A case of SLE with quadriparesis and skin manifestations

Dr. K.A. Sudharshana Murthy, Dr. Kiran.H.S., Dr. Thippeswamy, Dr. Vijay Cheluvaraj, Dr. Aparna.A.N., Dr. Pramod Chebbi, Dr. Sriram Chandrudu

A 35 year old lady presented to the ER with history of joint pain of 6 months, intermittent fever of 6 months, quadriparesis of 15 days, and hyperpigmented skin lesions over exposed areas since 5 days. There was no history of similar episodes in the past. Patient was not a diabetic or a hypertensive.

On examination patient had facial puffiness, hyperpigmented lesions over the legs and forearms. Her radial artery pulsations were felt and was 84/min whereas her lower limb pulsations were absent bilaterally upto the popliteal artery. Her blood pressure in both the upper limbs was 104/80 mm of Hg. She had quadriparesis and generalized areflexia. There were no sensory system or cranial nerve involvement. Higher mental functions were normal.

Figure 1: Hyperpigmented ecchymotic patches over the legs with gangrenous changes of the toes

Patient was further evaluated with skin biopsy and blood investigations which revealed the following: Hb- 8.6g/dl, TLC - 10,800/cu mm, DLC-N78 L18 E4, ESR- 30mm, Platelet count- 2.05 lakh/cu mm,Urea- 128 mg/dl, Creatinine- 1.9 mg/dl, Na- 136 mmol/l, K- 5 mmol/l

Rheumatoid factor: negative, ANA: positive,ds- DNA: positive, HIV: non-reactive.Arterial and venous Doppler studies of both the lower limbs were normal. Renal artery doppler- normal. Nerve conduction study: not excitable b/l radial nerve at wrist, b/l ulnar nerve at wrist, b/l peroneal nerve.

Figure 2: Deposition of keratinocytes along the blood vessels and basement membrane

Figure 3: DIF studies show discontinuous granular deposits of IgG, IgA and IgM along basement membrane zone. Deposition of IgG and IgA are also seen around basement membrane of sweat glands and blood vessels -features suggestive of cutaneous small vessel vasculitis.

Discussion

Vasculitis is a spectrum of clinicopathological disorders defined by inflammation of the blood vessels, including arteries and veins of varying caliber, which result in a variety of clinical neurological manifestations related to ischemic injury of the central nervous system (CNS) and peripheral nervous system (PNS). Peripheral neuropathy is a well-recognized consequence of systemic vasculitis due to peripheral nerve infarction with Wallerian degeneration. Rarely, neuropathy is the sole manifestation of vasculitis, referred to as nonsystemic vasculitic neuropathy (NSVN). These conditions are defined pathologically by tissue biopsy demonstrating disruption or destruction of the vessel wall with inflammatory cell infiltrates.

The diagnosis of vasculitic neuropathy is straightforward in patients with an established diagnosis of systemic vasculitis and classic features of mononeuritis multiplex. Most patients have clinical features of a subacute, progressive, generalized but asymmetric, painful, sensorimotor polyneuropathy. Classically, patients have painful sensory loss and weakness in the distribution of multiple peripheral nerves, referred to as mononeuritis multiplex. Pain also may be described as burning and often occurs in the distribution of affected nerve territories (eg, ulnar or peroneal sensory nerve distributions).

Symptoms develop in a stepwise progressive pattern evolving over weeks to months; occasionally, patients worsen rapidly over days and develop a painful quadriparesis. Others, particularly elderly patients, have an indolent course with progression over many months or a year or more. The latter pattern is especially common in patients with nonsystemic vasculitic neuropathy (NSVN). Similarly, vasculitic neuropathy is virtually never a pure motor condition; patients have symptoms of numbness or paresthesias and findings of sensory impairment in clinically affected regions.

. Laboratory tests often indicate features of systemic inflammation, such as an elevated sedimentation rate or positive anti-neutrophil cytoplasmic antibody, and electrodiagnostic evaluation shows multiple mononeuropathies or a confluent, asymmetric axonal neuropathy.

Because partial ischemic lesions may occur along the course of several nerves simultaneously, it may not be appreciated that the majority of patients have a confluence of multiple mononeuropathies, producing a generalized but multifocal and asymmetric polyneuropathy, or a lumbosacral plexus neuropathy when limited to the legs. A few patients have a distal, symmetric, pure sensory or sensorimotor polyneuropathy.

Table 1: Primary and secondary Systemic Vasculitis Associated With Neuropathy: Clinical and Laboratory Features.

TREATMENT

Standard therapy is combined treatment with prednisone, 1.0 mg/kg/d, and either cyclophosphamide, 1–2mg/kg/d orally, or intravenous pulse cyclophosphamide at a dosage of 1g/m.

Azathioprine is used as adjunctive therapy for vasculitic neuropathy and is an effective steroid sparing agent and safer alternative to cyclophosphamide. The benefit may not be apparent for at least 6 months after initiating treatment. It is administered as a single daily dosage of 2 to 3mg/kg/d administered orally, starting at 50 mg/d for the first several days to a week, followed by slow-dosage escalation of 50 mg every few days to the desired dose.

Our patient was treated with steroids and cyclophosphamide. Patient’s power has improved and her progression of skin lesions has been disrupted due to medications.

CONCLUSION

Systemic lupus erythematosus is a common entity. A rare possibility of quadriparesis with skin manifestations should direct us towards diagnosing vasculitis and prompt treatment should be initiated to prevent worsening of the disease.