CASE
A 25-year-old female with a 9-year history of systemic lupus erythematosus (SLE) categorized by +ANA (1:640 speckled), +ds-DNA, +anti-Smith, +Anti-histone, palatal ulcers, hypocomplementemia (C3 and C4), malar rash, class V glomerulonephritis, discoid rash, non-scarring alopecia, arthralgias, myalgias and morning stiffness, was referred for management of severe desquamation of all finger tips, hyperextension of the thumbs, and violaceous hyperkeratotic plaques on the hands associated with shallow ulcerations and erosions (Figure 1). Medications for her SLE included aspirin (81 mg PO qd), belimumab (IV, q30d), hydroxychloroquine (400 mg PO qd), lisinopril (40 mg PO qd) and mycophenolate mofetil (500 mg PO bid).
Her distal extremity symptoms first presented as painless livedo reticularis and progressed to include painful and pruritic lesions. Chilblain lupus erythematosus (CHLE) was suspected because of the patients underlying SLE diagnosis and chronicity and the absence of triphasic skin color changes typically seen in Raynaud’s phenomenon (RP).4 Moreover, although the lesions initially worsened in the winter months and healed during the summer months, they chronically worsened until they were persistent year-round (Figure 2). On presentation, her symptoms had developed into painful ulcers that prevented her from completing activities of daily living or working. She had to keep her hands wrapped in bandages at all times.
Over a 5-year period, treatment with petrolatum ointment, neomycin and polymyxin ointment, mupirocin cream, 2.5% hydrocortisone cream, topical dapsone, pimecrolimus cream, betamethasone diproprionate ointment, clobetasol cream, pentoxifylline (400 mg tid), nifedipine (60 mg qd), prednisone (up to 40 mg qd), nitroglycerine paste, sildenafil (50 mg qd), and lifestyle modifications all failed. Workup and clinical evaluation were negative for evidence of systemic sclerosis (negative anti- Scl-70, anti-centromere antibodies, and no sclerodactyly) and antiphospholipid syndrome (APLS antibodies negative). Plain radiographs revealed soft tissue atrophy at the tip of the fingers and subluxation of right thumb interphalangeal joint. Upper extremity duplex studies did not reveal evidence of microvascular disease. Transthoracic echocardiogram failed to reveal valvular abnormalities.
Her distal extremity symptoms first presented as painless livedo reticularis and progressed to include painful and pruritic lesions. Chilblain lupus erythematosus (CHLE) was suspected because of the patients underlying SLE diagnosis and chronicity and the absence of triphasic skin color changes typically seen in Raynaud’s phenomenon (RP).4 Moreover, although the lesions initially worsened in the winter months and healed during the summer months, they chronically worsened until they were persistent year-round (Figure 2). On presentation, her symptoms had developed into painful ulcers that prevented her from completing activities of daily living or working. She had to keep her hands wrapped in bandages at all times.
Over a 5-year period, treatment with petrolatum ointment, neomycin and polymyxin ointment, mupirocin cream, 2.5% hydrocortisone cream, topical dapsone, pimecrolimus cream, betamethasone diproprionate ointment, clobetasol cream, pentoxifylline (400 mg tid), nifedipine (60 mg qd), prednisone (up to 40 mg qd), nitroglycerine paste, sildenafil (50 mg qd), and lifestyle modifications all failed. Workup and clinical evaluation were negative for evidence of systemic sclerosis (negative anti- Scl-70, anti-centromere antibodies, and no sclerodactyly) and antiphospholipid syndrome (APLS antibodies negative). Plain radiographs revealed soft tissue atrophy at the tip of the fingers and subluxation of right thumb interphalangeal joint. Upper extremity duplex studies did not reveal evidence of microvascular disease. Transthoracic echocardiogram failed to reveal valvular abnormalities.
DISCUSSION
CHLE is a very rare form of chronic cutaneous lupus erythematosus that presents with pernio persisting beyond the winter months.2,3 Although the pathogenesis is not completely understood, it is thought to be associated with vasoconstriction and microvascular injury provoked by exposure to cold and damp environments.3 On exam, patients classically present with pruritic, erythematous to violaceous macules, papules or plaques that can develop into blisters and ulcerations.3
Treatment options for CHLE are limited and mostly confined to
Treatment options for CHLE are limited and mostly confined to
