Orofacial granulomatosis presenting as bilateral eyelid swelling

Abstract

Orofacial granulomatosis (OFG) is an uncommon but increasingly recognized disease of unknown etiology. The typical presentation is chronic swelling of the perioral soft tissue, but eyelid edema can be the sole manifestation. Terminology of this disease can be confusing as it may also be referred to as granulomatous cheilitis and a monosymptomatic presentation of Melkersson-Rosenthal syndrome (MRS). Crohn's disease and sarcoidosis should also be considered in the differential as the histopathology can be similar. Corticosteroids are the mainstay of treatment but can lack efficacy. Atypical presentations and the possibility of systemic disease involvement can further challenge the management. We describe an unusual case in which OFG manifests solely as chronic eyelid swelling. This 69-year old Asian female patient had a delayed diagnosis that responded well to intralesional corticosteroid injection with surgical skin reduction. In addition to describing this unusual presentation of OFG, we review the relevant literature and evaluate the current terminology used to describe this entity.

Isolated bilateral eyelid edema in Melkersson‐Rosenthal syndrome

“…1,3 The facial swelling of MRS has also been categorized as an orofacial granulomatosis (OFG), which encompasses a spectrum of granulomatous inflammation of the orofacial region that are not associated with a systemic disease. [4][5][6] Histopathology in its early stages may present with subepidermal edema and a nonspecific perivascular lymphocytic infiltrate, while late lesions may demonstrate an additional mixed mononuclear infiltration with lymphohistiocytic aggregates and noncaseating granulomatous inflammation that may be intralymphatic. [7][8][9] MRS is a rare disease and its presentation as monosymptomatic periorbital swelling has not yet, to our knowledge, been described in the dermatopathology literature.…”

A case of Melkersson‐Rosenthal syndrome with endocrine disorders: Extraordinary efficiency of hydroxychloroquine and mechanism hypothesis

“…The classical complete triad is present in approximately 25% of cases, whereas the mono‐ or oligosymptomatic forms are more common [2]. Some scholars believe that it belongs to the subclassification of orofacial granulomas [3–6]. Existing case reports have mainly focused on the partial or complete symptoms of MRS [7–9].…”

Lesions of Lip and Tongue

“… Actinic cheilitis, affecting mostly the fair skinned individuals which have the potential to proceed to malignancy 9 .  Orofacial granulomatosis-characterized by orofacial swelling of unknown etiology 10 .  Lymphangioma-a developmental malformation occurring in the head and neck region 11 .…”