Recalcitrant Diffuse Cutaneous Sarcoidosis With Perianal Involvement Responding to Adalimumab

We report a black male in his 50’s who presented to our dermatology clinic with a 20-year history of sarcoidosis. His chief complaint included skin lesions on the trunk, extremities, and perianal/gluteal region. His prior treatment consisted of cyclic corticosteroids for many years with minimal resolution of his cutaneous lesions. Chest radiograph revealed bilateral perihilar adenopathy, pulmonary function tests were normal. Physical exam revealed large infiltrated plaques on the trunk and proximal extremities (Figure 1). On his initial visit, he was unable to walk secondary to severe pain from perianal erosions and ulcers (Figure 2) which were biopsied. Histological examination of the perianal lesions revealed dermal noncaseating granulomas consistent with sarcoidosis. Tissue staining and culture for acid-fast bacilli and fungi were negative. The Tuberculin Skin Test using the Mantoux technique 0.1ml of 5 Tuberculin Units (TU) purified protein derivative (PPD) solution was negative. Adalimumab 80mg loading dose was administered, 40mg for the first week, then 40 mg every 2 weeks thereafter. The patient experienced dramatic improvement within 2 weeks (Figure 3) and complete resolution of all lesions, including the perianal area, within 4 weeks (Figure 4), the patient declined photograph of the perianal lesions but stated they resolved completely. The patient’s cutaneous lesions remained in remission on adalimumab 40mg every 2 weeks for one year when he moved from our area.Several reviews have concluded that adalimumab and infliximab demonstrate increased efficacy over etanercept in the treatment of cutaneous sarcoidosis.^1-3 One prospective randomized clinical trial has been conducted to date demonstrating the efficacy of adalimumab in the treatment cutaneous sarcoidosis.⁴ The increased efficacy is thought to be due to the ability of infliximab and adalimumab to bind both soluble and membrane-bound tumor necrosis factor (TNF) whereas etanercept binds primarily to soluble TNF.⁵ Adalimumab and infliximab also induce apoptosis of cells presenting the bound form of TNF6 1 whereas etanercept does not. Adalimumab was chosen for our patient over infliximab due to advantages in drug delivery. Adalimumab can be administered via biweekly subcutaneous injection done in the office or by the patient at home whereas infliximab must be administered by monthly infusion requiring monitoring for infusion reactions. An additional advantage of adalimumab is that it is a fully human monoclonal antibody and less likely to result in the formation of antibodies to the medication, infliximab is a chimeric antibody. Our patients’ response is in line with prior case reports, case series, and the recent RCT conducted by Pariser et al. Our case is unique in that it is the first documented case of perianal ulcerative sarcoidosis