Case series of volar juvenile xanthogranuloma: Clinical observation of a peripheral rim of hyperkeratosis

Juvenile xanthogranuloma is a benign histiocytic tumor predominantly occurring in children as yellowish papules on the head and trunk. Presentations on the volar surfaces are rare and may cause diagnostic confusion with pyogenic granuloma, eccrine poroma and digital fibrokeratoma. We report two patients with unusual presentations of solitary juvenile xanthogranuloma on the palm or sole. Both had lesions lacking the classic yellowish color and demonstrating a well‐defined, peripheral hyperkeratotic rim. Histopathological evaluation revealed prominent orthokeratosis corresponding to the rim. Additional histological features, including dermal histiocytes and Touton giant cells, were consistent with the diagnosis of juvenile xanthogranuloma. Given the unusual locations and colors of the lesions, we conclude that histopathological evaluation is central to diagnosing volar juvenile xanthogranuloma. We additionally suggest that juvenile xanthogranuloma should be included in the differential diagnoses of volar lesions displaying a peripheral hyperkeratotic rim.     

Juvenile xanthogranuloma with angiomatous appearance and a peculiar immunophenotype

Juvenile xanthogranuloma is the most common form of non‐Langerhans cell histiocytosis in childhood. The clinical differential diagnosis of a solitary juvenile xanthogranuloma includes molluscum contagiosum, Spitz nevus, and melanoma. Lesions larger than 2 cm in diameter may be misdiagnosed as hemangiomas, but this is not typical of smaller juvenile xanthogranuloma. We report a case of solitary juvenile xanthogranuloma in a 10‐year‐old boy with angiomatous appearance and peculiar immunophenotype.     

Radiation port xanthogranuloma: solitary xanthogranuloma occurring within the irradiated skin of a breast cancer patient‐report and review of cutaneous neoplasms developing at the site of radiotherapy

Xanthogranulomas are benign lesions composed of macrophages in which some of the cells have vacuolated cytoplasm. They commonly occur in children, referred to as juvenile xanthogranulomata, and are histopathologically characterized by the presence of Touton giant cells. Xanthogranuloma can also occur in adults. A woman who developed a solitary xanthogranuloma within the field of radiotherapy following treatment of her breast cancer is reported. In addition to xanthogranuloma, other benign lesions of keratinocytes, mast cells, endothelial cells and lymphatic cells have been observed at the site of radiation treatment. Also, several malignancies, including hematopoietic cancers, sarcomas, metastatic cancers and common skin cancers, have been observed within radiation ports. A causal relationship in the development of our patient's xanthogranuloma is suggested by the temporal association between the treatment with radiotherapy and the subsequent appearance of a xanthogranuloma directly within the radiation field. Cohen PR, Prieto VG. Radiation port xanthogranuloma and solitary xanthogranuloma occurring within the irradiated skin of a breast cancer patient: report and review of cutaneous neoplasms developing at the site of radiotherapy.     

Solitary reticulohistiocytic granuloma--a report of three cases and a review of literature

Case 1 was a 20-year-old male with a nodule on the scrotum. Case 2 was a 14-year-old female with a dome-shaped, reddish-brown nodule on the nose. Case 3 was a 30-year-old male with a dome-shaped, reddish-brown nodule on the forearm. All of the excised specimens showed typical features of solitary reticulohistiocytic granuloma. There were histiocytes and multi-nucleated giant cells in the dermal tumorous nests. They were stained positively with PAS reaction and anti-lysozyme antibody, but were stained negatively with S-100 protein antibody. To clarify the nosology of the reticulohistiocytic granuloma, we reviewed the literatures of multicentric reticulohistiocytosis and adult xanthogranuloma. Multicentric reticulohistiocytosis was considered to be a systemic disease and different from solitary reticulohistiocytic granuloma. However, adult xanthogranuloma showed clinical similarities to solitary reticulohistiocytic granuloma instead of the differences in the histopathologic features.     

黄色肉芽肿样单发型朗格汉斯细胞组织细胞增生症一例

朗格汉斯细胞组织细胞增生症（Langerhans cell histiocytosis, LCH）是一种少见的病因不明的疾病，表现为单发型皮损的朗格汉斯细胞组织细胞增生症更是罕见,本文报道一例。该患儿7个月，表现为右侧肩部单发黄红色结节2个月，皮损与黄色肉芽肿非常相似，极易误诊为黄色肉芽肿。     

Severe congenital systemic juvenile xanthogranuloma in monozygotic twins

Abstract:  Juvenile xanthogranuloma, a histiocyte disorder, usually presents with a solitary cutaneous lesion. Juvenile xanthogranuloma with extracutaneous involvement is a rare disease in which significant morbidity and occasional deaths may occur. Monozygotic twins with congenital systemic juvenile xanthogranuloma who presented with multiple skin lesions, hepatosplenomegaly, liver failure, and bone marrow involvement were reported. The diagnosis of systemic juvenile xanthogranuloma was confirmed by histology and immunohistochemical stains of the skin with liver biopsies revealing dense infiltration of lymphohistiocytes with typical Touton giant cells staining positive for CD68 and negative for CD1a and S-100 protein. Both of them received systemic prednisolone 1 mg/kg/day which was gradually tapered off with time according to clinical and investigative responses. At the 17-month follow-up period, both patients showed remarkable regression in all symptoms and laboratory studies.     

