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CASE REPORT
Year : 2012  |  Volume : 57  |  Issue : 5  |  Page : 396-398
Spectacular skin nodules: Cutaneous necrobiotic xanthogranuloma without paraproteinemia


Rajawadi Hospital and Dr. D. Y. Patil Medical College, Mumbai, Maharashtra, India

Date of Web Publication3-Sep-2012

Correspondence Address:
Shimoni Kadakia
501- A, Adinath Apts, 281, Tardeo Road, Mumbai - 400 007, Maharashtra
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/0019-5154.100499

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   Abstract 

Necrobiotic xanthogranuloma (NXG) is a very rare, progressive variant of non-Langerhans cell histiocytosis. It is known to be associated with multisystem involvement and paraproteinemias. A 65-year-old female presented with chronic, slowly growing, asymptomatic periorbital nodules. The lesions had recurred after local excision elsewhere. No systemic involvement or paraproteinemias were detected. A provisional diagnosis of isolated cutaneous NXG was made which was confirmed by histopathology and immunohistochemistry staining. The lesions were surgically excised with excellent cosmetic and functional results. There was no recurrence over a period of 9 months. To our knowledge, this is the second case of NXG reported from India and the first without any systemic manifestations.


Keywords: Necrobiotic xanthogranuloma, periorbital nodules, Touton giant cells


How to cite this article:
Kadakia S, Nadkarni N, Sonavane S, Ghate S. Spectacular skin nodules: Cutaneous necrobiotic xanthogranuloma without paraproteinemia. Indian J Dermatol 2012;57:396-8

How to cite this URL:
Kadakia S, Nadkarni N, Sonavane S, Ghate S. Spectacular skin nodules: Cutaneous necrobiotic xanthogranuloma without paraproteinemia. Indian J Dermatol [serial online] 2012 [cited 2019 May 24];57:396-8. Available from: http://www.e-ijd.org/text.asp?2012/57/5/396/100499

What was known? 1. Necrobiotic xanthogranuloma (NXG) is characterized by multiple, plaque- like, yellow-brown lesions, with a predilection for the periorbital area . 2. Majority of the cases reported have an associated paraproteinemia.



   Introduction Top


Necrobiotic xanthogranuloma (NXG) was first described as a separate entity by Kossard and Winkelmann in 1980. It belongs to Class II b of non-Langerhans cell histiocytosis. [1] The disease is characterized by multiple, plaque- like, yellow-brown lesions, with a predilection for the periorbital area (spectacle-like distribution). The lesions are sometimes associated with atrophy, telangiectasias and ulceration. Majority of the cases reported have an associated paraproteinemia of the IgG-κ type.

About 100 cases have been reported in world literature. [2] To our knowledge, this is the first case of isolated cutaneous NXG being reported from India.


   Case Report Top


A 65-year-old female presented with a 3-year history of gradually enlarging, periorbital, asymptomatic lesions. One of the lesions had been excised surgically 2 years ago in another hospital. However, it recurred in a few months and was accompanied by new larger nodules. There was no history of ulceration or any involvement of other sites. There was no history of any eye complaints or any significant systemic symptoms. The patient's medical history was not pertinent.On examination, there were multiple, yellowish brown, firm nodules distributed symmetrically in the periorbital area. The size of the nodules varied from 3 to 7 cm in their widest dimensions. The surface showed a few telangiectasias and mild atrophy [Figure 1] and [Figure 2]. There was mild right-sided ectropion.
Figure 1: Yellow brown firm periorbital nodules

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Figure 2: Superficial telangiectasias and mild atrophy

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With this history and clinical findings, a provisional diagnosis of a xanthomatous disorder was made.

Histopathological examination of a surgical wedge biopsy showed ill-defined large foci of dense granulomatous infiltrate of large histiocytes and histiocytic foamy cells with lymphocytes and few plasma cells. Several histiocytic giant cells resembling Touton giant cells were present [Figure 3] and [Figure 4]. However, typical findings of necrobiosis were not seen.
Figure 3: Dense granulomatous infiltrate of histiocytes

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Figure 4: Touton giant cell

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Laboratory investigations were normal for blood counts, electrolytes, glucose levels, LFTs, except for S.triglycerides and S.cholesterol levels which were mildly elevated.

Serum protein electrophoresis was within normal limits. Immunohistochemistry was positive for CD 68 [Figure 5] and negative for CD 1a and S100.
Figure 5: CD 68 positive

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Thus, a final diagnosis of isolated cutaneous NXG without paraproteinemia or associated disorders was made.

Surgical excision of the xanthogranuloma was done with primary closure.

The patient was maintained on a regular follow-up of every 15 days for the first 2 months and then once a month for 9 months. No evidence of any recurrence was seen [Figure 6].
Figure 6: Post treatment (after 9 months)

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   Discussion Top


NXG is a rare, progressive, histiocytic disease that features destructive cutaneous lesions, a close association with paraproteinemia, and multisystem extracutaneous manifestations. NXG is a disease of adults with an average age of onset in the sixth decade. NXG begins as papules and nodules that slowly enlarge into indurated plaques ranging from 0.5 to 25 cm. Characteristic lesions have a red-orange or yellow color as well as telangiectasias, atrophy, scarsand ulcers. [3],[4] The most common site of involvement is the face, particularly the periorbital region (85% of cases); the trunk and proximal extremities are other affected sites. [3] Ophthalmological complications affect approximately 50% of cases and include orbital masses, conjunctival involvement, keratitis, scleritis, ectropion, uveitis and blindness. [3],[4]

NXG may involve other extracutaneous sites, including the heart, lungs, kidneys, liver, spleen, intestines, skeletal muscle and central nervous system. [5],[6] Paraproteinemia is closely associated with NXG, with approximately 80% of patients demonstrating a monoclonal gammopathy of IgG-κ on serum protein electrophoresis; furthermore, 10% of these patients develop multiple myeloma.[3] Other lymphoproliferative disorders such as Hodgkin's lymphoma have been associated with NXG. [7] Hepatosplenomegaly, lymphadenopathy, an increased erythrocyte sedimentation rate, leukopenia, hypocomplementemia, thrombocytopenia and cryoglobulinemia are other common findings. [3],[4] Bone marrow biopsy can show plasmacytosis, myeloma or myelodysplastic syndromes.

