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We present two pediatric patients who exhibited an unusual clinical presentation of cutaneous acute graft-versus-host disease (GVHD), characterized by livedo-like appearance. Such manifestations of cutaneous acute GVHD have not been previously documented.
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Epithelioid sarcoma is a rare soft-tissue tumor that occurs mainly in children and young adults. It typically presents as a subcutaneous or deep dermal mass in distal extremities. Due to its benign-appearing clinical presentation, infrequent occurrence, and histologic similarities with other pathologies, the diagnosis of epithelioid sarcoma in its early stages can be extremely difficult and can be easily confused with benign lesions such as warts or foreign body granuloma. In this paper, we report the case of a 12-year-old boy with a distal-type epithelioid sarcoma of the hand and wish to emphasize the difficulties of diagnosing this potentially lethal tumor both clinically and histologically.
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Papiloma , Sarcoma , Neoplasias de Tecidos Moles , Verrugas , Criança , Diagnóstico Diferencial , Humanos , Masculino , Sarcoma/diagnóstico , Neoplasias de Tecidos Moles/diagnóstico , Adulto JovemRESUMO
BACKGROUND/OBJECTIVES: Primary cutaneous lymphomas are rare in pediatric patients. The clinical and histopathological manifestations may differ from those in adults. Due to their low frequency and the insidious clinical picture, the diagnosis is usually delayed. The Spanish Primary Cutaneous Lymphoma Registry was initiated in 2016 as a multicenter registry that would allow better insight into the epidemiological, clinical, histopathological, and treatment response characteristics of patients with primary cutaneous lymphomas. METHODS: We conducted a prospective observational cohort study of primary cutaneous lymphomas in pediatric patients participating in the Spanish Academy of Dermatology and Venereology (AEDV) Primary Cutaneous Lymphoma Registry. RESULTS: At the time of the analysis, 10 patients under 18 years of age out of 799 all-age cases (1.25%) had been included in the registry (7 males, 3 females). The mean age at diagnosis was 9.7 years (SD: 4.8). Seven (70%) had mycosis fungoides, 2 of them had the folliculotropic variant; and 3 (30%) had primary cutaneous marginal zone B-cell lymphoma. CONCLUSIONS: Primary cutaneous lymphomas are extremely rare in pediatric patients and usually have a good prognosis. Therefore, a high level of suspicion is necessary for the diagnosis. We suggest management by experienced physicians and follow-up into adulthood.
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Dermatologia , Micose Fungoide , Neoplasias Cutâneas , Venereologia , Adolescente , Adulto , Criança , Humanos , Estudos Prospectivos , Sistema de Registros , Neoplasias Cutâneas/diagnóstico , Neoplasias Cutâneas/epidemiologia , Neoplasias Cutâneas/terapiaRESUMO
Dermatology for pediatric skin of color is the application of dermatology to the genetically diverse and distinctive segment of the pediatric population that includes children of non-White racial and ethnic groups with increased pigmentation including individuals of Asian, Hispanic/LatinX, African, Native American, Pacific Island descent, indigenous people among others with overlap in particular individuals, and mixtures thereof. The discipline of pediatric skin of color can be challenging with difficulty in diagnosis of common conditions due to underlying pigmentation, variations in common hair styling practices, and differences in demographics of cutaneous disease. Whereas some conditions are more common in children of color, other conditions have nuances in clinical appearance and therapeutics with regard to skin color. This article, the second of the series, focuses on inflammatory skin disease nuances, melanocytic disorders, and hypopigmented mycosis fungoides.
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Transtornos da Pigmentação , Dermatopatias , Criança , Etnicidade , Humanos , Pele , Dermatopatias/diagnóstico , Pigmentação da PeleRESUMO
Reflectance confocal microscopy is an innovative imaging modality, which noninvasively provides an optical image to aid in the diagnosis of skin lesions. While performing a skin biopsy for histopathologic analysis is the gold standard to definitively diagnose skin disease, this may prove to be more difficult in the pediatric population. This will occasionally necessitate sedation or general anesthesia as an approach, which carries a number of different risks. Reflectance confocal microscopy is an exciting new avenue in the support of diagnosing skin pathology, with the goal of improving pediatric patient tolerance and experience when examining epidermal and superficial dermal skin lesions. This review discusses the utility of reflectance confocal microscopy for pediatric dermatology patients pertaining to melanocytic and non-melanocytic skin neoplasms and inflammatory and infectious skin diseases in children.
