Chronic cutaneous lupus erythematosus, lewandowski-lutz epidermodysplasia verruciformis – case study

Abstract

Discoid lupus erythematosus (DLE) is the most common form of chronic cutaneous lupus erythematosus (80%). Only 1-2% of patients with localized DLE progress to systemic lupus erythematosus. Cutaneous lesions are most commonly located on the scalp, ears, cheeks, nose, and lips, in the form of destructive plaques with follicular prominence [1]. Lewandowski-Lutz syndrome, also known as epidermodysplasia verruciformis, is a rare genetic condition characterized by an increased susceptibility to infections with certain types of human papillomavirus (HPV), resulting in wart-like skin lesions that can disseminate, resembling tree bark. The condition is caused by mutations in the EVER1 and EVER2 genes and is inherited in an autosomal recessive manner. Dermatological conditions can present a variety of manifestations and can be associated, representing a complex and challenging field of medical practice, highlighting the complexity of diagnosis and management in such cases [2]. Aim of this article is to highlight the importance of a holistic and personalized approach in managing complex dermatosis cases, exemplified by the combination of chronic cutaneous lupus erythematosus, Lewandowski-Lutz epidermodysplasia verruciformis, and tinea corporis. By analyzing the detailed clinical presentation of the patient, we aim to underline the necessity for additional investigations and close collaboration between specialists to ensure an accurate diagnosis and effective treatment, thereby improving the quality of medical care provided. Case Presentation The patient, a 53-year-old male, presents with complaints of hyperkeratotic lesions on the fingers and toes, some with ulcerations, as well as erythematous macules with telangiectasias on an atrophic background on the nose and scalp. Physical examination also revealed other lesions on the lateral arm and back, in the form of large indurated oval plaques (9 cm in diameter), elevated, circumscribed, with a violaceous halo, as well as disseminated lesions on the trunk in the form of hypo-hyperpigmented macules with furfuraceous scaling. The disease history extends over 10 years, with a protracted course. Paraclinical findings showed elevated levels of C-reactive protein, ASLO, and ESR, as well as a positive fungal culture for Pityrosporum orbiculare. Skin biopsy revealed interface vacuolar dermatitis - consistent with the diagnosis of discoid lupus erythematosus. Based on anamnesis data, clinical examination, and paraclinical findings, the diagnosis was chronic discoid cutaneous lupus erythematosus, Lewandowski-Lutz epidermodysplasia verruciformis, and tinea corporis - pityriasis versicolor. Treatment consisted of Prednisolone 5 mg, 8 tablets/day, with subsequent gradual dose reduction, Plaquenil 200 mg, 2 tablets/day for 20 days, then 1 tablet/day for 40 days, and topical Ketoconazole shampoo. Discussion Differential diagnoses included chronic cutaneous lupus erythematosus, chilblain lupus erythematosus, epidermodysplasia verruciformis, and disseminated pityriasis versicolor. While awaiting biopsy results, management focused on symptom control and patient comfort. The treatment involved a holistic approach aimed at establishing a clear diagnosis and an appropriate treatment plan. Conclusion Complex cases of dermatosis require an individualized approach and close collaboration between specialists. Diagnosis and management of these conditions can be challenging and may necessitate additional investigations and attention to detail. Through a thorough understanding of the symptoms and clinical presentation of each case, physicians can provide optimal care and effective treatment for patients.