By Disease Name > Pityriasis lichenoides et varioliformis acuta

Pityriasis lichenoides et varioliformis acuta

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acute form of "pityriasis lichenoides" (see below)
AKA Mucha-Haberman disease, PLEVA
recurrent crops of variably purpuric papules that develop crusts, ulcers, vesicles or pustules
before spontaneously regressing over few weeks
acute lesions à may result in smallpox-like (varioliform) scars

 

PLEVA ddx:

in children:  Gianotti-Crosti syndrome, varicella
lymphomatoid papulosis:  a countable number of grouped papular or self-healing nodular lesions
PLC: the chronic form of "pityriasis lichenoides"

 

 

"Pityriasis Lichenoides"

reccurrent crops of spontaneously regressing papules
therefore polymorphous, lesions of different age
all age groups, but more common in first few decades
T-cell gene rearrangement demonstrate clonal populations of T-cells in select cases
but shows no significantly documented association with malignant lymphoma
PLC may regress in months or persist for years (PLEVA usually has a shorter duration)
spectrum of disease with a variable clinical presentation ranging from mostly acute lesions to mainly chronic lesions

 

treatment:

phototherapy provides the best therapeutic response
high dose tetracycline *not in children < 8years old, nor in pregnant women (2g/day) or erythromycin  X 2 months

 

histology:

(CD8 lymphocytes predominate in PLEVA;  CD4 in PLC)