Pharming Group N.V.

Regulatory Filings • Sep 2, 2014

P H A R M I N G

PHARMING TO SOUND THE GONG TO OPEN AMSTERDAM MARKET TO MARK A SUMMER OF SIGNIFICANT STRATEGIC PROGRESS FOR PHARMING

Leiden, The Netherlands, 2 September 2014 - Sijmen de Vries, CEO of Pharming Group, Jaap Blaak, Chairman of the Board of Supervisory Directors of Pharming Group and the Pharming management team, today visit Euronext Amsterdam to sound the gong to signal the opening of the market.

The gong ceremony marks a summer of significant strategic progress for Pharming:

• Last week, Pharming and its US partner, Salix, announced the initiation of a randomized double blind placebo-controlled, crossover, Phase II clinical study of RUCONEST®, (C1 Esterase Inhibitor [Recombinant]) 50 IU/kg, for prophylaxis of angioedema attacks in patients with hereditary angioedema (HAE).
• Earlier in August Pharming announced the acquisition of certain assets of Transgenic Rabbit Models SASU (TRM), a private French company in liquidation; for € 0.5 million in cash. Through the acquisition Pharming gained access to five potential new product leads (founder rabbits); recombinant- human (rh)-α-glucosidase for the treatment of Pompe's disease, rh-α-galactosidase for the treatment of Fabry's disease, rh-βcerebrosidase for the treatment of Gaucher's disease, rh-Factor VIII for the treatment of Haemophilia-A and rh-Factor IX for the treatment of Haemophilia-B. In addition, Pharming gained access to transgenic rabbit founder technology and know-how developed by TRM.
• In July Pharming announced that the Food and Drug Administration approved RUCONEST® (C1 Esterase Inhibitor [Recombinant]) 50 IU/kg for the treatment of acute angioedema attacks in adult and adolescent patients with hereditary angioedema (HAE) in the USA. A US\$20 million milestone payment from Salix will become payable upon the first commercial sale of Ruconest® in the US, representing significant non-dilutive funding to the company. Salix currently plans on making RUCONEST® available to patients during the fourth quarter of 2014.

Sijmen de Vries, Pharming CEO, said: we are delighted with the very significant regulatory and commercial progress we have made over the past summer, particularly the approval of RUCONEST® in the US for the treatment of acute angioedema attacks in patients with Hereditary Angioedema, the initiation of the clinical study with RUCONEST® for Prophylaxis of Hereditary Angioedema, and the acquisition of assets from TRM.

P H A R M I N G

About Pharming Group NV

Pharming Group NV is developing innovative products for the treatment of unmet medical needs. RUCONEST® (conestat alfa) is a recombinant human C1 esterase inhibitor approved for the treatment of angioedema attacks in patients with HAE in the USA, Israel, all 27 EU countries plus Norway, Iceland and Liechtenstein. RUCONEST® is distributed in the EU by Swedish Orphan Biovitrum. RUCONEST® is partnered with Salix Pharmaceuticals Inc. (NASDAQ: SLXP) in North America. The product is also being evaluated for various follow-on indications, including prophylaxis of angioedema attacks in patients with hereditary angioedema. Pharming has a unique GMP compliant, validated platform for the production of recombinant human proteins that has proven capable of producing industrial volumes of high quality recombinant human protein in a more economical way compared to current cell based technologies. In July 2013, the platform was partnered with Shanghai Institute for Pharmaceutical Industry (SIPI), a Sinopharm Company, for joint global development of new products. Pre- clinical development and manufacturing will take place at SIPI and are funded by SIPI. Pharming and SIPI initially plan to utilize this platform for the development of rhFVIII for the treatment of Haemophilia A. Additional information is available on the Pharming website; www.pharming.com.

Contact

Sijmen de Vries, CEO: T: +31 71 524 7400

FTI Consulting Julia Phillips/ John Dineen, T: +44 203 727 1136