Doctor, Why is My Skin So Thick Doctor, Why is My Skin So Thick?: A Not So Common Case of Sclerodactyly Lukas LaSyone, MD, MPHTM Rheumatology Fellow, PGY4 Olive View - UCLA Medical Center

HPI: 55M with HTN presented to ED with 3 months of progressive pain in his upper and lower extremities Described as a burning pain with radiation down into his fingers and toes During this time, he noted unusual changes to his skin. His forearms and shins had become thick and he noted that his skin “pulled into lines” when he raised his arms.

HPI continued: He denied joint pains, swelling or stiffness, but could no longer close his fist fully. ROS: Noted 30 lbs unintentional weight loss. Denied fevers, chills, night sweats, cough, difficulty breathing or swollen glands. He had no weakness, Raynaud’s or other rashes.

HPI continued: Previously evaluated in the ED 3 months prior for similar complaints and chest pain. EKG at the time with non-specific changes CK normal B12 low Started on B12 supplementation, ASA, atorvastatin and discharged home Outpatient treadmill stress testing was later negative At the time, drinking 6-7 glasses of whiskey nightly No EtOH since hospital discharge Prior ½ PPD smoking history Denies FHX of autoimmune disease

Exam: Exam notable for diffuse woody edema involving bilateral upper and lower extremities, notably sparing hands and feet. Characteristic skin changes shown below

Labs: CBC: 15,000/14/40/500,000 ANC 1:160 (speckled, nucleolar) ESR 40 ANC 7.6 (50%) ALC 2.6 (17%) Absolute eosinophil count 3.7(25%) ANC 1:160 (speckled, nucleolar) ENAs all neg aPL labs all neg ESR 40 CRP 73 SPEP/UPEP with hypergammaglobulinemia

Workup continued: NEXT STEPS? DDx? Rx? MRI: Right and left leg subcutaneous edema/inflammation is mainly along the deep fascial plane. Minimal subfascial inter-muscular soft tissue edema/inflammation also present. No drainable fluid collections. No significant myositis. NEXT STEPS? DDx? Rx?

Case continued: Underwent full-thickness LLE skin biopsy 2 weeks later Pathology demonstrated skin, subcutaneous tissue and fascia with septal fibrosis and edematous changes of fascia with mixed inflammatory infiltrate consisting of lymphohistiocytes, plasma cells, some eosinophils and neutrophils Low (left) and high (right) power images of fascia demonstrating chronic inflammatory infiltrate

Case continued: Given his classic skin findings, characteristic MRI, and full-thickness skin biopsy with chronic inflammatory infiltrate including eosinophils, he was started on prednisone 60 mg daily for presumed diagnosis of eosinophilic fasciitis (EF). Additional workup to rule out underlying malignancy was unrevealing.

Case continued: With minimal improvement in his skin induration at hospital follow-up four weeks later, his prednisone was increased to 100 mg daily (1 mg/kg). Methotrexate 20 mg weekly was added as a steroid-sparing agent. He was able to make a fist with his hands at interval follow-up four weeks later. Given some mild softening of the skin, a slow prednisone taper of 20 mg per day every two weeks was initiated.

Eosinophilic Fasciitis First described by Shulman in 1974, EF is a rare connective tissue disease of unknown etiology characterized by progressive and symmetric subcutaneous edema and ultimately induration. Although typically involving only the distal extremities, EF can rarely be widespread with truncal involvement. The lack of distal digit involvement is a classic hallmark that can be used as a distinguishing feature from other sclerosing skin disorders, including scleroderma.

Eosinophilic Fasciitis Peripheral eosinophilia is present in up to 80% of cases. A small number of patients may also present with thrombocytopenia, aplastic anemia, myelodysplasia and leukemia. Treatment is aimed at preventing complications. High-dose prednisone (up to 1 mg/kg) and methotrexate may result in gradual improvement over many months.

Take-away EF is a rare scleroderma mimic that presents as symmetric skin induration that spares that digits and is not associated with Raynaud’s or abnormal nailfold capillaroscopy.