Documente Academic
Documente Profesional
Documente Cultură
Aurora Parodi a
a
DISSAL, Department of Dermatology, IRCCS AOU San Martino-IST, University of Genoa, Genoa, and
b
Department of Health Sciences, University of Milano-Bicocca, Monza, Italy
Classic sporadic HHV-6/7 trunk and limbs 12 – 90 16 ≥69 parakeratosis, spongiosis 45 days bed rest
systemic (epidermis); extravasated
reactivation red blood cells, lymphocyte
infiltrate (dermis)
Relapsing relapsing HHV-6/7 trunk and limbs 0 14 100 (less parakeratosis, spongiosis 15 days acyclovir
systemic (lesions reduced severe than (epidermis); extravasated
reactivation in size and the classic red blood cells, lymphocyte
number compared form) infiltrate (dermis)
to the classic
form)
Persistent persistence of trunk and limbs 75 75 92 parakeratosis, spongiosis >12 weeks acyclovir
HHV-6/7 plasma (epidermis); extravasated
viremia red blood cells, lymphocyte
infiltrate (dermis)
Pediatric longer activity of trunk and limbs 58 35 48 parakeratosis, spongiosis 16 days bed rest
HHV-6/7 infection (epidermis); extravasated
(recent primary red blood cells, lymphocyte
infection) infiltrate (dermis)
Pregnancy HHV-6/7 trunk and limbs: 50 16 ≥69 (more parakeratosis, spongiosis 45 days bed rest and
reactivation and widespread lesions severe if PR (epidermis); extravasated (8 – 12 weeks close follow-
their possible if PR develops develops red blood cells, lymphocyte if PR starts up
intrauterine within 15 weeks of within infiltrate (dermis) within
transmission gestation 15 weeks of 15 weeks of
gestation) gestation)
PR-like reaction to a drug/ trunk, limbs, face: 0 50 0 interface dermatitis and 2 weeks after drug
eruption vaccine diffuse and eosinophils discontinu- withdrawal
confluent lesions ing the drug
number, distribution, site, severity of symptoms, and years, without statistically significant variation between
course. Overall, the previous classifications of PR are sexes [5, 6]. Although a higher prevalence in cold seasons
mainly based on its atypical morphological features rather has been claimed [7, 8], controversial opinions remain
than on the pathogenetic mechanisms that underlie their [9]. In our experience of 613 consecutive PR patients re-
different presentations. Notably, most of the morphologi- cruited between January 2003 and December 2014 at the
cally atypical forms follow a course amenable to the classic Department of Dermatology, University of Genoa, Ge-
form. We therefore proposed a simplified and compre- noa, Italy, PR occurs uniformly throughout the year.
hensive classification of the PR variants, including the The typical PR begins in 12–90% of cases [17] with a
atypical forms, based on the differences in pathogenesis, solitary patch (HP), which is a medallion-like erythema-
clinical features, and course of the disease (table 1). tous patch with slightly elevated finely scaling borders
and a paler and slightly depressed center. It usually occurs
on the trunk – more rarely on the limbs – and progres-
PR Classification sively enlarges, reaching 3 cm or more in diameter. It re-
mains isolated for about 2 weeks, after which the general-
Classic PR ized eruption brusquely develops. This secondary erup-
The Typical Eruption tion is characterized by patches that are similar to the
The prevalence of classic PR (CPR) has been estab- initial one but smaller and symmetrically oriented with
lished at 1.3% [10], but it is probably underestimated be- their long axes along the cleavage lines (Christmas tree
cause of the occurrence of atypical forms and the number distribution). Oral lesions, occurring in 16% of Caucasian
of patients who are misdiagnosed by nondermatologists. patients [5], can be categorized into 5 groups: punctuate
The age of maximum incidence is between 10 and 35 hemorrhages, erosions or ulcerations (which are the most
Prodromal symptoms – present (coryza, fever, generalized muscle pain, fatigue, malaise, arthralgias,
gastrointestinal symptoms); transient/barely noticeable; absent or unreliable history from patient
HP – absent; obscure; single; multiple; gigantic; HP only as manifestation; the disease does not
progress beyond the HP
Number – few lesions (less than 5, oligo-lesional); many lesions; suberythrodermic; erythroderma
due to PR
Distribution – relatively unilateral; absolutely unilateral; localized; regional; mucosal (mainly oral and
genital); acral; PR inversus (PR mainly involving many flexures); shoulders and hips (limb-girdle PR); CPR
fewer but larger lesions localized to axillae and groins (pityriasis circinata et marginata of Vidal); (typical and
actinic (photo-distributed); photo-spared atypical
eruptions)
Axis 3 – size, morphology, and orientation of lesions
Orientation – typical, i.e. along the lines of skin creases (lines of langer); atypical, i.e. not along lines
of skin creases: scattered; Blaschkoid (along lines of Blaschko); segmental (along a dermatome)
Clinical course – too short (less than 2 weeks); too long (more than 6 months)
Recurrent PR
PR in pregnancy
PR-like eruption
Fig. 1. Comparison between the previous classification of PR PR form. PR in children usually lasts for about 12 days (short
(main body) and our classification system (arrows and panels). course). PR in pregnancy and PR-like eruptions do not fit into
CPR, in its typical and atypical eruptions, includes all the mor- any of the previously cited variants of the exanthem. PR in preg-
phological variants of the previous classification. Considering the nancy may be a CPR, or it may have a particularly aggressive
course of the disease, we introduced a recurrent and a persistent course.
genesis and in the clinical and laboratory features. Re- HHV-6/7 infection has occurred more recently and, ac-
markably, the shorter course of the eruption compared to cordingly, the virus activity is still intensive. PR in preg-
adults is not explained by an equally shorter persistence of nancy can be included in CPR, or it can have a more ag-
HHV-6/7 plasma viremia. Conversely, in children, the gressive course with unusually widespread skin lesions,
HHV-6/7 average plasma viremia persists even after re- long duration, and severe constitutional symptoms. These
covery. Moreover, both during the acute phase and after atypical forms require a closer follow-up since they may
resolution, plasma viremia are very much higher in chil- be associated with a prolonged viral reactivation in the
dren than in adults [15], probably because the primary plasma, eventually determining a HHV-6 intrauterine
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