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Importance of dermoscopy in incipient lesion of neviform circumscribed lymphangioma in children

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ORIGINAL ARTICLE

ZÁU, Aline Sales Mendes [1], ROCHA, Ana Paula Coelho [2], SANTOS, Luciana Mendes dos [3]

ZÁU, Aline Sales Mendes. ROCHA, Ana Paula Coelho. SANTOS, Luciana Mendes dos. Importance of dermoscopy in incipient lesion of neviform circumscribed lymphangioma in children. Revista Científica Multidisciplinar Núcleo do Conhecimento. Year. 07, Ed. 01, Vol. 06, p. 133-139. January 2022. ISSN: 2448-0959, Access link: https://www.nucleodoconhecimento.com.br/health/incipient-lesion ‎

ABSTRACT

Background: Neviform Circumscribed Lymphangioma (LCN)[4] is a rare benign lymphatic malformation that corresponds to a hamartoma and occurs in the first years of life. It is clinically characterized by multiple vesicular micropapules of clear and/or sero-hematic content and has several differential diagnoses. Problem: Faced with a rare disease, with a clinic similar to that of numerous dermatoses, dermoscopy can be an important tool in order to guide and improve the diagnosis. Objective: We report the case of an 8-year-old girl, who for 5 years was being treated as Molluscum Contagiosum for presenting grouped and asymptomatic vesicular normochromic micropapules in the abdomen. The analysis and interpretation of the dermoscopic patterns of the lesions allowed the hypothesis of LCN, which was confirmed by the histopathological examination. Methodology: A complete anamnesis, dermatological physical examination, including dermoscopic evaluation and incisional biopsy of the patient in question were performed, and it was found to be a case of LCN. For the construction of the article, a brief literature review was also carried out on the dermoscopic aspects of this lesion. Conclusion: It is concluded that dermoscopy is an important tool to improve the diagnostic accuracy of a rare pathology such as LCN, and it is necessary to know its dermoscopic aspects in order to exclude other diagnoses and avoid unnecessary treatments without a definitive diagnosis.

Keywords: Lymphangioma circumscribed, Lymphatic malformation, Dermoscopy, Molluscum contagiosum, Vesicular micropapules.

INTRODUCTION

LCN is a rare benign lymphatic malformation, which corresponds to a hamartoma, as described by Domínguez (2013), clinically characterized by the presence of multiple vesicular micropapules of clear and/or serohematic content according to Jucá et al. (2011), which can coalesce, form crusts and eventually have an almost verrucous appearance. According to Guerrero (2013) they usually appear in childhood, and there is no predilection for race or sex.

This malformation should be remembered as an important differential diagnosis of molluscum contagiosum (MC), verruca vulgaris, angiomas, angiokeratomas, lymphangioendothelioma and angiosarcomas. In this context, the present article has the following problem: in the face of a rare disease, with a clinic similar to that of numerous dermatoses, dermoscopy can be an important tool to distinguish them in order to guide and improve clinical and diagnostic suspicion.

Therefore, it aims to report the case of an 8-year-old girl, who for 5 years was being treated for molluscum contagiosum because she had grouped and asymptomatic vesicular normochromic micropapules in the abdomen. The analysis and interpretation of the dermoscopic patterns of the lesions allowed the hypothesis of LCN, which was confirmed by the histopathological examination. A complete anamnesis, dermatological physical examination, dermoscopic evaluation and incisional biopsy were performed, and it was found to be a case of LCN.

METHODOLOGY

The patient under study was seen at the outpatient clinic of the dermatology service of the Hospital Universitário Getúlio Vargas in Manaus/AM with a complaint of cutaneous lesions in grouped and asymptomatic vesicular normochromic micropapules in the abdomen. She was submitted to a complete anamnesis, dermatological physical examination, including dermoscopic evaluation and incisional biopsy, and it was found to be a case of Neviform Circumscribed Lymphangioma.

