Case 1: 35-year-old Mestizo female

Bernardo Pons-Estel

A 35-year-old Mestizo female was diagnosed with systemic lupus erythematosus (SLE) in 2005 based on polyarthritis, malar rash, photosensitivity, mucosal ulcerations, positive ANA and anti-dsDNA, and low complement. She was treated with prednisone 20–30 mg/day and hydroxychloroquine (HCQ) 400 mg/day. In June 2010, lupus pneumonitis was diagnosed.

In July 2018, she was first admitted to our hospital. She was cushingoid and had fever, fatigue, malar rash, oral ulcers, alopecia, polyarthritis, oedema, multiple purplish-red streaks, and active erythematous, palpable and painful subcutaneous indurated nodules/plaques located on her face, proximal lower extremities and abdomen; some were ulcerated.

Laboratory tests RBC 3.8 (x10¹²/L), hemoglobin 11.8 g/dl, WBC 2,3 (x10⁹/L), platelets 62 (x10⁹/L), ESR 8 mm, CRP 0.8 mg/L, serum ferritin 1,487 ng/ml, ALAT 70 UI/ml, ASAT 26 UI/ml, GGT 64 U/L, BUN 43 mg/dl, serum creatinine 1.54 mg/dl, GFR 67 mL/min, cholesterol 186 mg/dL, triglycerides 149 mg/dl, proteinuria 210 mg/24 h, ANA 1/320, speckled pattern, anti-Sm (+) and anti-dsDNA, anti-U1RNP, anti-Ro and anti-La all (-). C3 75 mg/dl, C4 10, Coombs test (-), procalcitonin 0.29 ng/ml (<0.5). VDRL and viral serologies were negative. A cutaneous ulcer culture showed proteus mirabilis. Her SLEDAI was19. Three skin biopsies of indurated lesions all showed lobular panniculitis.

She was treated with IV methylprednisolone (500 mg/day/3 days), and high-dose intravenous immunoglobulin, and discharged with mycophenolate mofetil 1 g/day, HCQ 400 mg/day, prednisone 20 mg/day and TMS-SMX 860/160 BID for 5 days. A week later she was re-admitted with fever, pancytopenia, hepatosplenomegaly, lymphadenopathy and several painful, indurated erythematous lesions. Her SLEDAI was 13. A bone marrow aspiration and biopsy was interpreted as a macrophage activated syndrome in the context of SLE exacerbation and treated with IV dexamethasone, colony stimulating factor, and rituximab 1000 mg. Despite treatment, she remained severely ill with fever, asthenia, petechiae and purpura on her abdomen and thighs, and pancytopenia. Due to disease severity, treatment with etoposide was indicated. Finally, the patient presented an acute episode of respiratory distress followed by death.

Discussion Points

• Interpreting the different skin manifestations in a patient with SLE

• Analyzing complications and differential diagnoses with other associated diseases

Discussion Points Case 2: 28-year-old Caucasian female

Annegret Kuhn

A 28-year-old Caucasian female was diagnosed with systemic lupus erythematosus (SLE) in 1996 and presented with severe, erythematous, scarring discoid lesions on the scalp, face, and hands. She had been resistant to a high number of therapeutic agents including hydroxychloroquine, chloroquine, methotrexate, isotretinoin, and cyclophosphamide for several years and had developed various side effects. Moreover, the long-term medication for SLE had not influenced her skin lesions in the past months. In December 2009, the patient received alitretinoin, which was administered orally with a daily dose of 30 mg. The patient showed continuous improvement of the discoid skin manifestations over a period of 5 months and finally a total clearance of most lesions. No serious adverse events were recorded during treatment with alitretinoin; however, the patient experienced recurring headache, which was successfully treated with nonsteroidal anti-inflammatory drugs. Therefore, alitretinoin was reduced to a daily dose of 10 mg.

The vitamin-A derivative, alitretinoin, has been approved for use in severe chronic hand eczema unresponsive to treatment with potent topical corticosteroids. This case suggests that alitretinoin could also be an effective alternative in the treatment of cutaneous manifestations in SLE; however, randomized controlled trials are needed to prove the efficacy and evaluate the safety of alitretinoin in this disease.

Discussion Points

• Treatment of therapy-resistant cutaneous manifestations in SLE

• Therapeutic guidelines in cutaneous lupus erythematosus

Learning Objectives

• Explain different possible skin manifestations in patients with SLE

• Discuss complications, differential diagnosis with allied diseases and treatment approaches

• Describe the spectrum of manifestations in cutaneous lupus, including: ACLE, CCLE, SCLE and ICLE

• Explain the therapeutic guidelines in cutaneous lupus

• Describe preventive strategies in cutaneous lupus including photoprotection

• Describe topical treatment options in cutaneous lupus

• Discuss common and experimental systemic treatment options in cutaneous lupus

Reference

1. Kuhn A, Patsinakidis N, Luger TA. Alitretinoin for cutaneous lupus erythematosus. J Am Acad Dermatol 2012;67:123–126.