Psoriasiforme Onychopachydermoperiostitis der Großzehen: Das OP3GO-Syndrom

Abstract

A 46-years old male patient suffered for several years from painful swelling of the distal soft tissues of the great toes associated with onychodystrophy. Mycological studies were negative. By means of radiological and scintigrafic examination and because of the typical clinical signs, the diagnosis of psoriatic onycho-pachydermo-periostitis of the great toes was established. The syndrome which is highly evocative of rheumatoid psoriasis was first described by Fournié and co-workers in 1989. Oral retinoids were helpful in diminishing both pain and soft tissue swelling of the great toes.

Successful treatment of psoriatic onycho‐pachydermo periostitis (POPP) with adalimumab

“…Based on the features psoriatic onychodystrophy and connective tissue thickening of the distal phalanx, including a periosteal reaction (1), the patient was diagnosed as having POPP. Of note, concomitant features characteristic of psoriatic arthritis as observed in our patient have also been described in other patients (5), and in 6 of the 15 cases reported so far, HLA–B27 was positive (1–12).…”

“…POPP can be extremely painful and frequently causes significant functional impairment. It has been recognized as an uncommon subset of psoriatic arthritis and, to date, only 15 cases have been described worldwide (1–12). Therapy for this rare disease is based on experiences with treatment of psoriatic arthritis but, according to the few published case reports, nonsteroidal antiinflammatory drugs and sulfasalazine showed inconsistent and unsatisfactory results (1–3).…”

The psoriatic great toe or the psoriatic onycho-pachydermo-periostitis of great toe (OP3gt)

“…Però si tratta di un rilievo caratteristico dell'AP, difficilmente ritrovabile in altre affezioni, a parte il facile riconoscimento dell'osteoartropatia ipertrofica primaria. Per cui, la sua presenza ha un importante valore diagnostico, soprattutto quando associata ad onicopatia (13,14).…”

Onycho-Pachydermatit with extensive bone marrow edema predominant in the metacarpals: a “forme fruste” of POPP?

“…It has been described by several authors as a combination of findings as psoriatic onychodystrophy, connective tissue thickening above the distal phalanges accompanied with a periosteal reaction [1][2][3][4][5][6][7][8].…”

“…Psoriatic onycho-pachydermo-osteo/periostitis (POPP) syndrome is documented as a rare form of psoriatic arthritis [1][2][3][4][5][6][7][8]. The striking clinical features of the syndrome include psoriatic onychodystrophy, connective tissue thickening, and periostitis of the distal phalanges, producing a drumstick-like deformity.…”