Category: Immune Response & Autoimmune Disorders

ANAESTHESIA – LOCAL AND GENERAL WITH BRAND NAMES AND COMPANY MANUFACTURER

DR .SAJAL SHARMALeave a comment

Anaesthetics – local & genral
….
1.Bupivacaine

Used as

👉 Percutaneous infiltration anaesthesia ,

👉 peripheral nerve block

👉Sympathetic nerve block
,
👉retrobulbar block ,

👉Cadual block

👉Lumbar epidural block

Brand names.

🙏Buloc by celon
Inj – 0.25 % & in 0.5 % ( 20ml )
.
🙏Bupivan by Sun pharma
Inj :- 0.25% (20ml)
0.5% ( 20ml )
0.5% ( 4ml )
.
🙏 Marcain by AHPL
Inj:- 0.5 % ( 20ml )
Inj :- 1 % ( 2ml )
.

  1. Halothane
    Inhalation anesthesia

👉 used in Induction & maintenance of general anaesthesia
.
🙏Fluothane by AhPL
I:vap :- 100% in ( 200 , 250 , 30, 50 ml ) soln
.
3.Isoflurane
Inhalation anaesthesia

👉 Induction & maintenance of general anaesthesia
.
🙏 Forane by abbott
Inhalant :- 100% in ( 100, 250 ml )

🙏Isorane by AhPL
I:sol :- 5mg/5ml in ( 100,250,30 ml )
.

  1. Ketamine
    Intramuscular
    & Intravenous anesthesia


.
,🙏Ketam by sun
Inj 10mg/ ml (10ml )
Inj 50mg / ml ( 2ml )
.
🙏Ketmin by Themis medicare
Inj 50mg /ml ( 10 ml )
Inj 50 mg/ ml ( 2ml )
.
🙏Ketsia by celon
Inj 100mg ( 2ml )
Inj 500mg ( 10ml )

Abbreviation
I sol :- inhalation solution
Ivap :- inhalation vapour

  1. Lidocaine ( used as )
    Epidural
    👉 as Epidural anesthesia
    Injection
    👉Pulp dilatation during phaco-emulsification cataract surgery
    Intraspinal
    👉Spinal anaesthesia
    as Intravenous
    👉Intravenous regional anaesthesia
    Parenteral
    👉 Sympathetic nerve block
    👉 Peripheral Nerve Block
    👉 Percutaneous infiltration anaesthesia
    Urethral
    👉Surface anesthesia
    Mouth / throat
    👉Surface anesthesia
    as for Opthalmic region
    👉Surface anesthesia
    Rectal & topical / cutaneous
    .
    Company names
    …
    …
    ..

🙏Gesican 2% gelly by AHPL ( 30ml )

🙏Lidopatch by zydus cadila
T:patch- 5%

🙏Xylocaine by AstraZeneca
T:sol:- 2% 100ml
Oint :- 5% w/w ( 20mg )
Jelly :- 2% w/w ( 30mg )
..

🙏Xylocard 2 % by AstraZeneca
Inj (21.3mg/ml ) 50ml soln
.

🙏 Xylocaine viscous by astra zeneca
T:sol :- 21.3mg/ml ( 100ml )

🙏 Xylocaine topical 4% by AstraZeneca
T:sol :- 42.7mg/ml ( 30ml )

🙏Nummet by icpa
Spy :- 15% w/w ( 100g )

.
Some Combinations
Lidocaine + epinephrine

🙏 Lignosafe by stedman
( Lignocaine hcl 21.3mg & adrenaline 0.0125mg/ml )
Inj in 30ml

🙏 Xylocaine with adrenaline 2% by AstraZeneca
( Lidocaine hcl 21.3mg , adrenaline 0.005mg , nacl 6mg /ml )
Inj 30ml
.

Some other combination
🙏 Xylocaine 5% heavy ( lignocaine hcl 53.3mg/ml , Dextrose 75mg ) inj in 2ml
.

