Though Red ear syndrome (RES) was first described by Lance in 1994, there is relatively less awareness among dermatologists till date. It often runs a chronic course and is less responsive to therapy, as a consequence of which patients often shuffle between various specialties. Though various theories have been proposed, it lacks a definitive etiopathophysiology, and is a challenging condition to treat. Herein, we report a case of RES who was undiagnosed for 10 years, and the therapeutic challenges that we had to address.
A 50-year-old woman presented with unilateral, episodic attacks of redness and swelling accompanied by a burning sensation involving the right pinna, with a heightened sensitivity to light touch, pressure, and sunlight, since last 10 years, with waxing and waning episodes.
When the patient presented to us, the tenderness of the involved right ear was out of proportion to the physical signs (mild erythema with absence of nodularity or induration), with a worsening of erythema and tenderness on exposure to sunlight for 5 minutes [Figure 1a and b]. RES and granulomatous conditions were considered. A thorough ear and neurological evaluation was normal.
We subjected the patient to repeat incisional biopsy from ear lobe which revealed a subepidermal homogenization of the dermal collagen in the form of solar elastotic degeneration. Superficial dermal perivascular lymphocytic infiltration was present, with no granulomas in dermis [Figure 2].
Based on the clinical and histopathological findings, a diagnosis of RES was considered in our patient.
The patient had taken multiple treatments in the past including topical, intralesional, and oral steroids with partial benefits. Since sunlight was an aggravating factor, and biopsy revealed solar elastosis, we started the patient on hydroxychloroquine 200 mg BD along with pregabalin 75 mg BD. A mild symptomatic improvement was noted by the patient initially; however, she presented after 2 months with worsening of symptoms and hence we considered botulinum toxin injection.
Botulinum A toxin 100 units vial was diluted with 2.5 ml of 0.9% normal saline. Around 40 units of botulinum A toxin was used.Injections were given 0.5 cm apart all over the right pinna intradermally using a 26-ga needle. The patient was followed up after 1 week and showed significant improvement in redness and symptoms [Figure 3], which is still sustained after 4 months of follow-up.
A diagnosis of RES was made mainly on clinical grounds. A median age of onset of around 44 years, with a female preponderance is seen. The frequency and severity of symptoms in RES vary, and sometimes, there may be year-long remission periods. Various triggering factors have been identified such as heat, touch, movement, sneezing, coughing, exercise, chewing, stress, exposure to cold, certain dietary factors, and drugs.[2,3]
The pathophysiology of RES is complex, and various theories have been put forth, which could be largely grouped as central and peripheral. In the central mechanism, there is dysregulation of brainstem trigemino-autonomic or vascular connections, while in the peripheral mechanism, there is dysfunction of cervical nerves (C3 root). Given the rich sensory innervation of the ear, the sympathetic sensory system–mediated vasodilation is accompanied by sensory symptoms adding to the patient’s distress. A The condition has been associated with migraine, cervical, and cranial neuralgias upper cervical spine disorders, temporomandibular joint disorders, thalamic syndrome, and pleomorphic adenoma of carotid body. However, our patient had intense sensory symptoms without any systemic association.
Histopathology of RES is sparsely reported in literature. According to Brackenrich et al., histopathology showed perivascular lymphocytic inflammation, edema, and arteriolar smooth muscle hyperplasia. Similar results were reported by Castellanos-Gonzalez et al. In our case, additional biopsy findings included subepidermal homogenization of dermal collagen, which has not been reported in literature till date.
Various treatments for RES have been used with varying success. Injection botulinum toxin is a newly described modality. It acts by blocking the parasympathetic system and inhibits the release of other neurotransmitters and affects the transmission of afferent neuronal impulses. It is used in the treatment of headaches where it blocks the neuronal signaling pathway activated during headache. As our patient did not respond favorably to earlier therapy and was emotionally disturbed given the chronic course of disease, we considered botulinum injection in her which is reported to produce a rapid relief in symptoms sustained over a longer period of time. However, she was still advised to avoid all triggers and the chance of possible recurrence was explained.
This report will increase the awareness about RES amongst clinicians. Though our patient did not have any associated systemic condition, she had suffered for the last 10 years. Her response to botulinum was favorable, and we propose it as a therapeutic option in such patients.
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Conflicts of interest
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