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[Epidermodysplasia verruciformis (2 cases). Immunological study (author's transl)]
Annales De Dermatologie Et De Venereologie
|July 1, 1980
Summary
Epidermodysplasia Verruciformis (EDV) patients with Human Papilloma Virus (HPV) type 5 infection developed pityriasis-like lesions. Immune defects and HPV-5 are implicated in EDV, suggesting retinoid therapy for prevention.
Area of Science:
- Dermatology
- Virology
- Immunology
Background:
- Epidermodysplasia Verruciformis (EDV) is a rare genetic disorder characterized by susceptibility to specific human papillomavirus (HPV) types.
- Patients with EDV often develop widespread, persistent HPV infections, increasing the risk of skin malignancies.
Observation:
- Two EDV patients presented with flat, wart-like lesions and pityriasis-like macules.
- Human Papilloma Virus (HPV) type 5 was identified as the causative agent for the pityriasis-like lesions in both patients.
- Immunological assessments revealed elevated IgE, decreased IgM in one patient, and depressed mitogenic responses to PHA and ConA.
Findings:
- HPV type 5 infection was directly linked to the pityriasis-like lesions and subsequent bowenoid transformation (a precursor to skin cancer) in the EDV patients.
- Cell-mediated immunity defects, including decreased T-cell percentages and impaired mitogenic responses, were observed.
- Treatment with aromatic retinoid (Ro 10-9359) normalized the mitogenic response in one patient.
Implications:
- Both the specific HPV type (HPV-5) and underlying immune deficiencies play critical roles in the pathogenesis of EDV and its malignant progression.
- Long-term preventive treatment with retinoic acid derivatives may be beneficial for EDV patients infected with HPV type 5.
- Understanding the interplay between viral factors and host immunity is crucial for managing EDV and preventing cancer development.