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)