British Association of Dermatologists' Guidelines for the Management of Pemphigus Vulgaris 2017

K.E. Harman; D. Brown; L.S. Exton; R.W. Groves; P.J. Hampton; M.F. Mohd Mustapa; J.F. Setterfield; P.D. Yesudian

Disclosures

The British Journal of Dermatology. 2017;177(5):1170-1201. 

In This Article

Pemphigus Vulgaris in Children (Strength of Recommendation D, Level of Evidence 3)

Even though PV affects adults predominantly, it can occur in children. A review suggested a further subdivision of this group into childhood PV, referring to disease in children aged < 12 years, and juvenile PV, affecting adolescents aged 12–18 years.[265] This subclassification helps delineate the potential adverse effects of medications used in these subgroups. A self-limiting form of PV can occur in neonates born to mothers with PV, due to transplacental transfer of autoantibodies.

Systemic corticosteroids are the treatment of choice in both childhood and juvenile PV,[67] but children are more susceptible than adults to the potential adverse effects of corticosteroids. Growth retardation is the most important adverse effect in children on long-term oral corticosteroids. In both children and adolescents, the height will need to be recorded regularly and expert advice is prudent if high-dose corticosteroids are used long term. Regular checks for signs of adrenal suppression are recommended.[266]

In a series of 33 patients with childhood PV, prednisone was used in 26, with the dose ranging from 12 to 500 mg per day (mean 88·3). Other immunosuppressant medications used included gold (n = 2), azathioprine (n = 6), dapsone (n = 4), cyclophosphamide (n = 2), ciclosporin (n = 1), rituximab (n = 2), mycophenolate mofetil (n = 1) and IVIg (n = 1). Six patients (18%) achieved complete recovery and 79% (26 of 33) had partial remission, with minor relapses while on maintenance therapy. Of concern was the high rate of serious side-effects, with cushingoid features in 65%, growth retardation in 50% and infection in 50%.[267]

Juvenile PV has features similar to adult PV, but disruption of biological and social development due to the skin disease raises particular concern during adolescence. The largest series of juvenile PV included 47 patients, with 42 requiring systemic corticosteroids. Corticosteroid-sparing agents used included azathioprine (n = 1), intramuscular gold (n = 1), dapsone (n = 3), cyclophosphamide (n = 2), mycophenolate mofetil (n = 2) and rituximab (n = 3). IVIg was reported in eight patients, for four of whom it was used as monotherapy. All 47 patients responded to treatment, with adverse effects reported in 19%. Infection (9%), weight gain (11%) and cushingoid appearance (6%) were the main side-effects, associated mainly with systemic corticosteroids.[67,268] Relative youth may be a positive factor in terms of prognosis and mortality.[268]

There have been only 18 anecdotal reports of the use of rituximab in PV affecting children.[263,269–272] It may have a role in childhood PV when treatment with systemic corticosteroids and other immunosuppressants has failed to confer any benefit. It has been used as monotherapy or in combination with systemic corticosteroids and other immunomodulatory drugs.

IVIg therapy has been reported to be effective in children with juvenile PV.[273,274] It can be used as monotherapy or in combination with other systemic agents.[268] IVIg is an attractive second-line option for juvenile PV as the risks of thromboembolic events and renal failure are considered to be much less compared with adults.[274]

Summary

The course of PV in children is generally favourable, with a better prognosis compared with adult PV.[263] Due to its rarity, there are no RCTs in the use of systemic agents in this condition. Overall, its treatment after initial systemic corticosteroids is similar to adult regimens and the same adjuvant therapies can be used.

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