Livedo-like acute cutaneous graft-versus-host disease in two children.

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.

Distal-type epitheloid sarcoma mimicking a wart in a child: A diagnosis not to be missed.

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.

Primary cutaneous lymphomas in children: A prospective study from the Spanish Academy of Dermatology and Venereology (AEDV) Primary Cutaneous Lymphoma Registry.

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.

Review article: Emerging issues in pediatric skin of color, Part 2.

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.

Reflectance confocal microscopy in pediatric dermatology: A state-of-the-art review.

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.

Neurocutaneous melanocytosis-associated malignant melanoma presenting with peritoneal seeding.

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.

Examination of use and barriers for five sun protection strategies in parents and their children.

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.

A pediatric approach to management of skin growths in basal cell nevus syndrome.

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.

Severe vemurafenib-induced photosensitivity in a 6-year-old boy.

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.

Malignant transformation of neurocutaneous melanosis (NCM) following immunosuppression.

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.

Primary cutaneous CD4+ small- to medium-sized pleomorphic T-cell lymphoproliferative disorder in a pediatric patient successfully treated with low-dose radiation.

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.

Folliculotropic mycosis fungoides in a pediatric patient mimicking black dot tinea capitis.

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.

Poikiloderma with neutropenia and associated squamous cell carcinoma: A case report.

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.

Ulcerated congenital plexiform fibrohistiocytic tumor: Case report and literature review.

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.

Aggressive melanoma in an infant with congenital melanocytic nevus syndrome and multiple, NRAS and BRAF mutation-negative nodules.

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.

A pediatric case of squamous cell cancer in situ in the setting of sclerodermatous graft-versus-host disease and voriconazole treatment.

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.

Woringer-Kolopp (Pagetoid Reticulosis) disease successful response to bexarotene gel.

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.

A novel approach for inguinal lymph node dissection without inguinal skin incision for invasive extramammary Paget disease.

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.

Daylight photodynamic therapy with methyl-aminolevulinate for the treatment of actinic cheilitis.

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.