plaque disease that consists of palisading elongated fingerlike patches that follow the dermatome and are most prominently displayed on the lateral thorax and abdomen. (See the images below.)
Large plaque parapsoriasis manifests as faint erythematous patches with arcuate geographic borders. Each lesion often is greater than 6 cm in diameter. Lesions are scattered on the proximal extremities and the trunk and often show a bathing-suit distribution. Surfaces of the lesions have a faint red-to-salmon color; show flaky thin scales; and have an atrophic, cigarette-paper or tissue-paper, wrinkling quality. (See the image below.)
Diagnosis of Parapsoriasis
The differential diagnosis for parapsoriasis includes the following:
A complete blood cell count with differential should be performed, and a high lymphocyte count or the presence of Sézary cells suggests mycosis fungoides/cutaneous T-cell lymphoma (MF/CTCL).
Skin biopsy with immunophenotyping analysis and gene rearrangement studies should be performed.
Histopathology of small plaque parapsoriasis shows a mild superficial perivascular lymphocytic infiltrate with a nonspecific inflammatory infiltrate of CD4+ and CD8+ T cells. However, CD4+ T cells are predominant. The epidermis may show mild spongiosis, focal hyperkeratosis, scale crust, parakeratosis, and occasional exocytosis. Often, the pattern is not diagnostic and is nonspecific. Lymphocytes are small and do not show atypical features.
In large plaque parapsoriasis, a superficial dermal inflammatory infiltrate consists predominantly of lymphocytes. Numerous lymphocytes abut the dermal-epidermal junction and single lymphocytes can be observed in the epidermis. Lymphocytes are generally small and do not show atypical nuclei. Blood vessels are dilated, and melanophages can be present. The epidermis shows flattening of the rete ridges when epidermal atrophy is prominent on clinical examination. Acanthosis of the epidermis and irregular hyperkeratosis of the cornified layer are present. In contrast to small plaque parapsoriasis, spongiosis is absent.
Gene rearrangement studies can assist in excluding MF or CTCL.
Treatment of Parapsoriasis
Parapsoriasis can be managed conservatively on the basis of symptoms, and often, topical treatment is effective.
Small plaque parapsoriasis
Small plaque parapsoriasis usually is asymptomatic. Treatment should be based on alleviation of symptoms associated with scaliness, and patients should be reassured of the benign self-limiting nature of the disease.
Emollients may be sufficient to treat scaliness; however, a trial of midpotency topical steroids (class 3-5) may lead to greater clinical responsiveness.
Phototherapy is effective in treating lesions that are widely scattered. Broad- or narrow-band UV-B can be effective and can lead to remission.[12, 13] More recalcitrant presentations can be treated with psoralen and long-wave ultraviolet radiation (PUVA).
Annual follow-up is recommended. An increase in the number of lesions, an increase in the size of lesions, or the development of induration or epidermal atrophy should prompt a repeat skin biopsy to consider a diagnosis of mycosis fungoides (MF) in evolution.
Large plaque parapsoriasis
Large plaque parapsoriasis should be treated, because treatment may prevent progression to MF (cutaneous T-cell lymphoma [CTCL]). Therapy includes mid- to high-potency topical steroids (class 2-4), topical nitrogen mustard, and topical carmustine (BCNU). Patients using topical treatment need follow-up every 2-3 months.
Phototherapy with either broad- or narrow-band UV-B or PUVA can be effective in inducing remission. Phototherapy requires an evaluation to response after every 8-12 visits or monthly.
Large plaque parapsoriasis requires closer follow-up than small plaque parapsoriasis. Follow-up frequency is determined by the treatment modality used. Follow-up every 6 months is recommended. Increasing number of lesions, increase in lesion size, or the development of induration or epidermal atrophy should prompt a repeat skin biopsy to consider a diagnosis of MF in evolution. If patients remit or do not desire treatment, follow-up is still recommended to assess for recurrence or progression.
Consult with a dermatologist specializing in cutaneous lymphoma to coordinate medical care if progression to MF (CTCL) occurs.
Administration of topical chemotherapy agents may result in development of contact dermatitis.
Small plaque parapsoriasis may persist in a stable pattern for years to decades and then resolve spontaneously. A small number of cases may progress to mycosis fungoides (MF).
Large plaque parapsoriasis remains indolent for many years. The disease may progress to cutaneous T-cell lymphoma (CTCL) with transformation of lymphocytes from benign small size to larger atypical lymphocytes. The 5-year survival rate, however, still remains high and is greater than 90%.
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