EPIDERMOLYSIS BULLOSA
® It is an uncommon group of dermatologic diseases that may have familial
predHection.
Types
A- Acquired B- Genetic - inherited
• ·Dystrophic (oral features are common)
• Junctional (life threatening disease)
• Simplex
Non hereditary ................................ .. Hereditary
Pathogenesis:
• Linear IgG deposits in the basement The inherent defect is located in:
membrane & binds to coHagen - Anchoring CT filament
located below the level of - Hemidesmosome
lamina densa. - Basal cells
• Circulating antibodies are The circulating antibodies are not
involved in the pathogenesis involved in the pathogenesis of the
of the disease. disease.
Age of onset:
Adults Infants - children
lSkin: bullae develop on minor trauma & healing maby associated by scarring
Simplex:
intraepithelial blisters
improved at puberty
no oral abnormalities
(autosomal dominant)
Junctional:
subepithelial blisters- generalized
death is common in infancy
(autosomal recessive)
D!'stroplric:
subepithelal blisters ~ scarring
(autosomal dominat)
Orai manifesftall:iimm: Oral manifestation
uncommon * common & severe in dystrophic & junctional form
oral buUeae , ulceration and * oral ulcer heal by scar which induce;
scarring ~ restriction of mouth opening
~ restriction of tongue movement
~ obliteration of oral vestibule.
;~ Rudimentary or congenitally absent teeth.
* Severe disturbance of enamel & dentin
fon~Jation.
Treatment:
Avoid trauma ,steroids, cytotoxic drugs & Dapsone may help . . ,
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DIAGNOSIS OF PATIENTS WITH BLEEDING DISORDERS
An adequate history is the single most important part of the evaluation of patients with abnormal
bleeding tendency, clinical examination is also necessary, but the hematological tests are needed
to confinn the diagnosis.
1.History
Any suggestioB of a hemorrhagic tendency must be taken seriously.
The history should include.
:n.. Bleeding problem h"D uelatives: may reveal hereditary disease as hemophilias.
1. Bleedb~g puoblem following ope_ration (e.g. tonsilectomy) or after dental extractioo.
Pervious dental extraction provide~ ~~:fu!_gtiide, but prolonged bleeding (1-2 days) as 2llL
isolated episode is usuaUy the result oflocal factors, which are the most common cause o-:
excessive bleeding.
3. Bleedirag puoMem following trauma : Small cuts in patient with coagulation disorders
may not cause excessive immediate bleeding. Bleeding is usually delayed due 'cc
vasoconstriction, extra vascular pressure and platelets plugging proceed nonnaHy.
However, small cuts in patients with
--
platelet or vascular
- --
deficiencies usually resuH ir::
----------------
---~--~~--------
immediate excessive bleeding.
4.1l1edicatim'J e!ui.t !liJUEY cause bleeding problems:
m. Anticoagulants e.g. Heparin and coumarin. Heparin prevents thrombin formation 2.!:'1C
in large dose inhibits platelets aggregation. Cumarin: It antagonizes vit K, it depresse::
dotting factors H, VII, IX and X in the liver.
'ibl. History of Aspirin or Aspiring - containing drugs: the dose and duration shouJd b~
determined.
c. History of recent ]prolonged administration of broad spectrum antibiotic therapy whid:
may disturb ~he intestinal flora and interferes with vitamin K synthesis
tdl. Cytotoxic drugs
5. Presem:e @j driserf!Jse that may have associated bleeding problems:
o Biliary tract obstruction
o Malabsorption
o Leukemia
o Parenchymal liver disease
o Hemophilia
o Congenital heart disease,cogestive heart failiure.
o Cancer, radiation therapy, anticancer chemotherapy.
Hi7-
6oHi:5torry spontaneous bleeding from nose, mouth, gingiva, ears, urinary
tract, G.I.T., rectal, pulmonary or vaginal.
7JH.ilstmry of abundant menstrual bleeding or menses lionger trrmaliTl 7 mays
§Jtnstory of excessive bleeding during delivaryo
9 onstory of easy bryjsing
11t History of prior blood transfusion
110 Cliinicall Examination
o The skin and mucous membrances should be examined for
petechia, ecchymosis, hematoma, angiomas and jaundice.
o The lymph node sholJ,ld be examined and the mobility ofthe joint
should be obsereveCi.
o Platelets and vascular defect give rise to petechiae and
ecchymosis on the skin and mucous membranes
o Physical exam rarely useful except for petechiae or severe hemophiliac arthropathy
JIJI.IL Screeing Laboratory Tests:
One or more or all the following laboratory test should be ordered to confinn the diagnosis of the
bleeding disorders:
1. Hemgram
RBC/cu.mm
Hemoglobin %
Total & defferential WBC count.
Platelets count.
2.Prothrombin time.
3. Thrombin time.
4.Partial thromboplastin time.
S.Tourniquet test.
6.Bleeding time.
7 .Determination of the level of clotting factors when indicated.
IlVo Surgical Procedures:
excessive bleeding following surgery may be the first clue of underlying bleeding problem.
1€i8-
Serum iron
- Transferrin saturation= ---------------------------------,=less than 10 % i.e. the serum
Total iron binding capacity
iron carrier,transferrin is< than 10 %,
The ratio in normal individuals is approximately 35 %.
Treatment:
The cause of iron deficiency should be identified and eliminated.
Ferrous sulphate tablets (600 mg daily contain 120 mg ferrous iron) best
absorbed when the patient is fasting. If nausea, diarrhea or constipation occur, advice
the patient to : ·
a. have the tablet with food.
lh. use other drug with low dose of iron e.g. ferrous gluconate (600 mg daily
contain 70 mg ferrous iron).
Parentral iron has no advantage and is useful mainly when inflammatory bowel
disease i.s aggravated by oral iron. Iron stores are replaced quickly with parentral iron
but hematologicafrespOii'iSe-is not quicker. Oral iron may be given for 3 months or more
to replenish marrow i.ron stores. The response to iron therapy can the monitored by
increasing reticulocytic count and Hb rises 1 gr/week.
Dental implication:
1. CBC with differential should be ordered for anemic patient.
2. Avoid surgical procedures in severly anemic state because ofi.ncreased
bleeding & impaired wound healing. If hemoglobin below 10 g/dl, the
low oxygen tension affect rheologic interaction between cellular
components of blood, mainly platelets & endothelium, decreasing their
ability to dot effectively.
3. Avoid G.A ifHb < lOg/d~
~ 1P1LUMMER-VINSON SYNDROME
11
1 lPA'JI'TERSON-KELLEY SYNDROME
P]ummer-Vinson syndrome is one manifestation of iron deficiency anemia
Plummer-Vinson syndrrome is characterized bv:
1) Smooth. ;ted painful tongue with atrophy of filliform and later by the fungifonn
papillae.
2) Atrophy of the mucosa of the mouth, pharynx and upper esophagus.
3) Angular cheilitis ,c&'1aidal infection,oral ulceration .
. 4) Dysphagia or feehng of food sticking in the throat. ,_. - ~.:c~
L "-· . D~ia is dlue to muscular degeneration in the esophagus and stenosis or webs of
/ the esophageal mucosa.
The depletion of iron stores in the body may be the direct cause of mucosal
atrophy, since the integrity of the epithelium is dependent upon adequate serum iron
leveL Patients with these syndrome should be followed up-closely- for any oral or
pharyngeal changes that may be early indicat()! <:>f~~inoma.
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