Pediatric Dermatosis: Nevi😍

Pediatric Dermatosis: Nevi

1. Verrucous Epidermal Nevi (VEN)

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  • Along Blaschko's lines
  • Verrucous plaque
  • Asymptomatic
  • Present soon after or after birth
  • Treatment: lasers

2. Inflammatory Linear Verrucous Epidermal Nevi (ILVEN)

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  • Aka: Eczematous Epidermal Nevi
  • Linear and inflammatory
  • More red, eczematous
  • Resistant to usual treatment
  • Needs topical steroid

3. Becker's Nevus

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  • Aka:
    • Becker's Melanosis
    • Pigmented Hairy Epidermal Nevi
  • Presents in adolescence
  • Starts as hyperpigmented lesion
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  • Develops hypertrichosis on lesion
  • Role of androgen suspected
  • Not congenital melanocytic nevi

4. Infantile Hemangioma

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  • Three stages:
    • Growth:
      • Usually at 3 months
    • Maturation:
      • Usually by 3-9 months
    • Resolution:
      • Starts at 9 months
      • Leaves telangiectasias
  • Capillary proliferations
  • Appear as:
    • Vascular lesions with telangiectasias
    • Erythematous plaques
    • Anywhere on body
  • On nose:
    • Pinocchio or Siren nose appearance
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  • Spontaneous resolution
  • Treatment required if:
    • Over vital organs
    • Undergo ulcerations
    • Bleeding
  • Drugs for treatment:
    • Systemic steroids
    • propranolol

5. Capillary Malformations

Salmon Patch

  • Aka: Erythema Nuchae, Stork Bite
  • Autosomal dominant inheritance
  • Very common condition
  • Reddish patch
  • On neck or face
  • Usually resolves by 1 year
  • Area of erythema and telangiectasia
  • On nape of neck or face
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Port-wine Stain

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  • Aka: Nevus Flammeus
  • Capillary malformation
  • Strict midline demarcation
  • Stable course (keeps growing)
  • Does not resolute
  • Strictly unilateral
  • Commonly on face
  • Very vascular reddish patch

Treatment:

  • Lasers (pulse dye laser)

Associated with Sturge-Weber Syndrome:

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  • Neurocutaneous are AD except
    • Sturge WeberSporadic → GNAQ mutation

Seen in
GNAS
Mccune Albright
Cardiac Myxoma
GNAS 1
• Pseudohypoparathyroid/ Albright Hereditary Osteodystrophy
GNAQ
Sturge Weber (Sporadic)

  • Port-wine stain in trigeminal distribution
  • Leptomeningeal Angiomatosis (ipsilateral)
    • cavernous angioma (vascular malformation).
    • Convulsions (Focal)
    • behavioural problems,
    • Mental retardation
  • Choroidal Hemangiomas
    • Ocular complications
      • ipsilateral glaucoma,
      • blindness,
      • congestion
      • Buphthalmos
  • Unilateral weakness
    • d/t tumor compressing corticospinal pathway.

CT scan:

  • S-shaped intracranial calcifications
  • S for Sturge-Weber, S for S shaped
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  • Tram track appearance or rail road calcification
    • NOTE: Also seen in
      • membranoproliferative glomerulonephritis,
      • bronchiectasis.
  • Mnemonic: Web of storage (Sturg Weber) of Portwine factories () → connected by rail tram tracks (Tram track apearance)

Urticaria Pigmentosa

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  • Urticarial wheals with pigmentation
  • Form of cutaneous mastocytosis (increased mast cells)
  • CD 117/CKIT
  • Usually in children < 2 years
  • No systemic involvement
  • Typical urticarial itchy wheals on trunk
  • Heal with pigmentation
  • Itching due to increased mast cell

Darier's sign:

  • Not a feature of Darier's disease
  • Stroking skin with blunt object causes linear urticarial wheals
  • Due to histamine release from mast cells

Biopsy caution:

