Case 1: Blisters in systemic lupus erythematosus Skin lesions can be very heterogeneous in systemic lupus erythematosus (SLE). Clinicopathological correlation is crucial to identify the type of cutaneous lesion since it provides prognostic and therapeutic implications. Blistering lesions are rare in lupus erythematosus; however, they can be difficult to characterize and the cutaneous differential diagnosis is wide. The term ‘bullous lupus’ can be confusing since it may even reflect different immune mechanisms that need specific management.
A 42-year-old with a 10-month history of hand arthritis and positivity for antinuclear antibodies (ANA), anti-RNP and anti-Sm, presented with a 2-month history of cutaneous lesions affecting her upper trunk and upper limbs. Vesicle and blisters were observed on the aforementioned sites. She had been treated with non-steroidal anti-inflammatory drugs and short pulses of prednisone that improved partially her skin lesions. The lesions resolved without scarring but leaving hypopigmentation.
Demonstrate the clinical differential diagnosis of blisters in SLE
Describe the main immunopathologic findings of blistering eruptions in SLE
Explain the main features of bullous systemic lupus erythematosus
Discuss the extracutaneous associations of blistering eruptions in SLE
Describe the treatment of bullous systemic lupus erythematosus
Case 2: Hand lesions in SLE Hand lesions are relatively common in patients with SLE. Since these lesions are not always biopsied (especially those of the digits) a correct characterization may be lacking. These lesions can appear in patients with or without SLE. The differential diagnosis includes vasculitis, vasculopathy, chilblain lupus and palmoplantar lupus. Although localized, they are usually painful with functional impairment of loss of quality of life. Among them, the most common lesions are chilblain lupus. Response to topical treatment and antimalarials is typically poor. Chilblain lupus may not respond as well to systemic therapy compared to other visceral manifestations of SLE, hence being a treatment challenge both for rheumatologists and dermatologists.
A 40-year-old woman, with an 8-year history of cutaneous lesions in the face and arms (diagnosed of cutaneous lupus erythematosus by biopsy), arthritis and ANA+, treated with hydroxychloroquine and variable doses of prednisone, was admitted in the rheumatology department due to headache and visual loss with bilateral papilledema. Magnetic resonance imaging was normal but the ophthalmological exploration showed inferior branch retinal vein thrombosis in the left eye. The autoimmunity study showed ANA 1/1280 and positivity for anti-dsDNA, anti-RNP and anti-Sm. With the possible diagnosis of SLE-related pseudotumor cerebri, prednisone dose was increased, hydroxychloroquine was maintained and acetazolamide was added, with progressive vision recovery. Two months after discharge, she started to progressively present papulosquamous lesions on the back, neckline, back of the hands and fingertips, the latter being painful.
Describe the differential diagnosis of hand lesions in SLE
Understand the prognostic implications of hand lesions in SLE
Discuss how cutaneous manifestations may not respond to systemic treatments aimed at controlling active SLE
Explain chilblain lupus and its treatment
Describe available treatments strictly directed to the cutaneous component of lupus erythematosus
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