Pharming Group Announces Receipt of $10 Million Milestone Payment

LEIDEN, THE NETHERLANDS--(Marketwire - November 26, 2012) - Biotech company Pharming Group NV ("Pharming" or "the Company") (NYSE Euronext: PHARM) today announced that following the announcement by Pharming and Santarus on November 7, 2012 that the pivotal Phase III clinical study of RUCONEST® (recombinant human C1 esterase inhibitor) 50 U/kg for the treatment of acute attacks of angioedema in patients with Hereditary Angioedema (HAE) met its primary endpoint, and in accordance with the terms of the license agreement between Pharming and Santarus, a US$10 million milestone has now been paid to Pharming.

An additional US$5 million milestone will be payable to Pharming upon U.S. Food and Drug Administration (FDA) acceptance of the Biologics License Application (BLA) for review. Pharming and Santarus expect to submit the BLA for RUCONEST to the FDA in the first half of 2013.

Sijmen de Vries, Pharming CEO, commented: "We are pleased to have received this US$10 million milestone from our partner Santarus, which significantly strengthens our balance sheet and signals the beginning of a new chapter in the development of the Company."

About RUCONEST® and Hereditary Angioedema

RUCONEST® (INN conestat alfa) is a recombinant version of the human protein C1 inhibitor (C1INH). RUCONEST is produced through Pharming's proprietary technology in milk of transgenic rabbits and is approved in Europe for treatment of acute angioedema attacks in patients with HAE. RUCONEST® is an investigational drug in the U.S. and has been granted orphan drug designation for the treatment of acute attacks and prophylaxis of HAE, a genetic disorder in which the patient is deficient in or lacks a functional plasma protein C1 inhibitor, resulting in unpredictable and debilitating episodes of intense swelling of the extremities, face, trunk, genitals, abdomen and upper airway. The frequency and severity of HAE attacks vary and are most serious when they involve laryngeal edema, which can close the upper airway and cause death by asphyxiation. According to the U.S. Hereditary Angioedema Association, epidemiological estimates for HAE range from one in 10,000 to one in 50,000 individuals.

About Pharming Group NV

Pharming Group NV is developing innovative products for the treatment of unmet medical needs. RUCONEST® (RHUCIN® in non-European territories) is a recombinant human C1 inhibitor approved for the treatment of angioedema attacks in patients with HAE in all 27 EU countries plus Norway, Iceland and Liechtenstein, and is distributed in the EU by Swedish Orphan Biovitrum. Rhucin® is partnered with Santarus Inc (NASDAQ: SNTS) in North America where the drug has completed Phase III clinical development. The product is also being evaluated for various follow-on indications. Pharming has a unique GMP compliant, validated rabbit platform for the production of recombinant human proteins that, with the EU approval of Pharming's rhC1 inhibitor, has proven capable of producing industrial volumes of high quality recombinant human protein in a significantly more economical way through low upfront capital investment and manufacturing costs, compared to current cell based technologies. Pharming now plans to utilise this platform for the development of rhFVIII for the treatment of Haemophilia A.

Additional information is available on the Pharming website, www.pharming.com.

This press release contains forward looking statements that involve known and unknown risks, uncertainties and other factors, which may cause the actual results, performance or achievements of the Company to be materially different from the results, performance or achievements expressed or implied by these forward looking statements.

Press release (PDF): http://hugin.info/132866/R/1660435/537717.pdf

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Source: Pharming Group N.V. via Thomson Reuters ONE

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