Generalized Pustular Psoriasis in Childhood

with Exogenous Cushing’s Syndrome

Fitri Azizah

, Yusnita Rahman

, Rahadi Rihatmadja

,Triana Agustin

Rinadewi Astriningrum

, Githa Rahmayunita

Department of Dermatology and Venereology Faculty of Medicine Universitas Indonesia/

Dr.CiptoMangunkusumo National General Hospital, Indonesia

Keywords: Pustular Psoriasis, Childhood, Cushing’s Syndrome, Steroid Misuse

Abstract: Pustular psoriasis is characterized clinically by yellowish sterile pustules surrounded by erythema. Pustular

psoriasis in children is very rare and could be appear following steroid withdrawal in psoriasis vulgaris.

Psoriasis in children usualy mild and can be managed with topical treatment, which is considered as the first-

line therapy. Misuse of topical and systemic steroid in children could lead to Cushing’s syndrome. It is a

multisystem disorder resulting from prolonged exposure to excess glucocorticoids, both systemic or topical.

Children could easily develop systemic reactions to topical steroids due to their higher ratio of total body

surface area to body weight. Here we reported a case of generalized pustular psoriasis (GPP) in a 14-year-old

girl who had suffered from Cushing’s syndrome due to misuse of oral and topical steroid.

1 INTRODUCTION

Since the introduction in 1950s, topical steroids have

become the most commonly prescribed drugs by

dermatologists in outpatient setting. Used properly,

they are safe and effective, with minimal side effects.

Easy access to the drugs and practice of self-

medication has resulted in widespread misuse of

topical corticosteroids.(Meena S et al., 2015) In case

of childhood psoriasis, topical corticosteroids

remained the first line therapy and majority of

children can be managed only with topical treatment.

(Bronckers et al., 2015) Excess intake of

glucocorticoids, either systemic or topical could lead

to Cushing’s syndrome, which characterized

clinically by moon face, appearance of dorsocervical

and supraclavicular fat pads (buffalo hump), and

stretch marks. Cushing’s syndrome in children could

also manifests as growth deceleration.(Stratakis

CA,2018)

Over the last 35 years, there are 43 cases reported

of iatrogenic Cushing’s syndrome due to misuse of

very potent topical steroid usage in children and adult,

particularly in developing countries. The most

common purpose of steroid use in the children group

is infants with diaper dermatitis and in adult group is

psoriasis.(Sahana PK et al., 2015). Pustular psoriasis

is a rare disease, especially in children. Patients with

the generalized type of von Zumbuschs and

erythroderma have higher risk of mortality due to

systemic involvement. Few complications of

generalized pustular psoriasis (GPP) are septicemia,

hyperthermia, liver damage, and acute renal failure.

(Al Aboud DM et al., 2019) Psoriasis vulgaris can

either progressed into generalized pustular psoriasis

or appear after pustular psoriasis. Various

precipitating factors have been reported to trigger or

flare acute generalized pustular psoriasis, including

corticosteroid use and withdrawal. (Al Aboud DM et

al., 2019) Here we reported a case of exogenous

Cushing’s syndrome resulting from years of topical

and oral corticosteroids misuse in a pustular psoriasis

patient.

2 CASE

A 14-year-old girl came to our outpatient clinic 5

months agowith generalized erythematous plaques

worsening3 months prior to admission. Her lesions

first appeared when she was 4-year-old and was then

self-treated with various ointments. The plaques

never fully subsided until approximately 5 years later.

Inthe age of 11, she had her first menstrual period and

the lesions flared up all over her body and scalp.Her

262

Azizah, F., Rahman, Y., Rihatmadja, R., Agustin, T., Astriningrum, R. and Rahmayunita, G.

Generalized Pustular Psoriasis in Childhood with Exogenous Cushing’s Syndrome.

DOI: 10.5220/0009986002620266

In Proceedings of the 2nd International Conference on Tropical Medicine and Infectious Disease (ICTROMI 2019), pages 262-266

ISBN: 978-989-758-469-5

mother usually bought so-called bitter antipruritic

pills from pharmacy to relieved her of the

symptoms.She continously took the medication

everyday for almost a year. The plaques recurred

temporarily and she had never had her period again

since 4 years ago.A year ago, the lesions flared up

again and she bought very potent topical steroidto

apply all over the body twice daily for about 7

months. Her lesions were not improving so she went

to a dermatologist 8 months ago and was sent for

biopsy. The histopathologyresult was psoriasis. She

was then given 8 mg methylprednisolone twice daily

along with 10 mg cetirizine and mometasonefuroate

cream. In 2018, she had topical steroids for almost 10

months and oral steroids for around 3 months.

