Necrobiotic xanthogranuloma This presents as multiple sharply demarcated, indurated, non-tender dermal or subcutaneous nodules and plaques. Periorbital involvement is common. Lesions vary from violaceous to red orange, and enlarge to form infiltrative plaques that may show central atrophy. Though most of the lesions are asymptomatic, some may be pruritic or painful. Ulceration and scarring are ...

This case report originated from a case of neonatal multisystemic juvenile xanthogranuloma (JXG). The patient presented with blue muffin rush, cervical mass, bone destruction, lung nodule, hepatosplenomegaly, and coagulopathy and was successfully treated with Langerhans cell histiocytosis (LCH) based chemotherapy treatment. Similar cases in literature were reviewed and it seems that JXG, a rela...

CASE REPORT A 25-year-old woman noticed a painless yellow-orange mass on her right eye. Her visual acuity was 20/20 in both eyes, and a slit-lamp examination showed a yellow-orange mass located at the superior limbus of the right eye. No other ocular abnormalities were observed. DISCUSSION Surgical excision was carried out and the lesion was sent for histological examination. This showed a gr...

Neurofibromatosis is a systemic hereditary disorder with varied manifestations in bone, soft tissue, the nervous system and skin. Cutaneous manifestations of neurofibromatosis are characterized by café-au-lait macules, multiple neurofibromas, Lisch nodules and intertriginous freckling. Some benign or malignant tumors such as juvenile xanthogranuloma, pheochromocytoma, and malignant melanoma can...

Necrobiotic xanthogranuloma (NXG) is a rare granulomatous disorder of the skin. It is characterised by indurated nodules and erythematous, yellow plaques mostly affecting the periorbital region. In recent years, NXG has been increasingly associated with paraproteineamia and haematological disorders, such as multiple myeloma. Hereunder we report two cases with histological and clinical findings ...

©2017 by Anais Brasileiros de Dermatologia Dear Editor, A 77-year-old Caucasian male patient was referred to our Service for presenting brownish and erythematous-violet papules (4 to 8 mm), located bilaterally in arms, armpits, lower abdominal quadrants and thighs (Figure 1). There was no involvement of the face, dorsal surface of the joints or mucous membranes. The lesions, which were always a...

RATIONALE Xanthomatosis associated with monoclonal gammopathy includes hyperlipidaemic xanthoma (HX), normolipidaemic xanthoma (NX) and necrobiotic xanthogranuloma (NXG). All three pathologies are characterized by skin or visceral lesions related to cholesterol accumulation, monoclonal immunoglobulin (MIg) and hypocomplementemia. The pathophysiology underlying NXG remains unknown although the i...

oids (5, 6). In our patient the titre of circulating anti-ICS antibodies was low, which might indicate a prolonged initial phase of PV before generalized development of bullae. The massive plasma cell in®ltrate seen in the lesional upper dermis may be a factor for the localization of the lesions. Another possibility is that an unknown mechanism prevents the generalization of PV, resulting in th...

806 Ann Dermatol Received March 16, 2016, Revised November 3, 2016, Accepted for publication November 7, 2016 Corresponding author: Hei Sung Kim, Department of Dermatology, Incheon St. Mary’s Hospital, College of Medicine, The Catholic University of Korea, 56 Dongsu-ro, Bupyeong-gu, Incheon 21431, Korea. Tel: 82-32-280-5700, Fax: 82-32-506-9514, E-mail: [email protected] This is an Open Ac...

Necrobiotic xanthogranuloma (NXG) is a rare systemic disease primarily associated with cutaneous plaques; these plaques can manifest anywhere, but most commonly affect the face and periorbital regions. It has a distinct histopathology with extensive necrobiosis and infiltrates of inflammatory cells. Patients with NXG are often found to have paraproteinemia or hematologic disorders, including mu...