[Experience with Denosumab in central giant-cell granuloma]. / Experiencia con Denosumab en granuloma central de células gigantes.

INTRODUCTION: Central Giant Cell Granuloma is an infrequent bone lesion located mainly in the maxillary bone. The main treatment is surgery with wide margins, so it sometimes causes great morbidity and esthetic al terations. Denosumab, a RANK-ligand inhibitor monoclonal antibody, has been presented as a valid therapeutic alternative in the treatment of these lesions. OBJECTIVE: to describe the clinical and radio logical response after treatment with Denosumab in a patient with unresected giant cell granuloma. CLINICAL CASE: 12-year-old boy who consulted due to a 24-hour maxillary swelling, without other associated symptoms. Examination revealed a tumor in the upper left maxilla with bulging of the ip- silateral gingiva. A CT scan was performed which showed a large expansive intraosseous lesion in the maxillary alveolar ridge. The biopsy of the lesion was compatible with Central Giant Cell Granuloma. Due to the size and location of the lesion, initial treatment with Denosumab, a human monoclonal antibody with action on RANK-ligand, was indicated. After 10 months of treatment, the patient showed a favorable clinical and radiological response, with a size decrease of the lesion and metabolic activity. As an adverse effect, the boy presented mild hypocalcemia, resolved after supplementation with calcium. CONCLUSION: the use of Denosumab as the first line of treatment in Giant Cell Granu loma may be an adequate therapeutic option in adolescents with lesions that are difficult to resect.

Alternative pharmacologic therapy for aggressive central giant cell granuloma: denosumab.

In the search for new pharmacologic therapies for central giant cell granuloma (CGCG), proteins that are essential to osteoclastogenesis are intriguing potential targets. In the present case report, we describe a 25-year-old patient with an aggressive CGCG of the maxilla, who was successfully treated with the antiresorptive agent denosumab, after other pharmacologic treatment had failed to achieve regression or stabilization of the tumor. Denosumab could be a promising alternative to potentially mutilating surgery for CGCG. However, more research is needed before definite conclusions can be drawn about the potential role of this agent in the treatment of CGCG.

Successful treatment of generalized elastolytic giant cell granuloma with psoralen-ultraviolet A.

Elastolytic giant cell granuloma (EGCG) is an infrequent granulomatous skin disorder with variable response to different therapeutic regimens. Information on the benefit of phototherapy is very scarce as this therapy has seldom been tried in the affected patients. We present the results achieved in two female patients after undergoing psoralen-ultraviolet A (PUVA). Two 54-year-old otherwise healthy female patients received a course of PUVA after trying other alternatives. Complete clearance was achieved in the two patients with excellent tolerance and no adverse effects. We consider PUVA is a well-tolerated, safe, and effective treatment for patients with EGCG.

[Annular elastolytic giant cell granuloma]. / Granulome élastolytique annulaire à cellules géantes.

BACKGROUND: Annular elastolytic giant cell granuloma (AEGCG) is a rare form of granulomatous dermatosis. It is characterised histologically by phagocytosis of elastic fibres by multinucleated cells. We report a favourable outcome in a case of AEGCG following PUVA therapy and treatment with synthetic antimalarials. PATIENTS AND METHODS: A 67-year-old retired wine grower presented with highly pruritic annular lesions with raised borders on the shoulders and trunk that had been present for several months. Histological examination of a biopsy sample from the erythematous border was characteristic of AEGCG. Various topical treatments proved ineffective and systemic corticosteroids attenuated the patient's pruritus but had no effect on the skin lesions. PUVA therapy resulted in regression of lesions on the trunk, but the rash spread to the patient's arms and was covered with epidermal microcysts. PUVA therapy was discontinued and treatment with a synthetic antimalarial (hydroxychloroquine 400mg/d) was initiated, resulting in complete regression of the lesions. DISCUSSION: AEGC was isolated in 1979 by Hanke et al. on the basis of five cases seen in females. This is a rare form of dermatosis with some 30 cases being reported in the English literature. The clinical aspect is fairly evocative, with erythematous papular lesions, either alone or in groups, with a raised border and a lighter centre tending towards atrophy. In most cases, the lesions are found predominantly in areas exposed to sunlight. The histological appearance is characteristic, with an image of giant cell elastophagic granuloma from which the name of the disease is taken. This appearance allows the disease to be differentiated from a number of other granulomatous diseases. The aetiology is unknown and treatment is empirical. Spontaneous cure can occur and consistent results have not been obtained with any treatments. In our case, PUVA was partly successful, and the synthetic antimalarials resulted in complete regression of residual lesions.

Limited regression of central giant cell granuloma by interferon alpha after failed calcitonin therapy: a report of 2 cases.

Central giant cell granuloma (CGCG) is a benign lesion of the jaws with a sometimes locally aggressive behaviour. The most common therapy is surgical curettage which has a high recurrence rate, especially in lesions with aggressive signs and symptoms (i.e. pain, paresthesia, root-resorption and rapid growth). Alternative therapies such as interferon alpha (INFalpha) or calcitonin are described in the literature. In this study 2 patients with an aggressive CGCG are presented who were treated with INF mono-therapy. INF mono-therapy was capable of terminating the rapid growth of the lesion in both patients and induced a partial reduction. Total resolution, however, was not obtained and alternative treatment is still necessary.

Sarcoidosis associated with psoriasis vulgaris.

We report a 70-year-old patient with sarcoidosis associated with psoriasis vulgaris. He had a nodule on the medial lower lid of his right eye. Oral corticosteroid for the sarcoid lesions and oral PUVA for psoriasis were employed. The cutaneous lesion disappeared within two months after starting the therapy. No relapse of sarcoidosis has been seen for eight years. The association of sarcoidosis with psoriasis has been previously reported; however, it is still unclear whether this association coincidental or meaningful.

Adult T cell leukemia accompanied by annular elastolytic giant cell granuloma.

We report a 74-year-old Japanese patient with adult T-cell leukemia who concurrently developed annular elastolytic giant cell granuloma. Initially, itchy granulomatous lesions developed on his face, nape of the neck and dorsa of the hands, but gradually erythematous plaques appeared on the back and lower limbs. The histology of the granulomatous lesions revealed coexistence of an epithelioid cell granuloma with giant cells that phagocytosed elastic fibres in the dermis and Pautrier's microabscesses in the overlying epidermis. Subsequent sequential histological studies of an erythematous plaque revealed the development of granulomatous changes in pre-existing lymphomatous lesions. Laboratory data revealed the presence of antibody to human T cell leukemia/lymphoma virus I and 14,200 white cells/mm3 in the peripheral blood with 2% atypical lymphocytes which eventually amounted to 30%, one month before his death.