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 Table of Contents  
CASE REPORT
Year : 2016  |  Volume : 28  |  Issue : 3  |  Page : 342-345

Pigmentation of oral cavity: A clinical enigma; A rare case report


1 Department of Oral Medicine and Radiology, Bharati Vidyapeeth Deemed University Dental College and Hospital, Pune, Maharashtra, India
2 Department of Oral and Maxillofacial Pathology, Dasmesh Institute of Research and Dental Sciences, Faridkot, Punjab, India
3 Department of Oral Medicine and Radiology, Ragas Dental College and Hospital, Chennai, Tamil Nadu, India
4 Private Practice, Bhopal, Madhya Pradesh, India

Date of Submission05-Oct-2015
Date of Acceptance06-Dec-2016
Date of Web Publication13-Dec-2016

Correspondence Address:
Dr. Shams Ul Nisa
Department of Oral Medicine and Radiology, Bharati Vidyapeeth Deemed University Dental College and Hospital, Pune - 411 043, Maharashtra
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/0972-1363.195657

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   Abstract 

The diagnosis of pigmented lesions of the oral cavity and perioral tissues is challenging for the clinicians. The term orofacial pigmentation refers to a wide range of lesions and conditions, which change the color of the orofacial tissues. Even though some lesions may definitively be diagnosed on clinical grounds alone, the final diagnosis usually requires clinical, biochemical, and histopathologic evaluation. Clinical tests, such as diascopy and radiography, and laboratory investigations, such as blood tests, can be used to confirm a clinical impression and reach a definitive diagnosis. The oral cavity is the mirror of the body and is often the area where systemic diseases first present. Hereby, we are presenting a rare case of macular hyperpigmentation with lace-like pattern of oral cavity and macular hyperpigmentation of the face, skin, and palms, with a diagnosis of pigmentation due to iron-deficiency anemia.

Keywords: Iron-deficiency anemia, oral cavity, orofacial pigmentation, systemic diseases


How to cite this article:
Nisa SU, Saggu TK, Sangeetha R, Harchandani N. Pigmentation of oral cavity: A clinical enigma; A rare case report. J Indian Acad Oral Med Radiol 2016;28:342-5

How to cite this URL:
Nisa SU, Saggu TK, Sangeetha R, Harchandani N. Pigmentation of oral cavity: A clinical enigma; A rare case report. J Indian Acad Oral Med Radiol [serial online] 2016 [cited 2021 Oct 16];28:342-5. Available from: https://www.jiaomr.in/text.asp?2016/28/3/342/195657


   Introduction Top


The oral cavity reflects the overall status of the body and other organs. The signs of systemic disease are often manifested in the oral cavity before the systemic disease itself is suspected.[1] Human oral mucosa is not uniformly colored. Exogenous pigmentation of the oral mucosa is commonly caused by foreign material implantation such as amalgam tattoo and drugs. Endogenous pigments include melanin, hemoglobin, hemosiderin and carotene.[2],[3] Here, we are presenting a rare case with macular hypo and hyperpigmentation with lace-like pattern of buccal mucosa and diffuse pigmentation of the facial skin, palms, and nails with a clinical enigma in diagnosis.


   Case Report Top


A 40-year-old, female patient reported to our department with a chief complaint of generalized discoloration of the entire oral cavity since 5 years. Patient also complained of pain and burning sensation since 6 months. The patient gave a history of pain or burning sensation, which was gradual in onset, of dull aching type, and intermittent in nature. The pain and burning sensation aggravated during meals only and subsided on its own. Patient had a history of frequent nausea, vomiting, and constipation with loss of appetite, abdominal pain and muscular pain in the hands and legs since one year. Patient did not give any history of tissue abuse habits. The patient gave a history of irregular menstrual cycle (one cycle in three months).

Extra-oral examination revealed pallor of skin and conjunctiva with generalized diffuse macular hyper pigmentation on the palms. The bronze color nails of the fingers and toes were observed without any dystrophy. Diffuse hyper pigmented macular lesions were seen in the right and left side of the face [Figure 1]. On palpation, the skin was rough and wrinkled.
Figure 1: Extraoral picture showing generalized diffuse macular hyperpigmentation on the right and left side of the face and palms with bronze color nails

Click here to view


Intraoral examination revealed diffuse areas of hypo and hyperpigmentation present bilaterally on buccal mucosa with a radiating lace-like pattern resembling Wickham's striae. Generalized blackish brown pigmentation present in the entire oral cavity with localized areas of hyperpigmentation was noted in the palatal and labial mucosa [Figure 2]. Cracks and fissures were noted in the right and left commissural area of the lips with erythematous surface without tissue tags. On palpation, the entire oral mucosa was soft in consistency without any evidence of bleeding. The commissural area of lips bled on palpation bilaterally. Correlating history and clinical examination, the case was provisionally diagnosed as pigmented type of lichen planus with angular cheilitis.
Figure 2: Intraoral picture showing hypo and hyperpigmentation presenting bilaterally on the buccal mucosa with a radiating lace-.like pattern with generalized macular blackish brown pigmentation

