Even the most severe of lupus skin lesions can heal

This 21-year-old woman presented with flaring of malar rash and rash on the anterior chest wall after discontinuation of her drugs due to an inter-current infection. Two years ago, she had been diagnosed with systemic lupus erythematosus on presenting with fever, polyarthritis, Raynaud’s, malar rash, chill-blain lesions, dyspnoea, tachycardia and hypotension. In addition, she had altered mentation in the form of disorientation, hallucinations and delusions. Blood tests had revealed cytopenias, positive ANA (3+ nuclear fine speckled on IFA), positive dsDNA, low complements, global myocardial hypokinesia on echocardiography, normal MRI brain and CSF analysis. At that time, a diagnosis of acute SLE with myocardial involvement and NPLSE (possible organic brain syndrome) was made, and she had been treated with pulse methyl-prednisolone, followed by oral prednisolone and monthly CYC pulses. After 3 weeks of her third CYC pulse, she was re-admitted with high-grade fever, cytopenias and hyperferritenemia, diagnosed as macrophage activation syndrome and started on ciclosporin at 2 mg/kg.

At this time there was erythematous rash with haemorrhagic crusting and desquamation involving the malar area, angle of mouth, upper and lower lips, and periorbital area (Fig. 1). Blood tests revealed pancytopenia (haemoglobin 6.9 g/dl, white blood count 3.3 × 10^9/l and platelets 1.20 × 10^9/l), mild transaminitis (AST 139, ALT 87 IU/l), elevated titres of ds DNA (665 IU/ml) and hypocomplementemia (C3 and C4, 32 and 3 mg/dl). In hospital she again developed disorientation, delusions and abnormal behaviour. A diagnosis of severe cutaneous (similar to previous reports) [1, 2] and neuropsychiatric flare of SLE was made, and she was treated with three pulses of intravenous methylprednisolone (500 mg) followed by 1 mg/kg oral prednisolone with taper to 7.5 mg over 12 weeks along with monthly intravenous cyclophosphamide (600 mg, planned for six cycles) and rituximab (1 g *2 doses, 15 days apart). This decision for dual immunosuppression was made considering her severe course of illness. She is planned for maintenance with rituximab 500 mg every 6 months. After two months, the patient had near complete resolution of skin lesions and her behaviour had normalized.

Figure 1.

Clinicopathological facets of the patient along with the dramatic response within 6 weeks. Lupus skin lesions (A) before and (B) after therapy. (C) Skin biopsy showing lymphocytic exocytosis with basal cell vacuolation and degeneration indicating interface dermatitis and bullae formation (red arrow) at dermoepidermal junction (200$×$⁠, hematoxylin and eosin). (D) Direct immunofluorescence demonstrating 3 + intense granular positivity for Ig G, Ig M along dermoepidermal junction (200$×$⁠, fluorescein isothiocyanate)

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This case highlights the complete resolution of even severe skin lesions with appropriate therapy in a lupus patient.

Informed consent was provided for the publication of this manuscript.

Data availability

All the relevant data to the clinical image have been shared. Additional information on patient follow up shall be made available on request.

Funding

No specific funding was received from any bodies in the public, commercial or not-for-profit sectors to carry out the work described in this article.

Disclosure statement: The authors have declared no conflicts of interest.

References

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