Persistent Lip Sucking Mimicking Puetz - Jeghers Syndrome - A Rare Presentation of Two Cases.

INTRODUCTION

Many habits that are affecting the orofacial regions are commonly seen in the young children’s and a few of them are consider normal up to the particular age of the child.¹ The persistence of these habits beyond that age will cause considerable effects on the normal growth of the orofacial structures.²

Lip sucking habit is one of the habit usually seen in the young children’s. Persistent lip sucking habit will cause microchelia, smooth glossy lips, and crowding or retroclination of anterior teeth.^3,4 Lip sucking with excessive force over a time can cause the scarring and pigmentation of the skin around the lips over the area where the incisal edges of the anterior teeth meet the skin surface.

The perioral scarring and pigmentation caused by lip sucking habit must be differentiated with the perioral scarring and pigmentation associated with condition and syndrome particularly like Puetz - Jeghers syndrome.⁵

Puetz - Jeghers syndrome is an autosomal dominant hereditary syndrome characterized by the perioral and intraoral melanin pigmentation with intestinal polyposis.⁶

This syndrome can manifest anywhere from infancy to an adulthood. Perioral melanotic pigmentation is usually associated with the buccal, palatal and tongue regions. Since, this syndrome is associated with increases incidence of malignancies in later stages.^5,6

The proper diagnosis is important in the situations wherein the two young children’s of the same parents presented with perioral pigmentation due to lip sucking habit which mimicks Puetz - Jeghers syndrome.

CASE REPORT

A 6 year old male patient reported to the private clinic with the chief complaint of perioral pigmentation. On eliciting the history, the pigmentation was noticed 2 years back and from past 1 year scar appeared over the pigmentation. Medical history and family history of the patient was non-significant. Similar complaint was noticed with the elder brother of the patient. He was 9 years age. On examination, perioral pigmentation was less in elder patient compared to the younger patient. There was no scar present. On eliciting the personal history, it was revealed that, the elder boy has a habit of lip sucking from past 4 to 5 years. it was found that he also had similar presentation around 2 years back. The scar disappeared and pigmentation reduced in intensity gradually as he reduced his lip sucking habit from past one and half years. In younger boy, it was found that he also started to indulge in lip sucking about 3 to 4 years of his age. On examination, the linear perioral scar was seen on upper and lower lip over the skin in the younger boy [Fig 1] and mild pigmentation was seen on the lower lip over the skin in the elder boy [Fig 2]. In both cases, there was no intra oral melanin pigmentation seen [Fig 3]. Interestingly, in both boys the pattern of lip sucking was similar to a greater extent [Fig4]. The Peutz-Jeghers syndrome was consider as the differential diagnosis. In Peutz- jeghers syndrome, the melanotic freckles are seen around the vermillion boarder of the lips with intra oral melanin pigmentation on the buccal mucosa, labial mucosa and the tongue.

Based on the above findings like negative medical, family history and positive personal history of the two cases, we finally arrived to the diagnosis of perioral pigmentation and scarring secondary to the persistent intensive lip sucking habit.

DISCUSSION:

Lip sucking habit is a non-nutritive oral habit usually seen in the children’s in the age group of 3 to 6 years.7 Persistent of this habit beyond this age group is the matter of concern.8 Prolonged lip sucking habit usually causes microchelia, smooth glossy lips, redness and cracking of the lips. In some cases based on intensity, duration and frequency of the habit, there may be pigmentation and scarring of the lips. The pattern of the pigmentation and scarring usually will be linear following the incisial edges of the anterior teeth. In our cases, the younger patient had two linear scars were seen around the mouth, on the upper lip and the lower lip ( Fig 1). The upper and lower scars are separated near corner of the mouth. In elder patient, the brownish pigmentation seen over the lower lip (Fig 2). Melanin pigmentation was not seen over the vermillion border of the lips and intra orally in either of the cases.

In Peutz-Jegher syndrome, the melanotic freckles present on the vermillion border of the lips along with intra oral melanin pigmentation.^{5, 6, 9}

In our cases, the pigmentation seen over the skin not on the vermillion border and it was continuous, with no intra oral pigmentation. The two children’s had an age difference of 3 years. Initially lip sucking habit was noticed in elder boy and much later on in the younger boy. This explains the difference in the clinical presentation of the cases.

In elder boy, only mild pigmentation was seen due to decreased in the frequency of the habit. In younger boy, the intensity and frequency of the habit was much more when compared to the elder one. Usually children’s with lip sucking habit will suck one lip, commonly the lower lip at a time.¹⁰ But in our cases, it is very interesting to see that, both had habit of sucking upper lip and lower lip simultaneously (Fig4).

Perioral pigmentation and scarring is of a diagnostic challenge in the pediatric patients. A through medical, family and personal history is of great importance in the proper diagnosis and management of these conditions.