063 Headache with periorbital ecchymosis; a rare clinical entity

Objectives Primary headache disorders are common with migraine and tension headache accounting for the vast majority of cases. A smaller proportion suffer from trigeminal autonomic cephalgia (TAC). We present a 23-year-old Caucasian female who described characteristic, episodic headaches starting with a dull retro-orbital/bi-frontal pressure evolving, over the course of 1 minute, to experience florid periorbital ecchymosis. While this phenomenon has been described in the literature, the characteristics of our case are unique and noteworthy of reporting. Methods We reviewed the literature surrounding this rare entity by using PubMed/OVID databases and the search terms ‘Headache AND ecchymosis’. Results Case reports exist in older patients1–3, where the headache is side locked and associated with other autonomic characteristics such as periorbital oedema, conjunctival injection and tearing. Our case is a young female with only ecchymosis in a unilateral and/or bilateral manner and no other autonomic or indeed migraine features. The patient underwent vascular/cranial imaging and blood tests to exclude haematological, autoimmune, vasculitic causes for this presentation which were unrewarding. Conclusion Variations on this clinical entity are described;1–4 we hope this report may bring attention to this fascinating phenomenon. The pathophysiological process is likely to be similar to those implicated in TACs, namely activation of the trigemino-neurovascular system and facial autonomic pathways. The release of neuromediators such as CGRP, VIP and Substance P cause blood vessel fragility resulting in diapedesis. Optimal treatment regimens are unknown but various agents have been trialled. Our patient declined treatment and continues to be followed. References DeBroff B, et al. Migraine Associated with Periorbital Ecchymosis. Headache 1990;30:260–263. Dafer R, et al. Atypical Chronic Headache and Recurrent Facial Ecchymosis: A Case Report. Neuro-Ophthalmology 2011;35:76–77. Nozzolillo D, et al. Migraine associated with facial ecchymoses ipsilateral to the symptomatic side. J Headache Pain ( 2004) 5:256–259. Sethi PK, et al. Teaching neuroimages: Red forehead dot syndrome and migraine revisited. Neurology 2015;85;e28.