Chronic, progressive enlargement of the lower lip in a healthy girl
- Author(s): Bartell, Holly L;
- Harting, Mandy;
- Eldin, Karen W;
- Hollier, Larry H;
- Metry, Denise W
- et al.
Published Web Locationhttps://doi.org/10.5070/D36382c0n2
Chronic, progressive enlargement of the lower lip in a healthy girlBaylor College of Medicine, Houston, Texas
Holly L Bartell MD, Mandy Harting MD, Karen W Eldin MD, Larry H Hollier MD, Denise W Metry MD
Dermatology Online Journal 13 (2): 20
A 14-year-old girl presented to pediatric dermatology with a 5-year history of painless, progressive enlargement of her lower lip. Prior magnetic resonance imaging ruled-out a vascular etiology. Past treatments attempted included topical, intralesional and systemic corticosteroids, which were unsuccessful. She had a history of eczema and an allergy to cats which occasionally caused mild periorbital edema. Otherwise, her medical and family history, and a review of systems, was unremarkable.
Physical examination showed a healthy-appearing girl with firm enlargement of the entirety of her lower lip, causing eclabium. Rugations were present of the central inner lip (Figs. 1, 2). She had no other skin, mucous membrane, or other physical examination findings.
|Figure 1||Figure 2|
Histopathology showed a diffuse lymphocytic infiltrate in the dermis and submucosa, with perivascular, poorly formed granulomas. Mucosal acanthosis, patchy parakeratosis, and mild submucosal fibrosis were also noted. No acid fast organisms or fungal organisms were seen on AFB and PAS staining, respectively (Figs. 3, 4).
|Figure 3||Figure 4|
Based on the clinical and pathologic findings, a diagnosis was made of granulomatous cheilitis
Granulomatous cheilitis, also known as Miescher's cheilitis granulomatosa, was first described in 1945 . This rare, idiopathic condition is characterized by painless enlargement of one or both lips secondary to granulomatous inflammation. The histology may be nonspecific, but classically shows non-caseating granulomas with edema and a perivascular lymphocytic infiltrate .
Granulomatous cheilitis is considered a subtype of orofacial granulomatosis, and is most commonly regarded as a monosymptomatic finding of Melkersson-Rosenthal syndrome, characterized by the triad of cheilitis granulomatosis, peripheral facial nerve palsy, and fissured tongue. However, it has been estimated that only 25 percent of patients manifest the complete triad . Granulomatous cheilitis is a diagnosis of exclusion as various differentials are capable of producing a similar clinical and histologic picture such as Crohn's disease, foreign body reactions, chronic granulomatous diseases, sarcoid, mycobacterial infections, and allergic reactions. Hence, a thorough work-up is necessary and the full spectrum of orofacial granulomatous diseases should be considered .
Granulomatous cheilitis is also a well-documented manifestation of Crohn's disease . However, the need for aggressive evaluation for this possibility in a patient with isolated granulomatous cheilitis is controversial. A review of 13 such patients by Van der Waal et al. concluded that in the absence of gastrointestinal complaints, no further investigation is necessary . However, multiple studies have shown that cheilitis granulomatosa may precede the onset of Crohn's by several years; therefore, regular followup is advised .
The management of cheilitis granulomatosa is often challenging, with recurrences frequent despite treatment. Topical corticosteroids are the most commonly used modality, followed by intralesional and systemic corticosteroids. Other systemic therapies reported include clofazimine, hydroxycholoroquine, and sulfasalazine . Reduction cheiloplasty is the treatment of choice for medically unresponsive cases . Our patient underwent a labial reduction performed via full-thickness V-shaped wedge excision, with excellent results.
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