Case Number : Case 2620 - 22 July 2020 Posted By: Dr. Hafeez Diwan

I had never heard of CALME syndrome before, so I guess it is extremely rare.  The reported associated histology is really of normal constituents of vulvar tissue.  Here we have lymphangiectiasiae, fibrosis and rather sarcoidal granolomata.  I think this might be cutaneous Crohn's disease.

Interesting case. Those small aggregates of epithelioid cells can be intravascular Langerhan cells (to be confirmed by IHC). Is there a consideration for Langerhan cell histiocytosis?

There were two lesions biopsied.  The top left and bottom right panels are from the same lesion.  The other images are of the other lesion, which was pedunculated and fleshy.  There is no history of Crohn's disease.  These were interpreted as lymphangioma.  The fleshy lesion in addition shows intravascular histiocytosis, which is probably reactive in this setting.

CALME stands for "Childhood asymmetry labium majus enlargement."

I wanted to thank Dr. Sarah Al-Awami (who provided the photomicrographs and the history).

Interesting case. Certainly I've seen some very lymphangioma-like lesions in patient's with Crohn's or other reasons for lymphatic obstruction e.g. radiotherapy, obesity, prior surgery and pressure related etc.