Lymphomatoid papulosis: an update and review

Abstract

Lymphomatoid papulosis (LyP) is a benign chronic often relapsing skin condition that belongs to the CD30‐positive cutaneous lymphoproliferative disorders. LyP typically presents as crops of lesions with a tendency to self‐resolve, and morphology can range from solitary to agminated or diffuse papules and plaques to nodules or tumours. The clinical–histological spectrum can range from borderline cases to overlap with primary cutaneous anaplastic cell lymphoma (pcALCL). Histology and immunophenotype commonly show overlap with other CD30‐positive disorders and sometimes may be identical to pcALCL, making its diagnosis more difficult. Patients with LyP have an increased risk of developing a second neoplasm such as mycosis fungoides, pcALCL and/or Hodgkin lymphoma. Clinical correlation allows its proper classification and diagnosis, which is fundamental for treatment and prognosis. This review focuses on the clinical appearance, histopathological features, diagnosis, differential diagnosis and management of LyP.

Agminate papules and nodules on left lower limb

“…LyP presents as recurrent, self‐healing crops of polymorphous lesions that may include papules, papulonodules, or crusts, usually pink to violaceous, and less than 1 cm in diameter 2 . They are most commonly located on extremities, 3 followed by the trunk, and less frequently on the face and acral sites 2 . There are several clinical variants with a wide differential diagnosis.…”

“…As mentioned, LyP usually has a benign course and good prognosis. Thus, the main management strategy is an active nonintervention 2 . For widespread or symptomatic forms, most recommended treatments are potent topical corticosteroids, methotrexate, or phototherapy, although numerous therapies have been used.…”

“…For widespread or symptomatic forms, most recommended treatments are potent topical corticosteroids, methotrexate, or phototherapy, although numerous therapies have been used. Long‐term follow‐up is recommended since around 20% of patients may develop a second lymphoproliferative disorder, mainly mycosis fungoides, primary cutaneous anaplastic large cell lymphoma, and Hodgkin disease 2 …”

Frequency, risk factors and prognosis of systemic haematologic malignancies, cutaneous and other neoplasms in lymphomatoid papulosis: where are we now?

“…11 It has been reported but not confirmed that subtypes A and D of LyP have been associated with a lower risk for developing a second malignancy while subtypes B and C present a higher risk. 2 Although the histopathological subtypes have not been included in the article of Melchers et al to rule out or confirm these findings, a manuscript of the same group including a large part of these same patients showed that there are no prognostic differences between the different histological subtypes, considering in particular A and C subtypes. 11 Other prognostic markers that has been suggested as useful to predict the development of a second lymphoid neoplasm such as frequent relapses of LyP lesions, involvement of the face, an older age, the expression of fascin by CD30+ large cells, high blood levels of soluble CD30, CD25, interleukin (IL) 6, and IL-8 and a detectable T-cell clone 3 have not been highlighted by this study, and the risk factors for developing a second HM remains pending.…”

“…1 It is well known that patients with LyP have a lifelong increased risk to develop some haematologic malignancies (HM) including mycosis fungoides (the most common one), cutaneous or systemic anaplastic large cell lymphoma (ALCL), or Hodgkin lymphoma. 2 In spite of these data, the disease-specific survival of LyP patients after 5 years reaches almost 100% and the prognosis of the disease is considered excellent. 3 In this number of JEADV, Melchers et al 4 investigate the frequency and prognosis of associated malignancies in a large cohort of 504 LyP patients (i.e.…”