Dr Theresa Raju*Dr Sajitha Nair *Dr Gopikrishnan Anjaneyan * Dr C Jayakumar*
Department of Pediatrics* and Dermatology**AIMS KOCHI
Fifteen year-old male boy presented with erythema, scaling and flaking of skin from 20 days of life managed with topical applications developed high-grade intermittent fever and cough of 7 days duration. He came to AIMS as fever and worsening of skin lesions after Ayurvedic and treatment from a near hospital (picture:1)
Picture 1
Clinically he had pustular lesions over face, neck, abdomen and bilateral upper extremities. There was erythema and scaling over bilateral lower extremities with thick scaling over the scalp, nail pitting, erythema and mild exfoliations over palms and soles. Systemic examination was normal
Labs TC:19.9K/Ul, N:85.7%, L:9.5%) with microcytic hypochromic anaemia
CRP of 107.41mg/L
Procalcitonin of 8.07ng/ml.
RFT and TFT were normal.
LFT TSB(3.35mg/dl), DSB(0.42mg/dl), ALP (478.0IU/L) with normal transaminases. Peripheral smear showed microcytic hypochromic RBCs with neutrophilic leucocytosis with shift to left.
DCT was negative.
Serum calcium(6.16mg/dl) and phosphorus were low with raised PTH and low 25OH Vitamin D.
CXR done showed increased bronchovascular markings
ABG at admission was normal.
After stabilisation in paediatric HDU 10% calcium gluconate was given followed calciriol and shelcal.
skin biopsy was taken.
Topicals (saline compression, hydrocortisone cream, liquid paraffin and emollients) were started
Gram stain from the lesion were negative. After sending cultures from pus, blood and urine, he was started on Inj Ceftriaxone and clindamycin.
Over the next 24 hours, the child showed rapid worsening of skin lesions.
There was exfoliation and peeling of skin associated with erythema. There was also conjunctival congestion and mild erythema of oral cavity(picture 2 and 3)
Picture 2
Picture 3
Differentials
Atopic dermatitis with secondary bacterial infection
Exanthematous pustulosis
Icthosiform erythroderma
Generalised pustular psoriasis
Repeat labs showed increasing counts (TC: 32.08K/Ul , N: 82.1%, L:12.6%) and CRP(260mg/L) and procalcitonin 23.14.
Inj ceftriaxone was hiked into Inj Piptaz and clindamycin was continued. His retina was normal
Pus/blood/urine culture were sterile.
At this juncture,Inj Dexamethasone was added. and he showed signs of improvement both clinically and lab wise Skin biopsy showed features of Psoriasiform dermatitis favouring Psoriasis vulgaris.
After clinical improvement.Dexa was changed to oral prednisolone
He was started with acitretin (Retinoids and VIT A-derivative) prior to discharge.
Whole exome sequencing was done and result awaited
Antibiotics were withdrawn after 10 days
Need for multidisciplinary consultation follow up was counselled
Skin lesions had settled except for erythema and mild scaling
Pustular Psoriasis
It is a chronic inflammatory disease that classically affects the skin and joints.
Plaque psoriasis is the most common form of psoriasis vulgaris.
Psoriasis can also involve the nails and scalp or progress to erythroderma.
Generalized pustular psoriasis(GPP) is also known as Von Zumbusch type.
65% of cases occur in patients with prior diagnosis of psoriasis vulgaris.
It is characterized generalised sterile pustule formation with widespread inflammation and erythema. The pustules often expand and coalesce forming lakes of pus. Acute GPP is also associated with systemic symptoms such as fever, chills, malaise, anorexia, nausea and severe pain. Other findings include subungual pustules and geographic tongue. Systemic nature of the disease is reflected in laboratory abnormalities including hypocalcemia and hypoalbuminemia.
Pathophysiology: Various precipitating factor have been reported to trigger or flare acute GPP, such as corticosteroids use, impetigo herpetiformis, upper respiratory tract infection, non-steroidal anti-inflammatory drugs, terbinafine, tumor necrosis factor alpha inhibitor.
Treatment: The Medical Board of National Psoriasis Foundation recommended acitretin, cyclosporine, or methotrexate. Severe and extensive disease is likely to be most effectively be treated with infliximab or cyclosporine. Recommended second like therapies include adalimumab, etanercept, topical corticosteroids, topical calcipotriene and topical tacrolimus. Anakinra, an IL-1 receptor antagonist has been described to successfully treat GPP and gevokizumab an IL-1 beta inhibitor showed some promise in treating two patients with GPP. The unique treatment Of GPP with granulocyte and monocyte adsorption aphresis has also been described.
Take home message
Pustular psoriasis is an eminently treatable condition and multidisciplinary treatment optimises the management