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Chapter 98: Epidermal Nevi

Daniel M. Klufas; Jeffrey L. Sugarman

EPIDERMAL NEVI

Therapeutic Approach

Epidermal nevi (EN) comprise a heterogenous group of benign, hamartomas of the skin. Clinical appearance varies largely based on cell or structure of origin, which includes keratinocytes, the pilosebaceous unit, apocrine or eccrine glands, or smooth muscle cells. While some EN may occur as solely cutaneous lesions, they can be associated with a variety of extracutaneous manifestations involving numerous organ systems, particularly the central nervous system, skeletal system, and ocular system. The occurrence of EN in conjunction with extracutaneous findings is best umbrellaed under the term “epidermal nevi syndrome” (ENS). Management must be individually tailored and may be aimed at improving cosmesis, improving quality of life, reduction of tumor potential, reducing functional disability and potential life-threatening sequalae. See Table 98-1.

Table 98-1Epidermal Nevus Treatment Table
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Table 98-1 Epidermal Nevus Treatment Table
MEDICATION NAME INDICATIONb MECHANISM OF ACTION DOSING ADVERSE EFFECTS SUGGESTED MONITORING LEVEL OF EVIDENCE (REFERENCE)
Topical Therapy
Retinoids KEN, ILVEN, PEODDN, NC Anti-inflammatory; ECM repair properties Tretinoin cream 0.1%; tazarotene gel 0.1% (+ photodynamic therapy) for PEODDN Skin irritation None IV1–3
Retinoid + antibiotic NC Anti-inflammatory + antimicrobial Tretinoin 0.025% gel + clindamycin 1% gel Skin irritation None IV4
Keratolytics KEN, NC Softening and shedding of stratum corneum Ammonium lactate 12% once daily (NC), salicylic acid Skin irritation None IV5,6
Lovastatin + cholesterol CHILD Inhibition of HMG CoA reductase/cholesterol synthesis (lovastatin) and cholesterol substitution for normal stratum corneum (cholesterol) 2% lovastatin/2% cholesterol lotion twice daily None None III7
Topical or intralesional steroids ILVEN Anti-inflammatory Variable—aimed to treat pruritus Skin atrophy None IV8
Crisaborole ILVEN PDE-4 enzyme inhibitor 2% ointment twice daily Skin irritation None IV9
Synthetic vitamin D derivatives NC, ILVEN Inhibition of cell proliferation and induction of cell differentiation Calcipotriene, calcipotriene (+tazarotene for NC)   None IV6,10
Systemic Therapy
Oral retinoid ILVEN Regulation cell proliferation, differentiation, and apoptosis Acitretin 25 mg twice daily Xerosis, hypertriglyceridemia, teratogenicity

CBC + diff, CMP, lipid panel, bHCG, liver function tests

Monthly × 3 mo then q3mo

 IV11
Burosumab CSHS Anti-FGF23 monoclonal antibody 0.3 mg/kg every 3 wk Hyperphosphatemia PO4 serum level every 3 wk several days before dosing, then once stable q3mo IV12
Miransertib (ARQ 092) PS Allosteric pan-AKT inhibitor 5 mg/m2/d Dry mouth, mucositis, pharyngitis, sinus tachycardia, headache, pain, rash, elevated liver enzymes

CBC + diff, CMP, lipid panel, urinalysis, EKG, echocardiogram

Monthly × 3 mo, then q3mo

 IIB13,14
Sirolimus CLOVES, PROS mTOR inhibitor 0.6 mg/m2 sirolimus twice daily, which was then titrated to reach a target level of 5–12 ng/mL; 0.8 mg/m2 per dose twice daily, with pharmacokinetic-guided target serum trough levels of 10-15 ng/mL Hypercholesterolemia, hypertriglyceridemia, hyperkalemia, transaminases, mouth sores, GI upset, thrombocytopenia, infections (cellulitis, abscess, pneumonia, MRSA-bacteremia, CMV)

Complete blood count (CBC), liver ...

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