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Dermatological causes of white patches

 Lichen planus:
      Lichen = primitive plants composed of symbiotic algae and fungi
      Lichen planus = dry lichen-like appearance of affected skin
      It is the commonest dermatological disease that may result in oral white
      lesions
      It is a chronic mucocutaneous disease that involves skin and mucous
      membranes
      Clinical features:
          Skin lesions:
           o Site: any area in the skin may be involved but the commonest site
             is the flexer surface of the wrist
           o Fingernails may be involved mostly with vertical ridging in 10%
             of the cases
           o Scalp might be affected in females leading to alopecia
           o Clinical appearance:
                -   Purple, pruritic, papules (PPP) with white streaks on
                    the surface “Wickham’s striae” which are
                    characteristic of this condition
                -   Papule = circumscribed solid elevation of skin with no
                    visible fluid
                -   Papules show variable patterns (discrete, linear,
                    annular, bullous)
                -   Lesions may also present as widespread rash (in which
                    skin gets warm, swollen, and maybe painful)
           o Lesions develop slowly and 85% of them resolve in almost 18 months, however lesions may
             recur in some patients
               ** When lesions go they leave a pigmented scar behind that take considerable time to resolve




          Oral lesions:
           o In contrast to skin lesions, oral lesions pursue a much more chronic course, sometimes
             extending over many years (average duration 4.5 years)
           o May occur alone or in combination with skin lesions
                                                   1/6
o Site: lesions mostly affect buccal mucosa (90%), but may also affect the tongue, gingiva,
         palate, and lips. The floor of the mouth involvement is relatively uncommon
       o Lesions are often bilateral and show wide spectrum of presentations
       o Clinical appearance:
            -   Non-erosive lesions:
                 Reticular, annular, papular, plaque-like
                  ** Reticular lesions have white streaks arranged in lace-like pattern
                  ** Plaque-like lesions are white patches resembling leukoplakia clinically
                  ** Papular lesions are small white papules that may coalesce
                  ** Bullous lesions are subepithelial bullae
                 They are usually asymptomatic
                 They may show hyperkeratosis with NO atrophy & NO ulceration




Reticular                     Annular                      Papular                      Plaque-like

            -   Erosive/atrophic lesions:
                    ** Erosive lesions are extensive areas of shallow ulceration
                    ** Atrophic lesions are diffuse red lesions resembling Erythroplakia
                 Erosion and atrophy are usually present together and lesions have a red glazed
                  appearance with areas of superficial ulceration which may take several weeks to heal
                 Occasionally, ulcers are preceded by bullae (bullous type) that then rupture
                 Lesions are often associated with typical areas of non-erosive lichen planus
                 They are usually symptomatic
                 Pain and discomfort may be severe (especially when eating spicy/acidic foods)




            -   Gingival lesions:
                 Lichen planus involving the gingiva often presents as a desquamative gingivitis


                                              2/6
 Desquamative gingivitis is a clinical descriptive
                term that doesn’t infer any specific underlying
                pathology (so that it has many causes!)
               Gingival lesions may occur alone (in 10% of
                cases) or with other oral lesions
Prevalence:
o   Lichen planus is a relatively common disease (affects 0.5-2% of general population)
o   There's a worldwide distribution
o   Peak incidence is found between ages 30-50
o   Lesions are more common in females (60% of cases)
o   Oral lesions are detected in ~50% of patients with initial skin lesions
o   Skin lesions are detected in ~10-50% of patients with initial oral lesions!!
    This may be due to:
    - Asymptomatic nature of oral lesions in many cases
    - Inconstant relationship of oral and skin lesions (oral lesions may occur BEFORE, AFTER
        or AT THE SAME TIME as skin lesions)
Histopathological features:
o Orthokeratosis or Parakeratosis
o Epithelial atrophy or acanthosis
o Acanthosis results in irregular elongation and widening
  of the rete ridges in a “saw-tooth pattern”
o Dense, well-defined band of subepithelial mononuclear
  infiltrate (mainly T-lymphocytes)
o Liquefactive degeneration of the basal cell layer with
  edema and lymphocytic infiltration
o The degenerating cells appear as hyaline shrunken bodies,
  called Civatte bodies
o Basal cell degeneration may result in subepithelial bullae
  formation and ulceration because of the lack of cohesion
  between epithelium and lamina propria following the
  degeneration




