Case Number : Case 2571 - 14 May 2020 Posted By: Saleem Taibjee

Difficult. It's a subcutaneous, faintly granulomatous, histiocytosis, whereas LCH is usually dermal with epidermotropism. It's a single lesion, whereas cutaneous LCH is usually multifocal ( can be solitary when arises in bone )...but my spot is Langherans Cell Histiocytosis.

it is a granulomatous inflammation exclude infections like tuberculosis. I have not seen any idiopathic facial granulomatous inflammation  in my part of the world. if tb is negative i would also do s100, Cd68 to see whether it is  Non LCH type histiocytic infiltrate then do BRAFF mutation etc

Same thoughts here.. I like the idea of Idiopathic facial aseptic granuloma (IFAG).

14 hours ago, Krishnakumar subramanian said:

it is a granulomatous inflammation exclude infections like tuberculosis. I have not seen any idiopathic facial granulomatous inflammation  in my part of the world. if tb is negative i would also do s100, Cd68 to see whether it is  Non LCH type histiocytic infiltrate then do BRAFF mutation etc

I never saw this pathology, as well. 

Epithelioid fibrous histiocytoma

This was a patient I had seen in clinic, the haemorrhagic quality and initial progression of the lesion rather confused me, and this was compounded by an ultrasound scan in which the radiologist also considered a vascular lesion.

However, the histology was rather granulomatous, special stains were negative.

Hence, I too concluded that this was a case of 'idiopathic facial aseptic granuloma'. I have seen a few cases over the years.

The original description was from a group from France. Boralevi et al. Idiopathic facial aseptic granuloma: a multicentre
prospective study of 30 cases. Pediatric Dermatology 2007. The clinical images from the original publication are shown below.

Cases are exclusively in children, and generally resolve spontaneously, as in this case. The original paper shows the anatomical distribution in their cases, cheek is far and away the most common. Also see below.

BW
Saleem

Nice case! Have never seen it before! Thanks for sharing!