Pediatric Dermatology Vol. 32 No. 1 109–112, 2015
Congenital Melanotic Macules of the Tongue. A Case Report and Brief Review of the Literature Francesco Savoia, M.D.,* Lorenza Ricci, M.D.,† Annalisa Patrizi, M.D.,† and Giuseppe Gaddoni, M.D.* *Dermatology Unit, Azienda Unit a Sanitaria Locale della Romagna, Ravenna, Italy, †Dermatology Unit, Department of Specialist, Diagnostic and Experimental Medicine, S. Orsola-Malpighi Hospital, Bologna, Italy
Abstract: Congenital melanotic macules of the tongue (CMMT) are a rare and benign condition that is probably underestimated. We report the case of an African infant with multiple congenital hyperpigmented macules of the tongue. To avoid a difficult-to-perform procedure such as a tongue biopsy, focused clinical monitoring was performed every 3 months for 30 months to detect significant changes. A clinical diagnosis of CMMT was made in the absence of concomitant systemic diseases using the clinical findings, the location on the tongue, the negative family history for melanoma, and the absence of drugs and toxic exposure. Clinical follow-up may be sufficient to monitor CMMT rather than performing a tongue biopsy.
A 10-week-old healthy infant boy of Senegalese origin, skin phototype VI, was referred to our outpatient clinic for multiple congenital hyperpigmented macules of the tongue. Physical examination revealed five flat, well-defined, dark-brown macules. Four lesions were round, ranging from 3 mm to 5 mm in diameter, and were located on the left side of the dorsal surface of the tongue; the fifth lesion was linear, was located in the midanterior left portion of the tongue, and was approximately 12 mm 9 4 mm (Fig. 1). Dermoscopy could not be performed because of the lack of compliance of the infant. These lesions, present since birth, were asymptomatic and had grown proportionally with the normal
body growth of the patient. Physical examination was otherwise unremarkable. Family history was negative for melanoma, intestinal polyposis, von Recklinghausen’s disease, Addison’s disease, and toxic agent or drug exposure during pregnancy. The parents reported that similar lesions of the oral mucosa and tongue were frequent in their African tribe, including some members of their families, although the father and mother did not have them. Because of the clinical features of the macules, previous data from the literature, and the reliability of the family, we made a clinical diagnosis of congenital melanotic macules of the tongue (CMMT).
Address correspondence to Lorenza Ricci, M.D., Dermatology Unit, Department of Specialist, Diagnostic and Experimental Medicine, S. Orsola-Malpighi Hospital, University of Bologna, Via Massarenti 1, 40138 Bologna, Italy, or e-mail: lorenza.ricci3@ gmail.com. DOI: 10.1111/pde.12472
© 2014 Wiley Periodicals, Inc.
109
2 months
1 month
Male
Male
Male
Dohil et al (3)
Dohil et al (3) Azorin et al (4)
Female
Male
Marque et al (5)
Marque et al (5)
1 month
10 months
3 months
2 months
Female
Male
Marque et al (5) Marque et al (5)
3 months
Male
Marque et al (5)
4 months
6 months
Male
Dohil et al (3)
6 months
5 months
Female
Female
3 days
Male
Dohil et al (3)
12 years
Female
Anavi et al (1) Menni et al (2) Dohil et al (3)
Age
Sex
Reference
White
White
South American
White
Nord African
White
Hispanic
Hispanic
White
White
White
African American White
Ethnicity
None
None
None
None
None
None
None
None
None
None
None
None
None
Family history
Right dorsum
Left dorsum
Left dorsum
Tip
Left dorsum
Midanterior dorsum Left dorsum
Bilateral dorsum
Left dorsum
Midportion
Right dorsum Left dorsum Left anterior surface
Location on the tongue
1
1
3
1
3
3
6
Multiple
Multiple
1
1
3
3
Number
Brown
3.5 9 3 mm
5 mm
Few mm
5 mm
Few mm
3–5 mm
2–6 mm
Brown
Brown
Brown
Brownish
Brownblack
Dark brown Dark brown
Bluishblack
—
3–4 mm
Dark brown
3–4 mm
Irregular, isolated
Irregular, flat
Asymptomatic, confluent, nonpalpable Round, mildly depressed Smooth, irregular shape, welldefined
Palpable
Circular
Irregular
Irregular, flat without bleeding and ulceration —
—
—
—
ND
ND
+
ND
ND
ND
ND
NN
+
ND
NN
ND
ND
NN
+
+
NN
+
+
+
+
+
+
NN
Subepithelial melanophages
+
+
Increase in melanocytes
+
+
+
+
—
—
Basilar hyperpigmentation
Other clinical characteristics
3 mm
Color Blackbrownish Blue-black
1–3 cm
Size
Histologic findings
TABLE 1. Review of the Cases of Melanotic Macules of the Tongue with Congenital Onset Reported in the Literature
ND
NN
ND
ND
+
+
+
+
+
+
+
NN
Hyperkeratosis
Unchanged after 9 months Spontaneous brightening of one macule, all stable in color and contour No recurrence or surgical consequence after 2 years from total excision At 8 months, spontaneous, almost total, regression of hyperpigmentation, leaving a less pigmented atrophied area
No recurrences during 9 months of follow-up —
Mild increase in size according to child’s growth Mild increase in size according to child’s growth —
Unmodified at 6 months Mild increase in size according to child’s growth —
—
Evolution
Oral corticosteroid therapy for threat of preterm delivery
No drug exposure
No drug exposure No drug exposure
No drug exposure
No toxic or drug exposure
—
—
—
—
—
—
—
Pregnancy
110 Pediatric Dermatology Vol. 32 No. 1 January/February 2015
Unmodified after 30 months of follow-up ND
Normal
Figure 1. Multiple melanotic macules on the left dorsum of the tongue in a 2-month-old African infant.
