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    Linear Iga Dermatosis / Chronic Bullous Disease of Childhood

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    Hoc Lim See
    Linear IgA Dermatosis and Chronic Bullous Disease of Childhood are subepidermal bullous diseases characterized by linear IgA deposits at the dermoepidermal junction. Linear IgA Dermatosis typically affects adults over 40 and presents with annular or grouped vesicles and bullae in a symmetric distribution, while Chronic Bullous Disease of Childhood typically affects children under 5 and presents as tense bullae occurring in clusters. Both diseases can involve mucosal surfaces but are usually self-limiting, with Chronic Bullous Disease of Childhood often resolving by puberty.

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    Linear Iga Dermatosis / Chronic Bullous Disease of Childhood

    Uploaded by

    Hoc Lim See
    29 views31 pages
    Linear IgA Dermatosis and Chronic Bullous Disease of Childhood are subepidermal bullous diseases characterized by linear IgA deposits at the dermoepidermal junction. Linear IgA Dermatosis typically affects adults over 40 and presents with annular or grouped vesicles and bullae in a symmetric distribution, while Chronic Bullous Disease of Childhood typically affects children under 5 and presents as tense bullae occurring in clusters. Both diseases can involve mucosal surfaces but are usually self-limiting, with Chronic Bullous Disease of Childhood often resolving by puberty.

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    LIGA CBDC

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    Linear IgA Dermatosis and Chronic Bullous Disease of Childhood are subepidermal bullous diseases characterized by linear IgA deposits at the dermoepidermal junction. Linear IgA Dermatosis typically affects adults over 40 and presents with annular or grouped vesicles and bullae in a symmetric distribution, while Chronic Bullous Disease of Childhood typically affects children under 5 and presents as tense bullae occurring in clusters. Both diseases can involve mucosal surfaces but are usually self-limiting, with Chronic Bullous Disease of Childhood often resolving by puberty.

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    © All Rights Reserved
    Download as PPTX, PDF, TXT or read online from Scribd
    Download as pptx, pdf, or txt
    29 views31 pages

    Linear Iga Dermatosis / Chronic Bullous Disease of Childhood

    Uploaded by

    Hoc Lim See
    Linear IgA Dermatosis and Chronic Bullous Disease of Childhood are subepidermal bullous diseases characterized by linear IgA deposits at the dermoepidermal junction. Linear IgA Dermatosis typically affects adults over 40 and presents with annular or grouped vesicles and bullae in a symmetric distribution, while Chronic Bullous Disease of Childhood typically affects children under 5 and presents as tense bullae occurring in clusters. Both diseases can involve mucosal surfaces but are usually self-limiting, with Chronic Bullous Disease of Childhood often resolving by puberty.

    Copyright:

    © All Rights Reserved
    Download as PPTX, PDF, TXT or read online from Scribd
    Download as pptx, pdf, or txt
    of 31

    Linear IgA

    Dermatosis / Chronic
    Bullous Disease of
    Childhood
    2
    EPIDEMIOLOGY

    ▫ Linear IgA Dermatosis


    ⬝ Age: After 4th decade of life
    ⬝ Gender: F>M

    ▫ Chronic Bullous Disease of Childhood


    ⬝ Age: <5 years old
    ⬝ Gender: F>M
    3
    CLINICAL FINDINGS

    ▫ Linear IgA Dermatosis


    ⬝ Combinations of annular or grouped papules,
    vesicles and bullae
    ⬝ Distributed SYMMETRICALLY on extensor
    surfaces including elbows, knees and buttocks
    ⬝ Very pruritic -> numerous crusted papules
    ⬝ Rarely, may present with an acute febrile illness
    with arthritis, arthralgia and generalized malaise.

