Clinical Guide to Oral Diseases. Crispian Scully
characteristics of capillary and cavernous hemangiomas?
1 Increased proliferation of endothelial cells
2 Calcified thrombus formation (phlebolith)
3 Fibrous septa
4 Dense accumulation of inflammatory cells within the submucosa
5 Cholesterol crystals
Answers:
1 Endothelial cell hyperplasia with or without lumen formation is characteristic of hemangiomas, especially at their proliferative phase. The endothelial cells are dense and form clusters or small vascular channels prominent at the proliferative phase (capillary hemangiomas) or they form large cystic dilated vessels with thin walls (cavernous type).
2 No
3 Fibrous septa separate the neoplastic vascular lumens and are more numerous and dense at the involuting phase of the hemangiomas.
4 No
5 No
Comments: Cholesterol crystals are associated with chronic vascular inflammation, as well as being commonly seen in atheromatous plaques and in the inflamed cystic wall of dental cysts, but not in hemangiomas. Inflammation of the fibrous hemangioma's stroma is rare and mainly consists of chronic inflammatory cells, mast cells and a few macrophages. Phleboliths are formed from clots within the lumen of cavernous hemangiomas only.
Q3 Which of the syndromes below is/or are associated with cavernous hemangiomas?
1 Sturge‐Weber syndrome
2 Maffucci syndrome
3 Blue rubber bleb nevus
4 PHACE syndrome
5 Klinefelter syndrome
Answers:
1 No
2 Maffucci syndrome is associated with multiple enchondromas, mainly on the bones of hands and feet as well as hemangiomas.
3 Blue rubber bleb nevus syndrome is characterized by multiple hemangiomas (cavernous in majority) in the skin and visceral organs.
4 PHACE syndrome is a cutaneous syndrome characterized by multiple congenital abnormalities of posterior fossa, hemangiomas, and other vascular abnormalities, cardiac, and eye defects, sternal clefts and supraumbilical raphe syndrome.
5 No
Comments: Klinefelter syndrome is characterized by a number of oral abnormalities apart from hemangiomas and occurs exclusively in males, while Sturge‐Weber syndrome is characterized by capillary lesions of leptomininges and facial skin (nevus flammeus) along the distribution of ophthalmic and maxillary divisions of the trigeminal nerve.
3 Brown Lesions
Brown lesions are commonly seen in the oral mucosa and characterized by accumulations of endogenous or exogenous pigments in the superficial subepithelial connective tissue. These lesions appear as punctuate (macular) or diffuse and have a variety of clinical courses and prognosis (Figure 3.0a and b). Some of them are innocent lesions like racial, physiological mucosal pigmentation or those induced by drugs, diseases like Addison's or syndromes like Peutz‐Jeghers, and others are dangerous like complex nevi or melanomas.
Table 3 shows the most common oral brown pigmented lesions.
Figure 3.0a Brown discoloration of neck skin after radiation.
Figure 3.0b Hydroxyurea‐induced oral brown pigmentation.
Table 3 The most common oral brown lesions.
| In brown oral lesions the pigmentation (exogenous or endogenous) is superficial, while in black or blue lesions is deep into the submucosa |
| Endogenous pigmentation |
| Related to melaninIncreased melanin production onlyRelated to: raceRacial pigmentosaHormone alterationsChloasmaAddison's diseaseEctopic ACTH productionNelson syndromeAcanthosis nigricansLaugier‐Hunziker syndromeLeopard syndromeSpotty pigmentation, myxoma, endocrine overactivity syndromeVon Recklinghausen's diseaseAlbright syndromeUse ofDrugsOral contraceptivesCytostaticsAntimicrobialsAntiarrythmicsFoodsSun exposureFrecklesSolar lentiginesSmokingBetel nut chewingSmoker's melanosisInflammation LP melachrosisBMMP melachrosisEM melachrosisInfections HIVSyndromes Peutz‐Jegher syndrome Abnormal distribution of melanin TumorsBasal cell carcinomasMelanoacanthomaIncreased number of melanocytes Lentigines simplexNeviMelanoma |
| Exogenous pigmentation |
| Foreign materialsTattoosAmalgamGraphiteTribalTarMedicationsLocalPotassium permanganate |
Case 3.1
CO: A 43‐year‐old woman was evaluated for multiple brown lesions scattered in her mouth.
HPC: The brown lesions were first noticed by her dentist 10 years ago, but had remained unchanged since then. The lesions were asymptomatic and did not cause any patient concern until two years ago when she lost her sister from lung cancer and started her menopause.
PMH: She suffered from carpal tunnel syndrome on her right hand, exacerbated due to her job as a secretary and allergic keratitis on both eyes relieved with steroid eye drops (in crisis). She used no other medications or other known allergies. She was a chronic smoker of 8–10 cigarettes daily, and an occasional drinker.
OE: A middle‐aged lady with dark complexion, with no other lesions on her mouth apart from a few pigmented brown lesions on her gingivae, buccal mucosae, floor of the mouth (Figure 3.1) and lips. The discoloration was more obvious on the gums and floor of the mouth. No other lesions were found on her skin or other mucosae. Blood pressure and hormone tests were within the normal range.
Q1 What is the cause of the discoloration?
1 Addison's disease
2 Racial pigmentation
3 Drug‐induced discoloration
4 Heavy metal poisoning
5 Melasma
Answers:
1 No
2 Racial pigmentation is a common condition among darker skinned individuals, and is presented as multifocal brown to black discoloration of gingivae (mainly) but also the tongue (dorsum and inferior) (