J Cutan Pathol 2010: 37: 932 – 937 doi: 10.1111/j.1600-0560.2010.01570.x John Wiley & Sons. Printed in Singapore Copyright 2010 John Wiley & Sons A/S Persistent pruritic papules and plaques: a characteristic histopathologic presentation seen in a subset of patients with adult-onset and juvenile Still’s disease Background: ‘Persistent pruritic papules and plaques’ of Still’s disease represents a recently described eruption seen in a subset of patients. Most cases reported in the literature to date have been associated with adult-onset Still’s disease. Methods: We present the clinical and histopathologic examinations of three female patients ranging in age from 15 to 54 years. Results: Our three patients each presented with clinical findings consistent with Still’s disease. The youngest patient suffered from the juvenile form of Still’s disease (systemic-onset juvenile rheumatoid arthritis). Each patient had a persistent, pruritic eruption with histopathologic findings of dyskeratosis confined to the upper layers of the epidermis as well as a sparse superficial dermal infiltrate containing scattered neutrophils. Conclusions: These cases confirm the characteristic clinical and histopathologic findings of ‘persistent papules and plaques of Still’s disease’ and show the potential for this eruption in both the adult and juvenile age groups. Fortna RR, Gudjonsson JE, Seidel G, DiCostanzo D, Jacobson M, Kopelman M, Patel RM. Persistent pruritic papules and plaques: a characteristic histopathologic presentation seen in a subset of patients with adult-onset and juvenile Still’s disease. J Cutan Pathol 2010; 37: 932 – 937. 2010 John Wiley & Sons A/S. Ryan R. Fortna 1 , Johann E. Gudjonsson 2 , Gregory Seidel 3,4 , Damian DiCostanzo 3,4 , Mark Jacobson 3,4 , Margaret Kopelman 2 and Rajiv M. Patel 1,2 1 Department of Pathology, 2 Department of Dermatology, University of Michigan Medical Center, Ann Arbor, MI 48109, USA, 3 Department of Pathology, Montefiore Medical Center, Albert Einstein College of Medicine, Bronx, NY 10461, USA and 4 Dermpath Diagnostics, Port Chester, NY 10573 Rajiv M. Patel, Departments of Pathology and Dermatology, University of Michigan Medical Center, M3261 Medical Sciences I, 1301 Catherine Rd., Ann Arbor, MI 48109, USA Tel: +734 764 4460 Fax: +734 764 4690 e-mail: rajivpat@med.umich.edu, pate0057@gmail.com Accepted for publication April 19, 2010 Still’s disease is an idiopathic inflammatory disorder with varied clinical manifestations. Its diagnosis is one of exclusion and is often difficult and delayed. The four cardinal features of the disor- der include polyarthritis/polyarthralgia, high spiking fevers, leukocytosis with neutrophilia, and evanes- cent skin rash. Whether the disease presents in adulthood [adult-onset Still’s disease (AOSD)] or childhood [systemic form of juvenile idiopathic arthritis (systemic JIA)], skin manifestations are often a critical component of disease recognition. The classic Still’s rash consists of an evanescent, non-pruritic, salmon-colored morbilliform eruption; however, a distinct type of Still’s rash has recently been described, which consists of persistent (fixed), pruritic papules and plaques which often contain fine scale. 1–5 The clinicopathologic features of the latter eruption appear to be sufficiently characteristic as to be useful in aiding the diagnosis of the disorder. We present three patients with a clinical diagnosis of 932