Tratamiento quirúrgico del xantogranuloma palpebral. Caso clínico <span class="so-article-trans-title" dir="auto"> Translated title: Surgical treatment of eyelid xanthogranulomas. Case report </span>

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Abstract

Resumen El xantogranuloma es una lesión poco frecuente que caracteriza al amplio grupo de las histiocitosis de células no Langerhans (NLCH). Se describen diferentes variantes de NLCH: xantoma, xantogranuloma juvenil, xantoma disseminatum, xantogranuloma necrobiótico, retículohistiocitosis, enfermedad de Rosai Dorfman y un conjunto heterogéneo de enfermedades infrecuentes que comparten manifestaciones cutáneas e histopatológicas comunes y que se describen como enfermedad xantogranulomatosa orbitaria del adulto (EXOA). La enfermedad de Erdheim Chester (EEC) está incluida en este último grupo. Nuestro objetivo es describir un caso clínico con gran compromiso sistémico y lesiones cutáneas en ambos párpados superiores e inferiores que invalidaban a la paciente física y psicológicamente. Presentamos su resolución quirúrgica con resección amplia de lesiones y reconstrucción con injertos de piel total con un muy buen resultado estético y funcional.

Translated abstract

Abstract Xanthogranuloma is a rare lesion that characterizes the large group of non-Langerhans cell histiocytosis (NLCH). Different variants of NLCH: xanthoma, juvenile xanthogranuloma, xanthoma disseminatum, necrobiotic xanthogranuloma, reticulum histiocytosis, Rosai Dorfman disease, and a heterogeneous set of rare diseases that share common cutaneous and histopathological manifestations are described as orbital xanthogranulomatous disease of the adult (EXOA). Erdheim Chester disease (ECD) is included in this last group. Our objective is to describe a clinical case with great systemic involvement and skin lesions in both upper and lower eyelids that invalidate the patient physically and psychologically. We present its surgical resolution with wide resection of lesions and reconstruction with total skin grafts with a very good aesthetic and functional result.

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Most cited references 22

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CNS involvement and treatment with interferon-α are independent prognostic factors in Erdheim-Chester disease: a multicenter survival analysis of 53 patients.

Laurent Arnaud, Baptiste Hervier, Antoine Néel … (2011)

Erdheim-Chester disease (ECD) is a rare form of non-Langerhans histiocytosis, with noncodified therapeutic management and high mortality. No treatment has yet been shown to improve survival in these patients. We conducted a multicenter prospective observational cohort study to assess whether extraskeletal manifestations and interferon-α treatment would influence survival in a large cohort of ECD patients. To achieve this goal, we thoroughly analyzed the clinical presentation of 53 patients with biopsy-proven ECD, and we performed a survival analysis using Cox proportional hazard model. Fifty-three patients (39 men and 14 women) with biopsy-proven ECD were followed up between November 1981 and November 2010. Forty-six patients (87%) received interferon-α and/or PEGylated interferon-α. Multivariate survival analysis using Cox proportional hazard model revealed that central nervous system involvement was an independent predictor of death (hazard ratio = 2.51; 95% confidence interval, 1.28-5.52; P = .006) in our cohort. Conversely, treatment with interferon-α was identified as an independent predictor of survival (hazard ratio = 0.32; 95% confidence interval, 0.14-0.70; P = .006). Although definitive confirmation would require a randomized controlled trial, these results suggest that interferon-α improves survival in ECD patients. This may be seen as a significant advance, as it is the first time a treatment is shown to improve survival in this multisystemic disease with high mortality.

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Adult xanthogranulomatous disease of the orbit and ocular adnexa: new immunohistochemical findings and clinical review.

F V Buffam, Z Currie, Andrew V. White … (2006)