& Xylocaine soln ( same dosage as above ) T:Sol 100ml by AstraZeneca

🙏 Xylocaine spray by AstraZeneca
( Lidocaine hcl 100mg , ethanol 28.29% ) 500ml
.
🙏Xicaine by icpa
( Lignocaine 2 percent , adrenaline 0.022mg) inj 30ml
&
( Lignocaine hcl 2% , adrenaline 0.009 mg ) inj 30ml
.
🙏Asthesia by unichem
( Lidocaine 2.5% w/w , prilocaine 2.5% )
CRM (15,30,5 )g
.
Abbreviation
Crm :- cream
Tsol :- topical solution

CYTOKINES

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Cytokines are soluble protiens/polypeptides, produces by a variety of hematopoetic and non hematopoetic cell types. They are responsible for the regulation of immunological, inflammatory and reparative host responses.

Cytokines tend to bind to high affinity receptors and the target cells and mediate their effects. Specific cytokines mediate specific reactions.

Reference: Arvind Arora

Cytokines basically mediate immune response. But some cytokines are also pro inflammatory.

These are:-

Il-1, TNF alpha and IL-6.

IL-1 is the most important pro inflammatory cytokine.

Anti inflammatory cytokines are involved in the resolution of inflammation. These include:

IL-4, IL-10 IL-13 and TGF beta.

IL-4 has mainly anti-inflammatory properties with some pro-inflammatory properties.

TGF-beta is the most important fibrogenic factor.

T- Cells

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T- cells are among the two important cells of the adaptive immune system (others are B- Cells). T-cells arise in the bone marrow and mature in the thymus.

T- cells constitute 60-70% of the circulating peripheral lymphocytes.

T- cells categories:-

1.) Helper or Inducer T-cells:

60% of T- cells are helper T- cells. They have CD4 surface marker and are MHC II restricted. Types of CD4 helper cells are:

A) Effector cells- TH1, TH2 and TH17 cells. The TH1 cells are activated by IFN- gamma and themselves produce IL-2, IFN-gamma and IL-12. They are the primary cells involved in delayed hypersensitivity, cell mediated immunity, macrophage activation and killing of intracellular microbes. TH2 cells are activated by IL4 and themselves produce IL4, IL5, IL6 and IL13. They produce all antibodies except for IgG2b and also provide defence against helminthic parasites. TH17 cells are powerful recruiters of neutrophils and monocytes to play a role in severe inflammatory diseases.

B) Memory cells- They retain the antigenic affinity of previously activated T-cells and are used in a second immune response.

2.) Cytotoxic T-cells:

30% of the total T-cells. They have CD8 surface marker and MHC I restricted. They kill amd lyse target cells which include tumour cells, virus infected cells and allograft.

THE RATIO OF CD4:CD8 T-cell is normally 2:1.

3.) Suppressor T-cells:

They have CD8 surface marker and are MHC I restricted. They are responsible for down regulation of the immune response.

T–CELL RECEPTOR (TCR)

TCR is a molecule found on the T-cell which is responsible for recognising the antigen bound to MHC molecule. It is a heterodomer made up of an alpha and a beta chain. The presence of TCR gene rearrangements demonstrated by molecular analysis is a marker of T-cell lineage.

REITER’S SYNDROME

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Reiter’s syndrome is associated with urethritis, balanitis, conjunctivitis, and mucocutaneous lesions.

 It is a disease of unknown aetiology, although there is evidence of an infec- tious origin.

It is one of the most common complications of non-specific urethritis and it clinically mimicks gonorrhoea, although the urethral discharge is negative for Neisseria.

CLINICAL FEATURES

>Reiter’s syndrome is more prevalent in young adult men, usually between 20 and 30 years of age. 

>The male-to-female ratio is 9:1. 

>There is a typical tetrad of manifestations: non- gonococcal urethritis, arthritis, conjunctivitis, and mucocutaneous lesions. 

>Urethritis may be the first sign. The urethral discharge is usually associated with itching and burning sensation. 

>The arthritis is often bilaterally symmetrical and usually polyarticular.

 >Conjunctivitis is often so mild as to be overlooked. 

>The skin lesions are similar to those seen in keratoderma blennorrhagica and consist of red or yellow keratotic macules or papules which eventually desquamate.

Oral Manifestations

Sites—it is seen on the buccal mucosa, lips and gingiva.

Oral lesions appear as painless, red, slightly elevated areas, some- times granular or even vesicular, with a white circinate border on the buccal mucosa, lips, and gingiva. 

The palatal lesions appear as small, bright red purpuric spots, which darken and coalesce, while the lesions on the tongue closely resemble ‘geographic’ tongue.

Laboratory Findings

The patients usually have a mild leukocytosis, an elevated erythrocyte sedimentation rate, and pyuria.

