Atrophic involution of juvenile xanthogranulomas

INTRODUCTION: We report an unusual atrophic involution of juvenile xanthogranulomas. CASE REPORT: A newborn boy presented with 5 papular, nodular and necrotic lesions located on the upper part of the body. The diameter of the lesions ranged between 1 and 3 cm. Light microscopy showed an infiltrate with foamy and Touton cells. Langerhans' cell histiocytosis was eliminated because none of these cells showed reactivity for S100 protein and CD1a. At the age of 8 years, all the lesions had spontaneously regressed leaving unusual atrophic scars that had the same size as the active lesions. DISCUSSION: We compared the clinical, histological and evolution data of our patient with 251 published cases. The most significant clinical feature of juvenile xanthogranuloma is the spontaneous involution without any trace. However, hyperpigmentation, anetoderma or atrophy may occur. Atrophy is not frequent and can result from 2 mechanisms. Inflammation of the hypodermic tissue, which becomes atrophic and atrophy that may also result from collagen remodeling anomalies during the scarring process.     

Multiple juvenile xanthogranulomas in a 13-year-old

A 13-year-old Caucasian girl presented with a 1(1/2) month history of multiple, asymptomatic, discrete, orange-yellow to skin-colored, dome-shaped, smooth, 3 to 6 mm papules on the arms, legs, trunk, and buttock. Pathology showed a dermal infiltrate of Touton-type giant cells, scattered lymphocytes, and macrophages. The patient was diagnosed with juvenile xanthogranuloma. We present this patient because of the uncommon presentation of multiple juvenile xanthogranulomas in a 13-year-old. Although juvenile xanthogranuloma generally occurs in infancy, it must be included in the differential diagnosis for an older child. This entity can less frequently occur in adults and typically a solitary lesion is found. Multiple juvenile xanthogranuloma is rare in older children and adults. Work-up should include a thorough review of systems, physical examination, and ophthalmology examination. Pediatricians and dermatologists should be aware that juvenile xanthogranulomas might occur in older children.     

HMB-45 expression in the epidermis overlying juvenile xanthogranulomas

Aim HMB-45 staining of melanocytes has been described in vascular proliferations and surgical scars. We studied this staining on inflammatory tumours. Methods Histiocytic tumors were stained with HMB-45 monoclonal antibody. Results Only juvenile xanthogranulomas stained with HMB-45. Conclusion HMB-45 expression may be induced by cytokines synthesized by inflammatory cells.     

Multiple juvenile xanthogranulomas successfully treated with CO2 laser

Juvenile xanthogranulomas are a rare disease entity within the spectrum of cutaneous non‐Langerhans cell histiocytoses characterized by single or multiple cutaneous xanthogranulomas without further organ involvement. A 16‐year‐old girl developed multiple xanthogranulomas in various skin regions, especially the trunk and the flexural areas, over a period of 6 months. The clinical diagnosis was confirmed by H&E histology, showing a histiocytic infiltrate admixed with foam cells and Touton giant cells, and by immunohistochemistry using anti‐bodies to stabilin‐1 whose expression is highly specific for cutaneous non‐Langerhans cell histiocytoses. No diabetes insipidus, pituitary hyperplasia or paraproteinemia were observed. Skin lesions were treated with a CO₂ laser and did not reappear during a 5 years follow‐up period.     

Clinicohistopathologic comparison of adult type and juvenile type xanthogranulomas in Korea

Xanthogranuloma (XG) is an uncommon benign disorder characterized by solitary or multiple yellow-red papulonodules on the skin, and occasionally, in other organs. It is predominantly a disease of infancy or early childhood, although adults may rarely be affected. To compare the clinicohistopathological featues of juvenile-type xanthogranulomas UXGs) and adult-type xanthogranulomas (AXGs) (>14 years) in Korea, 30 cases of JXGs and 15 cases of AXGs were compared clinically and histopathologically. Except for the fact AXGs were more often solitary and larger and showed neither other associated systemic diseases nor spontaneous regression, the clinical features such as color, mean number, or site of the lesions in AXGs were not statistically different from JXGs. Histologically, AXCs were not significantly different in amounts of foamy cells, giant cells including Touton cells, and inflammatory cells, although subcutaneous involvement was seen only in the two infant cases. In conclusion, in contrast to AXGs, JXGs need special attention to accompanying systemic diseases and do not need excisional procedures, considering their frequent spontaneous regression.     

