This document discusses Pemphigus, a rare autoimmune blistering disease of the skin and mucous membranes, in children. It notes that the most common variant seen in children is Pemphigus Vulgaris. Treatment considerations for children include using less carcinogenic drugs and minimum steroid doses to reduce side effects while not affecting fertility. For severe Pemphigus Vulgaris, methylprednisolone pulse therapy or dexamethasone may be used. Various adjuvants like azathioprine, gold, and rituximab can be used when high steroid doses are needed to control the disease. Pemphigus Foliaceus is also seen in children, particularly the endemic F

1. PEMPHIGUS IN CHILDREN

2. • Rare, all variants described
• Most common- PV excluding Fogo Selvagem
• C/F same as adults
• Treatment – special consideration: less
carcinogenic drugs, minimum dose of steriods
to decrease S/E, shouldnot affect fertility.

3. Pemphigus Vulgaris
• Mean age of onset – 12 yrs ( boys earlier)
• Slightly increased predilection for males (vs
adults)
• Increased incidence around puberty
• Triggers- drugs (enalapril, monteleukast,
captopril, pencillamine), thiol groups –
tannins, ICT, Immunisations- DPT

4. • Treatment- Bjarnson et al:
-Initial: 2- 3 mg/kg/day slowly tapered within 2 w to .5- .8
mg/kg/day and further tapered by A/D schedule
-Severe: methylprednisolone pulse (1g/day i.v. X 5 days)
dexamethasone (136mg/d i.v x 3d)
faster drying of lesion, lesser steroid S/E
-Single recalcitrant lesion: i/l or topical steroids
-Iv IG 2g/kg/cycle - monotherapy
-Adjuvants: 1)when high dose steroids are needed to
control the disease
2) when repeated attempts to taper steroid
associated with recurrence.

5. Adjuvants used
• Azathioprine
• Gold
• Cyclosporine
• Methotrexate
• Dapsone
• Rituximab
• Plasmapheresis

6. Pemphigus foliaceus
• Sporadic- rare
• Endemic- fogo selvagem common in children,
river banks of Brazil, spread by simulium
nigrimanum
• Circinate, arcuate, polycyclic lesions – unique
• Treatment- Steroids, Dapsone ( Mc Adjuvant)
Erythromycin, Chloroquine, Methotrexate,
Sulfapyridine, Azathioprine, Hydroxychloroquine

7. • Pemphigus vegetans, erythematosus, IgA
pemphigus – very rare, features similar to
adults
• Paraneoplastic pemphigus-
- most common malignancy- Castleman’s
disease
- in children mucocutaneous disease was more
lichenoid than blistering

8. Neonatal pemphigus and Still Births
• Due to transplacental transfer of IgG
antibodies
• Neonatal skin more sensitive than maternal
skin
• Neonatal skin similar to adult mucosa- hence
only PV has been described, PF not seen.
• few still births (around 8 m)- Dsg antibodies
were detected
• No progression to adult PV

9. • Differential diagnosis
- Other immunobullous diseases
- Impetigo, impetigenous eczema
- Erythroderma ( r/o AD, Seb derm, PRP, SSSS)
• Side effects of steroids
- Growth retardation
- Weight gain, hypertension, DM
- acne, menstrual irregularities
- Conduct disorder
- Cushingoid habitus, cataract, infections
- Osteopenia