Abstract
An eight-year-old female presented with one year history of rash on the face, hands, elbows, and feet. Associated symptoms included intermittent fevers, joint pain of the lower extremities, and cervical adenopathy. Clinical examination was significant for erythematous plaques with scale along the philtrum, cheeks, and oral commissures, periocular erythema, well-demarcated orange-hued plaques on the extremities, and hyperkeratosis of the feet with associated onychodystrophy of the right fifth digit. Her work up included multiple negative tests for influenza and COVID-19 and negative allergy skin prick testing. Labs, including CBC, CMP, CRP, ESR, CK, aldolase, and ASO titer, were normal. She trialed oral antibiotics, antihistamines, and topical anti-inflammatory agents (hydrocortisone ointment 2.5%, calcipotriene cream 0.005%, halobetasol propionate-tazarotene cream 0.01-0.045%, tacrolimus 0.03% ointment) without improvement. Initial biopsy performed at an outside institution was reported as spongiotic dermatitis. She was subsequently started on dupilumab and received three doses; despite this treatment, had progression of her symptoms. The findings were clinically consistent with pityriasis rubra pilaris (PRP) or Wong-type dermatomyositis, neither of which could be reconciled with the prior biopsy results. A repeat biopsy was obtained from the left upper extremity, which showed regular epidermal hyperplasia with acanthosis, hyperkeratosis with alternating areas of compact orthokeratosis and parakeratosis, and mild superficial perivascular lymphohistiocytic inflammation. Final pathologic findings were most consistent with PRP. Here, we present a case of juvenile PRP with systemic symptoms, which was initially diagnosed as spongiotic dermatitis and progressed on therapies. Our case underscores the importance of clinical and dermatopathology congruency in informing treatment and management.