Parapsoriasis Overview of

Parapsoriasis
Author: Henry K Wong, MD, PhD; Chief Editor: Dirk M Elston, MD
Updated: Jul 02, 2014

Overview of Parapsoriasis
Parapsoriasis describes a group of cutaneous diseases that can be characterized by
scaly patches or slightly elevated papules and/or plaques that have a resemblance
to psoriasishence 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-cellpredominant 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.[1] 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.[5]
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.[6] 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.[7]

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.
Human herpesvirus type 8 has been 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.[8]

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. Large plaque parapsoriasis may
be greater than the reported incidence of mycosis fungoides (MF), 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.[9]
Small plaque parapsoriasis is associated with male predominance. The male-tofemale 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.

Clinical Presentation
Patient history
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.

Physical examination
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.[10] (See the images below.)

Small plaque parapsoriasis.

Small plaque parapsoriasis.

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.)

Large plaque parapsoriasis.

Diagnosis of Parapsoriasis
The differential diagnosis for parapsoriasis includes the following:

Contact Dermatitis, Allergic

Cutaneous T-Cell Lymphoma

Nummular Dermatitis

Pityriasis Alba

Pityriasis Lichenoides

Pityriasis Rosea

Psoriasis, Guttate

Syphilis

Laboratory studies
A complete blood cell count with differential should be performed, and a high
lymphocyte count or the presence of Szary cells suggests mycosis
fungoides/cutaneous T-cell lymphoma (MF/CTCL).

Skin biopsy
Skin biopsy with immunophenotyping analysis and gene rearrangement studies
should be performed.

Histologic findings
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 dermalepidermal 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. [11, 12] 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 midto 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.[13]
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.

Consultations
Consult with a dermatologist specializing in cutaneous lymphoma to coordinate
medical care if progression to MF (CTCL) occurs.

Complications
Administration of topical chemotherapy agents may result in development of
contact dermatitis.

Prognosis
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%.

References
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Parapsoriasis en plaques: its potential for progression to malignant
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lymphoma, even an 'abortive' one, it must be mycosis fungoides!. Arch
Dermatol. 1996 May. 132(5):562-6. [Medline].
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8. Kreuter A, Bischoff S, Skrygan M, Wieland U, Brockmeyer NH, Stcker

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