Disseminated clustered juvenile xanthogranuloma: an unusual morphological variant of a common condition

Juvenile xanthogranuloma (JXG) is a benign, self-healing non-Langerhans cell histiocytosis of unknown aetiology, most commonly occurring in infants and children. JXG is characterized by solitary or multiple yellowish cutaneous nodules, the two common clinical variants being a small nodular form and large nodular form, which frequently coexist. Unusual morphological presentations include keratotic, lichenoid, pedunculated, subcutaneous, clustered, plaque-like and giant lesions. We describe the first reported case of 'disseminated' clustered juvenile xanthogranuloma presenting in an infant.     

Solitary Mastocytoma on the Palm

Abstract: We report a solitary mastocytoma with a recurrent tense bulla on the palm of a 4-month-old male infant. Histologically there were numerous mast cells infiltrating the dermis. The bulla subsided without treatment in 2 weeks, remaining an oval reddish patch. The present case shows that solitary mastocytoma can occur on the palm, but to our knowledge, this site is unusual.     

单发性成人黄色肉芽肿2例

报告2例单发性成人黄色肉芽肿。两例患者均在面部出现黄红色结节,皮损组织病理示真皮层泡沫样组织细胞浸润,CD68(+),S-100(-)。手术切除皮损后未见复发。     

不伴副球蛋白血症的渐进性坏死性黄色肉芽肿一例并文献复习

报告1例不伴副球蛋白血症的渐进性坏死性黄色肉芽肿并复习相关文献。患者,女,56岁。双上眼睑黄色丘疹10年,泛发加重1年,不伴副球蛋白血症。皮损组织病理检查示：真皮内肉芽肿伴渐进性坏死。     

Juvenile xanthogranuloma underneath a toenail

A 1 1/2-year-old boy developed a dystrophic toenail shortly after trauma to the foot. At the age of three years, it was recognized that the nail deformity was caused by a subungual tumor. The tumor proved to be a juvenile xanthogranuloma. The lesion was solitary, and no systemic associations or visceral involvement were found.     

Multiple adult xanthogranuloma

Juvenile xanthogranuloma is an infrequent, benign, normolipemic, non-Langerhans cell histiocytosis, which primarily affects young children. Clinically, it is characterized by the appearance of one or several brownish-yellow papulonodular lesions on the upper body, especially on the head and neck. Xanthogranulomas are less frequent in adults, and generally present as solitary lesions. The diagnosis of multiple xanthogranulomas in adults is exceptional. We describe the case of a female patient diagnosed with this rare disease.     

Juvenile xanthogranuloma associated with contralateral lymphadenopathy

Juvenile xanthogranuloma is an uncommon, benign histiocytic condition, primarily affecting children less than 1 year of age. Although usually only cutaneous lesions are found, systemic manifestations of the disease have been reported. We present a child with juvenile xanthogranuloma of the right cheek associated with contralateral cervical lymph node histiocytic infiltration.     

Solitary giant xanthogranuloma and benign cephalic histiocytosis — variants of juvenile xanthogranuloma

Summary Sequential biopsies taken from a patient with a solitary giant xanthogranuloma, an exaggerated macronodular (>5 cm in diameter) variant of juvenile xanthogranuloma, and from a patient with benign cephalic histiocytosis, revealed a characteristic time sequence of histopathological findings. Early stages of the diseases showed a monomorphous infiltrate of mononuclear vacuolated histiocytes positive for KiM1p. HAM56 and factor XIIIa and were characterized by clusters of comma-shaped bodies. This was followed by a polymorphous mixture of various mononuclear and mullinucleate histiocytes additionally labelling with KP1 (CD68) and, in occasional cells, for the adherence of peanut agglutinin. A variety of ultrastructural changes were found, including dense and regularly laminated bodies or lipid droplets. Our findings indicate that both entities are variants of a xanthogranulomatous reaction.     

