Work

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

Year: 2014

Type: article

Abstract: 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.

Source: Dermatology Online Journal

DOI: 10.5070/d3201021241

Authors: Philip R Cohen, Razelle Kurzrock

Institutions: University of California San Diego, Moores Cancer Center

Language: English

Topic: Histiocytic Disorders and Treatments

Subfield: Physiology

Field: Medicine

Domain: Health Sciences

SDG: Good health and well-being

Open Access status: hybrid

Estimated APC paid: $300