                                            3/6
o Oral lesions may show little superficial resemblance to skin lesions clinically even though the
  basic histological changes are similar, because of the modifying environment of the oral cavity
  by:
    -   Continuous presence of saliva
    -   Secondary infection by oral organisms
    -   Repeated trauma
o Almost all cases run a benign course
o Malignant transformation has been described in a very small proportion (0.5%-2.5% over 5 years)
o Some studies suggest that atrophic/erosive forms are more likely for such transformation
  because of the decreased barrier presented to potential carcinogens
o Other studies found malignant transformation more likely with plaque lesions
Etiology & pathogenesis:
o It is NOT fully understood
o In most cases the precipitating factors are unknown and the disease is Idiopathic
o It is widely accepted that cell-mediated immune responses to an external antigen, or internal
  antigenic changes in epithelial cells, are involved since response resembles type IV
  hypersensitivity reaction which is T-cell mediated
o Cytotoxic lymphocytes damage the basal epithelium
    ** Possible immunological mechanism in lichen planus:
    External antigen challenge and/or modified antigenic structure of epithelial cells  induces
    cytokines release from langerhans cells & keratinocytes  chemotaxis of lymphocytes which
    accumulate in the basement membrane zone & basal epithelium  antigen presentation to CD4
    helper cells  activation of CD8 Cytotoxic cells  basal cell degeneration
o Lichen planus has been associated with some systemic diseases, in many of these, a cause-and-
  effect relationship has not been established (e.g. there is strong association of the disease with
  chronic liver disease especially hepatitis C virus)
o Oral & skin lesions resembling lichen planus are also seen as a part of graft-versus-host
  reaction (immune reactions in patients receiving transplants), in such cases, the transplanted T
  cells react to antigens on host epithelial cells
o Lichenoid reaction:
  - Lichenoid = lichen-like
  - In some patients, lesions similar to lichen planus may appear
      triggered by hypersensitivity reaction (IV) to certain drugs
      (NSAIDs) or dental materials (amalgam)
  - These lesions are clinically and histologically similar to
      Lichen planus, but tend to be reversible so that they resolve
      upon withdrawal of the offending agent
        ** Lichenoid reactions aren't idiopathic as lichen planus
        ** Lichenoid reactions are usually unilateral while lichen planus is usually bilateral




                                             4/6
 Lupus Erythematosus (LE):
      It is a chronic mucocutaneous disease that involves skin and mucous membranes and believed to be
      due to an autoimmune process
      It has two main forms:
       1. Chronic discoid lupus erythematosus  localized
       2. Systemic lupus erythematosus  disseminated
      Lesions are more common in females
      Clinical features:
       1. Chronic discoid lupus erythematosus (DLE):
           o It is the localized form of the disease in which skin lesions occur without any systemic
             involvement
           o Site: lesions are usually restricted to the skin, and confined to the face
           o Clinical presentation:
               -   Skin lesions:
                    Lesions appear as scaly or crusted red patches that heal with scar
                    Sometimes facial lesions have a symmetrical distribution over the nose and cheek,
                     the so-called (butterfly rash)
                       ** This butterfly rash can be seen in both forms
                    Follicular plugging “hair follicles being plugged and prevented from going out and
                     growing leading to hair loss)




               -   Oral lesions:
                    Lesions are found in up to 50% of cases
                    Buccal mucosa is most frequently affected
                    There's considerable variation in the usual
                     presentation of oral lesions but the most common
                     is a discoid area of Erythema or ulceration
                     surrounded by white Keratotic border
                     sometimes with radiating striae (resembling
                     lichen planus)
           o Histopathology:
               -   Orthokeratosis or Parakeratosis

                                                  5/6
-   Epithelial atrophy or acanthosis
       -   Keratin plugging
       -   The subepithelial lymphocytes are aggregated
           in follicles and don’t show the clear band-like
           arrangement as in lichen planus
       -   Liquefactive degeneration of basal cells
       -   Circulating autoantibodies are found in one-
           third of patients




2. Systemic Lupus Erythematous (SLE):
   o It is the disseminated form of the disease in which
     skin lesions occur with systemic involvement
   o Site: skin lesions typically affect the face
     “cheeks” and the hands
   o Photosensitivity may be implicated in lesions
     eruption
   o May be fatal
   o Clinical presentation:
       -   Skin lesions:
            Skin rashes (maculopapular)
            Sometimes facial lesions have a symmetrical distribution over the nose and cheek,
             the so-called (butterfly rash)
       -   Oral lesions:
            They are variable
            Superficial erosions and erythematous patches on the buccal mucosa
            White Keratotic areas are not so frequently seen as in DLE
   o Histopathology:
       -   Non specific diffuse inflammatory infiltrate
       -   A variety of circulating autoantibodies are almost always present (e.g. antinuclear
           antibodies (ANAs))




