Papa Syndrome and Cystic Acne

The acronym of PAPA syndrome (pyogenic sterile arthritis, pyoderma gangrenosum and acne) is suggested for newly recognized pleiotropic autosomal dominant disorder.

Cystic acne, aseptic arthritis and pyoderma gangrenosum are clinically distinct disorders within the broad class of inflammatory diseases. A three generation kindred with autosomal dominant transmission of these three disorders has been reported as PAPA syndrome (OMIM #604416). These three clinically distinct disorders may share a genetic etiology. Data further indicates that the role of genes outside the major histocompatibility locus in inflammatory disease [66].

It has been recently mapped to chromosome 15q mutation. The patients with PAPA syndrome responded well to intravenous gamma globulin and intra-articular corticosteroid therapy [8(5)].

In PAPA syndrome, typically joint disease precedes the onset of skin disorder which seems to develop during puberty.

Affected persons may have sulfonamide induced pancytopenia and abscesses at injection sites, diabetes and renal dysfunction may also be associated conditions. Several associations between skin and joint diseases have been described.

Like other acne-musculoskeletal syndromes, PAPA syndrome included corticosteroid responsive arthritis. In contrast to other acne-musculoskeletal syndromes pyoderma gangrenosum is an important feature of PAPA.

This distinctive lesion often occurs with inflammatory bowel disease, chronic active or persistent hepatitis, seronegative polyarthritis, rheumatoid arthritis, myeloproliferative disorders and severe acne.

The pathogenesis of PAPA syndrome is currently unknown but clinically it results form immunologic reactivity between a common skin microbe Propionibacterium and a component of skin and joints analogous to group A streptococcal induced rheumatic fever [72].

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