At the time she went to our outpatient clinic, she

had moon face, her body weight was 46 kgs andher

height was only 137 cm which was short for her age

(BMI 24.5). Her skin was thin along with multiple

red-purple striae all over her body. She also had an

ulcer from a previous motorcycle accident which did

not heal completely. Multiple erythematous scaly

plaques were found on her scalp, neck, back, and

thighs (Figure 1). Dermoscopic findings show regular

dotted vessels distributed uniformly across the plaque

along with white scales (Figure 2). On her first visit,

we tapered off the oral and topicalsteroid.

Figure 1.Moon face, striae, and erythematous plaques with

scales.

Figure 2.Dermoscopy showed regular dotted vessels

(black circle) and white scales (yellow arrows).

Figure 3.Erythroderma with multiple pustules,lake of pus,

and yellow crust.

Until one month later she could not came back to our

outpatient clinic. Her medication ran out for the past

one week, both oral and topical steroid. Four months

ago, she was admitted to the emergency unit with

generalized erythematous plaques and multiple

pustules with lake of pus and yellow crust (Figure 3).

She was moderately ill and tachycardic but without

any fever. Her laboratory studies showed elevated

white blood cell count of 16,400/µL, low morning

cortisol level of 1.5 µg/dL (3.7 – 19.4), and normal

level of liver and kidney function. She was given 50

mg cyclosporine twice daily and a change course of

oral steroid to hydrocortisone under pediatric

endocrinologist’s supervision. She had

hydrocortisone 40-40 mg and 40-20 mg, every other

day and a decrease of 25% of her dose each week was

planned to tapered off. She also had petroleum jelly

and coconut oil for her scalp.

After two to four weeks of 100 mg cyclosporine

per day, her lesions were getting better but then she

experienced a flaredup which was suspected from

stress due to her school’s exam.After two months of

treatment, her lesions were not fully subsided so her

dose was titrated to 125 mg/day and her condition has

Generalized Pustular Psoriasis in Childhood with Exogenous Cushing’s Syndrome

263

improved a lot up until now (Figure 4).Currently her

oral steroid is still tapered off, changing back to

methylprednisolone 3 mg and 2 mg every other day.

Her gynecology ultrasound revealed uterus and

ovarium hypoplasia and low level of LH 0.8 mIU/mL,

normal level of FSH 6.7 mIU/mL, normal level of

estradiol 16.6 pg/mL, and normal level of anti

Mullerian hormone 1.39 ng/mL. She still has not had

her period and currently on obstetrician-gynecologist

supervision because her clinical and laboratory

studies did not add up.

Figure 4. Improvement in both moon face and skin lesions

after 3 months.

3 DISCUSSION

Pustular psoriasis is characterized clinically by

widespread yellowish sterile pustules surrounded by

erythema. The acute generalized type (von

Zumbusch) usually presents with systemic symptoms

and might evolve into erythroderma.(Al aboud DM.,

2019). Pustular psoriasis in children is very rare,

according to a review from Australia, there were only

0.6% cases with pustular variants in 1262 childhood

psoriasis patients.(Wang Q, 2017). Various

precipitating factors have been reported to trigger

acute generalized pustular psoriasis, including

corticosteroid use and withdrawal. (Hyde K et al.,

2016). Cutaneous lesions characteristics of psoriasis

vulgaris can be present before, during, or after an

acute pustular episode. In childhood psoriasis,

majority of children can be managed only with topical

treatment, such as topical steroids. .(Bronckers et al.,

2015) But when stopped abruptly after long period of

usage, corticosteroids withdrawal could triggered the

flare of generalized pustular psoriasis. In our case,

initially she had psoriasis vulgaris but later developed

generalized pustular psoriasis when her medication

stopped.

Misuse of topical steroids in children could easily

lead to systemic reactions because of their higher

ratio of total body surface area to body weight.

Topical corticosteroids were easily absorbed through

normal intact skin. Damaged or inflamed skin may

increase percutaneous absorption of the drug.

Application of steroids to large surface areas,

occlusion, vehicle, location of application, and more

potent derivatives such as clobetasol propionate,

directly increase the risk of hypotalamic-pituitary-

adrenal (HPA) axis suppression.

As little as 2 g/day

of clobetasol propionate 0.05% cream can cause a

decreased morning cortisol level after only a few

days.(Hengge UR et al., 2016). (Rahmayunita G et

al.,2008) reported a case of Cushing’s syndrome due

to misuse of topical steroid in a childhood psoriasis

but fortunately the patient was able to recover from

Cushing’s syndrome completely only with permanent

striae. In childhood, the characteristics of Cushing’s

syndrome are truncal obesity, growth failure, striae,

hypertension, appearance of dorsocervical and

supraclavicular fat pads, and moon face. Clinically,

there is no difference between exogenous or

endogenous Cushing’s syndrome. (Stratakis

CA,2018;Lodish MB et al., 2018). Hypercortisolism

is also associated with bone age advancement and

premature epiphyseal maturation. Decreased bone

mineral density, osteoporosis, and related fragility

fractures have been reported in in almost 60%

patients with glucocorticoid excess.(Raff H et al.,

2014). The likelihood of catch up growth after the

cure of Cushing’s syndrome depends on bone age

advancement at the time of diagnosis, but final height

in patients with Cushing’s syndrome in childhood is

known to be compromised. (Raff H et al., 2014). Our

patient had low morning cortisol level, moon face,

striae, growth retardation, and secondary amenorrhea.