  • Anesthesia can degranulate mast cells

Urticaria and Angioedema

Urticaria

  • Erythematous, edematous, itchy papules and plaques
  • Evanescent
    • disappears in hours, no marks
  • High IgE causing mast cell histamine release

Acute Urticaria: < 6 weeks

  • Causes (mnemonic: 5 Is):
    • Infections
    • Injections (or drugs)
    • Inhalation
    • Ingestion (food items)
    • Idiopathic

Chronic Urticaria: > 6 weeks

  • Mostly physical in nature
  • Includes Urticarial Vasculitis

Physical Urticaria 

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4
4
  • Most common chronic type
  • Physical force causes urticaria

Dermographism (most common)

  • Skin writing, linear itchy wheals on stroking
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Other forms:

  • Cold Urticaria (cold)
  • Heat Urticaria (heat)
  • Solar Urticaria (sun)
  • Aquagenic Urticaria (water)
  • Cholinergic Urticaria (increase in core body temperature)
  • Delayed Pressure Urticaria (pressure)
    • Appears later, painful, lasts > 24 hours

Treatment of Urticaria

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  • First line: Second-generation H1 antihistamines (standard dose)
  • If no response: Increase dose to four-fold
  • If still no response:
    • Add omalizumab,
    • cyclosporin,
    • montelukast, or
    • first-generation antihistamines

4. Omalizumab

  • Monoclonal antibody against IgE
  • Used for
    • Urticaria
    • prophylaxis of severe persistent Bronchial asthma
  • Not effective in atopic dermatitis
  • Given subcutaneously
  • Kochinu asthma (asthma) vannapo maran Om Kreem (Om) kuttichathaennu prnj ammumma

Urticarial Vasculitis

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  • Differences from typical urticaria:
      1. Lesions more persistent (> 24 hours)
      1. Heal with pigmentation
      1. More tender than itchy
  • Form of chronic urticaria, actually vasculitis
  • Biopsy: Leukocytoclastic vasculitis
  • Associated with connective tissue disorders and malignancies

Angioedema

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  • Swelling of skin or mucosa
  • Deeper, less defined, more painful, less erythematous
  • Hypodermis and submucosa involvement
  • Takes longer to resolve
  • Can be:
    • Mast cell mediated:
      • With urticaria, treated like urticaria
    • Bradykinin mediated: Just angioedema

Hereditary Angioedema (HAE)

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  • Also: Quincke's Disease
  • Autosomal dominant
  • Bradykinin mediated
  • NOT HISTAMINE MEDIATED
  • Defect: C1 esterase inhibitor deficiency
    • Leads to uncontrolled complement cascade
    • C1 inhibitor normally controls C1 pathway.
    • Deficiency → uncontrolled complement → episodic swelling (edema).
  • Types based on C1 inhibitor:
    • Type 1: Low C1 inhibitor protein concentration
    • Type 2: Loss of C1 inhibitor function (quantity normal)
    • Type 3: Normal C1 inhibitor → Factor 12?? (unknown defects)
  • Presentation:
    • Recurrent laryngeal edemas
    • Colicky abdominal pain
    • Recurrent angioedema
    • Positive family history
  • Evaluation:
    • Screening: Low C4 level
    • Specific: C1 inhibitor levels
  • Treatment:
    • Acute: Airway management
    • IV C1-esterase inhibitor concentrate (C1-INH)
    • Fresh frozen plasma (contains C1 inhibitor)
    • DOC: Danazol
    • Icatibant, Ecallantide
    • Lanadelumab
      • Ee kaatile Ee kallan Anade adyil
    • Prophylaxis: Tranexamic acid, anabolic steroids
    • Anabolic steroid Danazol may help.

