INTRODUCTION
Epidermodysplasia verruciformis (EV) is a rare genodermatosis
associated with persistent HPV susceptibility and the subsequent development of generalized verrucae
and flat warts with brown to reddish tinea versicolor-like lesions
commonly localized to the face, extremities, back, and in other sun exposed areas. These lesions begin during childhood and progressively increase in size and severity with 30-60% of patients experiencing cutaneous malignant transformations by the fourth decade.1 HPV genotypes 5 and 8 account for more than 90% of HPV associated malignancies.1 Several loci have been identified which suggests a dysfunction in the cell-mediated
immunity or a dysregulation in intracellular zinc transport.1,2 Current medical therapies have been aimed at stimulating the immune response and slowing the development and progression
of cancers with medications like imiquimod, retinols, cimetidine,
and 5- fluorouracil. These treatments can produce systemic side effects, require long-term application, and lesions
often reoccur with poor results upon discontinuation.3-5
The current treatment therapies for EV are not optimal. Ingenol mebutate, a medication recently reported as an effective treatment for actinic keratoses, is a potential candidate for treating EV.6 We report a case of imiquimod resistant EV with significant clinical improvement
after application of a 3-day course of ingenol mebutate.
CASE
A 38 year-old female with no prior medical history presented with multiple annular lesions/plaques on the head, torso, and
lower extremities with erosions and ulcerations localized to the face. The patient noted the lesions began 5 years ago and progressively worsened particularly along sun exposed areas. The patient was unemployed for several years and reported no previous radiation or chemical exposures. Her parents are non-consanguineous and there is no family history of similar skin lesions or cutaneous malignancies. Initial dermatological
examination revealed a 2 x 2.5 cm ulceration with pearly borders on the right scalp, multiple scattered hyperpigmented and crusted papules and erosions on the forehead, and several
discrete pink papules on bilateral knees, and upper back (Figure 1).
Multiple shave biopsies were taken for histologic examination.
Two biopsies from the right scalp and forehead were invasive SCC (Figure 2). A biopsy from the left thigh was a benign verruca with characteristic histologic changes of EV. Additional biopsies confirmed SCC in situ of the left forehead and left lateral hairline with associated changes of EV (Figure 3). The patient was consulted on minimizing sun exposure and advised to regularly apply UV protection. The two invasive
SCC were surgically excised. Medical treatment with 5% Imiquimod (Aldara) 5 times a week for 6 weeks was begun with minor improvement.
The patient returned three months later for additional biopsies of lesions on the face including a basal cell carcinoma (BCC) on the forehead, SCCIS on the right zygoma, and invasive