CASE REPORT

An 8-year-old female patient came to the outpatient clinic of the dermatology service of the Hospital Universitário Getúlio Vargas in Manaus/AM. The patient complained of a discrete skin lesion for 5 years, located in the abdomen, asymptomatic, with insidious growth in number, she underwent several previous treatments due to suspected diagnosis of molluscum contagiosum without clinical improvement. She denies itching, involvement in other areas of the body, changes in general condition or other complaints. The dermatological examination revealed the presence of multiple vesicular papules, millimetric, skin-colored, translucent, with a smooth and shiny surface, some of them vinous, grouped in the abdomen on a normochromic base (Fig. 1). Imaging was not performed to analyze the existence of a deep component. Dermoscopy showed a multiple lacunar pattern, separated by clear septa, with translucent and, to a lesser extent, serohematic content (Fig. 2). The aforementioned dermoscopic findings were compatible with the diagnosis of LCN, and, as a complement to the exams for diagnostic elucidation, an incisional biopsy was performed, which was confirmed by the histopathological study because it was LCN. Given the benignity of the diagnosis and the absence of clinical symptoms, it was decided to maintain an expectant management.

Figure 1: Multiple vesicular papules, millimetric, skin-colored, translucent, with a smooth and shiny surface, some vinous and coalescent.

Múltiplas pápulas vesiculosas, milimétricas, cor da pele, translúcidas, de superfície lisa e brilhante, algumas vinhosas e coalescentes
Source: HUGV dermatology service archive, 2019

Figure 2: Multiple lacunar pattern, separated by clear septa, with translucent and serohematic content.

Padrão lacunar múltiplo, separadas por septos claros, com conteúdo translúcido e serohemático
Source: HUGV dermatology service archive, 2019

Figure 3: Arboriform vessels in a crown pattern, with a yellowish white center.

Vasos arboriformes em um padrão de coroa, com centro branco amarelado
Source: https://dermagarrafblog.wordpress.com/2017/04/22/ficha-19-dermatoscopia-en-infecciones-e-infestaciones/

DISCUSSION

LCN is a rare and benign congenital microcystic lymphatic malformation of uncertain etiology. They commonly appear between 2-3 years of age as shown in Boon’s literature; Vikkula (2008). The peak incidence is up to 5 years of age, with 90% presenting before 2 years of age, as in the case presented here. It is usually located in the axillary, inguinal, extremities (GONTIJO, 2004) and mouth regions, and its location in the abdomen region is uncommon, as described in this patient.

The Whimster hypothesis (MENCHÓN, 2004) associates its origin with increased pressure in the lymphatic vessels of the skin, which would be connected to hypodermic lymphatic cisterns, thus justifying the occasional drainage of lymph. Its deep involvement, especially in well-developed and extensive lesions, makes the therapeutic approach a challenge. There is the possibility of watchful waiting for minor lesions, surgical excision, ablation with carbon dioxide laser, cryotherapy, electrofulguration, radiofrequency, embolization, among others, but none of them ideal and with the possibility of recurrence and unsightly scars (GUERRERO, 2013).

The clinical presentation of LCN is similar to that of angiomas, angiokeratomas, molluscum contagiosum, lymphangioendothelioma, angiosarcomas, warts, hidrocystoma and herpes simplex, and the dermoscopic findings are essential for the differential diagnosis. Lymphangioma presents two distinct dermoscopic patterns, the first with yellow lakes surrounded by a clear septum without blood vessels, and the other with yellow-reddish or red-blackish lakes containing blood (MASSA, 2015). In the case of molluscum contagiosum, a pathology for which the patient had been treated for about 5 years, dermoscopically, a center consisting of a whitish yellow amorphous area surrounded by linear or branched arboriform vessels in crown, which do not intersect the center, would be expected (Fig. 3), according to Pedrosa et al. (2013). Dermoscopy was essential for the diagnosis of a chronic lesion, but with an incipient clinic, so it is of fundamental importance that the specialist has knowledge about these findings in order to have the LCN as a diagnostic possibility.