  • Differential Diagnosis
  • • Geographic tongue and stomatitis—no skin changes, no visceral lesions are seen.
  • • Pustularpsoriasis—Auspitz’ssignpresent.
    • Behcet’ssyndrome—nourethritis,aphthaewithredhalo.
  • • Stevens-Johnson syndrome—acute appearance, moresevere clinical course, no arthritis or urethritis.
    • Benign mucosal pemphigoid—blister formation, nourethritis, found in older patients.
Histologic Features
The microscopic findings are not diagnostic. They consist of parakeratosis, acanthosis, and polymorphonuclear leukocyte infiltration of epithelium, sometimes with mi- croabscess formation similar to psoriasis. The connective tissue shows a lymphocyte and plasma cell infiltrate.
  • Management
  • • Spontaneous remission—many patients undergo spontaneous remission.• Antibiotics—incasesymptomaticpatient,doxycycline or minocycline may be given.
  • • Analgesics—nonsteroidalanti-inflammatorydrugsare given to manage arthritis.
  • • Immunosuppressiveagents—immunosuppressiveagents like azathioprine and methotrexate are given in cases of most resistant cases.

REFERENCE- SHAFER’S TEXTBOOK OF ORAL PATHOLOGY [8TH ED} AND ANIL GHOM TEXTBOOK OF ORAL MEDICINE

WEGNER’S GRANULOMATOSIS

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Wegener’s granulomatosis is a disease of unknown aetiology, which basically involves the vascular, renal and respiratory systems. It involves the nose, paranasal air sinuses, lower respiratory tract, gut, joints, nervous system, and kidneys. Involvement of the kidney is the common cause of death.

This disease is caused by an abnormal immune reaction secondary to a nonspecific infection or a hypersensitivity reaction to an unknown antigen.

Clinical Features

>Wegener’s granulomatosis may occur at any age, although the majority of cases are in the fourth and fifth decadesof life.

 >There is a slight predilection for occurrence in males.

 >It is best described as a multisystem disease, which is usually first characterized clinically by the development of rhinitis, sinusitis, and otitis or ocular symptoms.

 >The patient soon develops a cough and hemoptysis as well as fever and joint pain. 

>Hemorrhagic or vesicular skin lesions are also commonly present. 

>Granulomatous lesions of the lungs are found on the chest radiograph, while the glomerulonephritis, which develops ultimately, leads to uraemia and terminal renal failure.

 >In nervous system, sensory neuropathy may be an occasional finding.

>Prognosis—the disease is usually fatal, with mean survival time of 5 months. Death occurs due to involvement of kidney.

Oral Manifestations

Involvement of the gingiva has been the most common and characteristic manifestation, and is termed as strawberry gingivitis.Gingival lesions may manifest as ulcerations, friable granular lesions, or simply enlargements of the gingiva. 

The inflammatory process starts in the interdental papilla and spreads rapidly to the periodontal structure and leads to bone loss and tooth mobility. 

Palate—orallesionstypicallyincludeulcerationofthe palate by extension of nose lesions and destruction of nasal septum. This will lead to perforation of palate.

• Teeth—theremaybelooseningofteethwithinsomecases spontaneous exfoliation. After extraction of teeth patient is usually noticed poor healing.

Laboratory Findings

Laboratory findings include anaemia, leukocytosis, elevated erythrocyte sedimentation rate, and hyperglobulinaemia. Because of kidney involvement, haematuria is common, as well as the finding of albumin, casts, and leukocytes in the urine. Circulating immune complexes have been demonstrated in some patients, but this is not a consistent finding.

Histologic Findings

Wegener’s granulomatosis presents a pattern of mixed inflammation centred around the blood vessels. 

The lesions in the upper respiratory tract and lungs consist of giant cell necrotizing granulomatous lesions showing vasculitis. 

Oral biopsy specimens show pseudoepitheliomatous hyperplasia and subepithelial abscesses. The gingival and other lesions show a nonspecific granulomatous process with scattered giant cells.