    4
    CLINICAL FINDINGS

    ▫ Drug Induced LIGA


    ⬝ Erythema multiforme like findings and toxic
    epidermal necrolysis like presentation
    ⬝ Widespread bullae
    ⬝ (+) Koebner phenomenon
    ⬝ Vancomycin – most common
    ⬝ Other drugs: lithium, phenytoin,
    sulfamethoxazole-trimethoprim, furosemide,
    captopril, diclofenac, ketoprofen and infliximab

    5
    CLINICAL FINDINGS
    ▫ Chronic Bullous Disease of Childhood
    ⬝ Tense bullae on an inflammatory base
    ⬝ AOP: perineum and perioral region
    ⬝ Occur in clusters > "cluster of jewels”
    appearance
    ⬝ New lesions > appear around the periphery of
    previous lesions > '"collarette" of blisters
    ⬝ (+) Pruritus and/or a burning of the skin
    ⬝ Mucosal involvement is less often seen
    ⬝ Good Prognosis
    6
    CLINICAL FINDINGS

    ▫ Mucosal involvement
    ⬝ Asymptomatic oral ulcerations & erosions >
    severe conjunctival and oral disease
    ⬝ Oral lesions seen in 70% of patients with LIGA
    ⬝ Mucosal invasion with complications is less
    often seen in CBDC
    ⬝ Less prominent in patients with druq-
    induced linear IGA

    7
    DISEASE ASSOCIATIONS
    ⬝ Association with Gluten-sensitive enteropathy (?)
    ⬝ Clinical manifestations have not been controlled by the use of a gluten-free diet,
    ⬝ No circulating autoantibodies against tissue transglutaminase
    ⬝ Other conditions
    ⬝ Ulcerative colitis
    ⬝ Crohn's disease
    ⬝ Acute mononucleosis (CBDC)
    ⬝ Paecilomyces lung infection (CBDC)
    ⬝ Malignancies
    ⬝ IgA nephropathy
    ⬝ Physical exposures: Ultraviolet light
    DISEASE ASSOCIATIONS
    ▫ Drugs: Vancomycin, Lithium Phenytoin,
    Sulfamethoxazole/Trimethroprin, Furosemide,
    Atorvastatin, Captopril, And Diclofenac.

    ▫ *Vancomycin - most common drug


    ⬝ (+) have circulating lgA antibodies directed
    against the BP180, BP230, and LAD 285
    antigens.
    ⬝ Linear laA bullous dermatosis is the most
    common nonimmediate hypersensitivity
    reaction to vancomycin.
    HISTOPATHOLOGY

    ▫ Subepidermal blister
    ▫ with collections of neutrophils linearly aligned
    along DEJ, often accumulating at the papillary tips
    ▫ Superficial perivascular and interstitial neutrophilic
    infiltrates admixed with occasional eosinophils and
    lymphocytes
    ▫ WITHOUT any evidence of neutrophilic vasculitis

    ▫ DIF: LINEAR IgA deposits at DEJ

    10
    HISTOPATHOLOGY

    11
    12
    CLINICAL COURSE AND PROGNOSIS

    ⬝ Adults with LIGA - unpredictable course


    ⬝ Continues for years, with few episodes of remission
    ⬝ Patients with severe mucosal disease (eye)
    ⬝ symblepharon formation > structural problems with the eyelids and cornea

    ⬝ CDC is most often a self-limited disease, with most children going into remission
    within 2 years of the onset of the disease.
    ⬝ May persist well into puberty but often is less severe than the initial eruption.
    14
    Dermatitis
    Herpetiformis
    EPIDEMIOLOGY

    ⬝ M>F
    ⬝ May start at any age
    ⬝ most common: 2nd to 4th decade of life
    ⬝ Persists indefinitely with varying severity
    ⬝ Patients usually have asymptomatic gluten-sensitive
    enteropathy (celiac disease).
    ETIOLOGY AND PATHOGENESIS

    17
    CLINICAL FINDINGS
    ▫ Primary lesion: INTENSELY PRURITIC
    ⬝ Erythematous papule
    ⬝ Urticaria-like plaques
    ⬝ Vesicle (most common)
    ▫ Post inflammatory hypo or hyperpigmentation
    ▫ Localized stinging, burning, itching (8-12 hours
    ▫ before appearance of lesions)
    ▫ SYMMETRIC distribution: elbows, knees,
    ▫ buttocks, shoulders, and sacral areas
    ⬝ May affect scalp, posterior nuchal area, face
    ▫ Mucous membranes rare
    18
    CLINICAL FINDINGS

