Successful treatment of xanthoma disseminatum with pulsed dye laser: A case report.

Xanthoma disseminatum is a rare form of non-Langerhans cell histiocytosis with limited treatment options due to its unknown aetiology and diffuse skin lesions. This case report presents the successful treatment of a 31-year-old male with severe pan-facial xanthoma disseminatum lesions following a facial burn and traumatic brain injury resulting from a car accident. After 5 sessions of monthly pulsed dye laser treatment, there was a clinically significant reduction in the lesions. Over the course of 3 years, the patient underwent a series of monthly pulsed dye laser treatments, and the lesions were almost cleared. These findings suggest that pulsed dye laser therapy may offer an effective treatment option for managing xanthoma disseminatum. This is the first report on use of the pulsed dye laser for treatment of xanthoma disseminatum.

Progressive nodular histiocytosis: an unusual disorder.

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.

Multicentric Reticulohistiocytosis: Elective Excision of Symptomatic Hand Nodules With 1-Year Follow-Up.

Multicentric reticulohistiocytosis is a rare non-Langerhans cell histiocytic rheumatologic disorder characterized by multiple skin lesions, painful hand nodules, and destructive polyarthritis that results in arthritis mutilans in 20% to 30% of patients. In the current literature, there have been approximately 300 cases reported and the pathogenesis remains unclear. Currently, there is no diagnostic serologic test and no consensus on management. In this case report, we present a patient with multicentric reticulohistiocytosis who was treated with selective excision of 3 painful nodules on the hand. The patient was seen 1 year after surgery. One of the nodules reoccurred whereas the other 2 remained quiescent. This procedure has the potential to produce considerable pain relief and improve quality of life in a disease process for which optimal medical regimen remains to be determined.

Osteoarticular lesion in xanthoma disseminatum treated with total hip arthroplasty: a case report.

INTRODUCTION: Xanthoma disseminatum is a very rare disease classified as a benign non-Langerhans cell histiocytosis, which is rarely associated with osteoarticular lesions. There is only a report of tumor abrasion during treatment of osteoarticular lesions of this disease, artificial joint replacement has not been reported. We describe a patient in whom bilateral total joint replacement was performed for disseminated xanthoma lesions of the hip joints. CASE PRESENTATION: A 34-year-old Japanese woman had a chief complaint of bilateral coxalgia. She had been diagnosed as having disseminated xanthoma. Radiographs showed numerous 5-mm radiolucent bands that resembled worm-eaten tracks in the lower part of the femoral heads adjacent to the joint surface. In addition, short tau inversion recovery imaging scans showed high-intensity areas from the femoral head to the neck in both femurs, suggesting bone marrow edema. Total hip arthroplasty was performed for hip arthrosis on both hip joints caused by disseminated xanthoma. Deflection of the implants was a concern from the early stages postoperatively, but both the imaging and clinical findings have been satisfactory for 4 years of follow-up. CONCLUSIONS: A very unusual hip joint lesion of xanthoma disseminatum was replaced with a total artificial joint replacement, and the course over 4 years was good. Our patient's course will continue to be followed carefully.

Progressive nodular histiocytosis with normal karyotypic analysis.

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.

Progressive nodular histiocytosis.

Progressive nodular histiocytosis (PNH) represents a very rare type of non-Langerhans cell histiocytosis (NLCH). It can be distinguished from other types by the occurrence of yellow to red-brown papules and nodules measuring a few centimeters in size. Histologically the nodules represent spindle-cell xanthogranulomas. We report on two new cases of PNH with novel clinical symptoms. In the first case, the patient had microcytic anemia due to massive iron deposition in the nodules and a progressively worsening limp due to involvement of the soles. The complex therapeutic challenge was effectively managed by successive surgical ablation of all visible lesions. The second patient had epibulbar infiltrates. The clinical variability of PNH leads to considerable overlap with other forms of NLCH.

Solitary epithelioid histiocytoma (reticulohistiocytoma) of the glans penis.

Solitary epithelioid histiocytoma (previously called reticulohistiocytoma) is a rare benign dermal histiocytic proliferation characterized by the presence of large, eosinophilic histiocytes with 'glassy' cytoplasm. This entity assumes importance because of its close histologic resemblance to several benign as well as malignant cutaneous lesions. Involvement of the penis is extremely rare, and to our knowledge, only one case has been previously described in the literature. We report a case of solitary epithelioid histiocytoma in a 34-year-old man who presented with a nodule in the glans penis. Microscopy showed proliferation of large epithelioid histiocytes with abundant eosinophilic cytoplasm, which expressed CD68, CD163 and vimentin. This case highlights the significance of recognizing this unusual lesion and differentiating it from its histologic mimics.

A 38-year old woman with a dural based lesion.

Isolated intracranial xanthogranulomas arising from the dura mater are extremely rare.We present a case of a symptomatic large right frontoparietal dura based intracranial xanthogranuloma in a 38-year-old female. Xanthogranulomas are benign non-Langerhans cell histiocytic lesions. They are frequently described in the skin of infants and children. Extracutaneous manifestations especially in the central nervous system are highly uncommon. Dural xanthogranulomas usually arise in association with familial hypercholesterolemia, with Erdheim Chester disease (ECD), and with Weber-Christian disease. Our case however, had no such associations. In this report, the authors describe the clinical, radiological and microscopic presentation of this case and the differential diagnoses of intracranial xanthogranuloma.

Nonlipidized juvenile xanthogranuloma: an unusual variant with a potential diagnostic pitfall.

Juvenile xanthogranuloma (JXG) is a histiocytic inflammatory disorder that can present different histologic patterns. Classic JXG consists of sheets of foamy histiocytes and numerous multinucleated Touton giant cells. Nonlipidized JXG (NJXG) is one of the unusual variants of JXG, consisting of a diffuse monomorphic infiltrate of mononuclear histiocytes, suggesting an aggressive or malignant tumor due the high mitotic index. However, NJXG behaves clinically as classic JXG. We present an unusual case of a 6-year-old boy who presented an exophytic ulcerated nodule on the lower lip diagnosed as NJXG. The boy is currently well without recurrence three years after surgical excision.

Malignancy-associated multicentric reticulohistiocytosis.

Multicentric reticulohistiocytosis (MR) is a rare systemic disorder of unknown etiology. Up to 25% of patients with MR have associated malignancies. We are reporting a case of a patient with MR who initially had an excellent clinical response to oral methotrexate and prednisone. Subsequent attempts of prednisone taper failed because of disease flare-up. Workup for associated malignancy revealed localized squamous cell carcinoma of the lung. The tumor was resected; methotrexate and prednisone were tapered and discontinued with complete remission of MR. A review of literature and the new advances and treatment modalities for MR is provided.

[Necrobiotic xanthogranuloma: efficacy of surgery in 2 patients]. / Xantogranuloma necrobiótico: eficacia de la cirugía en dos casos clínicos.

Necrobiotic xanthogranuloma is a rare disease characterized by indurated nodules and yellowish-red plaques in the dermis or subdermal tissues particularly in the periorbital region; the lesions are often ulcerated. This disease is frequently associated with hematological disorders such as monoclonal gammopathy and lymphoproliferative disorders. Its pathogenesis is unknown and the small number of cases makes long-term studies difficult. We present 2 cases of periorbital lesions in which the biopsies established a diagnosis of necrobiotic xanthogranuloma. Both patients were treated with corticosteroids and cyclophosphamide, with no improvement. The lesions were excised and the periorbital regions were reconstructed with skin grafts. After 1 year of follow-up there are no signs of recurrence.