Double Lip with Hexadactyly in All the Four Limbs: A Case Report

Introduction

Double lip or macrocheilia is a rare entity that affects either the upper lip or lower lip or rarely bilabial involvement [1, 2]. It may be congenital or acquired and is characterized by excessive areolar tissue and non-inflammatory labia and mucous gland hyperplasia of the pars villosa in the lips [3]. As the upper lip goes in tension during movements like smiling, pars villosa hangs underneath pars glabrosa giving it a characteristic of double-lip appearance. Acquired double-lip deformity usually results from trauma [4].

Case Report

A 22-year-old male presented to the Department of Dentistry with chief complaint of deformed upper lip since 14 years. Patient never noticed the deformed upper lip during his childhood until it worsened when the upper front teeth started to erupt. The upper lip folds gradually increased in size. This growth was painless and was not associated with any neck swelling or eyelid edema. Patient also reported extra fingers in all the four limbs, addressing which, amputation of the extra digits of hands was done in infancy leaving skin tags on bilateral little fingers. His medical and familial history was irrelevant.

On general physical examination, patient had a normal built with no pallor, cyanosis, clubbing, blepharochalasis and thyroid enlargement. Tissue tags were seen on bilateral hands at lateral side of little finger (ulnar hexadactyly) with postaxial hexadactyly in bilateral lower limbs. Local examination revealed slightly convex profile with prognathic maxilla with competent lips. Inner mucosal folds of approximate length of 2.5 cm × 1 cm width were visible on upper lip on smiling with notching in labial frenum area as shown in Figs. 1, 2, 3. Patient also presented with high-arched palate with a fissured tongue.

Fig. 1.

Clinical extraoral frontal photograph of the patient showing double lip on smiling without blepharochalasis

Fig. 2.

Photographs of the hands showing skin tags (signs of previous surgery)

Fig. 3.

Photographs of the feet showing bilateral postaxial hexadactyly

The most remarkable clinical findings included the following: double upper lip, hexadactyly in all the four limbs, high-arched palate and fissured tongue.

Complete hematological investigations along with thyroid profile were within the normal range. Both lobes of thyroid showed normal size and echotexture on USG (ultrasonography). Radiographs of hands revealed no polydactyly, but postaxial hexadactyly was present in lower limbs.

Patient was counseled for a genetic analysis, but he was not keen for the same and refused any further investigations. The double lip was excised surgically under general anesthesia as the patient declined surgery under local anesthesia and there was no recurrence on a follow-up of 1 year.

Discussion

Double lip is a non-inflammatory enlargement of lip mainly due to glandular tissue hyperplasia. This enlargement can be a single continuous strand of tissue or can present with a central constriction due to the presence of upper labial frenum [5]. Usually, double lip compromises the aesthetics when the patient is smiling or attempts to show his/her teeth. When a double lip appears as an isolated anomaly, it is most often congenital [6] and is usually first noticed after the eruption of the teeth [7]. It has been suggested that, with time, the original anomaly may be enhanced by a reactive component secondary to a “sucking-in” of the tissue between the teeth.

Differential diagnosis of double lip includes Ascher’s syndrome which is constituted by recurrent edema of the lip and upper eyelid resulting in double lip and blepharochalasis. In 10% of cases, idiopathic nontoxic thyroid enlargement also occurs [8]. Congenital double upper and lower lips have been reported in association with hypertelorism; unilateral ptosis; blepharochalasis; and broad nose with broad nasal tip, high-arched palate and bilateral third finger clinodactyly [4]. But to the best of our knowledge, there is no case report of double lip and polydactyly in all the four limbs. Whether this is a mere coincidence or a definite association cannot be commented on in our case as the patient was not keen on further analysis. Although our patient refused to undergo genetic analysis, the report of this case calls for an attentive eye on future literature in case another such association is encountered. The only treatment option for the double lip is the surgical excision and long-term follow-up.

Conclusion

With this case report, we would like to bring attention to a new entity. The presence of upper double lip, hexadactyly in all the four limbs, fissured tongue and high-arched palate may just be a new association awaiting its identification.

Compliance with Ethical Standards

Conflict of interest

The authors declare no conflict of interest.

Ethics Statement/Confirmation of Patient’s Permission

Written consent was obtained from the patient for publication of his photographs.