Disseminated Superficial Actinic Porokeratosis in a Patient With Sjögren Syndrome

Margherita Terranova, MD; Lauretta Amato, MD; Daniela Massi, MD; Paolo Fabbri, MD

Skinmed. 2003;2(6) 

In This Article

Abstract and Introduction

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-pred-nisolone. An incisional biopsy taken from a typical lesion of the right leg was performed.

Multiple, brown, keratotic lesions and two purpuric macules on the right leg.

Magnification: The hyperpigmented ring enclose a new keratotic, central area.

Laboratory tests showed increased erythrocyte sedimentation rate (67), C-reactive protein (1.0 U/dL), fibrinogen (512 mg/dL), immunoglobulin (Ig) G (1648 mg/dL), and IgA (448 mg/dL). The ANA titer was 1:160. Characterization of extractalde nuclear antigens demonstrated antibodies SSA (2.24) and SS-B (1.79). Thyreoglobulin and peroxydase autoantibody, and rheumatoid factor were present.

Histopathologic examination showed a column of parakeratosis that formed an indentation within a hyperorthokeratosic epidermis. Near the cornoid lamella, the granular layer was thin, and vacuolized keratinocytes were present beneath this area. The dermis showed no gross abnormalities except a focal, superficial, lymphohistiocytic infiltrate (Figure 3). Beacause of clinical and histopathologic findings, the diagnosis of disseminated superficial actinic porokeratosis (DSAP) was made.

Parakeratotic columns nearby a parakeratotic epidermis; vacuolized and diskeratotic keratinocytes beneath this area; focal, lymphohistiocytic infiltrate in the superficial dermis.