                                          6/6

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5 dermatological causes of white patches

  • 1. Dermatological causes of white patches  Lichen planus: Lichen = primitive plants composed of symbiotic algae and fungi Lichen planus = dry lichen-like appearance of affected skin It is the commonest dermatological disease that may result in oral white lesions It is a chronic mucocutaneous disease that involves skin and mucous membranes Clinical features:  Skin lesions: o Site: any area in the skin may be involved but the commonest site is the flexer surface of the wrist o Fingernails may be involved mostly with vertical ridging in 10% of the cases o Scalp might be affected in females leading to alopecia o Clinical appearance: - Purple, pruritic, papules (PPP) with white streaks on the surface “Wickham’s striae” which are characteristic of this condition - Papule = circumscribed solid elevation of skin with no visible fluid - Papules show variable patterns (discrete, linear, annular, bullous) - Lesions may also present as widespread rash (in which skin gets warm, swollen, and maybe painful) o Lesions develop slowly and 85% of them resolve in almost 18 months, however lesions may recur in some patients ** When lesions go they leave a pigmented scar behind that take considerable time to resolve  Oral lesions: o In contrast to skin lesions, oral lesions pursue a much more chronic course, sometimes extending over many years (average duration 4.5 years) o May occur alone or in combination with skin lesions 1/6
  • 2. o Site: lesions mostly affect buccal mucosa (90%), but may also affect the tongue, gingiva, palate, and lips. The floor of the mouth involvement is relatively uncommon o Lesions are often bilateral and show wide spectrum of presentations o Clinical appearance: - Non-erosive lesions:  Reticular, annular, papular, plaque-like ** Reticular lesions have white streaks arranged in lace-like pattern ** Plaque-like lesions are white patches resembling leukoplakia clinically ** Papular lesions are small white papules that may coalesce ** Bullous lesions are subepithelial bullae  They are usually asymptomatic  They may show hyperkeratosis with NO atrophy & NO ulceration Reticular Annular Papular Plaque-like - Erosive/atrophic lesions: ** Erosive lesions are extensive areas of shallow ulceration ** Atrophic lesions are diffuse red lesions resembling Erythroplakia  Erosion and atrophy are usually present together and lesions have a red glazed appearance with areas of superficial ulceration which may take several weeks to heal  Occasionally, ulcers are preceded by bullae (bullous type) that then rupture  Lesions are often associated with typical areas of non-erosive lichen planus  They are usually symptomatic  Pain and discomfort may be severe (especially when eating spicy/acidic foods) - Gingival lesions:  Lichen planus involving the gingiva often presents as a desquamative gingivitis 2/6
  • 3.  Desquamative gingivitis is a clinical descriptive term that doesn’t infer any specific underlying pathology (so that it has many causes!)  Gingival lesions may occur alone (in 10% of cases) or with other oral lesions Prevalence: o Lichen planus is a relatively common disease (affects 0.5-2% of general population) o There's a worldwide distribution o Peak incidence is found between ages 30-50 o Lesions are more common in females (60% of cases) o Oral lesions are detected in ~50% of patients with initial skin lesions o Skin lesions are detected in ~10-50% of patients with initial oral lesions!! This may be due to: - Asymptomatic nature of oral lesions in many cases - Inconstant relationship of oral and skin lesions (oral lesions may occur BEFORE, AFTER or AT THE SAME TIME as skin lesions) Histopathological features: o Orthokeratosis or Parakeratosis o Epithelial atrophy or acanthosis o Acanthosis results in irregular elongation and widening of the rete ridges in a “saw-tooth pattern” o Dense, well-defined band of subepithelial mononuclear infiltrate (mainly T-lymphocytes) o Liquefactive degeneration of the basal cell layer with edema and lymphocytic infiltration o The degenerating cells appear as hyaline shrunken bodies, called Civatte bodies o Basal cell degeneration may result in subepithelial bullae formation and ulceration because of the lack of cohesion between epithelium and lamina propria following the degeneration 3/6
