Tongue Necrosis due to Giant Cell Arteritis / Necrose da língua por arterite de células gigantes

Giant Cell Arteritis is a vasculitis that mainly affects women over 50. The most common manifestations are headache, jaw claudication, and amaurosis. If not diagnosed early, it can lead to rare irreversible ischemic consequences, with tongue necrosis being one of these. We report a case of a previously undiagnosed patient with lateral tongue necrosis who responded well to oral corticosteroid treatment. The diagnosis is clinical, laboratory and histological and may be aided by imaging exams. Initial treatment is with oral corticosteroids, with methotrexate and tocilizumab as alternatives. Diagnostic suspicion and quick start of treatment favorably influence the prognosis of the disease (AU)

Apresentamos um caso de arterite de células gigantes com amaurose, seguida de necrose da língua, diagnosticado clinicamente com boa resposta terapêutica ao corticoide oral. Salientamos que os casos de necrose da língua são extremamente raros. A suspeita diagnóstica de arterite de células gigantes é de fundamental importância e mesmo na impossibilidade de realizar a biópsia de artéria temporal, não se deve retardar o início do tratamento sob pena de acarretar sequelas definitivas ou manifestações mais graves da doença (AU)

Enfermedad de Mucha-Habermann febril ulceronecrótica con respuesta favorable a minociclina, a propósito de un caso / Febrile ulceronecrotic Mucha-Habermann disease: report of one case

Pityriasis lichenoides et varioliformis acuta (PLEVA), pityriasis lichenoides chronica (PLC) and febrile ulceronecrotic Mucha-Habermann disease (FUMHD) are considered different manifestations of the same disease. Febrile ulceronecrotic Mucha-Habermann disease is a rare, and potentially lethal illness which is characterized by fast progression of numerous papules that converge, ulcerate and form a plaque with a necrotic center, together with hemorrhagic vesicles and pustules that are associated with high fever and variable systemic symptoms. We report a 16 years old male presenting with erythematous papules with crusts and fever. The diagnosis of febrile ulceronecrotic Mucha-Habermann disease was confirmed with the pathological study of the lesions. He was successfully treated with minocycline after a failed attempt of treatment with prednisone.

[Febrile ulceronecrotic Mucha-Habermann disease]. / Enfermedad de Mucha-Habermann febril ulceronecrótica con respuesta favorable a minociclina, a propósito de un caso: Report of one case.

Pityriasis lichenoides et varioliformis acuta (PLEVA), pityriasis lichenoides chronica (PLC) and febrile ulceronecrotic Mucha-Habermann disease (FUMHD) are considered different manifestations of the same disease. Febrile ulceronecrotic Mucha-Habermann disease is a rare, and potentially lethal illness which is characterized by fast progression of numerous papules that converge, ulcerate and form a plaque with a necrotic center, together with hemorrhagic vesicles and pustules that are associated with high fever and variable systemic symptoms. We report a 16 years old male presenting with erythematous papules with crusts and fever. The diagnosis of febrile ulceronecrotic Mucha-Habermann disease was confirmed with the pathological study of the lesions. He was successfully treated with minocycline after a failed attempt of treatment with prednisone.