Autosomal dominant epidermodysplasia verruciformis: a clinicotherapeutic experience in two cases.
Epidermodysplasia verruciformis (EV) is a rare genodermatosis characterized by a unique susceptibility to cutaneous infection by a group of phylogenetically related human papilloma viruses (HPVs). These patients show a defect in cell-mediated immunity specific toward the causative HPVs that lead to lifelong disease. The defect is usually inherited as autosomal recessive trait and presents clinically with plane warts, pityriasis versicolor-like lesions and reddish verrucous plaques. Dysplastic and malignant changes in the form of actinic keratoses, Bowen's disease and squamous cell carcinoma (SCC) are common but metastasis occurs rarely. A totally effective treatment against EV is as yet highly desirable. Two siblings having autosomal dominant EV presented with multiple actinic keratoses in addition to classic lesions. One of them had also developed well-differentiated SCC over forehead with metastases to regional lymph nodes. They were treated with combination of excision of small malignant/premalignant lesions, topical 5-flurouracil and sun protection. Additionally, elective excision/grafting of large SCC was performed after chemotherapy/radiotherapy in patient with metastatic SCC. Oral acitretin (25 mg/day) was of benefit in the other patient. Overall clinicotherapeutic experience in both the patients is discussed here.
Vohra
Surbhi
S
Department of Dermatology, Venereology & Leprosy, Indira Gandhi Medical College, Shimla, Himachal Pradesh, India.
Sharma
Nand Lal
NL
Shanker
Vinay
V
Mahajan
Vikram K
VK
Jindal
Nidhi
N
D000328:Adult; D000970:Antineoplastic Agents / Q000627:therapeutic use; D002294:Carcinoma, Squamous Cell / Q000235:genetics / Q000556:secondary / Q000628:therapy; D003131:Combined Modality Therapy; D004819:Epidermodysplasia Verruciformis / Q000235:genetics* / Q000473:pathology* / Q000628:therapy; D005192:Family Health; D005260:Female; D005799:Genes, Dominant; D006801:Humans; D008297:Male; D015580:Mohs Surgery; D035781:Siblings; D012878:Skin Neoplasms / Q000235:genetics / Q000473:pathology / Q000628:therapy; D055815:Young Adult
D000970:Antineoplastic Agents