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Anakinra-responsive lichen planus in a woman with Erdheim-Chester disease: a therapeutic enigma


Background:  Anakinra 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.

Purpose:  To describe the activity of anakinra in a patient with co-existing lichen planus and Erdheim-Chester disease.

Methods:  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.

Results:  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.

Conclusions:  Our 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.

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