Hyperacute paraplegia and neurovascular (immuno vasculotoxic) catastrophe of nicolau syndrome: Primum non nocere

Abstract

A case of Nicolau syndrome (NS) in a 36-year-old adult taking an unusual and devastating hyperacute irreversible paraplegia after an intramuscular injection of benzathine penicillin as a part of routine chemoprophylaxis of her rheumatic heart disease is reported. Although this syndrome is a considerably rare, iatrogenic and underappreciated dermatologic entity, we reiterate in this report, its extracutaneous systemic potential for a catastrophic neurovascular phenomenon and morbidity as well as its possible preventive measures. The apoplectiform onset of T10 flaccid areflexic paraplegia, with the cutaneous hallmark of “embolia cutis medicamentosa” was corroborated by magnetic resonance imaging evidence of centromedullary complete cord involvement from T10 to conus medullaris. Combination therapy with pulse methylprednisolone, low-molecular-weight heparin, and pentoxifylline infusion proved unsuccessful. The skin biopsy and direct immunofluorescence revealed features were consistent with NS with overlap features of leukocytoclastic vasculitis, hitherto not reported. The literature of this preventable and iatrogenic disorder is reviewed, and plausible etiology is discussed.

Nicolau syndrome: a review of case studies

“…Neurological signs are the vast proportion of the symptoms that could lead to unilateral or bilateral sensory and motor disorders. 38,70 Accordingly, these seem to occur due to axonopathy, secondary to vascular problems such as embolism and vascular occlusions. Moreover, these are ranged from burning and numbness, starting from the initial phase, to dystonia, paresthesia, paraplegia, and sensitivity.…”

“…7,27 In terms of cardiovascular signs, the coldness of the injection site and the absence of pulses in the area have been observed in some cases. 38,43,44 Table 4 summarizes all the symptoms of three phases of Nicolau syndrome and the treatments used for the studied patients.…”

“…17,19 Meanwhile, it was indicated that several cell-damaging factors such as lactate dehydrogenase, creatine kinase, and myoglobin have intensified in some patients with Nicolau syndrome. 20,38,49,53…”

Extensive Nicolau syndrome following intramuscular diclofenac sodium injection

“…Theories about its etiopathogenesis involve a combination of factors: stimulation of sympathetic innervation with vasospasm and ischemia; blockade of prostaglandin synthesis by NSAIDs, arterial embolic occlusion by inadvertent intravascular injection; perivascular inflammation due to cytotoxic drug reaction; mechanical injury caused by lipophilic drugs penetrating the vessels. 2 , 3 …”

“… 4 The prognosis is unpredictable, with reports of recovery and atrophic scarring at the affected site but also compartment syndrome, hyperkalemia, renal failure, paralysis of the affected limb, and death. 3 , 5 The diagnosis is clinical, highly suggestive when the lesion starts at the injection site, with distal progression over the injected limb. Histopathology is non-specific and may reveal fatty tissue necrosis and inflammation.…”