, not present; ND, not done; NN, not noted.
Figure 2. After 2 years of follow-up: the macules appear unmodified but have grown according to the patient’s body growth.
+, present;
Flat, welldefined Dark brown African 2 months Male Savoia et al (current case)
None
Left dorsum
5
3–12 mm
ND
ND
ND
Evolution Basilar hyperpigmentation Other clinical characteristics Color Size Ethnicity Age Sex Reference
TABLE 1. Continued
Family history
Location on the tongue
Number
Histologic findings
Increase in melanocytes
Subepithelial melanophages
Hyperkeratosis
Pregnancy
Savoia et al: Congenital Melanotic Macules of the Tongue 111
We did not perform a tongue biopsy for histologic examination, but we scheduled follow-up visits. Clinical pictures were taken every 3 months to detect significant macroscopic changes. After 30 months, the patient’s melanotic macules were unchanged except for proportional growth according to the patient’s body growth, suggesting the benign nature of the lesions (Fig. 2). Moreover, the remaining physical examination was always normal, and in particular, there was no submandibular lymphoadenopathy or other pigmented lesions in the oral cavity. DISCUSSION AND CONCLUSIONS Congenital melanotic macules of the tongue are a rare and probably underestimated benign entity with only few cases reported in the literature (1–5). Table 1 summarizes the clinical and histopathologic findings
112 Pediatric Dermatology Vol. 32 No. 1 January/February 2015
of those cases. CMMTs are usually multiple, asymptomatic, hyperpigmented macules on the dorsum of the tongue from birth. Diameters vary from 0.3 to 3 cm, and in most cases the lesions are located on the left side. Other diseases or conditions presenting with oral mucous hyperpigmentation, such as pigmented fungiform papillae, lingua nigra villosa, Laugier– Hunziker syndrome, Peutz–Jeghers syndrome, pigmented nevi, and malignant melanoma, can be easily excluded. Although some authors consider the disease to be more frequent in dark-skinned patients, our review of the literature shows that CMMT occurs in most cases in white patients (5). We could not find any association between CMMT and predisposing or causative factors occurring during gestation. Some authors suggest performing a tongue biopsy to exclude a malignancy (3), but in our opinion this procedure is difficult to perform in the first months of life and can be avoided because the clinical features of CMMT are distinctive and congenital melanoma has never been reported in the oral cavity. A simple follow-up can be performed, and if significant changes occur, a tongue biopsy should be done to exclude a malignant transformation, as in the case of congenital
melanocytic nevi. Only two cases of a melanoma developing over a melanosis of the mouth have been reported, but in two adult patients with an acquired oral melanosis (6,7). REFERENCES 1. Anavi Y, Mintz S. Unusual physiologic melanin pigmentation of the tongue. Pediatr Dermatol 1992;9:123–125. 2. Menni S, Boccardi D. Melanotic macules of the tongue in a newborn. J Am Acad Dermatol 2001;44:1048–1049. 3. Dohil MA, Billman G, Pransky S et al. The congenital lingual melanotic macule. Arch Dermatol 2003;139:767– 770. 4. Azorin D, Enriquez de Salamanca J, de Prada I et al. Congenital melanotic macules and sebaceous choristoma arising on the tongue of a newborn: epidermal choristoma? J Cutan Pathol 2005;32:251–253. 5. Marque M, Vabres P, Prigent F et al. Congenital melanotic macules of the tongue. Ann Dermatol Venereol 2008;135:567–570. 6. Taylor CO, Lewis JS. Histologically documented transformation of benign oral melanosis into malignant melanoma: a case report. J Oral Maxillofac Surg 1990;48:732–734. 7. Kahn MA, Weathers DR, Hoffman JG. Transformation of a benign oral pigmentation to primary oral melanoma. Oral Surg Oral Med Oral Pathol Oral Radiol Endod 2005;100:454–459.
