Omalizumab use for telangiectasia macularis eruptiva perstans
Question:
11/21/2020
I have a 32 year-old female with Telangiectasia Macularis Eruptiva Perstans dxd on biopsy by derm. We previously kept her itching and skin lesions under control with Cetirizine 20 mg bid. This is no longer working well. Is there any evidence of benefit from XOLAIR in this condition?
Previous info on this pt:
Derm skin biopsy c/w: telangiectasia macularis eruptiva perstans. 5/9/17: Tryptase 7.7. Many GI sx so referred to Hematology to see if needs bone marrow to r/o systemic mastocytosis vs cutaneous (TMEP). Bone marrow done by Hematology on 7/21/17 = "normal. There are no cytogenetic abnormalities (normal) karyotype, FISH negative for FIP1L1-PDGFRA). There are no molecular abnormalities (c-kit negative). Morphologically her bone marrow is normal - no evidence for an underlying bone marrow process like systemic mastocytosis. f/u as needed". 12/12/19: Tryptase 6.8. 10/15/20: Tryptase 7.8 NL. Would Xolair help control symptoms?
Answer:
Sokol and colleagues published a review of omalizumab use in mastocytosis with a case report of its use in an individual with TMEP. The authors noted that antihistamines are the standard treatment of TMEP. Their patient experienced a marked reduction in cutaneous lesions which they postulated could have been related to omalizumab use. Of note, their patient was 77 years old, and had heart disease, was on a beta blocker and had a severe reaction to a honey bee sting, so omalizumab was used concurrently with venom immunotherapy.
H1 and H2 antihistamines, leukotriene antagonists, topical and systemic steroids, oral disodium cromoglycate, ketotifen, PUVA and intense pulsed light have also been reported as treatment options.
Reference:
Sokol KC, Ghazi A, Kelly BC and Grant JA. J Allergy Clin Immunol Pract 2014;2:266-70.
I hope this information helps you with your patient.
Jacqueline A. Pongracic, MD, FAAAAI