Page 140 - The Netter Collection of Medical Illustrations - Integumentary System_ Volume 4 ( PDFDrive )

Plate 4-55 Integumentary System

ORAL MANIFESTATIONS Thrombocytopenic purpura, diffuse bleeding
IN BLOOD DYSCRASIAS

Many systemic hematological diseases have cutaneous
findings as well as oral mucosal findings that are unique
and can be the presenting clinical sign of the underlying
disease. Awareness of the oral manifestations of these
disorders is of paramount importance. Oral manifesta-
tions of blood dyscrasias can be seen in agranulocytosis,
pernicious anemia, leukemia, polycythemia vera, and
thrombotic thrombocytopenic purpura (TTP).
Clinical Findings: Agranulocytosis has been shown
to produce oral ulcerations and erosions. Many differ-
ent causes of agranulocytosis may result in these clinical
findings. Medication-induced agranulocytosis is the
most frequent cause of a decreased absolute neutrophil
count of less than 500/µL. Many medications can cause
this reaction, including dapsone, methotrexate, and a
host of chemotherapeutic agents. A rare autosomal
recessively inherited disease called infantile genetic
agranulocytosis or Kostmann disease has been described. Leukemia (chronic), gingival infiltration
These patients present in the first months of life with
recurrent oral ulcerations, multiple bacterial infections,
and severely depressed absolute neutrophil counts.
Death is the norm by 1 year of age unless the disease is
correctly diagnosed and treated. Successful therapy is
achieved with granulocyte colony-stimulating factor
(G-CSF) or, in more advanced cases, with bone marrow
transplantation. Even with successful G-CSF treat-
ment, patients still develop oral ulcerations and severe
periodontal disease. This is caused by the lack of an
antimicrobial peptide, which allows certain bacteria to
proliferate unabated. The species most frequently
found is Actinobacillus actinomycetes comitans.
Pernicious anemia is caused by a deficiency of vitamin
B 12 . This deficiency is most commonly seen in individu-
als with an inability to absorb vitamin B 12 or in strict
vegetarians. Pernicious anemia can manifest with a
macrocytic anemia and neurological complications. Agranulocytosis, multiple oral ulcers
Hunter’s glossitis is a form of atrophic glossitis that
affects the tongue, leading to atrophy of the tongue
filiform and fungiform papillae. The tongue takes on a
beefy red appearance with a smooth surface. Varying
amounts of glossodynia and decreased ability to taste
are also found.
Gingival infiltration with leukemic cells may be the
presenting sign of an acute leukemia, and gingival Pernicious anemia, smooth red tongue
bleeding is the most frequent oral manifestation of leu-
kemia. Oral ulcerations are commonly associated with
the gingival leukemic hypertrophy. The gums appear
red and swollen, with varying degrees of gingivitis. The
gums may grossly enlarge to cover the majority of the
teeth. This form of leukemic infiltration is seen almost
exclusively in acute myelomonocytic leukemia (M4) and
acute monocytic leukemia (M5). It is estimated to occur
in two thirds of patients with M5 disease and in 20% Polycythemia vera, beefy red tongue
of those with M4 disease. Other forms of leukemia have
been implicated in causing gingival enlargement to a
much lesser degree.
Polycythemia vera (previously termed polycythemia mucosa. The tongue may become slightly enlarged, caused by a hereditary defect in the ADAMTS13 gene
rubra vera) is caused by excessive production of red smooth, and hyperemic. Bleeding from the gingival or by decreased platelet levels caused by medications or
blood cells, which results in abnormally high hemoglo- mucosa can also be seen. The disease is manifested by autoimmunity. ADAMTS13 is a gene that encodes a
bin and hematocrit values. The majority of cases are many other systemic signs and symptoms. plasma metalloprotease, which is important in regulat-
complicated by thrombosis. Most of these patients have TTP is a rare, life-threatening disease that can ing von Willebrand factor function. Oral manifesta-
a mutation in the JAK2 gene, which encodes a Janus develop rapidly. It is manifested by the formation of tions of the disease include widespread petechiae and
family tyrosine kinase protein. The ability to test for microthrombi throughout the small vasculature. This ecchymosis of the tongue, gingival, labial, and buccal
these mutations has made diagnosis much easier. Oral causes multisystem organ failure and rapid death if not mucosa. Petechial hemorrhages of the gums may appear
manifestations are limited to the tongue and gingival promptly treated. Most cases have been found to be later in the course of the disease.

126 THE NETTER COLLECTION OF MEDICAL ILLUSTRATIONS