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Necrobiotic xanthogranuloma is a rare chronic condition, belonging to the group C non-Langerhans cell histiocytoses, which is relevant due to the possibility of extracutaneous involvement and association with systemic diseases, particularly hematologic malignancies. The case reported here was only diagnosed after nine years of evolution and was ass...
Context in source publication
Context 1
... clinical, histopathological, and laboratory findings allowed the diagnosis of necrobiotic xanthogranuloma associated with smoldering multiple myeloma. A six-month treatment was proposed with weekly cycles of 500 mg of cyclophosphamide + 40 mg dexamethasone and thalidomide 100 mg/day, resulting in decreased infiltration of the cutaneous lesions ( Figure 6) and reduction of serum levels of monoclonal protein. FIgure 2: Symmetrical yellowish plaques in the cervical and clavicular regions res 1, 2, and 3). ...
Citations
... Necrobiotic xanthogranuloma (NBX) is one of the most frequently reported subtypes and is characterized by the presence of necrobiosis in adnexal and frequently accompanying skin lesions [4]. Systemic findings include monoclonal gammopathy and associated hematologic malignancies [4,[10][11][12][13][14]. ...
... Patients presented with a median best corrected visual acuity (BCVA) of the (most) affected eye of 0.8 (range 0.16-1.25), a median intra-ocular pressure (IOP) of 16 mmHg (range [13][14][15][16][17][18][19][20], and a median Hertel value of the affected eye of 20 mm (range [16][17][18][19][20][21][22][23][24][25][26]. These values were not significantly different after treatment (p > 0.05, paired t-test). ...
... NBX is a subtype of non-LCH, histopathologically characterized by the presence of necrobiosis in the lesions. NBX is associated with extra-orbital lesions and with monoclonal gammopathy or other hematologic malignancies [4,[10][11][12][13][14]. In concurrence with the literature, patients with NBX in this study showed lesions of the skin, parotid gland, and lymph nodes. ...
Background:
Adult orbital xanthogranulomatous disease (AOXGD) is a group of rare disorders. Four subtypes are identified: adult-onset xanthogranuloma (AOX), adult-onset asthma and periocular xanthogranuloma (AAPOX), necrobiotic xanthogranuloma (NBX), and Erdheim-Chester disease (ECD). Therapy options vary and little is known about the long-term effect of the treatment. In this study, we will describe the clinical behaviour, effect of treatment, and long-term outcome in a consecutive series of patients with AOXGD.
Methods:
This is a descriptive, retrospective study with a long follow-up term of 21 patients with histologically proven AOXGD, treated between 1989 and 2021 in the Rotterdam Eye Hospital and Erasmus MC University Medical Center.
Results:
Twenty-one patients with histologically proven AOXGD were included. The follow-up ranged from 2-260 months (median of 67 months). Six of the nine patients with AOX were treated with surgery alone, with recurrence in two. Three received systemic therapy, with recurrence in one. All four patients with AAPOX received systemic treatment, the disease recurred in two. Two patients with NBX were treated with surgery alone, with recurrence in one. Four required additional therapy with recurrence in two. Both patients with ECD required systemic therapy.
Conclusions:
Recognition of AOXGD is important, in particular, because of the potential severe systemic locations in the different subtypes. Surgical excision might be a sufficient therapy for patients with AOX. Patients with AAPOX, NBX, and ECD warrant systemic therapy. Currently, there is no conclusive evidence for a superior treatment strategy, but further studies are necessary to investigate treatment options.
Background
Even though a plethora of systemic therapies have been proposed for necrobiotic xanthogranuloma (NXG), there is no systematic review on this topic in literature.
Objective
To review all existing literature on the systemic therapy of NXG in order to identify the most effective therapies.
Methods
All reported papers in the literature were screened for systemic treatments of NXG. Papers without proper description of the therapies, papers describing topical therapy, and articles without assessment of effectiveness were excluded. Subsequently, we analyzed 79 papers and a total of 175 cases.
Results
The most effective treatments for NXG are intravenous immunoglobulins (IVIG), corticosteroids, and combination therapies including corticosteroids.
Conclusions
Corticosteroids and IVIG should therefore be considered first-line treatments in patients with NXG.
Necrobiotic xanthogranuloma (NXG) is a rare chronic condition, belonging to the group non-Langerhans cell histiocytoses, which is relevant due to the possibility of extracutaneous involvement and association with systemic diseases, particularly monoclonal gammopathy, MGUS and multiple myeloma. The case reported here NXG was diagnosed after 1 years of evolution in patient with asymptomatic multiple myeloma. After treatment with bortezomib, lenalidomid and dexamethasone, there was evident abrupt decrease of monoclonal immunoglobulin to not measurable level (complete remission of multiple myeloma) and in the same time was evident disappearance of cutaneous and hepatic lesions of NXG on FDG-PET/CT. The etiopathogenetic association of monoclonal immunoglobulin with NXG is documented in this case report with disappearance of NXG in the time of disappearance of monoclonal immunoglobulin.
