Hosp Aeronáut Cent 2013; 8(2):107-112

Midline destructive lesion. Case report

1er Ten. (E. Med.) Ángeles Borsato*, Liliana Villalon**, Alberto Orden***, Roberto Mugnolo (h)**** Workplace: Clinical Medicine Residency. Hospital Aeronáutico Central. 3697, Ventura de la Vega St., City of Buenos Aires. * Former Chief Resident, Internal Medicine Department, Hospital Aeronáutico Central. ** Doctor, Pathology Service, Hospital Aeronáutico Central *** Head of the Rheumatology Department, Hospital Aeronáutico Central **** Consultant, Department of Internal Medicine, Hospital Aeronáutico Central

Resumen

Abstract

Introducción: El síndrome destructivo de la línea media es una

Introduction:

entidad caracterizada por un conjunto de signos y síntomas

characterized by a group of signs and symptoms, secondary to

secundarios

the condition, usually destructive and located in the middle of the

a

la

afección,

generalmente

destructiva,

de

Midline

destructive

syndrome

is

an

entity

localización medio facial.

face.

Objetivos: Presentación de caso clínico y revisión bibliográfica.

Objectives: Clinical case report and literature review.

Reporte de caso: Varón de 38 años procedente de Chaco con

Case Report: 38-year-old male patient, from Chaco, with

diagnóstico de Leishmaniasis mucocutanea que cursó internación

mucocutaneous Leishmaniasis diagnosis, who was hospitalized

por una lesión destructiva de la línea media que luego de

due to a midline destructive lesion. After multiple diagnostic

múltiples planteos diagnósticos y estudios complementarios se

options

llega al diagnóstico final de vasculitis granulomatosa con

granulomatosis with polyangiitis (Wegener's granulomatosis) is

poliangeitis (granulomatosis de Wegener).

reached.

Discusión Estos pacientes representan un desafío diagnóstico

Discussion: These patients pose a diagnostic challenge in

en medicina interna, debido a las múltiples etiologías capaces de

internal medicine due to the multiple etiology factors that can be

desarrollarlo, con manifestaciones clínicas similares pero con

responsible for its development, with similar clinical symptoms but

pronóstico y tratamiento diferente.

with a different treatment and prognosis.

Palabra

clave:

lesión

destructiva

de

la

línea

media.

and

follow-up

studies,

the

final

diagnosis

of

Key word: Midline destructive lesion. Wegener's granulomatosis.

Granulomatosis de Wegener. Received: March, 14th, 2013. Accepted: July 20th, 2013.

Introduction The midline destructive lesion is an entity

paranasal

characterized by a group of signs and symptoms

maxillary, inferior and medium turbinates, nose

secondary to pathologies that cause inflammation

and adjacent structures bones. It is described in 6

and necrosis of the upper respiratory tract both

of every 10,000 inhabitants1 and, more frequently,

suprapalatal

and

infrapalatal,

Hospital Aeronáutico Central Journal

orbital

sinuses,

ethmoid

bones,

vomer,

floor, 107

Midline destructive lesion. Case Report

within the fifth and sixth decades of life. It prevails 5

He started treatment with prednisone 1 mg/kg/day

in women and has been observed in all races .

plus amphotericin B as a double dose and, as his

It manifests as fast-progression ulcerations and

answer was favorable, he was released from

perforation, though, in some cases it may have

hospital. Soon afterwards, he presented frontal

chronic evolution and consequences in time.

headache and severe compromise of bilateral

Patients show symptoms that suggest diseases of

vision

the superior airways such as rhinorrheas with or

epistaxis and nasal septum destruction. A nuclear

without nose deformity, as well as ulceration of

magnetic resonance showed severe ocular and

nasal mucosa, mouth or gum. It is infrequent for

sinonasal lesions.

patients to show ocular symptomatology due to

Within that context, he was referred to our

4

with

necrotizing

scleritis,

panuveitis,

orbital compromise .

institution. On admission, physical examination

These patients pose a diagnostic challenge in

showed saddle nose, secondary to nasal septum

internal medicine due to the multiple infectious,

destruction (fig 1.), thorax pustular cutaneous

oncologic-hematological

rheumatologic

lesions, as well as other lesions with cicaticial

etiologies that may cause the disease, as well as

appearance (Fig 2), bilateral endophtalmitis with

to the aggressive behaviour many of them have.

blurred vision in both eyes. (Fig 3). Routine tests,

and

as well as other parameters that could guide the

Objectives

etiologic diagnosis, were required. The results Clinical case report and literature review that may

were

as

follows:

leukocytes

11,100

/uL.

serve as a guideline to assess patients with

Conserved formula, microglobulin 2300 ng/mL.

diseases affecting the central structures of the

Proteinuria 0.18 g/24 hs. Rheumatoid factor