Clinical Reference / Clinical Atlas / Lymphomatoid Papulosis

Lymphomatoid Papulosis

Definition

Macaulay first coined the diagnostic term “lymphomatoid papulosis” (LyP) in 1968, although the disease process was first published more than a decade earlier by Dupont and colleagues under the moniker “histiomonocytic reticulosis.” LyP is a rare primary cutaneous CD30+ lymphoproliferative disorder of unknown etiology, estimated to affect approximately 1.2-1.9 individuals per million. LyP is typified by a chronic and recurrent eruption that consists of grouped or disseminated papules and circumscribed nodules that either eventuate to ulceration, healing with a varioliform scar, or spontaneously regress over a few weeks, healing with dyspigmentation. LyP is composed of episodic flares of individual lesions that come and go over weeks to months, often repeating for many years. The disease may eventually enter remission, the culmination of an unpredictable clinical course, or it may smolder indefinitely for years or decades, all the while portending an excellent prognosis in terms of mortality.