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Dermatologia , Criança , Humanos , Microscopia ConfocalRESUMO
Neurocutaneous melanocytosis (NCM) is characterized by melanocyte deposition in the leptomeninges and brain parenchyma, primarily occurring in children with large or giant congenital melanocytic nevi (LCMN) or multiple congenital melanocytic nevi. Patients with NCM may develop hydrocephalus and increased intracranial pressure, which can be managed with ventriculoperitoneal (VP) shunting. We present the case of a 16-month-old girl who developed peritoneal carcinomatosis and malignant ascites following VP shunting for hydrocephalus secondary to NCM to increase awareness of this rare, but serious, complication of cerebrospinal fluid diversion.
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Melanoma , Melanose , Síndromes Neurocutâneas , Nevo Pigmentado , Neoplasias Cutâneas , Criança , Feminino , Humanos , Lactente , Melanoma/complicações , Melanoma/diagnóstico , Melanose/diagnóstico , Melanose/etiologia , Síndromes Neurocutâneas/complicações , Síndromes Neurocutâneas/diagnóstico , Nevo Pigmentado/diagnóstico , Neoplasias Cutâneas/diagnósticoRESUMO
BACKGROUND/OBJECTIVES: Sun protection starting in childhood is an important means of skin cancer prevention. Factors associated with sunscreen use have been previously described. However, less is known about factors associated with children's utilization of non-sunscreen sun protection strategies. We sought to examine parent and child characteristics, parental use of sun protection, and barriers associated with children's use of five sun protection strategies: sunscreen, shirts with sleeves, wide-brimmed hats, sunglasses, and shade. METHODS: A cross-sectional survey of parents of children entering kindergarten through grade 8. Survey participants were recruited at the 2016 Minnesota State Fair. RESULTS: A total of 409 parents were surveyed. The most common sun protection strategies parents reported for their child were wearing a shirt with sleeves (69.9%) and using sunscreen (61.9%), while protection via shade, wide-brimmed hats, and sunglasses were reported by less than one-quarter of parents. For each individual strategy, parents' own use of that strategy was strongly associated with that strategy in their children. Lack of child cooperation was associated with decreased use of sunscreen, shirts with sleeves, and wide-brimmed hats. CONCLUSION: Significant room for improvement exists in childhood sun protection. Parents should especially be encouraged to use shade, wide-brimmed hats, and sunglasses as methods of protection for their children. Advising parents to model seeking shade, applying sunscreen, and choosing appropriate clothing and hats may improve sun safety practices.
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Neoplasias Cutâneas , Queimadura Solar , Criança , Estudos Transversais , Humanos , Minnesota , Pais , Roupa de Proteção , Neoplasias Cutâneas/tratamento farmacológico , Neoplasias Cutâneas/prevenção & controle , Queimadura Solar/prevenção & controle , Protetores Solares/uso terapêuticoRESUMO
Little guidance on management of basal cell nevus syndrome in children exists. We report a case series of four patients diagnosed with BCNS in early childhood, in whom several highly suspicious lesions were biopsied, but several smaller and questionably concerning lesions were treated with therapies that are more tolerable for children, including topical imiquimod, 5-fluorouracil, cryotherapy, or touch electrodessication following topical anesthetic cream. These therapies were well tolerated, and all residual or persistent lesions were subsequently biopsied and found to be benign. This approach is often preferable for pediatric BCNS patients, in whom concerning lesions can be identified clinically and managed compassionately. However, any lesion that exhibits growth, bleeding, or symptoms should be biopsied for definitive diagnosis.
