Langerhans Cell Histiocytosis and Juvenile Xanthogranuloma

Abstract

Background: Histiocytoses represent a large, puzzling group of diseases which may involve the skin and other organs. At present, juvenile xanthogranuloma is the disorder most often confused with Langerhans cell histiocytosis. A complex overlap exists between juvenile xanthogranuloma and Langerhans cell histiocytosis, with lesions showing clinical and/or pathological features of both disorders. Observations: We report 2 patients affected by Langerhans cell histiocytosis who, during chemotherapy, presented cutaneous lesions with clinical and histological features of juvenile xanthogranuloma. During the therapy, in both cases, histological examination of new biopsies revealed the presence of Touton giant cells in the dermis with a few histiocytic cells; immunohistochemical staining was negative for CD1a, and no Birbeck granules were seen by ultrastructural examination. Results and Conclusion: A possible explanation for the link between Langerhans cell histiocytosis and juvenile xanthogranuloma regards the lineage development and the relationships of histiocytes. We suggest that chemotherapy can modify the production of cytokines by influencing the conversion or ‘maturation’ of pathological cells into macrophages or xanthomatous cells and fusing them to form multinucleated giant Touton cells. In our opinion, the modification of the cutaneous lesions during chemotherapy in Langerhans cell histiocytosis patients, as observed in our cases, could be a favorable prognostic factor.

Multiple Adult Xanthogranuloma: Case Report and Literature Review

“…Occasionally (10% of cases), visceral or mucosal lesions are present (bone, spleen, lung, liver, testicle or eye with a risk of glaucoma). JXG regresses spontaneously during the first few years (3–6 years), sometimes leaving residual pigmentation and atrophy [3,4,5,6]. Histologic examination initially reveals histiocytic proliferation without xanthomatous cells, associated with a moderate infiltrate (lymphoid cells and eosinophils) [3].…”

Juvenile Xanthogranuloma in a Pediatric Patient with Langerhans Cell Histiocytosis

“…This association is not well understood but has been noted in a number of case reports and case series. Patrizi et al [12] reported 2 cases of JXG presenting during chemotherapy for LCH; 1 of the 2 cases involved the eyelid. Strehl et al [10] reported 8 cases in which patients were diagnosed with JXG 3 months to 5 years following diagnosis and therapy for LCH, with a mean of 2.4 years following diagnosis and therapy for LCH; 4 of the 8 cases involved the eyelid, and the JXG lesions occurred between 1.5 years to 5 years after therapy for LCH.…”

Diffuse xanthogranulomatous dermatitis and systemic Langerhans cell histiocytosis: A novel case that demonstrates bridging between non-Langerhans cell histiocytosis and Langerhans cell histiocytosis

“…1,2,5,6,9,11,12,14,16,19 Moreover, the segregation of LCH from non-LCH is called into question when case reports appear that exhibit overlapping features of both conditions. 8,10,13,15,26 Depending on the timing of the biopsy, LCH can cytomorphologically resemble non-LCH and vice versa. For example, early lesions of XG are often devoid of TGC and lipid-laden histiocytes, and can even contain a few LC, whereas LC in older lesions of LCH often accumulate lipids.…”

“…For example, early lesions of XG are often devoid of TGC and lipid-laden histiocytes, and can even contain a few LC, whereas LC in older lesions of LCH often accumulate lipids. 8,15 Moreover, Birbeck granules, pathognomonic organelles of LC, are only identified in approximately half of LC in LCH. 8 Lastly, the S-100 and CD1a immunohistochemical stains routinely expressed by LC are occasionally expressed in non-LCH histiocytoses.…”

“…[3][4][5][6][7][8][9][10] Furthermore, cases exist that exhibit concomitant lesions of LCH and non-LCH, and cases where LCH cells were converted into mature histiocytes with chemotherapy; thereby providing further evidence that an overlap exists both within and between the various histiocytoses. 5,6,[8][9][10][11][12][13][14][15][16] Herein we present a patient who does not easily fit into either the non-LCH or LCH category, but rather displayed clinical and histologic features that bridged both conditions. Through this novel case we review what is currently known about the various conditions classified under the non-LCH and LCH rubric.…”