Adult xanthogranulomatous disease involving the ocular tissues is rare and poorly understood. Adult onset xanthogranuloma (AOX), adult onset asthma and periocular xanthogranuloma (AAPOX), necrobiotic xanthogranuloma (NBX), and Erdheim-Chester disease (ECD) are the four syndromes within this disorder, which is diagnosed by characteristic histopathology. Experience with eight cases prompted a multi-institutional effort to study the histopathology, immunohistochemistry, clinical findings, and systemic associations in this disorder. 22 cases, including histopathological slides, were compiled. Published reports were identified by an English language Medline search (1966-2005) and review of reference citations. Each case in this series and the literature was classified as one of four syndromes and then analysed for age onset, sex, skin xanthoma, orbital location, immune dysfunction, internal organ and bone lesions, treatment, and outcome. The histopathology in each of these cases was reviewed by two pathologists. Immunhistochemical stains (CD3, CD4, CD8, L26) were performed in 14 cases where unstained slides were available. 137 cases were compiled. There was no sex or age difference between syndromes. AOX, AAPOX, NBX affect the anterior orbit, ECD tends to be diffuse and intraconal. Skin lesions are found in all the syndromes. Immune dysfunction was noted in all cases of AAPOX and NBX; 11% of NBX and all ECD patients had internal organ disease. Treatment included surgery, corticosteroids, other chemotherapeutic agents, radiotherapy, and combinations of these. No AOX or AAPOX deaths occurred; 66% of ECD patients died. All 22 cases had xanthoma cells; most had Touton giant cells. Lymphocytes were present in all cases and occurred as aggregates (mostly in AAPOX) or diffuse populations mixed with fibroblasts (mostly in ECD). Immunohistochemistry revealed the majority of these to be CD8+. Necrosis was most marked in NBX. Adult xanthogranuloma of the orbit is rare, making prospective evaluation or meta-analysis impossible. The best treatment is unknown but seems to be with multiagent chemotherapy guided by histopathological, immunohistochemical, and systemic findings.

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Treatment of necrobiotic xanthogranuloma - a systematic review.

D. Miguel, J. Lukács, T Illing … (2017)

Necrobiotic xanthogranuloma (NXG) is an uncommon non-Langerhans cell histiocytosis involving skin and extracutaneous tissues. The lesions are usually asymptomatic and commonly appear in the periorbital area. Paraproteinemia is closely associated with NXG and its pathogenesis remains unclear. NXG prognosis is poor with several treatments showing variable results. Treatment of monoclonal gammopathy with alkylating agents does not necessarily influence the activity of the skin disease and vice versa. The aim of this systematic review is to summarize all reported treatments of necrobiotic xanthogranuloma of the skin, with or without underlying malignant condition and based on articles from the PubMed database using the query 'necrobiotic xanthogranuloma treatment', both in English and German, about 'human' subjects and published between 1980 and 2014, documenting adequate treatment for NXG. Mainly individual case reports, small case series and retrospective studies were found. Treatment options include topical and systemic corticosteroids, thalidomide, high-dose intravenous immunoglobulin (IVIG), chlorambucil, cyclophosphamide, fludarabine, rituximab, melphalan, infliximab, interferon alpha, cladribine, hydroxychloroquine, azathioprine, methotrexate, laser therapy, radiotherapy, surgery, PUVA, plasmapheresis and extracorporeal photopheresis. Randomized controlled trials and studies on long-term outcomes after treatment were not found and are necessary to focus on in the future.

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Journal

Journal ID (publisher-id): cpil

Title: Cirugía Plástica Ibero-Latinoamericana

Abbreviated Title: Cir. plást. iberolatinoam.

Publisher: Sociedad Española de Cirugía Plástica, Reparadora y Estética (SECPRE) (Madrid, Madrid, Spain )

ISSN (Print): 0376-7892

ISSN (Electronic): 1989-2055

Publication date (Print and electronic): June 2022

Volume: 48

Issue: 2

Pages: 207-216

Affiliations

[1] Durand Buenos Aires orgnameHospital C. Durand orgdiv1Servicio de Cirugía Plástica y Reparadora Argentina

[4] orgnameUniversidad de Buenos Aires Argentina

[3] Buenos Aires Buenos Aires orgnameUniversidad de Buenos Aires Argentina

[2] Buenos Aires Buenos Aires orgnameUniversidad de Buenos Aires orgdiv1Servicio de Cirugía Plástica y Reparadora Argentina

[5] orgnameUniversidad de Buenos Aires Argentina

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Publisher ID: S0376-78922022000200207 Publisher ID: S0376-7892(22)04800200207

DOI: 10.4321/s0376-78922022000200012

SO-VID: 55736b15-cd32-4f75-a6b9-da922d6b6df9

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This work is licensed under a Creative Commons Attribution-NonCommercial-ShareAlike 4.0 International License.

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Date accepted : 20 June 2022

Date received : 15 May 2022

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Figures: 0, Tables: 0, Equations: 0, References: 22, Pages: 10

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Tratamiento quirúrgico del xantogranuloma palpebral. Caso clínico Translated title: Surgical treatment of eyelid xanthogranulomas. Case report

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Most cited references 22

CNS involvement and treatment with interferon-α are independent prognostic factors in Erdheim-Chester disease: a multicenter survival analysis of 53 patients.

Adult xanthogranulomatous disease of the orbit and ocular adnexa: new immunohistochemical findings and clinical review.

Treatment of necrobiotic xanthogranuloma - a systematic review.

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