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Year : 2022  |  Volume : 2  |  Issue : 2  |  Page : 64-66

Lupus erythematosus with segmental anhidrosis

Department of Dermatology, AIIMS Raebareli, Raebareli, Uttar Pradesh, India

Date of Submission16-Feb-2022
Date of Decision06-Apr-2022
Date of Acceptance18-Apr-2022
Date of Web Publication25-Jan-2023

Correspondence Address:
Dr. K Geetha
Department of Dermatology, AIIMS Raebareli, Raebareli, Uttar Pradesh
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/ara.ara_5_22

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Systemic lupus erythematosus (SLE) is a multisystem connective tissue disorder that also affects all the components of the neurological system, such as autonomic, peripheral, and central nervous systems. There was no link between autonomic dysfunction and lupus activity or the presence of peripheral neuropathy. Anhidrosis and hypohidrosis are most usually caused by local skin injury (e.g., related to trauma, radiation, infection [e.g., leprosy], or inflammation) or gland atrophy caused by connective tissue illness (e.g., systemic sclerosis, systemic lupus erythematosus, and Sjögren syndrome). Drugs, particularly those with anticholinergic effects, can possibly induce it. Diabetic neuropathy, thyroid problems, and other factors might potentially contribute to it. Here is a rare case report of patchy segmental anhidrosis with compensatory hyperhidrosis as the presenting manifestation of SLE treated with topical sunscreen, topical glycopyrrolate, and oral hydroxychloroquine with mild clinical improvement. However, anhidrosis showed no improvement.

Keywords: Autononomic dysfunction, lupus erythematosus, segmental anhidrosis

How to cite this article:
Geetha K. Lupus erythematosus with segmental anhidrosis. Ann Rheumatol Autoimmun 2022;2:64-6

How to cite this URL:
Geetha K. Lupus erythematosus with segmental anhidrosis. Ann Rheumatol Autoimmun [serial online] 2022 [cited 2023 Feb 4];2:64-6. Available from: http://www.ara.ssr.com/text.asp?2022/2/2/64/367438

  Introduction Top

Anhidrosis or hypohidrosis can occur as a result of brain, spinal cord, sweat gland, or peripheral nerve damage, as well as leprosy, diabetic neuropathy, thyroid dysfunction and Sjögren syndrome, ectodermal dysplasia, autoimmune diseases, and anticholinergic drug side effects.[1] Autonomic nerve function can be affected in people with connective tissue diseases such as SLE, rheumatoid arthritis, and systemic sclerosis, although less frequently than peripheral somatic neuropathy.[2] Autoantibodies targeted against the sympathetic ganglia, the vagus nerve, and some other components of the autonomic nervous system (ANS) may play a significant role in neurological impairment. These autoantibodies appeared in isolation from other autoantibodies, indicating that the antigens they target are tissue-specific. Those autoantigens' nature is currently unknown. The immune-mediated pathogenesis of autonomic dysfunction in connective tissue illnesses is supported by increased clinical response to immunosuppressive medication.[2] Generalized anhidrosis has been reported as the first clinical presentation of systemic lupus erythematosus by Provitera et al.[3] Here is one such rare report with segmental anhidrosis as the first clinical presentation, followed by other symptoms in SLE.

  Case Report Top

A 21-year-old woman presented to the skin outpatient department with patchy segmental anhidrosis on the right upper and lower limb, as well as the left side of the face, along with compensatory hyperhidrosis on the opposite side for the past 3 years. She presented with a history of photosensitivity and intermittent itching with erythematous lesions on exposure to the sun. She experienced extreme discomfort and social embarrassment as a result of compensatory increased sweating over the right face and left side of the upper and lower limbs. During her initial presentation, she had no further symptoms suggestive of systemic lupus erythematosus. No history was suggestive of diabetes, thyroid dysfunction, or drug intakes that can cause SLE-like manifestations. No other symptoms or signs were suggestive of autoimmune dysfunction. There is no other family history of similar illness or autoimmune dysfunction. Ross syndrome was ruled out as the ophthalmic examination revealed no pupillary abnormalities. The mucocutaneous and systemic examinations were unremarkable. Her hematology reports and renal and liver function test reports were normal. A raised erythrocyte sedimentation rate, positive titer (antinuclear antibody) 2+ nucleolar at 1:100, positive ds-DNA (double-stranded DNA), negative ENA (extractable nuclear antigen), and an elevated TSH value of 9.92 (reference range, 0.4–4 miu/l) were found in the blood investigation reports. An magnetic resonance imaging (MRI) of the brain revealed no obvious intracranial abnormalities. The MRI of the cervical spine revealed no obvious compressive or noncompressive pathology. Starch-iodine autonomic testing revealed abnormal sudomotor responses over the face and limbs. Skin biopsy report from the anhidrotic upper limb showed features of subacute lupus erythematosus with focal epidermal hyperkeratosis, flattening of rete pegs, hydropic degeneration of basal cells with periadenexal lymphocytic infiltrate, thickened dermal collagen with minimal inflammation, and entrapping adnexal structures. After a few months, she began to experience urticarial symptoms and joint pain. Her clinical symptoms, other than anhidrosis, improved after she was started thyroxine 75 mcg, along with topical sunscreen and oral hydroxychloroquine 200 mg. Hyperhidrosis was treated with topical glycopyrrolate, with only minor improvement.

