MacrochileliaK13.05

Persistent enlargement of the lips of varying etiology that is clearly perceptible compared to the norm. Chronic macrocheilia is defined as persistent enlargement of one or both lips for at least 6 (8) weeks.

Macrocheilias due to filler materials belong to a special category.

Congenital disorders

• Occur, for example, as idiopathic familial macrocheilia or as a component of syndromes (e.g., Ascher syndrome).
• Multiple endocrine neoplasia 3 (MMN syndrome).
• Hereditary angioedema (episodic swelling).
• Vascular malformations (venous, arterial, lymphatic/mixed)
• Down syndrome
• Storage diseases (e.g. systemic amyloidosis)

Trauma/infections

• Hematomas
• Herpes simplex recidivans
• Recurrent erysipelas
• Bacterial cheilitis
• Leprosy lepromatosa
• Leishmaniasis

Tumor formations

• Multiple skin and mucous membrane neuromas in multiple neuroma syndrome ( MMN syndrome)
• Spinocellular carcinoma
• Pseudolymphoma
• Malignant lymphoma

Other

• Cheilitis granulomatosa (oro-facial granulomatosis)
• Cheilitis glandularis
• Non-hereditary angioedema (episodic swelling)
• Sarcoidosis
• Crohn's disease

In a larger study of 47 identified patients (Toumi A et al. 2021), the distribution pattern was as follows:

• 20 (43 %) cutaneous leishmaniasis
• 10 (21 %) cheilitis granulomatosa
• 5 (11 %) Melkersson-Rosenthal syndrome
• 5 (11 %) sarcoidosis
• 1 (2 %) lepromatous leprosy
• 1 (2 %) systemic amyloidosis
• 1 (2%) Crohn's disease.
• In 4 cases, the etiology remained unknown.

Ulcerations were significantly associated with leishmaniasis.

Macrocheilia has numerous causes. It can be congenital or acquired. It can occur in isolation or as a partial manifestation of a general disease (see classification).

1. Toumi A et al (2021) Chronic macrocheilia: a clinico-etiological series of 47 cases. Int J Dermatol 60:1497-1503.