Popp syndrome L40.50

Fournie 1989

POPP is the acronym for "psoriatic onycho-pachyderma-periostitis". POPP syndrome is characterized by psoriatic onychodystrophy, connective tissue thickening and periostitis of the distal phalanges (DPs), which leads to a drumstick-like deformity. Onycholysis often occurs simultaneously. In a larger series (n=31), the big toes (DPs) were affected in around 70% of cases and the distal interphalangeal joints (DIPs) in around 30%, with the damage often detectable radiographically (Sakellariou GT et al. 2021).

Radiologically, there is usually evidence of lamellar periosteal reactions in the acral areas of the affected distal phalanges.

m>w

In one larger study (n=31), the mean age was 44.9 years

The therapy is based on clinical experience in the treatment of psoriatic arthritis (see below Arthritis, psoriatic and psoriasis arthropathica). Initial: disease-modifying antirheumatic drugs(DMARD).

Alterative: sulfasalazine.

If insufficient, consider the use of methotrexate. Methotrexate (MTX) appears to be only moderately effective (Ochiai Tet al. 2006; Sakellariou GT et al. 2021).

Alternative: Success has been achieved with IL-17 inhibitors (secukinumab 150 mg) and TNF-alpha blockers. Case reports exist on adalimumab.

Regarding further treatment of psoriatic onychodystrophy in POPP syndrome, see also Nail psoriasis.

1. Anders HJ et al (2002) Psoriatic onycho-pachydermo periostitis Psoriatic onycho-pachydermo periostitis. Z Rheumatol 61:601-604
2. Fournie B et al (1989) Psoriatic onycho-pachydermo-periostitis of the big toe: anatomo-clinical study and physiopathogenic approach apropos of 4 cases. Rev Rhum Mal Osteoartic 56:579-582
3. Goupille P et al (1996) Incidence of osteoperiostitis of the great toe in psoriatic arthritis. J Rheumatol 23: 1553-1556
4. Ochiai T et al (2006) Psoriatic onycho-pachydermo-periostitis successfully treated with low-dose methotrexate. Med Sci Monit12: CS27 - CS30
5. Sakellariou GT et al (2021) Psoriatic onycho-pachydermo periostitis (POPP): a case report treated successfully with IL-17 blockade and a literature review on characteristics, pathogenesis, and treatment. Clin Rheumatol 40:4749-4757.
6. Schilling F(2003) Diskussionsbeitrag zur Kasuistik über die psoriatische Onycho-Pachydermo-Periostitis [Contribution to the discussion of a case of psoriatic onycho-pachydermoperiostitis]. Z Rheumatol 62:206-207.