Dermatitis Might Reflect a Systemic Autoinflammatory Syndrome

We are often asked to assess a relatively nonspecific dermatitis characterized by erythematous macules, papules, or plaques with little scale, which reveals mild spongiosis and a lymphohistiocytic perivascular infiltrate on biopsy examination. Pruritus is common; patch testing identifies a cause in some, but cases without an identifiable cause may be part of an autoinflammatory syndrome. Patients with autoinflammatory disease have systemic manifestations, including fever, arthritis, serositis, and weight loss, or elevated markers of inflammation, such as erythrocyte sedimentation rate or C-reactive protein. Autoinflammatory syndromes have been linked to genetic abnormalities.

These authors explored a link between an otherwise nonspecific der…