Multiple xanthogranulomas in an adult

Xanthogranulomas develop in adults as well as in children; however, adult cases with multiple lesions are very rare. We report an adult who developed both multiple cutaneous lesions on the face and trunk and lesions on the conjunctiva, oral mucosa and genitalia. We believe that this is the first such case described.     

Multiple xanthogranulomas in an adult: case report and literature review

An otherwise healthy 30-year-old male developed multiple xanthogranulomas over a 1-year period. A review of the literature on adult xanthogranulomas and a tabulated outline of the differential diagnosis is presented.     

Treatment of refractory necrobiotic xanthogranulomas with extracorporeal photopheresis and intravenous immunoglobulin

Necrobiotic xanthogranuloma (NXG) is a disease of fibrotic or telangiectatic granulomatous papules and nodules that can ultimately progress into ulcerated plaques. Although the exact cause of NXG is unknown, it most often occurs in patients with paraproteinemia secondary to a hematologic disease. Consequently, therapy for NXG is targeted at treating the underlying hematologic disease, and subsequent paraproteinemia, with alkylating agents, antimetabolites, radiation, and/or immunosuppressive agents. Cases refractory to these therapies often have poor outcomes. We report the successful treatment of two patients with refractory NXG with two different modalities: extracorporeal photopheresis (ECP) and intravenous immunoglobulin (IVIG). The first case shows a patient without paraproteinemia who had success with ECP and IVIG, and the second is a patient with paraproteinemia treated effectively with IVIG. The beneficial response of our patients to IVIG, as well as ECP, shows that they may be an effective treatment option for refractory NXG.     

Emperipolesis and S100 expression may be seen in cutaneous xanthogranulomas: A multi‐institutional observation

Cutaneous Rosai‐Dorfman disease (RDD) can be difficult to distinguish from other non‐Langerhans cell histiocytoses, particularly xanthogranuloma (XG). Pathologists use S100 immunoreactivity, abundant plasma cells, and the presence of emperipolesis to distinguish RDD from XG. However, S100 expression has been reported in XG and, in practice, we have occasionally observed emperipolesis in cases that were otherwise clinically and pathologically consistent with XG. We present 10 cases of XG with emperipolesis and variable S100 immunoreactivity. Histologically, 7 cases were most in keeping with XG, and a histologic differential of XG versus RDD was raised in the remaining 3 cases. All 10 cases were clinically consistent with XG. Notably, none of these cases showed abundant plasma cells. Nine cases showed variable S100 immunostaining, ranging from focal/weak expression, to focal/strong, diffuse/moderate, and diffuse/strong expression. Histiocytes in all cases were CD68 positive and CD1a negative. We conclude that emperipolesis and S100 expression in a skin biopsy cannot reliably distinguish XG from cutaneous manifestations of RDD. Clinical correlations are essential, as are histologic clues to a diagnosis of classic XG that include an abundance of foamy mononuclear cells, Touton giant cells, and an absence of pale‐stained histiocytes, abundant plasma cells, fibrosis, or vascular proliferation.     

Association of juvenile xanthogranuloma with juvenile myeloid leukemia

Multiple cutaneous xanthomas developed in a patient at the age of 10 months, and juvenile chronic myeloid leukemia (JCML) developed at the age of 30 months. The xanthomas were histopathologically consistent with a diagnosis of juvenile xanthogranuloma (JXG). A review of other cases of JCML with JXG indicates that the cutaneous lesions have many clinical and histopathologic similarities to sporadic JXG but are more often multiple or papular and confluent. In addition to JXG, a few children with JCML also have multiple café-au-lait spots and a family history of neurofibromatosis.     

Disseminated xanthogranulomas associated with adult T-cell leukaemia/lymphoma: a case report and review the association of haematologic malignancies

Xanthogranuloma (XG) is rarely observed in adults and has been reported to be associated with chronic myelogenous leukaemia (CML) and/or neurofibromatosis type 1 (NF1). A 68-year-old woman with adult T-cell leukaemia/lymphoma (ATLL) gradually developed disseminated XGs over the 3 years since disease onset. Histopathological examination of a skin biopsy revealed the presence of histiocytes in the dermis with a few Touton giant cells admixed with lymphoid cells. The lesions of XGs persisted despite chemotherapy with prednisolone and chlorambucil for her ATLL. This is the first report of disseminated XGs associated with ATLL. The association of disseminated XGs with haematologic malignancies was reviewed and the possible pathogenesis of this association will be discussed.