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Adalimumab induced chilblain lupus in a patient with rheumatoid arthritis

Dear Editor, A 40-year-old caucasian female with no personal history of interest who had been diagnosed of rheumatoid arthritis (RA) in 2007, under treatment with Adalimumab biosimilar since November 2017 after failure with treatment with methotrexate was referred to the Dermatology Service complaining purple, painful acral lesions on the dorsum of the fingers of both hands for 3 months (since November 2019) (Figure 1) that did not yield to treatment with topical corticosteroids (Prednicarbate cream twice/day). Histological examination revealed epidermis with intense hyperkeratosis and hypergranulosis with vacuolar degeneration of the basal layer and lymphocytic inflammatory infiltrate in the papillary dermis and of perivascular and periadnexal location (Figure 2/HEx5). On the middle and reticular dermis, there was a slight increase in positive alcian blue mucinous material. Direct immunofluorescence showed deposition in the basal layer of Ig G, A, and C3. Blood sample test showed results within normality with positive ANA (1:320; previously negative) being negative anti dsDNA and ssDNA performed. The rest of complementary tests (immunoglobulins, complement, and proteinogram) were normal. Determination of anti-ADA antibodies was negative so no possible correlation with the development of chilblain lupus (CHLE) could be stated. Combination treatment with Hydroxychloroquine 200 mg/day and amlodipine 5 mg/day was established and adalimumab was withdrawal and switched to Sarilumab 200 mg every 14 days with improvement on the clinical picture in 2 months. The decision to switch to Sarilumab was agreed between the Rheumatology and Dermatology Service given the effect on the quality of life of the patient and the time of evolution of the clinic. CHLE is a variety of cutaneous lupus that has normally been associated with other forms of lupus and exceptionally RA. Currently, only seven cases associated with treatment with TNF blockers (5 infliximab, 1 etanercept, and 1 golimumab) have been reported, being our case the first associated with adalimumab. The characteristics of the affected patients are reflected in Table 1. It was initially described by Hutchinson in 1888 and a familial and sporadic form have been considered. The first one is due to a heterozygous mutation in the TREX1 gene that encodes a 30-50exonuclease. The sporadic form, which includes that caused by different drugs, remains with unknown etiopathogenesis. Diagnosis is mainly clinical and based on the criteria proposed by Su in 1994, as our patient fulfilling the two major criteria and one of the minor (negative results of testing for cryoglobulin and cold agglutinins). Antibody profile is unespecified and does not correlate with the severity or development of systemic lupus erythematous. CHLE should be considered as part of the spectrum of TNF-a antagonist-induced LE. It is noteworthy that the reported cases are all from patients with rheumatologic diseases prone to autoimmune disorders, and none in patients treated for psoriasis or inflammatory bowel disease. It usually presents a more benign clinical course and clinical lesions are practically indistinguishable from simple perniosis. On the therapeutical approach, the first measure to consider would be to protect the acral areas from low temperaturas on cold seasons. Treatment is not fully standarized. Topical corticosteroids (alone or associated with short cycles of systemic corticosteroids), topical calcineurin inhibitors (pimecrolimus and tacrolimus), calcium channel blockers (which reduce pain and erythema), chloroquine, mycophenolate mofetil, and F IGURE 1 Erythematoviolaceous patches on dorsum of fingers of the right hand