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LETTER TO THE EDITOR
A Proposed Classification to
Identify the Oral Manifestations
of Genodermatoses
Sir,
Dermatology, being the specialized study of skin
diseases, comprises of an important subdivision of the
practice of medicine not only because of the various
primary diseases affecting the skin, but also because of
the common cutaneous manifestations of deeper
visceral or systemic diseases.1
There are several dermatologic disorders that have a
genetic etiology or a genetic predisposition. Such
genetically determined skin disorders are termed as
“genodermatoses” (geno: genetic + dermatoses: skin
lesions).2
A genodermatoses can be defined as “a cutaneous
phenotype caused by a single mutation, which may be a
point mutation, deletion or a chromosomal aberration”.3
The epidermis of the skin and the amelodentinal (the
enamel and dentine) components of the teeth are
derived from a common embryologic neural origin of the
ectoderm. As a result, there exist many primary
cutaneous diseases which find their manifestations in
the oral cavity affecting the oral mucosa and dentition.4
Therefore, it is of utmost importance for a dentist to
recognize that not only some dermatoses exhibit
concomitant lesions of the oral mucous membranes but
also manifestations of some diseases may be preceded
by oral lesions.
There are a plethora of genodermatoses with prominent
and characteristic manifestations in both hard and soft
tissues of the oral cavity. These genodermatoses
have been well identified and classified. Although
oral manifestations of genodermatoses have been
mentioned by various authors, a comprehensive
classification is required to segregate the manifestations
of genodermatoses in oral cavity.4,5 This can enable
dentists and dermatologists or any clinician, to identify
the underlying genetic dermatopathy.
We hereby, propose a classification for oral manifestations of genodermatoses. We have attempted to
accrue various genodermatoses in one classification
suggesting their manifestation in hard and soft tissues of
the oral cavity.6,7 We have also included the diseases
with multiple manifestations in both teeth and oral
mucosa.
Through this classification, we bring to light the various
genodermatoses with oral manifestations which usually
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go otherwise unnoticed. Therefore, we are identifying
and shedding light on the significance of oral
genodermatoses which can aid in early diagnosis of the
disease.
Proposed classification of oral genodermatoses
1. Genodermatoses affecting teeth and dentition
• Ichthyosis
• Sjogren-Larrson syndrome
• Incontinentia pigmenti
• Ehlers Danlos syndrome
• Focal dermal hypoplasia syndrome
• Gardner syndrome
• Ectodermal dysplasia
• Hyperimmunoglobulin E syndrome (Job syndrome)
2. Genodermatoses affecting periodontium and gingiva
•Ichthyosis
• Sjogren-Larrson syndrome
• Papillon Lefevre syndrome
• Tuberous sclerosis
• Chediak-Higashi syndrome
• Ehlers Danlos syndrome
• Focal dermal hypoplasia syndrome
3. Genodermatoses affecting oral mucosa
• Darier's disease
• Neurofibromatosis type 1 and 2
• Chediak-Higashi syndrome
• Ehlers Danlos syndrome
• Lipid proteinosis
• Focal dermal hypoplasia syndrome
• Multiple hamartoma syndrome (Cowden syndrome)
• Pachonychia congenita
• Epidermolysis bullosa
• Multiple endocrine neoplasia syndrome
• White sponge nevus
4. Genodermatoses affecting jaw bones and facies
• Mccune-Albright syndrome
• Ehlers Danlos syndrome
• Marfan syndrome
• Focal dermal hypoplasia syndrome
• Gardner syndrome
• Basal cell nevus syndrome
• Orofacial digital syndrome type I
5. Genodermatoses causing pigmentation of oral mucosa
• Carney complex
• Neurofibromatosis type 1 and 2
• Mccune-Albright syndrome
• Lipid proteinosis
• Pseudoxanthoma elasticum
• Peutz-Jeghers syndrome
• Congenital erythropoetic porphyria
• Hypomelanosis of ito
• Sturge-Weber syndrome
• Hereditary hemorrhagic telengiectasia syndrome
6. Genodermatoses with malignant potential
• Xeroderma pigmentosum
• Dyskeratosis congenita
Journal of the College of Physicians and Surgeons Pakistan 2016, Vol. 26 (7): 636-637
Letter to the editor
REFERENCES
1. Rajendran R. Diseases of the skin. Shafer's textbook of oral
pathology. 7th edition. New Delhi: Elsevier; 2012. p.807-44.
2. Rimoin DL, Connor JM, Pyeritz RE. Emery and Rimoin's
principles and practices of medical genetics. 3rd edition. New
York: Churchill Livingstone; 1996. p.1383-5.
3. Kumar S, Sehgal VN, Sharma RC. Common genodermatoses.
Int J Dermatol 1996; 35:685-94.
7. Spitz JL. Genodermatoses: A clinical guide to genetic skin
disorders. 2nd edition. Lipincott Williams & Wilkins; 2005.
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4. Freiman A, Borsuk D, Barankin B, Sperber GH, Krafchik B.
Dental manifestations of dermatologic conditions. J Am Acad
Dermatol 2009; 60:289-98.
5. Hand JL, Rogers RS. 3rd edition Oral manifestations of
genodermatoses. Dermatol Clin 2003; 21:183-94.
6. Bork K, Hoede N, Karting G. Diseases of the oral mucosa and
the lips. Saunders. p258-93.
Journal of the College of Physicians and Surgeons Pakistan 2016, Vol. 26 (7): 636-637
Manika Arora1 and Deepa Mane2
Department of Oral and Maxillofacial Pathology, NIMS Dental
College and Hospital, NIMS University, Shobha Nagar,
Jaipur-Delhi Highway, Jaipur-303121, Rajasthan, India.
Department of Oral Pathology and Microbiology, KLEVK
Institute of Dental Sciences and Hospital, Belgaum, Karnataka,
India.
Correspondence: Dr. Manika Arora, Senior Lecturer,
Department of Oral and Maxillofacial Pathology, NIMS Dental
College and Hospital, NIMS University, Shobha Nagar,
Jaipur-Delhi Highway, NH-11C, Jaipur 303121, Rajasthan,
India.
E-mail: [email protected]
Received: January 14, 2016; Accepted: February 25, 2016.
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