Click here to view


Patient was subjected to complete blood investigation, and an incisional biopsy was obtained. The histopathological diagnosis was increased melanotic activity. The patient was subjected to medical evaluation and laboratory tests to rule out any other systemic reasons for hyperpigmentation. Patient had a history of frequent nausea, vomiting and constipation with loss of appetite and muscular pain in the hands and legs since 1 year, and hence Addison's disease and Peutz–Jeghers syndrome were suspected. To rule out Addison's disease, the levels of ACTH, plasma cortisol, and serum electrolytes were estimated, which were under normal limits [Figure 1]. To rule out the underlying cause for Peutz–Jeghers syndrome, the patient was subjected to endoscopy, colonoscopy and enteroclysis. The result was negative for the same. The blood sample revealed decreased level of hemoglobin (6.5 g/dl), and the blood smear revealed normocytic hypochronic cells, suggesting iron-deficiency anaemia. Based on the clinical, biochemical, histologic findings, and gastrointestinal investigations, a final diagnosis of pigmentation due to iron-deficiency anemia was made. Patient was advised treatment for the same. With the reference of a general physician, the patient was advised dietary modifications with oral and parental iron supplements. Livogen Captabs/10 once a day was given for 6 months, and 1 ampule of intravenous injection of iron supplement with saline once a week for 1 month was given. The present case showed fading of oral pigmentation after 6 months of treatment, with no signs of recurrence after 1 year of follow-up.


   Discussion Top


The oral cavity is an important anatomical location with a role in many critical physiologic processes. Truly, the oral cavity is a mirror that reflects and unravels many of the human body's internal secrets. In the course of disease, the mucosal tissues can assume a variety of discolorations due to disease process with increased keratinization or in increased vascularization. Blue, brown and black discolorations constitute the pigmented lesions of the oral mucosa, and such color changes can be attributed to the deposition of either endogenous or exogenous pigments.[3],[4] Here, we report a rare case of pigmentation induced by iron-deficiency anemia, which is most common in females due to low inadequate dietary supplements and in their reproductive life.

Iron-deficiency anemia is mainly caused by inadequate dietary intake, faulty absorption and increased requirement for iron. Iron-deficiency anemia clinically present as cracks or fissure seen on the corners of the mouth, smooth and red painful tongue with atrophy of filiform papilla and fungiform papilla. The mucous membrane appears pale with glossitis and angular stomatitis is encountered in these patients along with lemon tinted pallor of the skin. Sometimes dysphagia is also a presenting feature.[5] Pigmentation in iron-deficiency anemia is because iron is intimately associated with melanin metabolism. The enzymes phenylalanine hydroxylase and homogentisic oxidase requires iron for their action on homogentisic acid and melanin quinines; because of iron deficiency, homogentisic acid accumulates, which in turn leads to excessive tyrosine formation followed by abnormal melanosis.[6] In the present case, pallor of skin and conjunctiva was observed. The generalized diffuse macular pigmentation on the palms and face with bronze color of nails of the fingers and toes were seen. This was consistent with the features of iron-deficiency anemia. However, on intraoral examination, a diffuse hypo and hyperpigmentation was seen bilaterally on the buccal mucosa, which resembled a lacelike pattern of Wickham's striae. They presented a symmetrical distribution, which was inconsistent with the features of iron-deficiency anemia. The clinical picture gives an impression of pigmented lichen planus, whereas other features such as cracks and fissures were noted in the right and left commissural area of lips with erythematous surface in the patient, which has also been seen in iron-deficiency anemia and is consistent with the present case.[6],[7]

In the presented case, the laboratory finding showed that there was decreased hemoglobin. Smear study was done for red blood cells (RBCs), white blood cells (WBCs), and platelets. The smear showed normocytic hypochronic RBCs with normal levels of WBCs and platelets, which is consistent with the earlier literature. The diagnosis of iron deficiency anemia not only requires low hemoglobin levels but also requires laboratory-confirmed evidence of low iron stores and low ferritin. The decreased levels of hemoglobin were correlated with decreased levels of low iron stores and low ferritin.[8]

For the present case, a differential diagnosis of pigmented lichen planus, vitamin B12 deficiency, Addison's disease, Peutz–Jeghers syndrome, and hemochromatosis was made. A provisional diagnosis of pigmented lichen planus was made because buccal mucosa showed diffuse areas of hypo and hyperpigmentation along with Wickham's striae that produced a lacelike pattern bilaterally distributed on the left and right buccal mucosa. Incisional biopsy was obtained that showed a flattened rete ridges and increased melanotic activity, whereas in oral lichen planus parakeratosis, hyperparakeratosis, acanthosis and “saw-tooth” rete ridges with juxtaepithelial band of inflammatory infiltrate are seen.[7] Thus, histopathologically, we ruled out the pigmented form of oral lichen planus.

