Korean J Dermatol.  1992 Feb;30(1):93-98.

A case of idiopathic midline destructive disease a subgroup of midline granuloma syndrome

Abstract

Idiopathic midline destructive disease(IMDD) is a subgroup of midline granuloma syndrome, which is a clinical entity characterized by a relentless uilcer ation of the upper air way involving the nose, the palate, and the face, with a fatal outcorne. A 32 year-old male had symptoms of nasal obstruct.ion and rhinorrhea for about 4 months prior to visiting the hospital ad was referred to our Department for evaluation of a verely destruct.ive skin lesions on his face. The patient, revealed unique clinopatholcgic featurs compat with IMDD. These were : 1) prescence of locally destructive lesion which were restricted to the upper respiratory tract. 2) abscence of systemic disease durin the follow up peroid, 3) The histologic features consisted of acute and chronic inflarnmation with a variable dearel of necrosis on the examination of repeated biopsy specimens, 4) inabiliy to demonst rate an infectious causative organisms on repeated laboratory tests.

Keyword

Idiopathic midline destructive disease; Lethal midline gr nuloma; Midline granuloma syndrome

MeSH Terms

Adult
Biopsy
Follow-Up Studies
Granuloma*
Humans
Male
Necrosis
Nose
Palate
Respiratory System
Skin
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