Location
Philadelphia, PA
Start Date
3-5-2023 1:00 PM
End Date
3-5-2023 4:00 PM
Description
Introduction
Neutrophilic urticarial dermatosis (NUD) is a cutaneous eruption that is most commonly seen in conjunction with systemic disease. The diseases include but are not limited to: adult-onset Still disease, Schnitzler syndrome, systemic lupus erythematosus (SLE), and systemic juvenile idiopathic arthritis. The eruption presents as rose colored to pink slightly raised plaques with minimal epidermal change. There are subtle morphologic differences in presentation when compared to the classic wheals seen in histamine mediated urticaria. This case demonstrates the importance of recognizing the transient clinical manifestations of NUD and confirming with histologic evaluation as a way to help identify underlying systemic disease and avoid subsequent complications and morbidity.
Case Report
We present a previously healthy 8-year-old female who was evaluated in the setting of transient pruritic cutaneous eruption and migrating inflammatory arthritis for a period of 3 months. Her history was complicated by multiple upper respiratory infections that were treated with oral antibiotics over that same time period. The intermittent cutaneous manifestations initially involved the distal lower extremities and would last for 1-2 days before self-resolving. The flares progressed to involve larger body surface areas with extension to the proximal extremities, trunk and face. When seen for initial evaluation by dermatology, clinical and histologic evaluation of the eruption were consistent with NUD. Given concern for underlying inflammatory disease, laboratory workup was expanded and she was seen to have clinically significant proteinuria. Serologies were repeated and came back positive for ANA and anti-dsDNA, and she met American College of Rheumatology Criteria for SLE. Induction therapy consisted of corticosteroids, cyclophosphamide, and rituximab due to subsequent renal biopsy showing active disease. She has responded well, but treatment remains ongoing.
Discussion
Prior to its’ establishment as a separate clinical entity, most cases of NUD fell under the umbrella of neutrophilic urticaria, which carries limited clinical significance. The lesions are similar from a morphologic perspective but exhibit slightly different histopathologic features. Many patients with NUD concomitantly have systemic inflammatory disease, but this is not always the case. There have been numerous reports of NUD in patients with SLE. In a few of these cases, NUD was the initial presenting symptom of SLE. NUD is often misdiagnosed clinically as urticaria, and failure of oral antihistamines to control cutaneous manifestations can be a helpful clue. The neutrophilic migration inhibitors dapsone and colchicine have shown promising effects.
Conclusion
We present a rare case of a young female diagnosed with SLE with both joint and kidney involvement. The cutaneous eruption of NUD may be due to similar cytokine mediated inflammatory pathways as its associated systemic inflammatory disease, but the exact pathogenesis remains unknown. Therefore, in the right clinical context, the diagnosis of NUD should prompt careful and thorough evaluation so that providers can monitor for and manage systemic disease as early as possible.
Embargo Period
6-6-2024
Neutrophilic urticarial dermatosis: The presenting sign of systemic lupus erythematosus in a pediatric patient
Philadelphia, PA
Introduction
Neutrophilic urticarial dermatosis (NUD) is a cutaneous eruption that is most commonly seen in conjunction with systemic disease. The diseases include but are not limited to: adult-onset Still disease, Schnitzler syndrome, systemic lupus erythematosus (SLE), and systemic juvenile idiopathic arthritis. The eruption presents as rose colored to pink slightly raised plaques with minimal epidermal change. There are subtle morphologic differences in presentation when compared to the classic wheals seen in histamine mediated urticaria. This case demonstrates the importance of recognizing the transient clinical manifestations of NUD and confirming with histologic evaluation as a way to help identify underlying systemic disease and avoid subsequent complications and morbidity.
Case Report
We present a previously healthy 8-year-old female who was evaluated in the setting of transient pruritic cutaneous eruption and migrating inflammatory arthritis for a period of 3 months. Her history was complicated by multiple upper respiratory infections that were treated with oral antibiotics over that same time period. The intermittent cutaneous manifestations initially involved the distal lower extremities and would last for 1-2 days before self-resolving. The flares progressed to involve larger body surface areas with extension to the proximal extremities, trunk and face. When seen for initial evaluation by dermatology, clinical and histologic evaluation of the eruption were consistent with NUD. Given concern for underlying inflammatory disease, laboratory workup was expanded and she was seen to have clinically significant proteinuria. Serologies were repeated and came back positive for ANA and anti-dsDNA, and she met American College of Rheumatology Criteria for SLE. Induction therapy consisted of corticosteroids, cyclophosphamide, and rituximab due to subsequent renal biopsy showing active disease. She has responded well, but treatment remains ongoing.
Discussion
Prior to its’ establishment as a separate clinical entity, most cases of NUD fell under the umbrella of neutrophilic urticaria, which carries limited clinical significance. The lesions are similar from a morphologic perspective but exhibit slightly different histopathologic features. Many patients with NUD concomitantly have systemic inflammatory disease, but this is not always the case. There have been numerous reports of NUD in patients with SLE. In a few of these cases, NUD was the initial presenting symptom of SLE. NUD is often misdiagnosed clinically as urticaria, and failure of oral antihistamines to control cutaneous manifestations can be a helpful clue. The neutrophilic migration inhibitors dapsone and colchicine have shown promising effects.
Conclusion
We present a rare case of a young female diagnosed with SLE with both joint and kidney involvement. The cutaneous eruption of NUD may be due to similar cytokine mediated inflammatory pathways as its associated systemic inflammatory disease, but the exact pathogenesis remains unknown. Therefore, in the right clinical context, the diagnosis of NUD should prompt careful and thorough evaluation so that providers can monitor for and manage systemic disease as early as possible.