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Systemic Lupus Erythematosus (SLE) presenting with peripheral neuropathy: A rare occurrence

  • Author(s): Rai, Vandana Mehta
  • Shenoi, SD
  • Pai, Sathish B
  • Choondlal, Vinod Joseph
  • et al.
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Systemic Lupus Erythematosus (SLE) presenting with peripheral neuropathy: A rare occurrence
Vandana Mehta Rai MD 1, SD Shenoi MD 1, Sathish B Pai MD 1, Vinod Joseph Choondlal MD 2,
Dermatology Online Journal 11 (3): 43

1. Dept of Skin & STD, Kasturba Medical College, Manipal 576104, Karnataka, India. vandanamht@yahoo.com2. Dept of Neurology, Kasturba Medical College, Manipal 576104, Karnataka, India

Neuropathies are a prominent feature of systemic vasculitides. The immediate cause of the vasculitic neuropathies is inflammation and occlusion of the vasa nervosum resulting in ischemia of the peripheral nerve. Peripheral neuropathy occurs in 6-21 percent of patients with SLE and can manifest as cranial neuropathy or as polyneuritis. Mononeuritis multiplex can sometimes be the initial presentation of SLE[1]. Confirmation of diagnosis is by nerve biopsy and nerve conduction studies.

We report a case of 34-year-old male who had multiple joint pains for 10 years, hyperpigmented skin lesions on face with photosensitivity for 4 years, oral erosions for 2 years, and tingling and numbness of the lower and upper extremities of 3 weeks duration. There was diminished sensory perception to touch, pain and temperature in the lower and upper limbs with motor weakness. Laboratory investigations revealed anaemia, proteinuria, granular casts with numerous RBCs and WBCs in the urine, elevated ANA, dsDNA, hypocomplementemia. DIF from the uninvolved skin showed the classic lupus band confirming the diagnosis of SLE. Nerve biopsy showed features of vasculitic neuropathy and nerve conduction velocity studies were consistent with sensory and mild motor and axonal demyelinating peripheral neuropathy. Treatment was initiated with cyclophosphamide pulse along with corticosteroids. After a total of eight pulses patient reported 80 percent improvement in sensory and motor symptoms. Renal status improved and the immunological parameters reverted to normal. Although the mainstay of treatment of peripheral neuropathy is corticosteroids, resistant cases may need cyclophosphamide, azathioprine or plasma exchange. Pulse cyclophosphamide has been successfully tried in two cases of mononeuritis multiplex in SLE[2] and in cases of neuropsychiatric lupus with peripheral neuropathy [3]. This case has been reported to highlight the importance of cyclophosphamide pulse in resistant cases of peripheral neuropathy secondary to SLE with lupus nephritis. What was peculiar in our case was only the hyperpigmentation on the face which was suggestive of SLE rather than the classical malar rash that has been described in literature. Hyperpigmentation on the face in asian skin can easily be mistaken for melasma could be a subtle marker of SLE in asians and should be kept in mind as a differential diagnosis.

References

1. Hughes RA, Cameron JS, Hall SM et al. Multiple mononeuropathy as the initial presentation of systemic lupus erythematosus- nerve biopsy and response to plasma exchange. J Neurol 1982;228:239-247.

2. Martinez- Taboada VM, Alonso RB , Armona J. Mononeuritis multiplex in systemic lupus erythematosus: response to pulse intravenous cyclophosphamide.Lupus 1996;5:74-76.

3. Neuwelt CM, Lacks S, KayeBR. Role of intravenous cyclophosphamide in treatment of neuropsychiatric systemic lupus erythematosus.Am J Med1995;98:32-41.

© 2005 Dermatology Online Journal