Ross Syndrome with Sweating Anomaly Associated with Sjögren Syndrome: An Infrared Thermo­graphic Case Study

Abstract

Ross syndrome is a rare partial dysautonomic syndrome of unknown aetiology, characterized by a one-sided or bi-lateral anhidrosis associated with Holmes-Adie syndrome (defined by tonic pupil and hyporeflexia of deep tendons) (1). Since it was first described in 1958 (2), approxima-tely 50 cases have been described in the literature (3). Dermatologists may be confronted with this disorder of thermoregulation, which is often revealed by a segmental “compensatory” hyperhidrosis. We describe here a case of Ross syndrome associated with Sjogren syndrome (SS) and discuss the potential relationship between these two entities. Thermography imaging exploration with an in-frared camera performed in different ambient temperature settings allowed better definition of the thermoregulatory sweating abnormalities in our patient. Moreover, to our knowledge, this examination showed, for the first time, an unexpected body “thermal pattern”, which may be characteristic of Ross syndrome. CASe RepoRTWe present here a case of a 35-year-old woman with a 10-year medical history of localized hyperhidrosis. Her medical history revealed autoimmune thyroid disease and SS. The hyperhidrosis appeared at an ambient tem-perature of approximately 25–27°C and was localized on the left side of her lower back, extending belt-like towards the side and in the controlateral lower back on the right side, as well as on the nape of her neck and shoulders. The rest of the skin was anhidrotic. She was also heat intolerant, with lack of sweating, abnormal increase in body temperature and generalized fati-gability when exposed to high ambient temperatures (32–34°C). Both pupils showed a slow reaction to light and a normal constriction to a near target, and deep tendon jerks were absent without motor deficits (Holmes-Adie syndrome). There were no other signs of autonomic dysfunction. punch biopsies obtained from hyperhidrotic and hypohidrotic skin areas were normal. The skin direct immunofluorescence test was negative. Blood explorations showed an isolated presence of anti-SSA, anti-SSB antibodies and anti-thyroid antibodies. Serum ganglionic (AChR) antibody was negative. Oral and conjunctival Sicca were objectively confirmed by Schirmer’s test. Salivary gland biopsy revealed lympho-cytic infiltration in both periglandular and perivascular regions. All of these Sicca findings fulfilled the diag-nostic criteria for primary SS (4). Brain and spinal cord magnetic resonance imaging (MRI), as well as thoracic and abdominal computed tomography scan findings, were normal. Electrophysiological explorations of both hands and forearms showed an absence or collapse of the sympathetic skin responses. Nerve conduction study findings, EEG and cardiac tilt-test (reflecting the adren-ergic sympathetic and parasympathetic functions) were normal. Thermographic explorations with an infrared camera (A320, FLIRsystems, Inc., Issy Les Moulineaux, France) allowed for evaluating the body’s surface tem-perature at 22°C and after a 20 min exposure to 32°C ambient temperature (Fig. 1). This examination was significantly more efficient than the Minor Starch Test and clearly delimited the skin thermoregulation abnor-malities associated with Ross syndrome. Hyperhidrotic and anhidrotic areas were also clearly visualized as the lowest and highest estimated skin surface temperature. This examination showed large dermatomal-like pat-terns in hyperhidrotic areas (lower back and shoulder regions), but also detected unexpected smaller patch-