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Year : 2017  |  Volume : 8  |  Issue : 5  |  Page : 370-372  

A rare case of ross syndrome

Department of Dermatology, Venereology, Leprology, Government Medical College, Kota, Rajasthan, India

Date of Web Publication15-Sep-2017

Correspondence Address:
Ramesh Kumar
Department of Dermatology, Venereology and Leprology, Government Medical College Kota, Rajasthan - 324 005
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/idoj.IDOJ_356_16

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How to cite this article:
Sharma MK, Gupta S, Yadav S, Kumar R. A rare case of ross syndrome. Indian Dermatol Online J 2017;8:370-2

How to cite this URL:
Sharma MK, Gupta S, Yadav S, Kumar R. A rare case of ross syndrome. Indian Dermatol Online J [serial online] 2017 [cited 2022 Jan 18];8:370-2. Available from: https://www.idoj.in/text.asp?2017/8/5/370/214721


A 32-year-old female presented to us with complaint of loss of sweating on most of the left side of the body along with generalized burning sensation on sun exposure. There was no history of headache, dyspnoea, palpitation, giddiness, hypotension, pain abdomen, or diarrhoea. There was no history of any trauma to the spine and syncopal attacks. Family history was not contributory.

Cutaneous examination showed anhidrosis over left side of the trunk, bilateral upper limbs, lower limbs, right side of face, palms, and soles associated with compensatory hyperhidrosis on right side of the abdomen [Figure 1] (dermatomal distribution T8–T12) and left side of face. Moreover, there was hypoplasia of bilateral 4th and 5th toe. On nervous system examination, deep tendon reflexes including knee, ankle, biceps, and supinater of both sides were absent. Blood pressure was normal in supine as well as sitting position. Rest of the cutaneous and systemic examination was normal.
Figure 1: Anhidrosis on the left side with compensatory hyperhidrosis on the right side (T8–T12)

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Pupillary examination showed anisocoria, with the left pupil larger than the right [Figure 2]. Left-sided pupil was dilated and sluggishly reacting to light [Figure 3]. Consensual light reaction of the right pupil was normal and absent in the left pupil. Bilateral visual acuity, color vision, accommodation reflex, external ocular movements, fundus examination, and visual fields were normal. There was no ptosis. Findings of eye examination suggest Adie's pupil in the left eye.
Figure 2: Sluggishly reacting left pupil and normally reacting right pupil to light

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Figure 3: Closer view of the dilated left tonic pupil

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Laboratory investigations including hemogram, liver and kidney function tests, urine analysis and thyroid function tests, HIV, VDRL, ANA, and rheumatoid factor revealed no abnormality. X-ray of chest and lumbar spine and MRI of spine was normal. Cerebrospinal fluid examination did not reveal any evidence of infection. Histology of the anhidrotic skin showed sparse sweat glands and of the hyperhidrotic region showed increased numbers of sweat glands [Figure 4].
Figure 4: (a) Anhidrotic skin showing absent eccrine glands; (b) Hyperhidrotic skin showing abundant eccrine glands; (H and E, ×100)

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Based on the above findings, diagnosis of Ross syndrome was made. Ross syndrome, first described by Ross in 1958, is an uncommon disorder of thermoregulation characterized by the triad of hyporeflexia or areflexia, tonic pupil, and segmental anhidrosis with or without compensatory hyperhidrosis.[1] The first two components define the Holmes–Adie syndrome and the last component defines the Harlequin syndrome.[2] Tonic pupil and reduced sweating can be attributed to affection of postganglionic cholinergic fibres projecting to the iris and sweat glands.[3] The number of sweat glands may be decreased in the anhidrotic skin.[4] Depression of deep tendon reflexes is due to dorsal root ganglionic degeneration and spinal interneuron loss. Anhidrosis may be accompanied by other alterations of the autonomic nervous system such as cardiac dysautonomia, diarrhea, and coughing.[5] Ross syndrome may be associated with cytomegalovirus infection, in which the symptoms improved partially with the infection resolution.[6]

Our patient presented with all typical features of Ross syndrome along with an additional finding of toe hypoplasia. The differential diagnoses of anhidrosis such as Shy Drager syndrome, multiple sclerosis, harlequin syndrome, diabetes mellitus, leprosy, and polyneuropathies were excluded as tonic pupil and areflexia are not features of these disorders. We also considered and ruled out the differentials of Holmes–Adie syndrome, Horner's syndrome, and hereditary sensory autonomic neuropathy [Table 1]. The case is reported due to its rarity.
Table 1: Differential diagnosis of Ross syndrome

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Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.

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Conflicts of interest

There are no conflicts of interest.

   References Top

Shin RK, Galetta SL, Ting TY, Armstrong K, Bird SJ. Ross syndrome plus: Beyond horner, Holmes-Adie, and harlequin. Neurology 2000;26:1841-6.  Back to cited text no. 1
Nolano M, Provitera V, Perretti A, Stancanelli A, Saltalamacchia AM, Donadio V, et al. Ross syndrome: A rare or a misknown disorder of thermoregulation? A skin innervation study on 12 subjects. Brain 2006;129:2119-3.  Back to cited text no. 2
Weller M, Wilhelm H, Sommer N, Dichgans J, Wiethölter H. Tonic pupil, areflexia, and segmental anhidrosis: Two additional cases of Ross syndrome and review of the literature. J Neurol 1992;239:1432-59.  Back to cited text no. 3
Metta AK, Athanikar SB, Ramchandra S, Mohammad S. Ross syndrome. Indian J Dermatol Venereol Leprol 2009;75:414-6.  Back to cited text no. 4
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Chemmanam T, Pandian JD, Kadyan RS, Bhatti SM. Anhidrosis: A clue to an underlying autonomic disorder. J Clin Neurosci 2007;14:94-6.  Back to cited text no. 5
Nagane Y, Utsugisawa K. Ross syndrome associates with cytomegalovirus infection. Muscle Nerve 2008:38:924-6.  Back to cited text no. 6


  [Figure 1], [Figure 2], [Figure 3], [Figure 4]

  [Table 1]

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