• | 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) |
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