  Discussion Top

SLE is an autoimmune illness that can impair any organ system, including the neurological system. Central neuropsychiatric lupus (NPSLE) covers a broad spectrum of CNS problems, including acute altered mental state, psychosis, cognitive disorders, headache, seizures, cerebrovascular disease, myelopathy, demyelinating syndrome, and meningitis. Peripheral NPSLE encompasses cranial, autonomic, and peripheral neuropathies and plexopathy, in addition to myasthenia gravis. Neurologic involvement in SLE has been hypothesized to signify a more aggressive manifestation of the disease, affecting up to 75% of patients.[4]

In lupus, autonomic neuropathy is not uncommon, and it can exist independently. There are currently no clinical markers that might be used to determine a patient's prognosis.[5] The involvement of the ANS in patients with autoimmune connective tissue disorders has been studied only infrequently. This could be due to the fact that manifestations of autonomic dysfunction are nonspecific and can affect a variety of systems including the gastrointestinal, cardiovascular, and nervous systems. Longitudinal studies need to be undertaken on the importance of autonomic dysfunction and be confirmed by laboratory testing.[6]

ANS is involved in 6%–93% of SLE patients with limited literature reports.[8] It is frequently asymptomatic and has no relationship to disease activity. Furthermore, apart from the presence of antiphospholipid antibodies, there is no link between ANS dysfunction and immunoserological abnormalities. The effect of autonomic symptoms on lupus patients' standard of living has yet to be studied.[7],[8]

According to the neuropsychiatric presentation and severity, a careful diagnosis and personalization of treatment are essential in neuropsychiatric systemic lupus erythematosus. Autonomic testing to determine the severity, electromyography and nerve conduction studies to characterize neuropathy, and additional laboratory tests to rule out DM, renal function, and vitamin deficiencies, plasma norepinephrine levels, serological testing for infectious diseases, tests to rule out celiac disease, and acetylcholine receptor levels are all required in SLE with ANS involvement.[9]

These patients are treated in the same manner as nonSLE patients with similar clinical symptoms. Exacerbating variables should be corrected; nonpharmacological therapies should be used along with symptomatic therapy as the first line of treatment. Glucocorticoids, immunosuppressive treatment, plasmapheresis, and intravenous immune globulin (IVIG) are commonly used to treat autonomic dysfunction in SLE.[10] With this case report, we want to conclude that segmental anhidrosis with autonomous nervous system involvement can be a rare manifestation of SLE.

Declaration of patient consent

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

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  References Top

Kumar P, Das A, Mondal AK, Kumar J, Chattopadhyay SS, Guha G, et al. A lady presenting with segmental anhidrosis and heat intolerance. Indian Dermatol Online J 2015;6:126-7.  Back to cited text no. 1
[PUBMED]  [Full text]  
Maule S, Quadri R, Mirante D, Pellerito RA, Marucco E, Marinone C, et al. Autonomic nervous dysfunction in systemic lupus erythematosus (SLE) and rheumatoid arthritis (RA): Possible pathogenic role of autoantibodies to autonomic nervous structures. Clin Exp Immunol 1997;110:423-7.  Back to cited text no. 2
Provitera V, Lubrano E, Piscosquito G, Manganelli F, Santoro L, Nolano M. Generalized anhidrosis as first clinical presentation of systemic lupus erythematosus. Lupus 2018;27:2296-7.  Back to cited text no. 3
Fathalla MM, El-Badawy MA. Upper extremity subclinical autonomic and peripheral neuropathy in systemic lupus erythematosus. Egypt Rheumatol Rehabil 2015;42:87-93.  Back to cited text no. 4
  [Full text]  
Shalimar G, Handa R, Deepak KK, Bhatia M, Aggarwal P, Pandey RM. Autonomic dysfunction in systemic lupus erythematosus. Rheumatol Int 2006;26:837-40.  Back to cited text no. 5
Stojanovich L. Autonomic dysfunction in autoimmune rheumatic disease. Autoimmun Rev 2009;8:569-72.  Back to cited text no. 6
Sherer Y, Gorstein A, Fritzler MJ, Shoenfeld Y. Autoantibody explosion in systemic lupus erythematosus: More than 100 different antibodies found in SLE patients. Semin Arthritis Rheum 2004;34:501-37.  Back to cited text no. 7
Magarò M, Mirone L, Altomonte L, Zoli A, Angelosante S. Lack of correlation between anticardiolipin antibodies and peripheral autonomic nerve involvement in systemic lupus erythematosus. Clin Rheumatol 1992;11:231-4.  Back to cited text no. 8
Magro-Checa C, Zirkzee EJ, Huizinga TW, Steup-Beekman GM. Management of neuropsychiatric systemic lupus erythematosus: Current approaches and future perspectives. Drugs 2016;76:459-83.  Back to cited text no. 9
Bertsias GK, Ioannidis JP, Aringer M, Bollen E, Bombardieri S, Bruce IN, et al. EULAR recommendations for the management of systemic lupus erythematosus with neuropsychiatric manifestations: Report of a Task Force of the EULAR Standing Committee for Clinical Affairs. Ann Rheum Dis 2010;69:2074-82.  Back to cited text no. 10


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