    19
    CLINICAL FINDINGS

    20
    HISTOPATHOLOGY
    ▫ Early Skin Lesion (Clinically non-vesicular)
    ⬝ Separation of papillary tips from overlying
    epidermis
    ⬝ Dermal papillary collection of neutrophils
    (microabscesses)
    ⬝ Neutrophilic fragments
    ⬝ Varying numbers of eosinophils
    ⬝ Fibrin
    ⬝ Superficial to mid perivascular lymphohistiocytic
    infiltrates with some neutrophils and occasional
    eosinophils
    21
    HISTOPATHOLOGY
    ▫ Older Skin Lesion
    ⬝ Subepidermal vesicles
    ⬝ DDx: (subepidermal bullous eruptions)
    ⬝ Bullous Pemphigoid
    ⬝ Erythema Multiforme
    ⬝ Bullous Drug Eruption
    ⬝ Pemphiqoid Gestationis

    22
    HISTOPATHOLOGY
    ▫ - Immunoglobulin A (IgA)
    ⬝ GRANULAR IgA deposits in dermal
    papillary tips of normal appearing skin,
    perilesional, uninvolved skin
    ⬝ Most reliable criterion for diagnosis
    ⬝ Mostly IgA1 produced in bone marrow
    ⬝ IgA1 is the predominant subclass
    targeting dietary proteins of gut secretions
    w/ DH
    ⬝ IgA2 produced in mucosal sites

    23
    SULFONES
    ⬝ Dapsone (Diaminodiphenyl sulfone)
    ⬝ Initial: 100-150mg/d OD
    ⬝ Rarely: 300-400mg/d for initial improvement
    ⬝ Take the minimal dose required to suppress signs & symptoms
    ⬝ Some pts: 25mg/week is sufficient
    ⬝ Sulfoxone (Diazone)
    ⬝ Not available in the US
    ⬝ Sulfapyridine
    ⬝ 1-1.5g/d
    ⬝ Indications: Dapsone intolerance, Elderly, Cardiopulmonary problems
    ⬝ Not available in the US
    TREATMENT

    ⬝ Symptoms abate in 3 hours to few days after 15 pill taken


    ⬝ No new lesions after 1-2 days of treatment
    ⬝ Exacerbation occur hours to days after cessation of treatment
    26
    GLUTEN FREE DIET
    ⬝ Effect on Small Intestine
    ⬝ Intestinal lesions respond to dietary gluten withdrawal
    ⬝ Time course of response in adults w/ DH same as adults w/
    Celiac Disease
    ⬝ Effect on Skin
    ⬝ Decrease or complete elimination of need for medication for
    most but not all patients after variable periods of time (4months-l
    year)
    ELEMENTAL AND OTHER DIET THERAPY
    ⬝ Elemental Diet: free aminò acids, short-chain polysaccharides, small
    amount of triglycerides
    ⬝ Very beneficial in alleviating skin disease within a few weeks
    even if patient ingests large amounts of gluten
    ⬝ Difficult to tolerate for long periods
    ⬝ Atkins Diet: high protein, unlimited fat, low-carb diet
    ⬝ Complete resolution with adherence to this diet
    CLINICAL COURSE AND PROGNOSIS
    ⬝ Persists indefinitely in most patients
    ⬝ Wax and wane in severity
    ⬝ Spontaneous remissions occur
    ⬝ NSAIDS may exacerbate the disease
    SUMMARY
    ⬝ DERMATITIS HERPETIFORMIS
    ⬝ Intensely itchy, chronic papulovesicular eruption distributed symmetrically on
    extensor surfaces
    ⬝ Histologically: dermal papillary collections of neutrophils (microabscesses)
    ⬝ Diagnostic: Granular immunoglobulin (lg) A deposits in normal-appearing skin
    ⬝ Dominant Autoantigens: Epidermal transglutaminase (eTG)
    ⬝ Most, if not all, DH patients have an associated gluten-sensitive enteropathy
    ⬝ Responds rapidly to dapsone therapy and strict adherence to a gluten-free diet
    Thank You!

    31

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