Diagnosis

  • Clinical diagnosis—typical strawberry gingivitis with necrotic ulceration in the oral cavity.
  • Laboratorydiagnosis—cytoplasmiclocalizationispresent with Wegener’s granulomatosis. Histopathologically chronic inflammatory cells and multinucleated giant cells are found.
  • Differential Diagnosis
  • Agranulocytosis, leukemia, lymphoma—diagnosis by blood picture, possibly histology.
  1. Management
    • Cotrimoxazole—it is combination of trimethoprim and sulfamethoxazole. It has proved to be effective as an adju- vant or sole therapy in both localized and generalized forms.
    • Corticosteroids—regimenofcyclophosphamide12mg/ kg body weight/day with prednisolone 1 mg/kg body weight have been utilized to obtain complete remission.
    • Others—other treatment modalities includes cyclo- sporine, intravenous pooled immunoglobulin, and local irradiation.

REFERENCE- SHAFER’S TEXTBOOK OF ORAL PATHOLOGY {8TH ED} AND ANIL GHOM TEXTBOOK OF ORAL MEDICINE

Systemic lupus erythematosus{SLE}

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  • SLE is a multisystem autoimmune inflammatory disorder of unknown etiology.
  • Main feature is the formation of antibodies to DNA, which may initiate immune complex reactions, in particular a vasculitis. 
  • Female to male ratio of 9:1
  • More common in persons of non-European descent.
  • Etiology
  • Geneticpredisposition—relativeofpatientshavehigher incidences of auto-antibodies, immune deficiency and connective tissue disease. This tendency is greatest among identical twins.
    • Immunological abnormality possibly mediated by viral infection—immune complex consisting chiefly of nucleic acid and antibody account for majority of the tissue changes.
    • Autoimmune disease—as these patients develop antibodies to many of their own cells.
    • Endocrine—thereishighincidenceinfemalesinpreg- nancy. This finding suggestive of increased estrogen level.
    • Biochemicalincreaseinexcretionofmetabolicproducts, particularly tyrosine and phenylalanine, in certain SLEpatient.
  • CLINICAL MANIFESTATIONS
  • Lupus is known as “the great mimic.”
  •  Skin lesions of lupus can be classified 
    • lupus-specific (having diagnostic clinical or histopathologic features) 
    • nonspecific lesions.
  • Three subtypes of lupus-specific 
    • Acute
    • subacute 
    • chronic. 
  • Acute cutaneous lupus occurs in 30 to 50% of patients and is classically represented by the butterfly rash-mask-shaped erythematous eruption involving the malar areas and bridge of the nose
  • Chronic cutaneous lupus occurs in 15 to 20% of cases and affects the skin of the face or scalp in about 80% of cases.
  • The least common subtype, subacute cutaneous lupus, occurs in 10 to 15% of patients and includes papulo­squamous (psoriasiform) and annular-polycystic eruptions, usually on the trunk and arms.
  • Nonspecific but suggestive skin manifestations of lupus are common and include 
    • alopecia (both scarring following discoid lesions and non-scarring)
    • Photosensitivity
    • Raynaud’s phenomenon
    • Urticaria
    • Erythema
    • Telangiectases
    • cutaneous vasculitis.

  • ORAL MANIFESTATIONS
  • Two predominant types of oral lesions are
    •  discoid lesions 
    • ulcerations.
  • Oral ulcerations associated with SLE  they occur with increased frequency on the palate and in the oropharynx and are characteristically painless.
  • Histologically, they are characterized by lymphocytic infiltrate at the base of the ulcer and in the perivascular distribution, which is similar to that observed in discoid lesions.
  • Discoid oral lesions, appear as whitish striae frequently radiating from the central erythematous area, giving a so-called “brush border.”
  • Buccal mucosa, gingiva, and labial mucosa are the most commonly affected intraoral sites.
  • Direct immunofluorescent staining for immunoglobulins and complement C3 factor is a useful aid to diagnosis. Granular deposition of IgM, IgG, and C3 along the basement membrane is characteristic

Diagnosis

• Clinical diagnosis—skin lesion with lesion present on oral mucosa which is atrophic and erythematous will suspect lupus erythematous. Oral and nasopharyngeal ulceration is major diagnostic criteria for SLE.

Laboratory diagnosis—L.E. cell inclusion phenomenon with surrounding pale nuclear mass apparently devoid of lymphocytes. Anemia, leukopenia and thrombocyto- penia, with sedimentation rate increased. Serum gamma globulin increased and Coomb’s test is positive.

Positive lupus band test—it shows deposition of IgG,IgM or complement component in skin.