Congenital Melanotic Macules of the Tongue. A Case Report and Brief Review of the Literature Francesco Savoia, M.D.,* Lorenza Ricci, M.D.,† Annalisa Patrizi, M.D.,† and Giuseppe Gaddoni, M.D.* *Dermatology Unit, Azienda Unit a Sanitaria Locale della Romagna, Ravenna, Italy, †Dermatology Unit, Department of Specialist, Diagnostic and Experimental Medicine, S. Orsola-Malpighi Hospital, Bologna, Italy
Abstract: Congenital melanotic macules of the tongue (CMMT) are a rare and benign condition that is probably underestimated. We report the case of an African infant with multiple congenital hyperpigmented macules of the tongue. To avoid a difficult-to-perform procedure such as a tongue biopsy, focused clinical monitoring was performed every 3 months for 30 months to detect significant changes. A clinical diagnosis of CMMT was made in the absence of concomitant systemic diseases using the clinical findings, the location on the tongue, the negative family history for melanoma, and the absence of drugs and toxic exposure. Clinical follow-up may be sufficient to monitor CMMT rather than performing a tongue biopsy.
A 10-week-old healthy infant boy of Senegalese origin, skin phototype VI, was referred to our outpatient clinic for multiple congenital hyperpigmented macules of the tongue. Physical examination revealed five flat, well-defined, dark-brown macules. Four lesions were round, ranging from 3 mm to 5 mm in diameter, and were located on the left side of the dorsal surface of the tongue; the fifth lesion was linear, was located in the midanterior left portion of the tongue, and was approximately 12 mm 9 4 mm (Fig. 1). Dermoscopy could not be performed because of the lack of compliance of the infant. These lesions, present since birth, were asymptomatic and had grown proportionally with the normal
body growth of the patient. Physical examination was otherwise unremarkable. Family history was negative for melanoma, intestinal polyposis, von Recklinghausen’s disease, Addison’s disease, and toxic agent or drug exposure during pregnancy. The parents reported that similar lesions of the oral mucosa and tongue were frequent in their African tribe, including some members of their families, although the father and mother did not have them. Because of the clinical features of the macules, previous data from the literature, and the reliability of the family, we made a clinical diagnosis of congenital melanotic macules of the tongue (CMMT).
Address correspondence to Lorenza Ricci, M.D., Dermatology Unit, Department of Specialist, Diagnostic and Experimental Medicine, S. Orsola-Malpighi Hospital, University of Bologna, Via Massarenti 1, 40138 Bologna, Italy, or e-mail: lorenza.ricci3@ gmail.com. DOI: 10.1111/pde.12472
© 2014 Wiley Periodicals, Inc.
109
2 months
1 month
Male
Male
Male
Dohil et al (3)
Dohil et al (3) Azorin et al (4)
Female
Male
Marque et al (5)
Marque et al (5)
1 month
10 months
3 months
2 months
Female
Male
Marque et al (5) Marque et al (5)
3 months
Male
Marque et al (5)
4 months
6 months
Male
Dohil et al (3)
6 months
5 months
Female
Female
3 days
Male
Dohil et al (3)
12 years
Female
Anavi et al (1) Menni et al (2) Dohil et al (3)
Age
Sex
Reference
White
White
South American
White
Nord African
White
Hispanic
Hispanic
White
White
White
African American White
Ethnicity
None
None
None
None
None
None
None
None
None
None
None
None
None
Family history
Right dorsum
Left dorsum
Left dorsum
Tip
Left dorsum
Midanterior dorsum Left dorsum
Bilateral dorsum
Left dorsum
Midportion
Right dorsum Left dorsum Left anterior surface
Location on the tongue
1
1
3
1
3
3
6
Multiple
Multiple
1
1
3
3
Number
Brown
3.5 9 3 mm
5 mm
Few mm
5 mm
Few mm
3–5 mm
2–6 mm
Brown
Brown
Brown
Brownish
Brownblack
Dark brown Dark brown
Bluishblack
—
3–4 mm
Dark brown
3–4 mm
Irregular, isolated
Irregular, flat
Asymptomatic, confluent, nonpalpable Round, mildly depressed Smooth, irregular shape, welldefined
Palpable
Circular
Irregular
Irregular, flat without bleeding and ulceration —
—
—
—
ND
ND
+
ND
ND
ND
ND
NN
+
ND
NN
ND
ND
NN
+
+
NN
+
+
+
+
+
+
NN
Subepithelial melanophages
+
+
Increase in melanocytes
+
+
+
+
—
—
Basilar hyperpigmentation
Other clinical characteristics
3 mm
Color Blackbrownish Blue-black
1–3 cm
Size
Histologic findings
TABLE 1. Review of the Cases of Melanotic Macules of the Tongue with Congenital Onset Reported in the Literature
ND
NN
ND
ND
+
+
+
+
+
+
+
NN
Hyperkeratosis
Unchanged after 9 months Spontaneous brightening of one macule, all stable in color and contour No recurrence or surgical consequence after 2 years from total excision At 8 months, spontaneous, almost total, regression of hyperpigmentation, leaving a less pigmented atrophied area
No recurrences during 9 months of follow-up —
Mild increase in size according to child’s growth Mild increase in size according to child’s growth —
Unmodified at 6 months Mild increase in size according to child’s growth —
—
Evolution
Oral corticosteroid therapy for threat of preterm delivery
No drug exposure
No drug exposure No drug exposure
No drug exposure
No toxic or drug exposure
—
—
—
—
—
—
—
Pregnancy
110 Pediatric Dermatology Vol. 32 No. 1 January/February 2015
Unmodified after 30 months of follow-up ND
Normal
Figure 1. Multiple melanotic macules on the left dorsum of the tongue in a 2-month-old African infant.
