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Woman with Erdheim-Chester disease: Anakinra-responsive lichen planus


Anakinra ( Kineret ) is a recombinant form of interleukin-1 receptor antagonist. It is the drug of choice for Schnitzler syndrome and cryopyrin-associated periodic syndromes.
It has also recently been demonstrated to have activity in the treatment of the non-Langerhans cell histiocytosis known as Erdheim-Chester disease.

It has been described the activity of Anakinra in a patient with co-existing lichen planus and Erdheim-Chester disease.

A 43-year-old woman with progressive Erdheim-Chester disease presented for management of her night sweats and chills, systemic skeletal bone pain, and neurologic ( diabetes insipidus ) manifestations.
She also had widespread cutaneous lichen planus.
Anakinra, 100 mg subcutaneously daily, was initiated for the treatment of her Erdheim-Chester disease.

Within 2 days of starting Anakinra, there was prompt resolution of her Erdheim-Chester disease-related symptoms. Subsequently, her bone pain resolved and her diabetes insipidus improved.
Also, the lichen planus-associated pruritus rapidly ceased and most of the skin lesions improved.

This experience confirms the efficacy of Anakinra for the treatment of Erdheim-Chester disease.
The concomitant improvement of her lichen planus on Anakinra suggests that this agent warrants additional study in this disorder. ( Xagena )

Cohen PR, Kurzrock R, Dermatol Online J 2014;20:21241

XagenaMedicine_2014



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