Metal implant‐induced skin ulcer mimicking scrofuloderma

changes and corresponding histopathology is unusual since the lesions had been observed already in the first years of life. Most patients with PXE-like PDE reported onset of disease after the fifth decade. Only one case in early middle age has been reported so far. Because of the late onset of the disease, some authors hypothesize PXE-PDE is related to intrinsic skin ageing and/or extrinsic ageing due to ultraviolet radiation. Using immunohistochemistry, loss of elastin and fibrilin-1 has been observed in PXE-like PDE. On ageing, however, there is only loss of fibrilin-1 while elastin remains normal or decreased. So far, one familial case of PXE-PDE has been documented in the literature, suggesting influence of genetic or inheritable factors. The history of the present case does not favour intrinsic or extrinsic ageing as pathogenetical factor but rather indicate that genetics may be of importance at least in certain cases of PXElike PDE. Notably, PXE-like PDE is characterized by exclusive female predominance. The differential diagnosis PXE is of great importance in the present case, but one have to consider also other rare elastolytic disorders including linear focal elastosis, white fibrous papulosis of the neck, and mid-dermal elastolysis. PXE is a rare genetic disorder, caused by a mutation in the ABCC6 gene. The cutaneous lesions in PXE are very similar to lesions seen in PXE-like PDE. The predominant pathological findings of PXE, consisting of fragmentation, clumping and calcification of the elastic fibres, are located in the mid and lower dermis. As also observed in the present case, the absence or marked loss of elastic fibres in the papillary dermis and the absence of calcifications and fragmentation of elastic fibres are characteristic features of PXE-like PDE. The collagen fibres are normal. Unlike PXE-like PDE, PXE usually develops during childhood, has systemic involvement, characterized by calcification of the elastic fibres of the skin, retina, and cardiovascular system, which may lead to serious complications. In the present case, there was no evidence for systemic involvement. In conclusion, we observed a case of early-onset PXE-like PDE. This observation, the exclusive female predominance documented in the literature, and the report of familial PXE-like PDE indicate genetical factors in the aetiopathogenesis of this very rare disease.