Table 5.

Recommendations for the treatment (standard therapies) of CD30⁺ LPD

PCALCL			LYP
Solitary or grouped lesion(s)	Multifocal lesions	Extracutaneous spread	Localized/regional or few lesions*	Numerous and/or generalized lesions
SE	Methotrexate	Single or multiagent chemotherapy‡	Observation	Observation
RT	Alternatives: retinoids, interferon†		Phototherapy§	Phototherapy§
			Topical steroids	Methotrexate
				Topical steroids
				Alternatives†: retinoids, interferon

^*For larger (defined as > 2 cm in diameter) and persistent (defined as duration of lesion > 12 weeks) lesions, SE or RT may represent alternatives.

^†These therapies are of low-level evidence other than expert opinion.

^‡In cases of skin and only local node involvement in PCALCL, one could consider addition of local nodal radiation ⁴

^§PUVA is best documented. Alternatively, treatment with other types of phototherapy (eg, UVB-narrow band) can be tried (evidence level 5).