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Síndrome do Nevo Basocelular , Neoplasias Cutâneas , Aminoquinolinas , Síndrome do Nevo Basocelular/diagnóstico , Síndrome do Nevo Basocelular/terapia , Criança , Pré-Escolar , Fluoruracila , Humanos , Imiquimode , Neoplasias Cutâneas/terapiaRESUMO
Vemurafenib, a selective BRAF kinase inhibitor, has been found to induce several cutaneous adverse effects, ranging from a keratosis pilaris-like reaction to squamous cell carcinoma. While photosensitivity has been well described as one of these manifestations, we report a case of a 6-year-old boy on vemurafenib who developed a severe blistering sunburn after only two 30-minute episodes of sun exposure. A brief review of other common cutaneous adverse effects of vemurafenib is also provided.
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Toxidermias/etiologia , Transtornos de Fotossensibilidade/induzido quimicamente , Inibidores de Proteínas Quinases/efeitos adversos , Vemurafenib/efeitos adversos , Criança , Humanos , Masculino , Queimadura Solar/etiologiaRESUMO
Neurocutaneous melanosis (NCM) is the condition of abnormal melanocyte deposition in the leptomeninges and brain parenchyma. Associated with congenital melanocytic nevi, NCM can result in neurologic deficits, hydrocephalus, and rarely, malignant transformation of cells. We present the case of a 16-year-old boy with NCM who developed malignant leptomeningeal melanoma following immunosuppression with a TNFα inhibitor. To our knowledge, this is the first reported case of a patient with known NCM undergoing malignant transformation after anti-TNF therapy for inflammatory bowel disease.
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Adalimumab/efeitos adversos , Transformação Celular Neoplásica/patologia , Melanose/patologia , Neoplasias Meníngeas/secundário , Neoplasias Meníngeas/cirurgia , Síndromes Neurocutâneas/patologia , Neoplasias Cutâneas/patologia , Adalimumab/uso terapêutico , Adolescente , Seguimentos , Humanos , Imunossupressores/efeitos adversos , Imunossupressores/uso terapêutico , Doenças Inflamatórias Intestinais/tratamento farmacológico , Doenças Inflamatórias Intestinais/patologia , Imageamento por Ressonância Magnética/métodos , Masculino , Melanose/diagnóstico , Melanose/terapia , Neoplasias Meníngeas/diagnóstico por imagem , Síndromes Neurocutâneas/diagnóstico , Síndromes Neurocutâneas/terapia , Nevo Pigmentado/diagnóstico , Nevo Pigmentado/patologia , Nevo Pigmentado/cirurgia , Doenças Raras , Medição de Risco , Neoplasias Cutâneas/diagnóstico , Neoplasias Cutâneas/terapia , Tomografia Computadorizada por Raios X/métodos , Resultado do TratamentoRESUMO
Primary cutaneous CD4+ small- to medium-sized pleomorphic T-cell lymphoproliferative disorder (PCSM-LPD) is a rare and low-grade form of cutaneous T-cell proliferation with the average age of diagnosis of 54 years. Because of its rarity, the etiology or exact clinicopathology of PCSM-LPD remains unclear, with < 10 pediatric cases reported. A 13-year-old boy presented to our clinic with a raised tumor with PCSM-LPD histology and was successfully treated with ultra-low-dose radiation therapy. While no standard of care has been established for pediatric PCSM-LPD, this report represents an example of achieving remission in a pediatric tumor with minimal potential for therapy-related long-term toxicity.
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Linfócitos T CD4-Positivos/patologia , Linfoma Cutâneo de Células T/radioterapia , Neoplasias Cutâneas/radioterapia , Adolescente , Humanos , Linfoma Cutâneo de Células T/patologia , Masculino , Pele/patologia , Neoplasias Cutâneas/patologiaRESUMO
Immunosuppression following organ transplantation is a known risk factor for the development of lymphoproliferative disorders. Mycosis fungoides, a rare entity in pediatric patients, has seldom been reported as a post-transplant lymphoproliferative disorder. We report a case of folliculotropic mycosis fungoides in a pediatric patient following liver transplantation that was initially diagnosed as tinea capitis.
