Facial erythromelalgia: A rare entity to consider in the differential diagnosis of connective tissue diseases

Autoimmune progesterone dermatitis mimicking facial erythromelalgia successfully treated with hysterectomy and bilateral salpingo-oophorectomy

“…A recent review showed that most patients with APD present with cutaneous reactions that resemble a type I hypersensitivity reaction, whereas far fewer present with type IV hypersensitivity. 1 Only 3 of 89 patients mentioned a burning and painful sensation associated with their rash, in stark contrast to our patient. Further supporting the rarity of these symptoms in APD, to the best of our knowledge, there have been no case reports of APD that include erythromelalgia on the differential diagnosis.…”

“…Although erythromelalgia usually presents on the hands and feet, it has rarely been reported on the face. 1 However, her daily flares while taking progestin-containing OCPs and the subsequent change in frequency to align with monthly progesterone surges after discontinuation of exogenous progesterone raised suspicion for APD. Complete resolution after TAH-BSO confirmed the diagnosis of APD.…”

Erythromelalgia involving the face

“…The head and neck are affected least often in 2% to 15% [3][4][5][6][7]. However, a PubMed search produced only four reported cases with facial involvement and sparing of the extremities [8][9][10][11].…”

“…Differential diagnosis varies depending on the location affected. The differential diagnosis of facial erythromelalgia includes rosacea, actinic damage, connective tissue disease, menopausal flushing, flushing related to carcinoid syndrome, and medication reactions [8,15]. For distal extremity involvement only, diagnostic considerations would include Raynaud phenomenon, complex regional pain syndrome, venous insufficiency, peripheral arterial vascular disease, and peripheral neuropathy [13].…”

An Atypical Erythromelalgia on the Chest

“…Erythromelalgia is usually triggered by heat exposure or exercise, and it tends to be alleviated by cooling 2 . Although it commonly involves the feet and hands, erythromelalgia can also occur on the face or ears 1,3 . Histopathological findings are often nonspecific 2 .…”