Multifocal squamous cell carcinoma arising in a Favre-Racouchot lesion – report of two cases and review of the literature

Abstract

Background: Favre-Racouchot syndrome (nodular cutaneous elastosis with cysts and comedones) is a cutaneous disease characterized by coexistence of cysts, comedones and elastotic nodules in actinically damaged skin, typically on the face. Ultraviolet radiation plays a significant role in the development of the disease. Unilateral lesions have been described.

Favre–Racouchot disease: systematic review and possible therapeutic strategies

“…In fact, five cases of postradiation FRD are described in the literature. Cysts and comedones developed in the irradiated region, after a variable time following radiotherapy, ranging from 2 weeks up to several years …”

“…Cysts and comedones developed in the irradiated region, after a variable time following radiotherapy, ranging from 2 weeks 14 up to several years. 15 Even though not necessarily symmetrical, the disease is generally bilateral and occurs in sun-exposed areas. [16][17][18][19] More than 95% of the cases were localized in the face ( Fig.…”

“…10,11,25,26 Interestingly, FRD was found to be associated with invasive squamous cell carcinoma (SCC) in three cases, two of which had multifocal SCCs arising in the context of FRD plaques. 14,15 Probably, the link between those two disorders may reside in the common pathogenesis. Both FRD and SCC share risk factors as UV exposure and smoking.…”

Favre–Racouchot syndrome: report of a case treated by plasma exeresis

“…Important preventive measures include avoidance of sun exposure and to stop smoking. 6 Main therapeutic options include topical retinoids and laser treatment. 7,8 Based on the idea that it is a useful instrument both for sebaceous cysts and active acneic lesions, 9 we present a case of FRD treated with plasma exeresis (PE), a new technology in the field of aesthetic dermatology.…”

“…Two HHD patients with the identical ATP2C1 mutation c.2126 + 1G>A, which was shown to result in a single aberrant transcript, were previously reported. 2,6 One of them was a case of type 2 segmental HHD. 6 The other patient had the mutation as a germline mutation, and the disease severity was described as mild according to the physician's global assessment of disease activity, although the detailed phenotype is unknown.…”