  • 4. o Oral lesions may show little superficial resemblance to skin lesions clinically even though the basic histological changes are similar, because of the modifying environment of the oral cavity by: - Continuous presence of saliva - Secondary infection by oral organisms - Repeated trauma o Almost all cases run a benign course o Malignant transformation has been described in a very small proportion (0.5%-2.5% over 5 years) o Some studies suggest that atrophic/erosive forms are more likely for such transformation because of the decreased barrier presented to potential carcinogens o Other studies found malignant transformation more likely with plaque lesions Etiology & pathogenesis: o It is NOT fully understood o In most cases the precipitating factors are unknown and the disease is Idiopathic o It is widely accepted that cell-mediated immune responses to an external antigen, or internal antigenic changes in epithelial cells, are involved since response resembles type IV hypersensitivity reaction which is T-cell mediated o Cytotoxic lymphocytes damage the basal epithelium ** Possible immunological mechanism in lichen planus: External antigen challenge and/or modified antigenic structure of epithelial cells  induces cytokines release from langerhans cells & keratinocytes  chemotaxis of lymphocytes which accumulate in the basement membrane zone & basal epithelium  antigen presentation to CD4 helper cells  activation of CD8 Cytotoxic cells  basal cell degeneration o Lichen planus has been associated with some systemic diseases, in many of these, a cause-and- effect relationship has not been established (e.g. there is strong association of the disease with chronic liver disease especially hepatitis C virus) o Oral & skin lesions resembling lichen planus are also seen as a part of graft-versus-host reaction (immune reactions in patients receiving transplants), in such cases, the transplanted T cells react to antigens on host epithelial cells o Lichenoid reaction: - Lichenoid = lichen-like - In some patients, lesions similar to lichen planus may appear triggered by hypersensitivity reaction (IV) to certain drugs (NSAIDs) or dental materials (amalgam) - These lesions are clinically and histologically similar to Lichen planus, but tend to be reversible so that they resolve upon withdrawal of the offending agent ** Lichenoid reactions aren't idiopathic as lichen planus ** Lichenoid reactions are usually unilateral while lichen planus is usually bilateral 4/6
  • 5.  Lupus Erythematosus (LE): It is a chronic mucocutaneous disease that involves skin and mucous membranes and believed to be due to an autoimmune process It has two main forms: 1. Chronic discoid lupus erythematosus  localized 2. Systemic lupus erythematosus  disseminated Lesions are more common in females Clinical features: 1. Chronic discoid lupus erythematosus (DLE): o It is the localized form of the disease in which skin lesions occur without any systemic involvement o Site: lesions are usually restricted to the skin, and confined to the face o Clinical presentation: - Skin lesions:  Lesions appear as scaly or crusted red patches that heal with scar  Sometimes facial lesions have a symmetrical distribution over the nose and cheek, the so-called (butterfly rash) ** This butterfly rash can be seen in both forms  Follicular plugging “hair follicles being plugged and prevented from going out and growing leading to hair loss) - Oral lesions:  Lesions are found in up to 50% of cases  Buccal mucosa is most frequently affected  There's considerable variation in the usual presentation of oral lesions but the most common is a discoid area of Erythema or ulceration surrounded by white Keratotic border sometimes with radiating striae (resembling lichen planus) o Histopathology: - Orthokeratosis or Parakeratosis 5/6
  • 6. - Epithelial atrophy or acanthosis - Keratin plugging - The subepithelial lymphocytes are aggregated in follicles and don’t show the clear band-like arrangement as in lichen planus - Liquefactive degeneration of basal cells - Circulating autoantibodies are found in one- third of patients 2. Systemic Lupus Erythematous (SLE): o It is the disseminated form of the disease in which skin lesions occur with systemic involvement o Site: skin lesions typically affect the face “cheeks” and the hands o Photosensitivity may be implicated in lesions eruption o May be fatal o Clinical presentation: - Skin lesions:  Skin rashes (maculopapular)  Sometimes facial lesions have a symmetrical distribution over the nose and cheek, the so-called (butterfly rash) - Oral lesions:  They are variable  Superficial erosions and erythematous patches on the buccal mucosa  White Keratotic areas are not so frequently seen as in DLE o Histopathology: - Non specific diffuse inflammatory infiltrate - A variety of circulating autoantibodies are almost always present (e.g. antinuclear antibodies (ANAs)) 6/6