  1. Differential Diagnosis
    • Lichenplanus—homogenouspicture,nodarkerythema and no telangiectasia. Mucosal changes are usually extensive and symmetrical.
    • Lichenoidreaction—historyofdrugisalwaysthere.
    • Ectopic geographic tongue—systemic manifestation present is lupus erythematous, which is absent in ectopicgeographic tongue.
    • Psoriasis—Auspitz’s’signispositive.
    • Electrogalvanic lesion—dissimilar restorations are seenin oral cavity.
    • Leukoplakiaanderythroplakia—lesionstendtomaintainsame appearance and there are no skin changes.
    • Geographic stomatitis—no skin changes, mucosal lesionschange location rapidly.
    • Benign mucous membrane pemphigoid—no systemiccomplain and serology test to be done.
  • TREATMENT
  • Corticosteriods are the cornerstone of therapy
  • A pulse i.v cyclophosphamide regimen for remission induction followed by quarterly infusions
  • Recently, mycophenolate mofetil and azathioprine
  • NSAIDs for arthritis relief
  • Antimalarial like hydroxychloroquinine – effective in cutaneous lupus 
  • DENTAL MANAGEMENT
  • Recommended prophylactic antibiotics if ANC count falls below 500 – 1000 cells/mm3
  • Adrenal supression –
  • Adenocorticotropic hormone supression test is used to evalute
  • Current guidelines – Replacement therapy with hydrocortisone is unnecessary

REFERENCE- ANIL GHOM TEXTBOOK OF ORAL MEDICINE; BURKIT TEXTBOOK OF ORAL MEDICINE AND GOOGLE[SLIDE SHARE]

COVID-19 (PART -2)

Dr.Urusa InamdarLeave a comment

Written by : Dr. Urusa I Inamdar

Using mask and sanitizer

  • Person not showing any symptoms :

A medical mask should not be used as it creates a false sense of security that can lead to neglecting other essential measures . Resort to other measures like – washing hands , cover your nose and mouth while sneezing , monitor your body temperature and oxygen levels .

  • So when should you use medical mask ( apart from medical health care workers ) :

When a person develops symptoms like cough or fever , while visiting a health care facility .

Taking care of an ill person .

If your family member is a suspected or confirmed case undergoing home care .

ENSURE PROPER FIT OF MASK
  • Washing your hands :

Washing your hands frequently is essential . Use soap and water for atleast 20 seconds for most effective results .

  • Sanitizing your hands :

Hand sanitizers are to be used , when hand wash was not possible . An alcohol based sanitizer with 70% alcohol content must be used for 20 seconds .

  • REFERENCES:
  1. Self notes
  2. World health organization
  3. Google.com
  4. ayush.gov.in
  5. IGOT ( Integrated Government Online Training )
  6. AIMS , New Delhi

BULLOUS PEMPHIGOID

Dr.Mehnaz1 Comment

A chronic, autoimmune, sub-epidermal blistering skin disease that rarely involves mucous membrane.

🔹Clinical Features:

Age: Elderly (>60 years)

Skin Lesions:

  • Generalised non-specific rash, commonly on Limbs.
  • Appears urticarial/eczematous; persist for several weeks to months.
  • Vesicles & bullae arise in prodromal skin lesion as well as Normal skin.
  • The blisters are thick walled and don’t rupture easily.
  • ruptured blisters are usually sensitive and painful, have raw eroded area which heals rapidly.

Oral Manifestations:

Vesicles appear gingivally👇🏻

Erythematous & desquamate as result of minor frictional trauma

👉🏻 Oral lesions comprise of bullae/vesicle that rupture to form erosions and ultimately leave out ulcerations

👉🏻 Other sites:

  • Buccal Mucosa
  • Tongue
  • Floor of the mouth
  • Palate

🔹Diagnosis:

Apart from evaluating history, clinical presentation, histopathological analysis is carried out followed by direct immunofluorescence study for the differential diagnosis and confirmation of the condition.

👉🏻Histopathology:

  • Acanthotic mucosa
  • Subepidermal non-specific vesicles with fibrous exudate

👉🏻Direct immunofluorescence is found to be the gold standard test. Deposition pattern of different types of immunoreactants differentiates the various immune-mediated diseases. Direct immunofluorescence shows presence of IgG and C3 deposits along the basement membrane zone.

