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Scholarly Works (76 results)

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Dermatology Online Journal, Volume 30, Issue 1 (2024)
Juvenile xanthogranuloma is the most frequent form of non-Langerhans cell histiocytosis in children. Clinically, it presents as well defined, yellowish papules that are typically located on the head, neck, upper trunk, and proximal region of the extremities. Although solitary lesions are the most common presentation, few cases of multiple juvenile xanthogranuloma have been described, more frequently associated with extracutaneous involvement. We report a 2-month-old girl with 22 cutaneous papules, clinically and histologically compatible with juvenile xanthogranulomas. Screening of visceral involvement was performed with no evidence of systemic disease. Identifying high-risk factors of systemic disease in patients with multiple juvenile xanthogranuloma is essential to perform an appropriate management of this entity.
    Cover page: Multiple juvenile xanthogranulomaCreative Commons 'BY-NC-ND' version 4.0 license
    • Article
    • Peer Reviewed
    Dermatology Online Journal, Volume 22, Issue 10 (2016)
    Juvenile xanthogranuloma (JXG) is a benign, non-Langerhans cell histiocytosis, which commonly manifests as a solitary papule or nodule in infants and young children. However, other less common clinical presentations have been reported including clustered, agminated or plaque-like. It is important to recognize these variants, as diagnosis is often clinical. We highlight a case of juvenile xanthogranuloma presenting as nodules on the trunk of an infant that gradually coalesced to form a larger plaque.
      Cover page: Coalescing nodules on the trunk of an infant: juvenile xanthogranuloma ‘en plaque’Creative Commons 'BY-NC-ND' version 4.0 license
      • Article
      • Peer Reviewed
      Dermatology Online Journal, Volume 23, Issue 7 (2017)

      Congenital juvenile xanthogranuloma (JXG) is an uncommon diagnosis and even more rarely presents with ulceration. We report such a case in a two-week-old girl. Biopsy was performed to rule out any concerning entities. Adequate treatment was provided with topical petrolatum and occasional miconozole or zinc oxide; the mass spontaneously regressed. Because congenital JXG has an excellent prognosis, insight into unique presentations such as this may provide useful information and avoid unnecessary surgical interventions.

        Cover page: Congenital juvenile xanthogranuloma with ulceration: a pediatric case reportCreative Commons 'BY-NC-ND' version 4.0 license
        • Article
        • Peer Reviewed
        UC San Francisco Previously Published Works (2016)

        Background

        Head and neck cancer is often managed with a combination of surgery, radiation therapy, and chemotherapy, and skin toxicity is not uncommon. Xanthogranuloma is a pathological finding resulting from an inflammatory reaction that has not been previously reported following head and neck radiation therapy.

        Case presentation

        A patient with squamous cell carcinoma of the oropharynx, treated with definitive chemoradiation and hyperthermia, presented at eight-month follow-up with an in-field cutaneous lesion in the low neck, initially concerning for recurrent tumor. Biopsy showed xanthogranuloma and the patient underwent complete resection with congruent surgical pathology. The patient remained free of malignancy but continued to experience wound healing difficulties at the resection site which resolved with specialized wound care and hyperbaric oxygen.

