[Pancreatic panniculitis with polyarthritis: PPP syndrome]. / Polyartritis, panniculitis en pancreatitis.

BACKGROUND: Diseases of the pancreas may present with extrapancreatic symptoms, such as (poly)arthritis or necrosis of subcutaneous fat. A combination of pancreatitis, panniculitis and (poly)arthritis is referred to as the PPP syndrome, which is associated with acute and chronic pancreatitis, as well as pancreatic malignancies. CASE DESCRIPTION: This article describes a patient which was admitted to our hospital with severe polyarthritis and panniculitis. A meticulous work-up revealed an underlying focal alcoholic pancreatitis. The clinical course in our patient illustrates the severity of the PPP syndrome and emphasizes the need of a multidisciplinary approach. CONCLUSION: Panniculitis and/or (poly)arthritis may be the first symptom of underlying pancreatic disease. Timely recognition and diagnosis is imperative for successful treatment and outcome. The multi-organ involvement in the PPP syndrome requires close collaboration across different medical departments.

Dermatological guidelines for monitoring methotrexate treatment reduce drug-survival compared to rheumatological guidelines.

BACKGROUND: Methotrexate (MTX) is widely used as disease modifying treatment for psoriasis and psoriatic arthritis (PsA). Rheumatological and dermatological guidelines to prevent MTX-induced adverse events diverge in the number and frequency of blood tests. These differences are not based on evidence indicating a higher risk for patients with psoriasis compared to PsA or rheumatic arthritis (RA). This raises the question if multiple testing increases safety, or results in false positive signals potentially leading to early withdrawal of an effective treatment. OBJECTIVE: Compare the effects of MTX monitoring strategies by rheumatologists and dermatologists regarding drug survival, reasons for withdrawal and safety. METHODS: Retrospective follow-up of all patients diagnosed with psoriasis by dermatologists or PsA by rheumatologists. Included were consecutive patients who started methotrexate (MTX) between 2006 and 2012 and had a scheduled follow-up by dermatologist or rheumatologist. Exclusions were: drug not started after the first prescription or incomplete availability of lab data. Data were extracted from electronic records: start and stop dates and dosing of MTX; treatment with folic acid and dose; reasons for withdrawal of MTX; numbers of blood sampling and types of laboratory tests performed for MTX safety; number of abnormal tests; occurrence of any serious adverse event (SAE). RESULTS: 190 Psoriasis and 196 PsA patients starting methotrexate (MTX) were included. Age and sex were comparable. PsA patients used higher initial and maximum doses of MTX and folic acid, but psoriasis patients had a higher frequency of abnormal laboratory results (0.14 vs 0.03 per treatment month, p<0.001), resulting in a striking difference in withdrawal of MTX for abnormal liver enzymes (15.8% vs 3.6%, p<0.001). In PsA patients MTX was withdrawn less frequently for ineffectiveness (15.8 vs 24.2%, p<0.05) leading to longer drug survival in the first course of treatment (37.4±30 vs 18.8±19.1 months). Repeated courses of MTX were more often prescribed by rheumatologists than by dermatologists. There were no significant differences in the occurrence of SAE (psoriasis 0.0041 vs PsA 0.0038 per treatment month) or death (1.6% vs 2.0%) between these groups. Hospital admissions related to infection were recorded in 6 (3.1%) PsA vs 4 (2.1%) psoriasis patients. CONCLUSION: Strict monitoring by dermatologists resulted in more abnormal findings, which reduced drug survival of MTX. The limited monitoring strategy by rheumatologists was not associated with more SAEs. Further research in other populations is needed to confirm whether reduced intensity of monitoring can optimize the use of MTX with sufficient long-term safety.

Discovery of a new human polyomavirus associated with trichodysplasia spinulosa in an immunocompromized patient.

The Polyomaviridae constitute a family of small DNA viruses infecting a variety of hosts. In humans, polyomaviruses can cause infections of the central nervous system, urinary tract, skin, and possibly the respiratory tract. Here we report the identification of a new human polyomavirus in plucked facial spines of a heart transplant patient with trichodysplasia spinulosa, a rare skin disease exclusively seen in immunocompromized patients. The trichodysplasia spinulosa-associated polyomavirus (TSV) genome was amplified through rolling-circle amplification and consists of a 5232-nucleotide circular DNA organized similarly to known polyomaviruses. Two putative "early" (small and large T antigen) and three putative "late" (VP1, VP2, VP3) genes were identified. The TSV large T antigen contains several domains (e.g. J-domain) and motifs (e.g. HPDKGG, pRb family-binding, zinc finger) described for other polyomaviruses and potentially involved in cellular transformation. Phylogenetic analysis revealed a close relationship of TSV with the Bornean orangutan polyomavirus and, more distantly, the Merkel cell polyomavirus that is found integrated in Merkel cell carcinomas of the skin. The presence of TSV in the affected patient's skin was confirmed by newly designed quantitative TSV-specific PCR, indicative of a viral load of 10(5) copies per cell. After topical cidofovir treatment, the lesions largely resolved coinciding with a reduction in TSV load. PCR screening demonstrated a 4% prevalence of TSV in an unrelated group of immunosuppressed transplant recipients without apparent disease. In conclusion, a new human polyomavirus was discovered and identified as the possible cause of trichodysplasia spinulosa in immunocompromized patients. The presence of TSV also in clinically unaffected individuals suggests frequent virus transmission causing subclinical, probably latent infections. Further studies have to reveal the impact of TSV infection in relation to other populations and diseases.