Pustules and Dystrophy of the Nails

Abstract

A 57-year-old Caucasian woman came to the outpatient clinic with a complaint of a 3-month history of redness, pain, and itching of her left fifth digit involving the periungual skin, dorsal fingertip, and skin over her distal interphalangeal joint. Prior therapy consisted of subungual injectable steroids with considerable pain and minimal relief of symptoms. Subsequently she noticed that the left first, third, and fourth digits and all of the digits of the right hand were similarly affected. Her past medical history was significant for asthma, depression, and fibromyalgia. Her family medical history was significant only for breast cancer in her mother. There was no family history of rash or chronic skin conditions. Her current medications included Estrostep, venlafaxine, and an albuterol inhaler as needed. She denied arthritic pain, fevers, chills, or any new sick contacts. Physical examination showed severe nail dystrophy, periungual edema, erythema of the distal digits, inflammatory plaques, and multiple small pustules (Figure ​(Figure11). We also noted patches with silver scaling involving her right ear and her right elbow and localized small lesions on the legs that, on further questioning, had begun in the month before presentation. Figure 1 Patient's fingers at initial presentation showed multiple pustules, erythema, edema, and severe nail dystrophy. Initial therapy consisted of psoralen and ultraviolet A light (PUVA) and subsequently an induction course of intravenous infliximab at 5 mg/kg on weeks 0, 2, and 6. Loratadine was added for itching relief. Minimal improvement was noted at 3 months, so the infliximab dose was increased to 7 mg/kg every 6 weeks. Methotrexate 10 mg/week was added at this time. She was also treated topically with Keralac and Aquaphor for skin protection. She showed some response with early nail regrowth and complete resolution of her ear, elbow, and leg lesions but still had persistent periungual edema and isolated pustules, so the infliximab dose was increased to 8 mg/kg intravenously every 6 weeks. She continued to improve, and topical tazarotene was added, as was clobetasone lotion around the cuticles twice daily. After 12 months of the current infliximab dose she had experienced significant resolution of disease, with three normal-appearing nails and only moderate dystrophy of the other nails. Unfortunately, the patient then developed gastroenteritis and discontinued her methotrexate. Her liver function tests began to rise, resulting in discontinuation of the infliximab. The patient admitted to heavy alcohol use during this time. Within 6 months of discontinuing therapy, she developed a severe flare of her skin disease, with involvement of all 10 digits and new inflammatory lesions on her buttocks, back, legs, and elbows. At this point her laboratory studies had normalized and she denied any further alcohol use. Infliximab was then reinstituted at 5 mg/kg along with wet compresses twice daily and halobetasol ointment with occlusion. Over the next month the infliximab was increased to 7.5 mg/kg every 6 weeks, resulting in marked improvement in her skin and nails. She currently has only a mild degree of erythema, scaling, and nail dystrophy, with early evident proximal normal nail regrowth (Figure ​(Figure22). Figure 2 Patient's fingers after several months of therapy showed significant improvement. What is her diagnosis? DIAGNOSIS: Acrodermatitis continua of Hallopeau.