The link between paraproteinemia and NXG remains unclear. Theories regarding pathogenesis include deposition of immunoglobulins and lipid complexes with a foreign body giant-cell reaction and monocyte activation with intracellular lipid accumulation. [8] Histopathological examination shows a palisading xanthogranuloma with areas of necrobiotic, degenerated collagen that infiltrates the mid-dermis with extension into the subcutaneous fat. Multiple xanthomatized histiocytes, touton giant cells, other bizarre giant cells, cholesterol clefts and lymphoid follicles are often present within granulomas. [9]

Only anecdotal reports are available to support treatment options for NXG. Improvement in skin lesions has been seen with low-dose chlorambucil, [9] melphalan with or without prednisolone, methotrexate, localized radiotherapy, [10] systemic glucocorticoids, [11] interferon α-2a,[12] and plasmapheresis with hydroxychloroquine. [13] Topically nitrogen mustard, BNCU, intralesional corticosteroids and intravenous immunoglobulin [2] have also been tried. Surgical excision has also been advocated. [14]

In our patient, we decided to surgically excise the lesions in view of absence of systemic involvement. We are gratified to observe no recurrence of any lesion over 9 months. Thus this could be considered as a viable therapeutic option in isolated cutaneous NXG.

 
   References Top

1.Chu T, D'Angio GJ, Favara BE, Ladisch S, Nesbit M, Pritchard J. Histiocytosis syndromes in children. Lancet 1987;1:208-9.  Back to cited text no. 1
    
2.Hallermann C, Tittelbach J, Norgauer J, Ziemer M. Successful Treatment of Necrobiotic Xanthogranuloma With Intravenous Immunoglobulin. Arch Dermatol 2010;146:957-60.  Back to cited text no. 2
[PUBMED]    
3.Mehregan DA, Winkelmann RK. Necrobiotic xanthogranuloma. Arch Dermatol 1992;128:94-100.  Back to cited text no. 3
[PUBMED]    
4.Finan MC, Winkelmann RK. Necrobiotic xanthogranuloma with paraproteinemia: a review of 22 cases. Medicine 1986;65:376-88.  Back to cited text no. 4
[PUBMED]    
5.Winkelmann RK, Litzow MR, Umbert IJ, Lie JT. Giant cell granulomatous pulmonary and myocardial lesions in necrobiotic xanthogranuloma with paraproteinemia. Mayo Clin Proc 1997;72:1028-33.  Back to cited text no. 5
[PUBMED]    
6.Shah KC, Poonnoose SI, George R, Jacob M, Rajshekhar V. Necrobiotic xanthogranuloma with cutaneous and cerebral manifestations. Case report and review of the literature. J Neurosurg 2004;100:1111-4.  Back to cited text no. 6
[PUBMED]    
7.Reeder CB, Connolly SM, Winkelmann RK. The evolution of Hodgkin's disease and necrobiotic xanthogranuloma syndrome. Mayo Clin Proc 1991;66:1222-4.  Back to cited text no. 7
[PUBMED]    
8.Matsuura F, Yamashita S, Hirano K, Ishigami M, Hiraoka H, Tamura R, et al. In vivo Activation of monocytes causes intracellular accumulation of lipoprotein-derived lipids and marked hypocholesterolaemia: a possible pathogenesis of necrobiotic xanthogranuloma. Atherosclerosis 1999;142:355-65.  Back to cited text no. 8
[PUBMED]    
9.Machado S, Alves R, Lima M, Leal I, Massa A. Cutaneous necrobiotic xanthogranuloma - successfully treated with low dose chlorambucil. Eur J Dermatol 2001;11:458-62.  Back to cited text no. 9
[PUBMED]    
10.Char DH, LeBoit PE, Ljung BM, Wara W. Radiation therapy for ocular necrobiotic xanthogranuloma. Arch Ophthalmol 1987;105:174-5.  Back to cited text no. 10
[PUBMED]    
11.Chave TA, Chowdhury MM, Holt PJ. Recalcitrant necrobiotic xanthogranuloma responding to pulsed high-dose oral dexamethasone plus maintenance therapy with oral prednisolone. Br J Dermatol 2001;144:158-61.  Back to cited text no. 11
[PUBMED]    
12.Georgiou S, Monastirli A, Kapranos N, Pasmatzi E, Sakkis T, Tsambaos D. Interferon alpha-2a monotherapy for necrobiotic xanthogranuloma. Acta Derm Venereol (Stockh) 1999;79:484-5.  Back to cited text no. 12
[PUBMED]    
13.Finelli LG, Ratz JL. Plasmapheresis, a treatment modality for necrobiotic xanthogranuloma. J Am Acad Dermatol 1987;17:351-4.  Back to cited text no. 13
[PUBMED]    
14.Spicknall KE, Mehregan DA. Necrobiotic Xanthogranuloma. Int J Dermatol 2009;48:1-10.  Back to cited text no. 14
[PUBMED]    

What is new? A case of isolated cutaneous Cutaneous Necrobiotic Xanthogranuloma Without Paraproteinemia is being reported.


    Figures

  [Figure 1], [Figure 2], [Figure 3], [Figure 4], [Figure 5], [Figure 6]



 

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