PASS syndrome is a rare inflammatory disease characterized by a chronic-relapsing course of pyoderma gangrenosum, acne vulgaris, hidradenitis suppurativa and ankylosing spondylitis. Here, we describe a case of a patient with spontaneously recurrent purulent skin lesions along with seronegative spondylarthritis consistent with the PASS syndrome. During his disease exacerbation, the patient displayed episodes of fever along with elevated serum levels of interleukin (IL)-1β. Skin lesions were characterized by sterile neutrophilic infiltrates and showed a rapid response to the IL-1 receptor antagonist anakinra (Kineret®) consistent with the autoinflammatory nature of this disease. However, unlike other autoinflammatory diseases such as PAPA and PAPASH, we did not find mutations in the gene PSTPIP1, raising the possibility that other specific mutations in the IL-1 pathway may be involved.
Cutaneous and subcutaneous cysts with ciliated pseudostratified columnar (respiratory) epithelium present a diagnostic dilemma. We report a case of a bronchogenic cyst occurring on the back. The differential diagnosis includes branchial cleft cyst, thyroglossal duct cyst, cutaneous ciliated cyst, and mature cystic teratoma. We review reports of extrapulmonary bronchogenic cysts and discuss their possible embryology.
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