Reflectance confocal microscopy for the noninvasive diagnosis of cutaneous juvenile xanthogranuloma

Juvenile xanthogranuloma is the most common type of non‐Langerhans cell histiocytosis. It appears primarily in infancy, where its differential diagnosis includes Spitz nevus, dermal nevus and molluscum contagiosum. We report the case of a 2‐year‐old boy with juvenile xanthogranuloma, as diagnosed using real‐time, in vivo reflectance confocal microscopy.     

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.     

Juvenile Xanthogranuloma in a Child with Insulin-dependent Diabetes Mellitus

ABSTRACT: A young girl who developed classical insulin-dependent diabetes mellitus at one year of age also developed carotinemia and a typical picture of juvenile xanthogranuloma beginning at five months of age which exhibited A waxing and waning courses. When her diabetes was well-controlled, her serum lipid levels were within normal limits. This is the first reported association of diabetes mellitus and juvenile xanthogranuloma, which together with previous reports of eye, cardiac, pulmonary, and posterior pituitary involvement in children with juvenile xanthogranuloma, suggests that the rare organ involvement in this disease, as well as the characteristic skin lesions, could result from similar etiologic factors which still remain to be elucidated.     

Cutaneous adult xanthogranuloma with a small portion of BRAFV600E mutated Langerhans cell histiocytosis populations: A case report and the review of published work

Histiocytoses, including Langerhans cell histiocytosis (LCH), juvenile or adult xanthogranuloma (AXG) and Rosai–Dorfman disease (RDD), are rare disorders characterized by the proliferation of cells derived from monocyte/macrophage lineages. A few cases of LCH coexisting with xanthogranuloma or RDD have been reported. The etiology of these diseases remains unclear. However, oncogenic BRAF^V600E mutations have been identified in LCH. Here, we report the case of a 26‐year‐old Japanese man with a 3‐month history of a solitary occipital nodule. No abnormality was detected in his other organs, and a total resection of the nodule was performed. Histopathological examination revealed the coexistence of LCH and AXG with prominent emperipolesis characteristic of RDD. Immunohistochemistry showed that most of the large histiocytes were positive for CD68, weakly positive or negative for S100, and negative for CD207 and CD1a, supporting the diagnosis of AXG. The tumor cells with emperipolesis did not show S100‐positive findings characteristic of RDD. The focally aggregated oval histiocytic cells were positive for CD1a, CD207, CD68 and S100, and were compatible with the immunophenotype of LCH cells. In addition, these cells were positive for BRAF^V600E mutation. The tumor cells in our patient exhibited a cellular morphology characteristic of multiple histiocytoses in a solitary cutaneous nodule, which may imply an etiological association among LCH, AXG and RDD. To our knowledge, this is the first report of a BRAF^V600E mutation‐positive case of LCH coexisting with AXG. Because patients with BRAF^V600E mutation have higher risks of multisystemic LCH and recurrence, we should carefully follow up the patient.     

Eruptive xanthogranuloma in a healthy adult male

Xanthogranuloma is a benign, non‐Langerhans cell histiocytosis primarily diagnosed in infants and children, although a subset occurs in adults. Multifocal eruptive presentation of xanthogranuloma is very rare with only 4 previous cases reported in the literature to our knowledge. We describe a case of eruptive xanthogranuloma in a 49‐year‐old man who presented with sudden onset of numerous asymptomatic, red‐yellow to orange papules on the face, scalp, axilla, flank and scrotum. Histologic features were consistent with xanthogranuloma with diffuse mixed infiltrate of foamy histiocytes, Touton giant cells and lymphocytes. Other than temporarily elevated non‐fasting triglycerides, lab values have been unremarkable including serum plasma electrophoresis; however, the patient will continue to be monitored for ocular and other extracutaneous involvement and hematologic malignancies.     

Adult orbital xanthogranulomatous disease: adult-onset xanthogranuloma of periorbital location

Adult orbital xanthogranulomatous disease (AOXGD) is a rare granulomatous disorder, which has four subtypes: adult‐onset xanthogranuloma (AOX), adult‐onset asthma with periocular xanthogranuloma, necrobiotic xanthogranuloma and Erdheim–Chester disease. We report a 42‐year‐old woman who presented with yellowish nonulcerative nodules on her eyelids. On histopathological examination of a nodule, mild degeneration of collagen fibres was seen, with surrounding infiltration of numerous foam cells and Touton giant cells in the deep dermis. Lymphoid follicles were seen in the reticular dermis. There was no apparent necrobiosis of collagen fibres. There were no clinical symptoms of asthma and no laboratory signs of paraproteinaemia during a follow‐up of more than 5 years. We diagnosed this case as AOX, but further long‐term follow‐up would be required for the differentiation from the other AOXGDs. Dermatologists should be aware of these rare granulomatous disease conditions with ocular/orbital location, because they may cause ophthalmological complications.