        Conclusions

        Skin toxicity is not uncommon in patients with head and neck cancer treated with radiation therapy. Awareness of unusual pathologic sequelae, such as xanthogranuloma, is needed to provide patient counseling while continuing appropriate surveillance for recurrent malignancy.
          Cover page: Xanthogranuloma in the heavily irradiated low neck in a patient with head and neck cancer
          • Article
          • Peer Reviewed
          Dermatology Online Journal, Volume 24, Issue 5 (2018)
          Subcutaneous juvenile xanthogranuloma (JXG) of the inguinal fold, an unusual location, was diagnosed in an infant. Subcutaneous JXG should be included in the differential diagnosis of subcutaneous nodules of the lower body, despite the absence of the characteristic yellowish hue usually associated with JXG.
            Cover page: Firm, hyperpigmented subcutaneous nodule in the inguinal fold of an infantCreative Commons 'BY-NC-ND' version 4.0 license
            • Article
            • Peer Reviewed
            Dermatology Online Journal, Volume 29, Issue 2 (2023)
            Herein, we present a patient with a lipidized fibrous histiocytoma, an underrecognized variant of dermatofibroma (cutaneous fibrous histiocytoma). Our patient presented with a nodule on the ankle that showed foamy histiocytes and hyalinized collagen bundles on histology. This case highlights a classic presentation and features of lipidized fibrous histiocytoma, raising further awareness of this distinctive variant of dermatofibroma that should be distinguished from xanthoma and xanthogranuloma.
              Cover page: A pruritic pedunculated pink nodule on the legCreative Commons 'BY-NC-ND' version 4.0 license
              • Article
              • Peer Reviewed
              Dermatology Online Journal, Volume 27, Issue 2 (2021)
              Progressive nodular histiocytosis (PNH) is a rare type of non-Langerhans cell histiocytosis of the xanthogranuloma group. Less than 20 cases have been reported. We report here a novel case of PNH with dermoscopic description and post-surgical outcome. Our patient presented with pruritic papules and nodules with progression over two years. Dermoscopic examination of large lesions showed multiple telangiectases without ulceration. There was no mucosal involvement. Histopathological analysis of a papule showed a dermal infiltrate composed of histiocytes and many Touton giant cells; a nodule revealed a dermis occupied by spindle cells arranged in a storiform pattern. The nodules were resected and the largest surgical defect, on the forehead, was corrected with A-T advancement flap. The papules were treated with cryosurgery achieving adequate cosmetic outcome without recurrence.
                Cover page: Progressive nodular histiocytosis: an unusual disorderCreative Commons 'BY-NC-ND' version 4.0 license
                • Article
                Pediatrics (2023)
                Neurofibromatosis type 1 (NF1) is an inherited tumor syndrome caused by heterozygous germline mutations in the NF1 gene, occurring in approximately 1/2600 individuals. A subset of patients with neurofibromatosis type 1 (NF1) develop juvenile xanthogranulomas (JXGs), a non-Langerhans cell histiocytosis, and some of these patients also develop juvenile myelomonocytic leukemia (JMML).Yet, these associations are poorly delineated.JXG is a benign proliferation of non-Langerhans cells histiocytes characterized by small yellow/brown papulonodules ranging from 1-20 mm in size. JMML is a mixed myeloproliferative-myelodysplastic disorder that affects children, most often before age 6.4. The first and only systematic review on this described therisk of developing JMML 20 to 30 times higher in patients with NF1 with JXG lesions compared to those without JXG. Since then, mostly isolated case reports have either refuted or confirmed this triple association.
                  Cover page: The association between juvenile xanthogranulomas in neurofibromatosis type 1 patients and the development of leukemia: A systematic review
                  • Article
                  • Peer Reviewed
                  Dermatology Online Journal, Volume 19, Issue 6 (2013)

                  Non-Langerhans cell histiocytoses (NLH) comprise a spectrum of diseases that includes sinus histiocytosis with massive lymphadenopathy, hemophagocytic lymphohistiocytosis, xanthogranuloma, and reticulohistiocytoma. Progressive nodular histiocytosis (PNH) is a rare NLH that microscopically mimics juvenile xanthogranuloma but presents with disseminated persistent and progressive papulonodules in adults. Herein, we describe a case of PNH presenting as diffuse, progressively enlarging papules, nodules, and pedunculated tumors in a 38-year-old male. The diagnosis is supported microscopically by the morphologic and immunohistochemical findings. Whereas conventional cytogenetic analysis of Langerhans cell histiocytosis and juvenile xanthogranuloma has previously been described, there are no reports of the karyotype of PNH. In our patient, conventional cytogenetic analysis of the tumor revealed a normal karyotype. Although these results may represent the overgrowth of normal stromal cells rather than lesional cells, we believe this to be an important finding, indicating karyotypic analysis will not allow for distinction between PCH and other NLH or Langerhans cell histiocytoses.

                    Cover page: Progressive nodular histiocytosis with normal karyotypic analysisCreative Commons 'BY-NC-ND' version 4.0 license
                    • Article
                    • Peer Reviewed
                    Dermatology Online Journal, Volume 11, Issue 4 (2005)
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