Parapsoriasis Flashcards
What decade of life does SPP peak?
5th
sex predominance?
male
what can be seen in superficial dermis? Of small plaque
sparse lymphoid infiltrates of CD4 with T cell clonality
histology of small plaque parapsoriasis ?
mild nonspecific spongiotic dermatitis with focal parakeratosis
-clinical feature of small plaque parapsoriasis ?
Patches with fine scales (טלאים עם קשקש עדין)
patches description?
round to oval, <5 cm, slightly erythematous, fine scale, yellow hue
most typical distinct presentation of small plaque parapsoriasis?
digitate dermatosis”-elongated finger like symmetrical patches on flanks, 10 cm or more
nature of disease of small plaque parapsoriasis ?
chronic, generally asymptomatic or pruritic
course of disease?
early- wax and wane. Late- persist and progress to be extensive
distribution of small plaque parapsoriasis ?
widespread or limited
what is special about the limited form of SPP?
sun protected areas
progression to MF? ( SPP)
very rare
how is SPP different with pityriasis rosea?
herald patch, spontaneous resolution within few months
treatment of SPP?
can be only followed, also topical CS and NBUVB
does LARGE PLAQUE PARAPSORIASIS have a risk to progress to MF?
yes, high risk
clinical features of large
erythematous patches on sun protected areas
how do you treat large plaque parapsoriasis?
- same as early MF
PITYRIASIS LICHENOIDES - population prevalence?
more in pediatric
pitreyasis lichenoides and sex ? is it common more among males or females?
male predominance
with what is lichenoides associated with?
infections and drugs
infections associated with PLEVA?
- viral (VZV, HIV, EBV,PVB19)
drugs associated with PLEVA?
estrogen-progesteron, infliximab, adalimumab, statins
what can explain the association of PL with lymphoproliferative disorders such MF?
T cell clonality and presence of CD 30 cells in some variants
what important evaluation do patients with PL and aberrant T cell phenotype have to undergo?
clinicopathological correlation, molecular studies of T cell clonality
clinical features? pitryasis lichenoides
recurrent crops of spontaneously regressing erythematous to purpuric papules
what is the course of PLC (PITYRIAIS LICHINOIDES CHRONICA) and the status post?
indolent course, regressing over weeks to months. May leave hypopigmented macules
what may be the presenting complaint in dark skin patients with PLC?
hypopigmented macules
what lesions we see in PLEVA and what is the course?
asymptomatic crusts, ulcers, occasionally vesicles or papules, resolve within weeks
in what way do they heal if dermal damage is extensive?
varioliform scars
is PL usually systemic or confined to skin?
confined to skin
what is febrile ulceronecrotic mucha haberman disease?
severe variant of PLEVA where large confluent necrotic lesions with mucosal, gastro, pulmonary involvement
what is important in predicting outcome of disease?
distribution of skin lesions
what is the usual course in children?
more persistent and widespread distribution
what is the relationship btw the course of disease and distribution?
shorter with diffuse distribution and longer with peripheral distribution
- what is the histology in an acute lesion? of PL
dense wedge shaped lymphocytic infiltrate in adnexal epithelia (eccrine,follicular) Epidermal parakeratosis , erythrocyte extravasation
what is the pathology of PL?
perivascular interface dermatitis
DD for PLC?
guttate psoriasis, LP, PR, 2nd syphilis, drug eruptions, lymphomatoid papulosis
what is the histology in a chronic lesion? of PL
parakeratosis, mild infiltrate, keratinocyte necrosis
DD for PLC?
-A- guttate psoriasis, LP, PR, 2nd syphilis, drug eruptions, lymphomatoid papulosis.
WHAT IS THE THE DIFFERENTIAL FOR SMALL PLAQUE PARAPSORIASIS?
Pityriasis rosea
Drug eruption, in particular pityriasis rosea-like
Pityriasis lichenoides chronica
Psoriasis, including guttate
Mycosis fungoides
Secondary syphilis
Nummular dermatitis
WHAT ARE THE TREATMENT FOR SMALL PLAQUE PARAPSORIASIS ?
Therapeutic approaches are based primarily on uncontrolled case series, case reports, or anecdotal evidence.
Need to reassure them that the risk for MF is low.
Patients may be followed without treatment if they prefer.
other options include: topical corticosteroids and NBUVB (but reoccurrence is high).
Patients may be followed without treatment if they prefer.
what is pitryasis lichenoides ?
uncommon disorder that encompasses:
-pityriasis lichenoides et varioliformis acuta (PLEVA)
- the febrile ulceronecrotic Mucha–Habermann disease (FUMHD) variant of PLEVA
- pityriasis lichenoides chronica (PLC).
These acute and chronic forms of pityriasis lichenoides exist on a disease spectrum with variable presentations that can pose diagnostic and therapeutic challenges.
They are papular, often clonal T cell disorders that may rarely be associated with MF.
which population is affected most by pitryasis lichenoides?
is more prevalent in the pediatric population,
it affects patients in all age groups, races, and geographic regions.
There is a male predominance.
what is the pathogenesis of Pitryasis lichenoides?
Pathogenesis is unkown.
There is some evidence to support PLEVA being associated with viral infections, especially varicella–zoster virus, HIV, parvovirus B19, and Epstein–Barr virus.
Additional reports have described its association with medications (e.g. estrogen–progesterone, infliximab, adalimumab, statins), radiocontrast dye, and vaccines
what are the clinical features of pitryasis lichenoides? talk about PLEVA and its manifestation
Pityriasis lichenoides presents as recurrent crops of spontaneously regressing erythematous to purpuric papules . The acute form (PLEVA) and the chronic form (PLC) exist on a disease continuum.
But Many patients have intermediate or mixed manifestations,
In patients with PLEVA: individual lesions develop crusts, ulcers, and occasionally vesicles or pustules, which may heal with varioliform scars if dermal damage is extensive.
Lesions are usually asymptomatic and typically resolve within weeks. Disease manifestations are confined to the skin…. except rarely, when acute lesions are associated with malaise, fever, lymphadenopathy, arthritis, and/or bacteremia.
The term febrile ulceronecrotic Mucha–Habermann disease (FUMHD) has been used to refer to such severe variants in which large, confluent necrotic skin lesions as well as mucosal, gastrointestinal, and pulmonary involvement can be observed. In the latter group, an associated mortality rate of up to 15% has been reported
what about PLC and its clinical manifestation?
In PLC, the papules are erythematous to red–brown and scaly.
They pursue a more indolent course, regressing over weeks to months. When these lesions subside, there may be residual hypopigmented macules; the latter are more obvious in individuals with darker skin phototypes and may be the presenting complaint.
How is the course of Pitryasis lichenoides?
Pityriasis lichenoides can resolve spontaneously after weeks to months or it may pursue a chronic relapsing course, sometimes interspersed with long periods of remission.
חשיבות הפיזור !!!
Some studies have suggested that the distribution of skin lesions is more important than their acute or chronic nature in predicting outcome
*diffuse distribution - shorter course
* peripheral distribution - longest clinical course.
in children its a bit different : more persistent course and widespread distribution of lesions :(
