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Chapter 114: Lymphomatoid Papulosis

Bradley D. Kwinta; Connor J. Stonesifer; Larisa J. Geskin

LYMPHOMATOID PAPULOSIS

Therapeutic Approach

Lymphomatoid papulosis (LyP) is a cutaneous lymphoproliferative disorder. LyP typically presents on the trunk and proximal extremities and is characterized by recurrent, dome-shaped, necrotic papules or nodules occurring in cropped or generalized eruptions.1 It follows a chronic, recurrent course, with lesions that appear and spontaneously regress after several weeks with crusting, ulceration, and possible scarring.1 LyP can be classified into 6 major subtypes depending on histologic presentation.2,3 Though LyP does not confer a change in survival, patients are at risk for secondary malignancies, particularly cutaneous or nodal lymphoid malignancies.2 National Comprehensive Cancer Network (NCCN) guidelines suggest observation is the preferred first-line therapeutic approach for asymptomatic lesions. In symptomatic lesions or for patients with numerous, disseminated, or stigmatizing lesions, the therapies below can be used, though observation is still an acceptable first-line option. No therapies have been proven to alter the course of the disease or prevent secondary malignancies.2,4,5 See Table 114-1.

Table 114-1Lymphomatoid Papulosis Treatment Table
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Table 114-1 Lymphomatoid Papulosis Treatment Table
MEDICATION NAME INDICATION MECHANISM OF ACTION DOSING ADVERSE EFFECTS SUGGESTED MONITORING LEVEL OF EVIDENCE
Topical Therapy
Corticosteroids Primary treatment optiona (all lesions) Anti-inflammatory High to super-high potency if involving the trunk/extremities; low potency if involving the face. Apply BID Skin atrophy, telangiectasias, striae, acneiform eruption, perioral dermatitis None III6-7
Tacrolimus Alternative therapy Antiproliferative Tacrolimus 0.1% ointment BID Skin irritation, hyperesthesia None III8
Bexarotene Alternative therapy Antiproliferative Bexarotene 1.0% gel nightly for the first week, 2-4×/d in subsequent weeks Teratogenic, skin irritation Pregnancy test 1 wk before initiating III7,9
Imiquimod Alternative therapy Locally immunomodulatory Imiquimod 5% cream 3×/wk Skin irritation None III7,10
Nitrogen mustard Primary treatment option (extensive lesions) Cytotoxic 0.016% gel or 10-20 mg ointment daily Skin irritation, PIH None III7,11
Carmustine Alternative therapy Cytotoxic 10 mg in dilute alcohol to active lesions daily until remission; maintenance with 2-4 mg/mL in 95% ethanol to new lesions. Consider 10 mg/100 g petrolatum ointment daily Skin irritation, skin atrophy/telangiectasias, myelosuppression None III12
Methotrexate Alternative therapy Antiproliferative 2.5 mg tablet dissolved in water, applied to affected area Unknown None III13
Hexadecylphosphocholine Alternative therapy Cytotoxic hexadecylphosphocholine 60 mg/mL solution once or twice daily Skin irritation None IIB, III14
Systemic Therapy
Methotrexate Primary treatment optiona (extensive lesions) Antiproliferative 2.5-37.5 mg per week with 1 mg folic acid daily Teratogenic, GI upset, hair loss, hepatotoxicity, myelosuppression LFTs and CBC 2×/mo × 1 mo, then q4-12wk. Pregnancy test 1 wk before initiating III5-7
Isotretinoin Primary treatment option (extensive lesions) Pro-apoptotic, keratolytic 0.5 mg/kg body weight daily Teratogenic, skin dryness, nausea, hyperlipidemia, hepatotoxicity Baseline and periodic LFTs and lipid panel. Pregnancy test before initiating, monthly, and 1 mo after discontinuation III15
Bexarotene Primary treatment option (extensive ...

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