Clinical Features and Treatment Outcomes of Patients With Necrobiotic Xanthogranuloma Associated With Monoclonal Gammopathies.
INTRODUCTION
Necrobiotic xanthogranuloma (NXG) is a rare chronic granulomatous disorder of the skin associated with a monoclonal gammopathy.


PATIENTS AND METHODS
The present report describes the findings from a single tertiary medical center retrospective study, including the clinical features of 35 patients with NXG and monoclonal gammopathy from 2000 to 2015 and their subsequent disease course and treatment response. The median age at diagnosis was 56 years (range, 26-88 years).


RESULTS
Most patients had a plasma cell dyscrasia consisting of monoclonal gammopathy of undetermined significance in 28 patients and smoldering multiple myeloma in 5 patients; the remaining 2 patients had chronic lymphocytic leukemia. An IgG isotype of monoclonal gammopathy was present in almost all the patients (97%). The most common site of cutaneous involvement of NXG was periorbital (66%). The treatments were heterogeneous and included excision, intralesional injection, radiotherapy, and systemic chemotherapy. The median follow-up period was 46 months (range, 4 to 234 months). The median overall survival had not been reached at the analysis, and 80% of the patients were still alive. Eight patients (23%) had disease progression to multiple myeloma at a median of 67 months (range, 21 to 107 months), demonstrating that although the clinical course is generally indolent, malignant transformation is not uncommon. At the last follow-up visit, 80% had signs of either clinical improvement or stable skin disease.


CONCLUSION
Cutaneous objective responses can be achieved with treatment of lymphoplasmacytic malignancies.
['Higgins|Larissa S|LS|', 'Go|Ronald S|RS|', 'Dingli|David|D|', 'Kumar|Shaji K|SK|', 'Rajkumar|S Vincent|SV|', 'Dispenzieri|Angela|A|', 'Buadi|Francis K|FK|', 'Lacy|Martha Q|MQ|', 'Lust|John A|JA|', 'Kapoor|Prashant|P|', 'Leung|Nelson|N|', 'Lin|Yi|Y|', 'Kourelis|Taxiarchis V|TV|', 'Gertz|Morie A|MA|', 'Kyle|Robert A|RA|', 'Gonsalves|Wilson I|WI|']
D000328:Adult D000368:Aged D000369:Aged, 80 and over D015415:Biomarkers D003131:Combined Modality Therapy D019468:Disease Management D018450:Disease Progression D005260:Female D005500:Follow-Up Studies D006801:Humans D007074:Immunoglobulin G D053208:Kaplan-Meier Estimate D008297:Male D008875:Middle Aged D008998:Monoclonal Gammopathy of Undetermined Significance D058252:Necrobiotic Xanthogranuloma D010265:Paraproteinemias D000072078:Positron Emission Tomography Computed Tomography D012189:Retrospective Studies D012867:Skin D016896:Treatment Outcome
Monoclonal gammopathies NXG Progression Dermatologic manifestations Novel agent therapies