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Micose Fungoide/diagnóstico , Neoplasias Cutâneas/diagnóstico , Tinha do Couro Cabeludo/diagnóstico , Criança , Diagnóstico Diferencial , Humanos , Transplante de Fígado/efeitos adversos , Masculino , Micose Fungoide/etiologia , Neoplasias Cutâneas/etiologiaRESUMO
Here, we describe a case of a patient with known poikiloderma with neutropenia who developed cutaneous squamous cell carcinoma in a chronically sun-exposed area at the age of 14. To date, there is only one other report of this association. This report highlights the need for routine skin cancer screening in patients with this diagnosis as well as the importance of a correct initial diagnosis.
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Carcinoma de Células Escamosas/complicações , Neutropenia/complicações , Anormalidades da Pele/complicações , Neoplasias Cutâneas/patologia , Adolescente , Carcinoma de Células Escamosas/patologia , Feminino , Humanos , Neoplasias Cutâneas/complicaçõesRESUMO
A newborn boy presented with a progressively infiltrating and painful congenital ulcerated plaque on the back of his left foot. A partial excision was performed and histopathologic examination confirmed a diagnosis of a plexiform fibrohistiocytic tumor. This rare tumor usually appears in children and adolescents, with congenital presentations even more uncommon. This case details the exceptional presentation of a congenital ulcerated plexiform fibrohistiocytic tumor with a review of the current literature.
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Histiocitoma Fibroso Benigno/patologia , Neoplasias Cutâneas/patologia , Neoplasias de Tecidos Moles/patologia , Pé/patologia , Humanos , Recém-Nascido , Masculino , Úlcera Cutânea/patologiaRESUMO
We report the case of a newborn boy with multinodular NRAS and BRAF mutation-negative congenital melanocytic nevi and cerebral lesions compatible with congenital intraparenchymal melanosis. Histopathology from skin lesions showed atypical nodular melanocytic proliferation with marked melanocytic atypia and a large number of mitoses and apoptosis, indicating aggressive proliferation. The child developed several new subcutaneous tumors and multiple internal lesions, which were confirmed to be metastases, and died at 5 months of age. This case may represent an infantile melanoma developing from a giant congenital melanocytic nevus or a congenital melanoma.
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GTP Fosfo-Hidrolases/genética , Melanoma/patologia , Proteínas de Membrana/genética , Nevo Pigmentado/patologia , Proteínas Proto-Oncogênicas B-raf/genética , Neoplasias Cutâneas/patologia , Evolução Fatal , Humanos , Lactente , Recém-Nascido , Imageamento por Ressonância Magnética , Masculino , Melanose/patologia , Mutação , Nevo Pigmentado/genética , Pele/patologia , Neoplasias Cutâneas/genética , UltrassonografiaRESUMO
Sclerodermatous graft-versus-host disease is a subtype of cutaneous chronic graft-versus-host disease that is characterized by sclerosis of the skin and subcutaneous tissue, resulting in debilitating contractures, among other life-threatening complications. Children with sclerodermatous graft-versus-host disease are at high risk of developing nonmelanoma skin cancer because of several risk factors, including young age at transplantation, prolonged immunosuppression, and exposure to photosensitizing antimicrobial prophylaxis such as voriconazole. The immunosuppression required to treat sclerodermatous graft-versus-host disease makes effectively treating nonmelanoma skin cancer and sclerodermatous graft-versus-host disease in the same patient challenging. We describe a challenging case of a 6-year-old boy with a history of sclerodermatous graft-versus-host disease and voriconazole exposure presenting with squamous cell carcinoma in situ on the left temple and actinic keratoses on the scalp treated with topical chemotherapy agents.
