Letterer – Siwe Disease

  • Rare, acute aggressive disseminated proliferative lethal disease of Langerhans cells that affects infants <2 years old.
  • Accounts for 10% of cases of Langerhans cell histiocytosis

Clinical features:-

  • Initial manifestation often skin rash involving trunk, scalp & extremities.
  • Recurrent pyoderma – like lesions with crusting and scaling, vesicopustular and purpuric eruption; resembling seborrhoeic dermatitis.
  • Denuded skin may facilitate microbial invasion, leading to sepsis.
  • Persistent low grade spiking fever with malaise & irritability.
  • Splenomegaly, hepatomegaly & lymphadenopathy are early manifestations.
  • Nodular lesions in body flexures and intraoral haemorrhage.
  • Myelophthisic anemia , leukopenia and thrombocytopenia may also be seen.
  • Bone lesions are not common initially.
  • Diffuse involvement of skeletal system occurs later in the disease.
  • The uvea may be affected; orbital involvement is most unusual
  • The pustules are sterile and Tzanck smear of the vesicopustules shows pale histiocytes which can be used as a rapid screening test.

Oral manifestations:-

  • Ulcerative lesions, gingivitis, loose teeth, ectopic teeth also may be seen.
  • Parents frequently report precocious eruption of teeth, when in fact the gums are receding to expose immature dentition
  • Diffuse destruction of maxilla & mandible.
  • Some cases extreme rapid course of disease; oral involvement does not occur.

Histologic Features:-

1.Very similar to HSC; histiocytic proliferation with or without eosinophils.

2.These histiocytes do not contain significant amounts of cholesterol.

3.‘Foam cells’ not a feature.

Treatment & Prognosis:-

  • Extremely poor; Course of disease rapid & terminates fatally in short time.
  • Poor prognostic features include younger age; dissemination of lesions; involvement of lungs, liver, CNS and RE system; associated infection; anaemia, thrombocytopenia and purpura
  • Chemotherapy coupled with radiation to localised bony lesions and supportive measures are useful in treatment.
  • Thymic extracts have been successfully used to treat LS disease

References:-

Shafer’s 8th edition

Oral manifestations of systemic diseases-II

Hematologic Diseases :-
• Hematologic diseases are disorders which
primarily affect the blood.
Anemia is usually defined as a decrease
in the amount of red blood cells (RBCs) or
hemoglobin in the blood.

Oral Manifestations:
– folate and vit. B12 deficiency
– iron deficiency
– glossitis
• red colour
• athrophic papilae
• recurrent aphthae
– candidal infection
– angular stomatitis
– oral pain

Leukemia is a group of cancers that
usually begins in the bone marrow and
results in high numbers of abnormal white
blood cells.

*Oral Manifestations:-

– gingival hypertrophy
– petechiae
– mucosal ulcers
– hemorrhage
Treatment of leukemia
– reactivation of herpes simplex virus – oral mucosistis.

References:-

1.Google- slideshare.

Oral manifestations of systemic diseases – I

Careful examination of the oral cavity may
reveal findings indicative of an underlying
systemic condition, and allow for early diagnosis
and treatment. Examination should include
evaluation for mucosal changes, periodontal
inflammation and bleeding, and general
condition of the teeth.

I.GIT Diseases
• Gastrointestinal diseases refer to diseases involving
the gastrointestinal tract, namely the esophagus,
stomach, small intestine, large intestine and rectum,
and the accessory organs of digestions, the liver,
gallbladder, and pancreas.

Crohn’s disease, also known as Crohn
syndrome and regional enteritis, is a type of
inflammatory bowel disease (IBD).
Ulcerative colitis is a form of inflammatory
bowel disease (IBD) that causes inflammation
and ulcers in the colon.
Gastroesophageal reflux is a chronic symptom
of mucosal damage caused by stomach acid
coming up from the stomach into the
esophagus.
Chronic liver disease in the clinical context is a
disease process of the liver that involves a
process of progressive destruction and
regeneration of the liver parenchyma leading to
fibrosis and cirrhosis.

1. Crohn disease:
– diffuse labial, gingival or mucosal swelling
– „cobblestoning“ of buccal mucosa and
gingiva
– aphtous ulcers
– mucosal tags
– angular cheilitis
– oral granulomas

2.Ulcerative colitis:-
– oral signs are present in periods of
exacerbation of disease
– aphtous ulceration or superficial
hemorrhagic ulcers
– angular stomatitis
– pyostomatitis vegetans, pyostomatitis
gangrenosum.

3.Gastroesophageal reflux:-
– reduction of the pH of the oral cavity below
5,5

– enamel damage
– damage of the dentin – higher sensitivity (to
temperature..), caries

4. Chronic liver diseases:-
– jaundice
– petechiae or gingival bleeding (hemostasis
disorder)

RREFERENCES:-

1.Google -slideshare

2.Davidson-22nd edition