©️jiaomr.in
👉🏻Electron Microscopy: In bullous pemphigoid (BP), the 180 kD antigen (BPAG2) was shown by immuno-EM to be a transmembrane molecule and to possess an autoantibody binding site outside the cell, suggesting a major pathogenic role for the BPAG2 in blister formation.

🔹Differential Diagnosis:

• Mucous membrane pemphigoid can be differentiated from BP by its predominant involvement of mucosal surfaces and positive Nikolsky’s sign.

• Lichen planus pemphigoides is clinically differentiated by the presence of lichen planus lesions in addition to tense blisters.

• Nikolsky’s sign is present in case of pemphigus and cicatricial pemphigoid, but not in the case of BP.

🔹 Treatment:

👉🏻Treatment is based on the degree of cutaneous and oral involvement. Mostly, topical steroid (clobetasol propionate) gives satisfactory result in case of smaller area of skin involvement, whereas larger area of skin involvement and recurrent cases are treated satisfactorily with systemic steroids and immunosuppressive agents.

👉🏻Recommended dosage for oral prednisolone is 0.3–1.25 mg/kg body weight/day, controls disease within 1–2 weeks, followed by which the dose is tapered. Dexamethasone (100 mg in 500 mL 5% dextrose i.v. over 2–3 h for three consecutive days) is the preferred steroid for pulse therapy, either administered alone or in combination with cyclophosphamide.

Other drugs for treating BP include new antibody modulators, rituximab 375mg/m2weekly over 4 weeks and omalizumab subcutaneously 300–375 mg for every 6 weeks.

👉🏻Higher doses of systemic corticosteroids seem to be associated with higher mortality rates, which led to the addition of corticosteroid-sparing agents to the treatment of BP. The most frequently used immunosuppressive agent is azathioprine (0.5–2.5 mg/kg body weight/day). Others being cyclophosphamide, methotrexate, cyclosporine A, combination tetracycline/minocycline along with nicotinamide, and more recently, mycophenolate mofetil, a DNA synthesis inhibitor, and methotrexate, a folate antagonist.

👉🏻IVIg – A dose of 1–2 g/kg for five consecutive day cycle of 0.4 g/kg/day, although a 3-day cycle may be used in cases that are nonresponsive to conventional therapy.

Dr. Mehnaz Memon🖊

References:

  1. http://www.jiaomr.in/article.asp?issn=0972-1363;year=2018;volume=30;issue=4;spage=432;epage=435;aulast=Aparna
  2. https://www.cidjournal.com/article/S0738-081X(00)00178-4/abstract
  3. Shafer’s Textbook of Oral Pathology, 7th edition
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Papillon–Lefèvre syndrome

muhadnoorman2 Comments

Muhad Noorman P – Dentowesome 2020

Papillon–Lefèvre syndrome (PLS) is a rare autosomal recessive disorder, characterized by diffuse palmoplantar keratoderma (hyperkeratosis) and precocious aggressive periodontitis, leading to premature loss of deciduous and permanent dentition at a very young age. Various etiopathogenic factors are associated with the syndrome, like immunologic alterations, genetic mutations, and the role of bacteria. Dentists play a significant role in the diagnosis and management of PLS as there are characteristic manifestations like periodontal destruction at an early age and an early eruption of permanent teeth. Here, we are presenting an elaborate review of PLS, its etiopathogenesis, clinical presentation, and management options.

Genetic studies of patients with Papillon-Lefèvre syndrome have mapped the major gene locus to chromosome 11q14-q21 and revealed mutation and loss of function of the cathepsin C gene. This gene is important in the structural growth and development of the skin and is critical for appropriate immune response of myeloid and lymphoid cells. Researchers believe that the loss of appropriate function of the cathepsin C gene results in an altered immune response to infection. In addition, the altered gene may affect the integrity of the junctional epithelium surrounding the tooth.

A closely related disease, Haim-Munk syndrome, also exhibits palmoplantar keratosis, progressive periodontal disease, recurrent skin infections, and several skeletal malformations. In this syndrome, the skin manifestations are more severe and the periodontal disease is milder. Studies have demonstrated that Haim-Munk syndrome and many examples of prepubertal periodontitis also exhibit mutation of the cathepsin C gene and represent allelic variants of the mutated gene responsible for Papillon-Lefèvre syndrome.

References: Shafer’s Textbook of Oral 9th edition

Photo credits: Dr Karthikeyan,PG final year Periodontics,Saveetha dental College, Chennai