CONCLUSION

Based on the literature and on the peculiarity of the case report presented, it is concluded that LCN is a rare congenital pathology that has several differential clinical diagnoses. Thus, dermoscopy is an important tool to improve the diagnostic accuracy of this pathology, it is necessary for the specialist to know its dermoscopic aspects in order to exclude other pathologies and avoid unnecessary treatments without a definitive diagnosis.

REFERENCES

BOON L, VIKKULA M. Vascular Malformations.In: Wolff K, Johnson RA, editors. Fitzpatrick´s Color Atlas And Synopsis Of Clinical Dermatology. Berkshire: McGraw Hill; 2008.p. 1651-66.

DOMÍNGUEZ, VG. Dermoscopia de linfangioma circumscriptum neviforme – relato de caso. Revista da Sociedade Portuguesa de Dermatologia e Venereologia, v. 70, n. 3, p. 369, 13 de janeiro de 2013.

GONTIJO, Bernardo et al. Malformações vasculares. Anais Brasileiros de Dermatologia, v. 79, n. 1, p.7-25, 2004. Disponível em: <https://doi.org/10.1590/S0365-05962004000100002>. Epub 17 Jun 2004. ISSN 1806-4841. https://doi.org/10.1590/S0365-05962004000100002. Acesso em: 18 jan. 2022.

GUERRERO, María et al. Linfangioma circunscrito zosteriforme. Presentación de un caso. Medisur, Cienfuegos, v. 11, n. 2, p. 195-200, abr.  2013.   Disponible en <http://scielo.sld.cu/scielo.php?script=sci_arttext&pid=S1727-897X2013000200012&lng=es&nrm=iso>. accedido en 19 enero 2022.

JUCÁ, Natália Braga Hortêncio et al. Linfangioma microcístico acral: diagnóstico diferencial em lesões verrucosas de extremidades. Anais Brasileiros de Dermatologia, v. 86, n. 2, p. 343-6, 2011. Disponível em: http://www.scielo.br/pdf/abd/v86n2/v86n2a20.pdf. Acesso em: 18 jan. 2022.

MARTÍNEZ-MENCHÓN, T et al. Lymphangioma circumscriptum: an example of Whimster‘s hypothesis. Pediatr Dermatol., v. 21, n. 6, p. 652-4, nov-dec 2004. DOI 10.1111/j.0736-8046.2004.21607.x. PMID: 15575849. Disponível em: https://pubmed.ncbi.nlm.nih.gov/15575849/. Acesso em: 18 jan. 2022.

MASSA, António Fernandes et al. Cutaneous Lymphangioma circumscriptum – dermoscopic features. Anais Brasileiros de Dermatologia, v. 90, n. 2, p. 262-264, 2015. DOI https://doi.org/10.1590/abd1806-4841.20153652. Disponível em: https://doi.org/10.1590/abd1806-4841.20153652. Acesso em: 18 jan. 2022.

PEDROSA, A.; MORAIS, P.; MOREIRA, C.; FERREIRA, O.; AZEVEDO, F. Dermoscopia – casos selecionados de aplicações sub explorados e revisão da literatura. Revista da Sociedade Portuguesa de Dermatologia e Venereologia, v. 71, n. 4, pág. 487-494, 6 de julho de 2014.

APPENDIX – FOOTNOTE

4. Linfangioma Circunscrito Neviforme.

[1] Graduate in medicine, third-year resident of the dermatology service. ORCID: 0000-0002-6561-376X.

[2] Graduate in medicine, third-year resident of the dermatology service. ORCID: 0000-0001-9895-706X.

[3] Advisor. ORCID: 0000-0002-3991-6732.

Submitted: January, 2022.

Approved: January, 2022.

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Aline Sales Mendes Záu

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