High cortisol levels could supress gonadotropin

releasing hormone (GnRH), luteinizing hormone

(LH), and follicle stimulating hormone (FSH) release

thus ended in amenorrhea. (Raff H et al., 2014). Due

to the inconsistent laboratory and clinical data, our

patient is currently planned to have more diagnostic

tests.

To slowly tapered down exogenous steroids, our

patient was given hydrocortisone by pediatric

endocrinologist from Department of Child Health.

Hydrocortisone are the least potent glucocorticoids

but with the highest mineralocorticoid property.

Thus, hydrocortisone and cortisone are generally

preferred for use in patients with adrenal

insufficiency. (Liu D et al., 2013). Recovery from

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264

steroid-induced adrenal insufficiency is time

dependent and the adverse effects are generally

reversible with lower dosage or discontinuation of

treatment. Abrupt cessation of the drugs may

precipitate adrenocortical insufficiency, therefore the

drugs must be gradually discontinued. (Stratakis

CA,2018;Lodish MB et al., 2018). The physiologic

dose of steroids should be given for about 6-9 months

because the recovery period of HPA axis suppression

is 3.49 ± 2.92 months.(

Tempark T et al., 2010). Our

patient was tapered down slowly over the past 3

months and was planned to have her morning cortisol

level check again in May 2019, 6 months after the

initial test.

Depends on the severity, treatments available for

childhood psoriasis are emollients, salicylic acid,

calcipotriol, coal tar, topical steroids, phototherapy,

retinoids, and cyclosporine (Rahmayunita G et al.,

2008). Topical steroids are not indicated in severe

psoriasis due to the possible side effects of excessive

usage. Whereas first line treatment for erythrodermic

or pustular psoriasis is acitretin, followed by

cyclosporine, phototherapy, methotrexate, anti-TNF

agents, and systemic steroids.(Bronckers et al., 2015;

Gudjonsson JE et al, 2012). Acute GPP has proven to

be, like many psoriasis variants, a difficult disease to

treat. (Hyde K et al., 2016). Severe and extensive

disease is likely to most effectively be treated with

infliximab (TNF-α inhibitor) or cyclosporine, given

the quicker onset of action with these drugs. (Hyde K

et al., 2016). Our patient was prescribed cyclosporine

with initial dose of 100 mg daily. Cyclosporine is an

calcineurin inhibitor which inhibits the T-cell

activation mediated by antigen. (Gudjonsson JE et al,

2012) It has a rapid onset of action of 2-4 weeks and

notable side effects are nephrotoxicity, hypertension,

nausea, and diarrhea.(Bronckers et al., 2015;

Gudjonsson JE et al, 2012) To date, the efficacy of

cyclosporine in pediatric psoriasis is limited. (Hyde K

et al., 2016).

Children may require higher dosage than

recommended in adults because of greater body

surface area to weight ratio. Therefore, the lowest

possible dose and shortest treatment period should be

used.(Bronckers et al., 2015;Al Aboud DM et al et

al., 2019;Hyde K et al., 2016).

Ideally high-potency topical steroids should be

obtained only with prescription in order to prevent

misuse of topical steroids.(Sahana PK et al., 2015)

Patients and especially parents should be informed

about the appropriate usage and the possible side

effects of the drug.Children are more susceptible to

side effects, thus parents should clearly understand

how to use topical steroid wisely. One of the side

effects of steroids is immunosuppression which may

increase the risk of opportunistic and bacterial

infections. Semiz et al reported a fatal case of

disseminated cytomegalovirus infection in a

Cushing’s syndrome patient due to topical steroid use

in the diaper area.(Semiz S et al., 2008). Therefore,

topical steroids should be used wisely especially in

children.

4 CONCLUSION

Adverse effects of steroid-induced adrenal

insufficiency is generally reversible and the recovery

is time dependent thus the cessation of exogenous

steroid should be gradual. During the gradual

discontinued of exogenous steroids, systemic agents

for psoriasis ie.acitretin orcyclosporine should be

initiated. Parents should be noted that misuse of

topical and oral corticosteroids could be harmful for

children and severe immunosuppression state could

end fatally.

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