Detection of Borrelia burgdorferi sensu stricto and Borrelia garinii DNAs in patient with Hyperkeratosis lenticularis perstans (Flegel disease)

Abstract

Determination of the causative agent of erythema-like skin lesions in case of nonspecific superficial perivascular dermatitis was supported by histological examination and led to the latter diagnosis of Hyperkeratosis lenticularis perstans (Flegel disease) in patient. The presence of antibodies against Borrelia burgdorferi in patient serum was confirmed by a routine ELISA method and verified by Western blot technique. Skin biopsy and blood specimens were analyzed by PCR and multilocus sequence analysis (MLSA). Western blot method revealed IgG antibody response against two specific antigens, 17 and 83 kDa proteins. The recombinant test detected IgG antibody response against p100 and p41 antigens. The sequence analysis of amplicons from the selected genomic loci obtained from skin biopsy and serum samples revealed the presence of two species from B. burgdorferi sensu lato complex as a co-infection in this patient-B. burgdorferi sensu stricto (s.s.) and Borrelia garinii.

Widespread and Long-Enduring Hyperkeratosis Lenticularis Perstans (Flegel’s Disease): Clinico-Pathological and Dermoscopic Features of a Rare Presentation

“…Flegel’s disease (FD), first described in 1958, is a rare cutaneous disorder that usually arises in the fourth/fifth decade of life, often confused with other cutaneous keratotic pathologies. To the best of our knowledge, about 80 articles dealing with FD cases have been reported in the literature [ 1 , 2 , 3 , 4 , 5 , 6 , 7 , 8 , 9 , 10 , 11 , 12 , 13 , 14 , 15 , 16 , 17 , 18 , 19 , 20 , 21 , 22 , 23 ]. According to the literature, there is a slight prevalence of the female gender with a ratio 1.6:1 and a median age of 50 years, ranging between 18 and 82 years [ 1 , 2 , 3 , 4 , 5 , 6 , 7 , 8 , 9 , 10 , 11 , 12 , 13 , 14 , 15 , 16 , 17 , 18 , 19 , 20 ].…”

“…To the best of our knowledge, about 80 articles dealing with FD cases have been reported in the literature [ 1 , 2 , 3 , 4 , 5 , 6 , 7 , 8 , 9 , 10 , 11 , 12 , 13 , 14 , 15 , 16 , 17 , 18 , 19 , 20 , 21 , 22 , 23 ]. According to the literature, there is a slight prevalence of the female gender with a ratio 1.6:1 and a median age of 50 years, ranging between 18 and 82 years [ 1 , 2 , 3 , 4 , 5 , 6 , 7 , 8 , 9 , 10 , 11 , 12 , 13 , 14 , 15 , 16 , 17 , 18 , 19 , 20 ]. The median duration of the disease before reaching the diagnosis is reasonably long, with a median of eight years.…”

Hyperkeratosis lenticularis perstans (Flegel’s disease): our experience and review of the literature

“…No clear data are available about trigger of HLP. According to some authors, HLP may be triggered by ultraviolet radiation, 27 and a single case report verified the presence of B. burgdorferi DNA in a patient with histologically confirmed diagnosis of FD, suggesting the possibility that this spirochete could be a trigger for HLP 28 . Some authors supposed a relationship between genes participating in oncogenesis and HLP genes.…”

“…By contrast, in older lesions because of the scarcity of inflammatory infiltrate, corticosteroids are ineffective. 5‐fluorouracil (5‐FU) cream 5%: the most consistent results have been reported with topical 5‐FU cream 5%. It has been suggested that the 5‐FU provokes a strong inflammatory response, leading to the sloughing of the HLP lesion 12,28,35 . However, some works reported a relapse after stopping the therapy 20,36 …”

“…12 These histological features differentiate HLP from similar keratotic diseases such as Kyrle's disease, stucco keratosis, dis- suggested that the 5-FU provokes a strong inflammatory response, leading to the sloughing of the HLP lesion. 12,28,35 However, some works reported a relapse after stopping the therapy. 20,36 4 Topical or systemic retinoids: topical retinoids have not been effective, showing only a slight reduction in roughness.…”