The differential diagnosis of vitamin B12 was given as hyperpigmentation of the skin and mucous membrane was seen, which is consistent with our case; however, to confirm, complete blood count was done which revealed normal levels of vitamin B12.[9] Because the patient complained of weakness, nausea, vomiting, abdominal pain, constipation, weight loss, and hypotension with extraoral and intraoral hyper pigmentation, Addison's disease was suspected.[9],[10] However, blood endocrinology and serum electrolyte levels were within normal limits, ruling it out. Because the patient had diffuse mucocutaneous pigmentation with abdominal pain and weight loss, endoscopy, colonoscopy and enteroclysis were done to rule out  Peutz-Jeghers syndrome More Details. The results were inconclusive. Rare, but another differential diagnosis of hemochromatosis was given as there was diffuse pigmentation of skin and mucous membrane.[10] This was ruled out by blood ferritin level, which was low in the present case, whereas in hemochromatosis it is elevated (values).

According to literature, there are various treatment modalities for iron-deficiency anemia. The dosage for the iron therapy required to treat iron-deficiency anemia in adults is 120 mg per day for 3 months; the dosage for children is 3 mg per kg per day up to 60 mg per day. Intramuscular iron sorbitol (a complex of iron, sorbitol, and citric acid) injection was used as a parenteral iron replacement for many years, but was discontinued in the UK in 2003. In general, approximately 10–20 deep intramuscular injections were given over 2–3 weeks. However, side effects are common and included pain, skin staining at the site of injection, and arthralgia. Newer intravenous iron preparations such as iron hydroxide sucrose and iron dextran may also be given intramuscularly.[11],[12] The patient was advised treatment with the help of general physician, dietary modifications, oral and parental iron supplements (Livogen Cap tabs/10) once a day for 6 months, and 1 ampule of intravenous injection of iron supplement with saline once a week for 1 month) was given. The patient's condition improved, and fainting of hyperpigmentation of the skin and mucous membrane was observed after 6 months, with no signs of recurrence seen after 1 year.


   Conclusion Top


Though a rare presentation, hyperpigmentation due to iron-deficiency anemia does mimic other pigmented conditions, which can mislead the diagnosis and management.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.

 
   References Top

1.
Urse GN. Systemic disease manifestations in the oral cavity. Osteo Fam Phys 2014;3:16-21.  Back to cited text no. 1
    
2.
Levane P, Powell FC. Oral pigmentation. J Eur Acad Dermatol Venereol 2000;14:448-65.  Back to cited text no. 2
    
3.
Kauzman A1, Pavone M, Blanas N, Bradley G. Pigmented lesions of the oral cavity: Review, differential diagnosis, and case presentations. J Can Dent Assoc 2004;70:682-3.  Back to cited text no. 3
    
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Wadhawan R, Solanki G, Sabir S, Palekar S, Pareekh A. Oral manifestations of systemic diseases: A review. J Sci 2014;4:233-41.  Back to cited text no. 4
    
5.
Darby WJ. The oral manifestations of iron deficiency. J Am Med Assoc 1946;130:830-5.  Back to cited text no. 5
    
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Ghosh K. Non haematological effects of iron deficiency- A perspective. Indian J Med Sci 2006;60:30-7.  Back to cited text no. 6
[PUBMED]  Medknow Journal  
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Sugerman PB, Savage NW. Oral lichen planus: Causes, diagnosis and management. Aust Dent J 2002;47:290-29.  Back to cited text no. 7
    
8.
Bermejo F, García-López S. Review guide to diagnosis of iron deficiency and iron deficiency anemia in digestive diseases. World J Gastroenterol 2009;15:4638-43.  Back to cited text no. 8
    
9.
Agrawala RK, Sahoo SK, Choudhury AK, Mohanty BK, Baliarsinha AK. Pigmentation in vitamin B12 deficiency masquerading Addison's pigmentation: A rare presentation. Indian J Endocrinol Metab 2013;17:254-6.  Back to cited text no. 9
    
10.
Frantzis TG, Sheridan PJ, Reeve CM, Oral manifestations of hemochromatosis. Report of a case. Oral Surg Oral Med Oral Pathol 1972;33:186-90.  Back to cited text no. 10
    
11.
Pasricha SR, Flecknoe-Brown SC, Allen KJ, Gibson PR, McMahon LP, Olynyk JK, et al. Diagnosis and management of iron deficiency anemia: A clinical update. Med J Aust 2010;193:525-32.  Back to cited text no. 11
    
12.
Todd JC, Sanford AH, Davidsohn I, Henry JB. Clinical Diagnosis and Management by Laboratory Methods. 16th ed. Philadelphia, PA: WB Saunders Company; 1979.  Back to cited text no. 12
    


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