Disseminated Superficial Actinic Porokeratosis in a Patient With Sjögren Syndrome

Abstract

A 76-year-old woman presented to our department with intense itchiness, begun 3 years earlier, widespread to total body, especially to her limbs. Physical examination of the skin revealed multiple, small, brown, round lesions, with a diameter of about 2-6 mm, and a hyperpigmented, keratotic, slightly raised, palpable border. The skin within the ring was atrophic, hypopigmented, or mildly reddened. The lesions arose into atrophic skin where it was possible to observe some irregular, purpuric macules of variable size (Figures 1 and 2). Due to a previous diagnosis of necrotizing vasculitis of the legs and Sjögren syndrome, the patient was treated from 1999 with 8 mg/d prednisone and topical application of methyl-prednisolone. An incisional biopsy taken from a typical lesion of the right leg was performed.

Disseminated Porokeratosis with Idiopathic Thrombocytopenia - Case Report and Literature Review of Porokeratosis and Related Disorders

“…On the other hand, various antibody-related autoimmune diseases treated with systemic steroids and other immunosuppressive conditions such as chronic liver disease, HIV and organ transplantations have also been implicated in its etiopathology [ 11 ]. Despite that, our literature review established cases of association between disseminated porokeratosis and pyoderma gangrenosum [ 10 ], a case of dermatomyositis [ 12 ], scleroderma [ 13 ], Sjogren syndrome [ 14 ] and rheumatoid arthritis [ 15 ]. Authors suggest that immunosuppressant therapy is the reason for the occurrence of the porokeratosis [ 10 ][ 11 ].…”