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Antifúngicos/efeitos adversos , Carcinoma de Células Escamosas/etiologia , Doença Enxerto-Hospedeiro/complicações , Neoplasias Cutâneas/etiologia , Voriconazol/efeitos adversos , Administração Tópica , Antifúngicos/uso terapêutico , Transplante de Medula Óssea/efeitos adversos , Carcinoma de Células Escamosas/tratamento farmacológico , Carcinoma de Células Escamosas/cirurgia , Dermatite Fototóxica/complicações , Fluoruracila/administração & dosagem , Doença Enxerto-Hospedeiro/tratamento farmacológico , Humanos , Imunossupressores/efeitos adversos , Imunossupressores/uso terapêutico , Ceratose Actínica/tratamento farmacológico , Ceratose Actínica/etiologia , Masculino , Pele/patologia , Neoplasias Cutâneas/tratamento farmacológico , Voriconazol/uso terapêuticoRESUMO
Woringer-Kolopp (WK) is a rare subtype of cutaneous T-cell lymphoma (CTCL) with limited treatment options. Bexarotene gel is a topical retinoid used in the treatment of CTCL. This report describes three female patients (mean age 66 years) with WK disease who had an effective treatment response to bexarotene 1% gel. This treatment could provide a safe alternative to other current treatment modalities which have higher risks of potential adverse effects and lack of access to other conventional treatments such as light therapy.
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Antineoplásicos/administração & dosagem , Reticulose Pagetoide/tratamento farmacológico , Neoplasias Cutâneas/tratamento farmacológico , Tetra-Hidronaftalenos/administração & dosagem , Administração Cutânea , Idoso , Idoso de 80 Anos ou mais , Bexaroteno , Biomarcadores Tumorais/análise , Biópsia , Feminino , Géis , Humanos , Imuno-Histoquímica , Pessoa de Meia-Idade , Reticulose Pagetoide/química , Reticulose Pagetoide/patologia , Indução de Remissão , Neoplasias Cutâneas/química , Neoplasias Cutâneas/patologia , Resultado do TratamentoRESUMO
Inguinal lymph node dissection (ILND) for skin cancer is associated with a high incidence of wound complications. The traditional skin approaches are associated with a high risk of wound/flap necrosis of the inguinal skin, which leads to wound dehiscence and wound infection. We report a novel approach for ILND without inguinal skin incision for patients with invasive extramammary Paget disease (EMPD) to minimize the wound complications inherent in conventional ILND. We totally performed this procedure in 3 patients with invasive EMPD with inguinal nodal metastases. No patient had complications, including flap necrosis, wound dehiscence, or wound infection. Our novel surgical approach would retain the vascular supply because there was no inguinal skin incision, preventing postoperative wound complications. In addition, ILND was easily performed with satisfactory exposure of the surgical field. However, the number of patients was small and the follow-up period was short. Further evaluation of a larger case series with longer follow-up is essential to investigate the effect, safety, and indications for this novel approach.
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Excisão de Linfonodo/métodos , Linfonodos/cirurgia , Doença de Paget Extramamária/cirurgia , Neoplasias Cutâneas/cirurgia , Idoso , Idoso de 80 Anos ou mais , Feminino , Humanos , Linfonodos/patologia , Metástase Linfática , Masculino , Invasividade Neoplásica , Doença de Paget Extramamária/secundário , Neoplasias Cutâneas/patologia , Retalhos Cirúrgicos , Resultado do TratamentoRESUMO
Actinic cheilitis (AC) is a common premalignant condition that requires an effective treatment to reduce the risk of malignant transformation. Photodynamic therapy (PDT) has been recently added to the armamentarium available for AC treatment. Daylight PDT (D-PDT) is a novel PDT modality in which the activation of the topical photosensitizer is induced by the exposure to natural daylight instead of artificial light sources without preliminary occlusion. This simplified procedure was found to be more tolerated as compared to conventional PDT. We report our preliminary experience on the use of D-PDT using methyl-aminolevulinate cream in 10 patients with refractory AC of the lower lip. Patients received two consecutive D-PDT sessions with an interval of 7-14 days. At 3 months after therapy, a complete response was observed in seven patients, with sustained results in five patients over an observational period of 6-12 months. Treatment was well tolerated.