Vector-Borne Diseases

Leishmaniasis

  • Protozoal disease
  • Vector: Sandfly
  • Three types:
    • Cutaneous,
    • Mucocutaneous,
    • Visceral (Kalazar)

Old World Cutaneous Leishmaniasis

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  • Endemic to India (NE, Rajasthan)
  • Also: Delhi Boil, Baghdad Boil
  • Caused by (mnemonic: MAIT):
    • L. Major
    • L. Tropica
    • L. Aethiopica
    • L. Infantum
  • Seen in exposed areas
  • Typical lesion: Annular lesion with crusting
    • Papule -> ulcerates -> crusts
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New World Cutaneous Leishmaniasis

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  • Seen in Mexico
  • Caused by L. Mexicana
  • Chronic ear cartilage ulcer: Chiclero's Ulcer

Mucocutaneous Leishmaniasis

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  • Destruction of nasal and oral mucosa
  • Grossly deformed condition: Espundia

Post-Kalazar Dermal Leishmaniasis (PKDL)

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  • Consequence of visceral leishmaniasis
  • Develops 1-2 years after treatment.
  • Presents as hypopigmented nodules.
  • History of kalazar (fever, splenomegaly, hepatomegaly) in childhood
  • Develops years later
  • Caused by: L. Infantum, L. Chagasi, L. Donovani
  • Endemic to NE India
  • Lesions:
      1. Raindrop-like hypopigmented lesions on trunk
      1. Succulent erythematous papules and nodules around muzzle area of face
  • Not leprosy (no loss of sensation)

Smear:

  • Leishman in Donovan bodies
    • LD bodies, amastigote form in macrophages
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Treatment:

  • DOC: Oral Miltefosine
  • Antimonials,
  • Amphotericin B,

Erythema Chronicum Migrans (ECM)

  • Cutaneous feature of Lyme Disease
  • Caused by spirochete Borrelia burgdorferi
  • Vector: Ixodes ticks

Presentation:

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  • Erythema
  • Central bite usually present
  • Expanding erythema from bite
  • Clears centrally within a week
  • Advances up to 50 cm diameter

Classical sign:

  • Bite in center, expanding ring

Common Tests

Test
Method
Applications
Diascopy
Press glass slide against skin
1. Differentiate purpura (non-blanchable) and erythema (blanchable)
2. Differentiate Nevus Depigmentosus and Nevus Anemicus
3. See apple jelly nodules in granulomatous disorder
purpura and erythema
purpura and erythema
Apple jelly nodules in granulomatous disorders
Apple jelly nodules in granulomatous disorders
Nevus dipigmentosa vs anemicus
Nevus dipigmentosa vs anemicus

Woods Lamb Examination

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Mnemonic:
  • NO (9% Nickel oxide) Bullshit (Barium silicate) in Woods
  • 365 days (365nm) in wood
  • Woman with green cap (tinea capitis green), yellow beaded pedant (yellow versicolor) around neck and red bikini (red → erythrasma) inside a forest full of wood (Woods lamp)
  • Low output mercury arc lamp
  • Covered with Wood’s filter (barium silicate + 9% nickel oxide)
  • Emits UV light (320–450 nm, peak 365 nm)
  • Clinical Uses:
    • Condition
      Wood’s Lamp Finding
      Vitiligo
      Lesions accentuate
      Tinea capitis
      Greenish fluorescence
      Pityriasis versicolor
      Yellow fluorescence
      Erythrasma
      Coral red fluorescence
      Congenital erythropoietic porphyria
      Reddish fluorescence
      Melasma
      Differentiates epidermal vs dermal types
Woods Lamp
Woods Lamp
Peak 365nm
Peak 365nm
Erythrasma: Coral red fluorescence
Erythrasma: Coral red fluorescence
 vitiligo
vitiligo
Congenital erythropoietic porphyrias
Congenital erythropoietic porphyrias

Skin Biopsy Types

  1. Incision biopsy:
      • Small part of lesion excised
  1. Shave biopsy:
      • Superficial;
      • seborrheic or actinic keratosis
  1. Punch biopsy:
      • Most common
      • Uses circular punch of various sizes
      • Area is sutured after removal
  1. Excision biopsy:
      • For suspected malignancy
      • Entire lesion is removed
Incision biopsy
Incision biopsy
Excision biopsy
Excision biopsy