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.


OBJECTIVE
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.
University of California San Dieog.
Philip R
PR
Cohen
Razelle
R
Kurzrock
D000328:Adult; D017332:Cetirizine / Q000627:therapeutic use; D003894:Deamino Arginine Vasopressin / Q000627:therapeutic use; D003919:Diabetes Insipidus / Q000188:drug therapy / Q000209:etiology; D003875:Drug Eruptions / Q000188:drug therapy / Q000209:etiology; D031249:Erdheim-Chester Disease / Q000150:complications / Q000000981:diagnostic imaging / Q000188:drug therapy*; D005260:Female; D019382:Human Growth Hormone / Q000172:deficiency / Q000627:therapeutic use; D006801:Humans; D053590:Interleukin 1 Receptor Antagonist Protein / Q000009:adverse effects / Q000627:therapeutic use*; D008010:Lichen Planus / Q000150:complications / Q000188:drug therapy* / Q000473:pathology; D011374:Progesterone / Q000627:therapeutic use; D011537:Pruritus / Q000188:drug therapy / Q000209:etiology; D011877:Radionuclide Imaging; D013967:Thyroiditis, Autoimmune / Q000150:complications / Q000188:drug therapy; D013974:Thyroxine / Q000627:therapeutic use; D014807:Vitamin D / Q000627:therapeutic use; D014808:Vitamin D Deficiency / Q000150:complications
D053590:Interleukin 1 Receptor Antagonist Protein; D019382:Human Growth Hormone; D014807:Vitamin D; D011374:Progesterone; C472067:levocetirizine; D003894:Deamino Arginine Vasopressin; D013974:Thyroxine; D017332:Cetirizine