scholarly journals Activating CARD14 Mutations Are Associated with Generalized Pustular Psoriasis but Rarely Account for Familial Recurrence in Psoriasis Vulgaris

2015 ◽  
Vol 135 (12) ◽  
pp. 2964-2970 ◽  
Author(s):  
Dorottya M. Berki ◽  
Lu Liu ◽  
Siew-Eng Choon ◽  
A. David Burden ◽  
Christopher E.M. Griffiths ◽  
...  
Keyword(s):  
Psoriasis Vulgaris ◽  
Pustular Psoriasis ◽  
Generalized Pustular Psoriasis

scholarly journals A Case of Old Age-Onset Generalized Pustular Psoriasis with a Deficiency of IL-36RN (DITRA) Treated by Granulocyte and Monocyte Apheresis

2015 ◽  
Vol 7 (1) ◽  
pp. 29-35 ◽  
Author(s):  
Chiharu Tominaga ◽  
Masaaki Yamamoto ◽  
Yasutomo Imai ◽  
Kiyofumi Yamanishi
Keyword(s):  
Receptor Antagonist ◽  
Dose Reduction ◽  
Old Age ◽  
Psoriasis Vulgaris ◽  
Serum Levels ◽  
Pustular Psoriasis ◽  
Generalized Pustular Psoriasis ◽  
Homozygous Mutations ◽  
Inflammatory Infiltrates

A 78-year-old woman who had been suffering from psoriasis vulgaris for 31 years was admitted to hospital because of her erythroderma. A toxic eruption was suspected and she was treated with prednisolone 30 mg daily. However, it was ineffective and, suspecting psoriatic erythroderma, cyclosporine 150 mg daily was administered with tapering of the prednisolone. Two weeks after a dose reduction of cyclosporine to 100 mg/day, erythroderma with widespread generalized pustules and fever developed. Histology of a biopsy revealed inflammatory infiltrates in the skin with a spongiform pustule of Kogoj, which was consistent with generalized pustular psoriasis (GPP). Her pustules improved with additional etretinate 20 mg/day, but the erythroderma persisted and she consulted us. Three sessions of granulocyte and monocyte apheresis once weekly were effective for her condition and decreased her serum levels of IL-6 and IL-8. She had homozygous mutations of c.[28C>T] in IL36RN which cause p.[Arg10Ter]. She is the oldest reported case of GPP with a deficiency of interleukin-36 receptor antagonist (DITRA), although GPP in DITRA has been suggested to usually occur in younger cases with no pre-existing psoriasis vulgaris.

scholarly journals Anti-laminin-gamma 1 Pemphigoid with Generalized Pustular Psoriasis and Psoriasis Vulgaris

2016 ◽  
Vol 96 (1) ◽  
pp. 120-121 ◽  
Author(s):  
Y Fujimura ◽  
K Natsuga ◽  
Y Hamade ◽  
Y Nomura ◽  
Y Kaku ◽  
...  
Keyword(s):  
Psoriasis Vulgaris ◽  
Pustular Psoriasis ◽  
Generalized Pustular Psoriasis

scholarly journals The Majority of Generalized Pustular Psoriasis without Psoriasis Vulgaris Is Caused by Deficiency of Interleukin-36 Receptor Antagonist

2013 ◽  
Vol 133 (11) ◽  
pp. 2514-2521 ◽  
Author(s):  
Kazumitsu Sugiura ◽  
Akemi Takemoto ◽  
Michiya Yamaguchi ◽  
Hidetoshi Takahashi ◽  
Yukiko Shoda ◽  
...  
Keyword(s):  
Receptor Antagonist ◽  
Psoriasis Vulgaris ◽  
Pustular Psoriasis ◽  
Generalized Pustular Psoriasis

scholarly journals Serum Procalcitonin and Presepsin Levels in Patients with Generalized Pustular Psoriasis

2018 ◽  
Vol 2018 ◽  
pp. 1-6
Author(s):  
Makoto Nagai ◽  
Yasutomo Imai ◽  
Yoshihiro Wada ◽  
Minori Kusakabe ◽  
Kiyofumi Yamanishi
Keyword(s):  
Psoriatic Arthritis ◽  
Psoriasis Vulgaris ◽  
High Serum ◽  
Serum Biomarkers ◽  
Pustular Psoriasis ◽  
Cutoff Value ◽  
Generalized Pustular Psoriasis ◽  
The Mean ◽  
Diagnosis Of Infection

Patients with generalized pustular psoriasis (GPP) often present with symptoms that must be differentiated from sepsis. Procalcitonin (PCT) and presepsin (P-SEP) are widely used as biomarkers for sepsis; therefore, we examined the serum PCT and P-SEP levels in patients with psoriatic diseases. The enrolled patients included 27 with psoriasis vulgaris (PV) (22 males, 5 females; mean age 47.7 years), 12 with psoriatic arthritis (PsA) (8 males, 4 females; mean age 51.3 years), and 15 with GPP (10 males, 5 females; mean age 63.7 years). The mean serum PCT levels in patients with PV, PsA, and GPP were 0.01 ng/mL (25th–75th percentile; 0.00–0.03), 0.013 ng/mL (0.00–0.03), and 0.12 ng/mL (0.05–0.18), respectively; the levels of PCT were higher for patients with GPP than with PV or PsA but were lower than the PCT cutoff value (0.5 ng/mL) for the diagnosis of infection. The mean serum P-SEP levels in patients with PV, PsA, and GPP were 144.9 pg/mL (25th–75th percentile; 78–181), 168.1 pg/mL (124–203), and 479.9 pg/mL (216–581), respectively. Unexpectedly, the levels of P-SEP in the patients with GPP were as high as the P-SEP cutoff value (317 to 647 pg/mL) used for the diagnosis of infection. We also found that neutrophils produced P-SEP, suggesting that the high serum P-SEP levels in patients with GPP might arise at least in part due to the P-SEP derived from neutrophils activated in GPP. Both serum PCT and P-SEP might therefore be useful as novel serum biomarkers for GPP because their levels were decreased by GPP treatments. However, the measurement of PCT might be more useful than the measurement of P-SEP for discriminating between GPP and sepsis.

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