Department of Dermatology and Venereology, University Hospital Hassan II, Route Sidi Hrazem, Faculty of Medicine and Pharmacy, Sidi Mohamed Ben Abdellah University, Fez, Morocco
Received Date: 21/02/2023; Published Date: 21/04/2023
*Corresponding author: Hashas Fatima Zahra, Department of Dermatology and Venereology, University Hospital Hassan II, Route Sidi Hrazem, Faculty of Medicine and Pharmacy, Sidi Mohamed Ben Abdellah University, Fez, Morocco
Epidermodysplasia verruciformis is a rare autosomal recessive genodermatosis characterized by immunogenetic susceptibility to infection by a specific group of human papillomaviruses and the frequency of associated skin carcinomas. We report a new observation of a 33-year-old man.
Epidermodysplasia verruciformis, also known as Lewandowsky-Lutz syndrome or tree man disease is a rare genetic skin disorder autosomal recessive genodermatosis disorder. It is characterized by abnormal susceptibility of the skin coating to human papillomaviruses (HPVs). which presents with hypo- or hyperpigmented macular lesions, pityriasis versicolor-like lesions, and an early tendency to transform into skin cancer especially on the sun-exposed areas.
A 33-year-old farmer, operated for a left temporal squamous cell carcinoma, was referred to Our dermatology department for the management of multiple asymptomatic skin lesions over the face, neck, chest, and upper arm. history included the notion of first-degree consanguinity. In addition, he was not taking any immunosuppressive medication and had no similar cases in his siblings. The skin examination showed pityriasis versicolor like macules on the trunk and arms dating back to childhood, whose histological and immunohistochemical study consisted with epidermodysplasia verruciformis, finely scaly erythematous patches on the face and the back of the hands histologically consistent with Bowen's disease, as well as actinic keratoses, multiple seborrheic keratoses on the face and warts on the hands.
There were no other suspicious lesions on the Clinical examination and the immunodeficiency test were negative. The search for Human Papillomavirus is in progress. The therapeutic management consisted of photodynamic therapy on Bowen's patches and actinic keratoses of the face, followed by a low-dose retinoid-based background treatment.
Figure 1: Hypochromic macules. pityriasis versicolor like on the trunk.
Figure 2: Erythematous scaly plaques histologically corresponding to Bowen's disease.
Figure 3: Clinical and dermoscopic appearance of wart on the back of the foot.
Epidermodysplasia verruciformis is a rare condition of usually autosomal recessive transmission characterized by the persistent presence of human papillomavirus group beta in the skin. There are two main types of epidermolysplasia verruciformis: a typical and an atypical in immunocompromised patients [1]. Our patient is from a 1st degree consanguineous marriage and does not have immunodepression. The cutaneous lesions begin in childhood around the age of 4 - 8 years and classically associate disseminated flat warts and scaly depigmented or pigmented macules resembling pityriasis versicolor as in the case of our patient [2,3]. Dysplastic lesions and cutaneous cancers condition the prognosis of this pathology which depends on the oncogenic potential of certain types of human papillomavirus, in particular HPV5 and HPV8, which affect 90% of patients. It occurs most often in clair phototype in photo exposed areas after the age of 30 years and include actinic keratoses, Bowen's disease, basal cell carcinomas and squamous cell carcinomas.
Several treatments have been tried (retinoids, interferon, cimetidine) with little or no reproducible success [4-6] .
Management is essentially based on sun protection, assiduous clinical surveillance and rapid excision of any lesion in the process of carcinomatous degeneration.
Epidermodysplasia verruciformis is a rare genodermatosis with risk of degeneration, it should be considered in front of pityriasis versicolor like lesions with precancerous lesions or cutaneous cancers.