, not present; ND, not done; NN, not noted.
Figure 2. After 2 years of follow-up: the macules appear unmodified but have grown according to the patient’s body growth.
+, present;
Flat, welldefined Dark brown African 2 months Male Savoia et al (current case)
None
Left dorsum
5
3–12 mm
ND
ND
ND
Evolution Basilar hyperpigmentation Other clinical characteristics Color Size Ethnicity Age Sex Reference
TABLE 1. Continued
Family history
Location on the tongue
Number
Histologic findings
Increase in melanocytes
Subepithelial melanophages
Hyperkeratosis
Pregnancy
Savoia et al: Congenital Melanotic Macules of the Tongue 111
We did not perform a tongue biopsy for histologic examination, but we scheduled follow-up visits. Clinical pictures were taken every 3 months to detect significant macroscopic changes. After 30 months, the patient’s melanotic macules were unchanged except for proportional growth according to the patient’s body growth, suggesting the benign nature of the lesions (Fig. 2). Moreover, the remaining physical examination was always normal, and in particular, there was no submandibular lymphoadenopathy or other pigmented lesions in the oral cavity. DISCUSSION AND CONCLUSIONS Congenital melanotic macules of the tongue are a rare and probably underestimated benign entity with only few cases reported in the literature (1–5). Table 1 summarizes the clinical and histopathologic findings
112 Pediatric Dermatology Vol. 32 No. 1 January/February 2015
of those cases. CMMTs are usually multiple, asymptomatic, hyperpigmented macules on the dorsum of the tongue from birth. Diameters vary from 0.3 to 3 cm, and in most cases the lesions are located on the left side. Other diseases or conditions presenting with oral mucous hyperpigmentation, such as pigmented fungiform papillae, lingua nigra villosa, Laugier– Hunziker syndrome, Peutz–Jeghers syndrome, pigmented nevi, and malignant melanoma, can be easily excluded. Although some authors consider the disease to be more frequent in dark-skinned patients, our review of the literature shows that CMMT occurs in most cases in white patients (5). We could not find any association between CMMT and predisposing or causative factors occurring during gestation. Some authors suggest performing a tongue biopsy to exclude a malignancy (3), but in our opinion this procedure is difficult to perform in the first months of life and can be avoided because the clinical features of CMMT are distinctive and congenital melanoma has never been reported in the oral cavity. A simple follow-up can be performed, and if significant changes occur, a tongue biopsy should be done to exclude a malignant transformation, as in the case of congenital
melanocytic nevi. Only two cases of a melanoma developing over a melanosis of the mouth have been reported, but in two adult patients with an acquired oral melanosis (6,7). REFERENCES 1. Anavi Y, Mintz S. Unusual physiologic melanin pigmentation of the tongue. Pediatr Dermatol 1992;9:123–125. 2. Menni S, Boccardi D. Melanotic macules of the tongue in a newborn. J Am Acad Dermatol 2001;44:1048–1049. 3. Dohil MA, Billman G, Pransky S et al. The congenital lingual melanotic macule. Arch Dermatol 2003;139:767– 770. 4. Azorin D, Enriquez de Salamanca J, de Prada I et al. Congenital melanotic macules and sebaceous choristoma arising on the tongue of a newborn: epidermal choristoma? J Cutan Pathol 2005;32:251–253. 5. Marque M, Vabres P, Prigent F et al. Congenital melanotic macules of the tongue. Ann Dermatol Venereol 2008;135:567–570. 6. Taylor CO, Lewis JS. Histologically documented transformation of benign oral melanosis into malignant melanoma: a case report. J Oral Maxillofac Surg 1990;48:732–734. 7. Kahn MA, Weathers DR, Hoffman JG. Transformation of a benign oral pigmentation to primary oral melanoma. Oral Surg Oral Med Oral Pathol Oral Radiol Endod 2005;100:454–459.
