PLEVA: Mucha-Habermann disease.
1.5.2 Pityriasis Lichenoides
Synonyms
Epidemiology
PLEVA: Children and adolescents.
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PLC: young adults M>F.
Definition
Acute (PLEVA), subacute or chronic erythemato-squamous or -papulo-necrotising (PLEVA) diseases; nosologic relationship to vasculitis and to lymphomatoid papulosis (LyP; spontaneously regressive papular-necrotic form of CTCL).
Aetiology & Pathogenesis
Unknown. Viral (Epstein-Barr and other viruses) and other infectious agents (upper respiratory tract) discussed as trigger factors.
Signs & Symptoms
PLC: Lenticular, erythematous, macular or papular lesions with scaling.
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PLEVA: Disseminated, small papules and plaques with scaling or superficial crust, sometimes hemorrhagic. Development to necrotic lesions and healing with varioliform scarring.
Localisation
Predominantly trunk> extremities
Classification
Variable: acute (PLEVA), subacute or chronic.
Laboratory & other workups
Looking for infections (upper respiratory tract), blood picture, biopsy.
Dermatopathology
Depending on acuity
- PLEVA: Focal hyperparakeratosis; apoptosis of single keratinocytes; interface-dermatitis (edema in the epidermal-dermal junction with vacuolization of basal keratinocytes); lymphocytic (wedge shaped) infiltrate in the dermis and extravasation of erythrocytes.
- PLC: Like in PLEVA, but less prominent.
Course
Weeks or months; maximum of 2 years.
Complications
None.
Diagnosis
Clinical and histologic findings.
Differential diagnosis
Psoriasis guttata, secondary syphilis, varicella, vasculitis, drug reaction.
Prevention & Therapy
No prevention. Skin care, prevention of superinfection; erosive lesions may be treated topically with combined antibiotic-glucocorticosteroid (grade I) cream.
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