Congenital Linear Steatocystoma Multiplex of the Nose

Abstract

We describe a case of steatocystoma multiplex with an unusual clinical manifestation. A 5-year-old girl had 8 papulonodular lesions arranged in a linear fashion on the left side of the nose. These had been present at birth. Histologically the lesions showed the typical features of steatocystoma multiplex. There was no family history of similar lesions. This is the second case of linear steatocystoma multiplex, a rare variant of steatocystoma multiplex.

Steatocystoma multiplex

“…Steatocystoma simplex is the sporadic solitary tumour counterpart to steatocystoma multiplex. The majority of cases are seen in the second and third decade of life, however cases can present at birth5 and as old as 78 years 6. Patients usually present with multiple, asymptomatic, yellow-coloured cysts.…”

Steatocystoma multiplex associated with bilateral preauricular sinuses

“…While in Sardana et al's case, ‘there was no family history of similar complaints’, in Barone's case the association was apparent in four generations with autosomal dominant inheritance . It is unlikely that in these two cases (as well as in ours), the sinuses were just a mere complication of steatocystoma multiplex: although steatocystomas can suffer rupture, inflammation, scarring and formation of sinus tracts, such phenomena happen once the steatocystomas are evident (usually in adolescence and in early adulthood, despite the fact that occasional cases of congenital steatocystoma multiplex have been reported). That is not true in these three cases, in which the sinuses were congenital, whereas the steatocystomas were a subsequent phenomenon (when the patient was 23 years old in Sardana's case, 15 years old in Barone's case and 14 years old in this case).…”

Eccrine Hidrocystoma in a Child: An Atypical Presentation

“…There is an interesting previously reported case of steatocystoma multiplex localized on the nose of a child 12 .…”