|Year : 2015 | Volume
| Issue : 4 | Page : 252-254
Juvenile generalized pustular psoriasis in a 10-year-old girl
Yugal K Sharma, Meenakshi Wadhokar, Kirti Deo, Sanjeev Gupta, Neha Tyagi
Department of Dermatology, Dr. D.Y. Patil Medical College and Hospital, Pimpri, Pune, Maharashtra, India
|Date of Web Publication||24-Sep-2015|
Queenstown Society, M 704, Behind Chinchwad Railway Station, Chinchwad, Pune - 411 033, Maharashtra
Source of Support: None, Conflict of Interest: None
Children ≤18 years of age have an overall prevalence of psoriasis ≈0.71%, the pustular type occurring in 0.6-7%. Out of the four distinct patterns of pustular psoriasis, annular type is the rarest and generalized pustular psoriasis/von Zumbusch (GPP), the severest. A 10-year-old girl presented with extensive crusted erosions-ostensibly a day after consumption of a solitary 250 mg tablet of azithromycin-over erythematous base symmetrically involving face, trunk, and extremities followed next day by innumerable pin-head sized pustules coalescing into lakes of pus. GPP was suspected clinically and corroborated histopathologically. The diagnostic criteria for GPP are, firstly; clinical findings as mentioned above, secondly; subcorneal spongiform pustules on histopathology, thirdly; any/ many of the laboratory alterations (leukocytosis with left shift, elevated ESR, C-reactive protein, antistreptolysin O antibodies, IgG or IgA, hypoproteinemia, hypocalcemia) and lastly; clinical/histopathological recurrence. After three days of prednisolone, a short course of cyclosporine followed by NB-UVB revealed an exceptionally prompt remission in this, probable de novo, case of pso- GPP without any family history which is being kept under follow up.
Keywords: Generalized pustular psoriasis, juvenile generalized pustular psoriasis, pustular psoriasis
|How to cite this article:|
Sharma YK, Wadhokar M, Deo K, Gupta S, Tyagi N. Juvenile generalized pustular psoriasis in a 10-year-old girl. Indian J Paediatr Dermatol 2015;16:252-4
|How to cite this URL:|
Sharma YK, Wadhokar M, Deo K, Gupta S, Tyagi N. Juvenile generalized pustular psoriasis in a 10-year-old girl. Indian J Paediatr Dermatol [serial online] 2015 [cited 2020 Sep 19];16:252-4. Available from: http://www.ijpd.in/text.asp?2015/16/4/252/165638
| Introduction|| |
Psoriasis affects approximately 1-3% of the population globally, 45% developing lesions before 20 years of age. Children ≤18 years of age have an overall prevalence of psoriasis ≈0.71%, the pustular type accounting for 0.6-7% of the patients.  The childhood onset and adult onset disease have some contrasting epidemiological and clinical features [Table 1].  Out of four distinct patterns of pustular psoriasis (PP), annular type is the rarest and generalized pustular psoriasis/von Zumbusch (GPP) is the severest - sometimes, even life-threatening; exanthematic, and localized PP are its remaining types.  Around 200 reports of GPP exist in the literature.  It presents in children aged 6-10 years, usually abruptly with widespread erythematous patches, followed by superficial yellow, confluent, sterile "pustules" along with constitutional symptoms of acute illness in some. Its differential diagnosis includes subcorneal pustular dermatosis, pustular drug eruptions, staphylococcal scalded skin syndrome and acute generalized exanthematous pustulosis (AGEP).
|Table 1: Comparison between childhood-onset psoriasis and adult-onset psoriasis |
Click here to view
| Case report|| |
A 10-year-old girl patient was brought by her father to our outpatients department with extensive (≈60%) eruptions over the face, trunk, and extremities revealing crusted erosions over erythematous base [Figure 1]a]. The lesions commenced ostensibly within 24 h of consuming a 250 mg azithromycin tablet for a single pustule over the right earlobe. Sore throat, cough, malaise and "feverishness" were present since a week. There was no past or family history of any dermatoses including psoriasis. She had tachycardia (110/min) and pyrexia (101°F). Nails and mucosae were uninvolved. Erythrocyte sedimentation rate (ESR) was 41 mm and leukocyte count, 12,000 cells/cmm. Suspecting drug rash, oral prednisolone (40 mg) was administered. Innumerable pin-head sized pustules coalescing into lakes of pus [Figure 1]b] manifested next day; cyclosporine (3 mg/kg/day) was now added, suspecting GPP. Pus/blood cultures were sterile. Presence of subcorneal spongiform pustule filled with neutrophils, and absence of papillary dermal edema/perivascular infiltrate on skin histopathology corroborated GPP and excluded AGEP [Figure 1]c]. On the 4 th day patient became afebrile, prednisolone was withdrawn, and cyclosporine tapered to 2 mg/kg/day. The latter, being unaffordable, was withdrawn after another week and the patient advised narrow-band ultraviolet B (NB-UVB) therapy as an outpatient. The follow-up after a fortnight revealed residual erythema and fine scaling [Figure 1]d].
|Figure 1: (a) Extensive erythema studded with thinly crusted erosions. (b) Innumerable, pin-head sized, pustules coalesced into lakes of pus. (c) Periphery of the pustule showing epidermal spongiosis (H and E, ×400). (d) Residual erythema and fine scaling after treatment with cyclosporine|
Click here to view
| Discussion|| |
The diagnostic criteria for GPP  are, firstly; clinical findings as mentioned above, secondly; subcorneal spongiform pustules on histopathology, thirdly; any/many of the laboratory alterations (leukocytosis with left shift, elevated ESR, C-reactive protein, antistreptolysin O antibodies, IgG or IgA, hypoproteinemia, hypocalcemia) and lastly; recurrence clinico-histopathologically. Its patients may have suffered from psoriasis vulgaris (pso + GPP) or may not have (pso− GPP) [Table 2].  Beylot et al.  classified GPP clinically into acute, annular and mixed types; only some of the patients of the acute type being ill with intermittent fever, malaise, and arthralgia, others remaining symptomatically well; all develop successive waves of discrete "pustules" on an inflamed, erythematous skin, likely coalescing soon into lakes of pus before drying off and desquamating profuse, thin scales. Annular, the most common type, has well-developed annular plaques, pustules, and scales. The mixed type combines features of the two. Cyclosporine is one of the first-line systemic therapies, others being acitretin, methotrexate, and glucocorticosteroids; the last resorted rarely for acute stage/life-threateningly severe disease. In recalcitrant cases, etanercept (0.4 mg/kg biweekly) is considered the first line, adalimumab and infliximab being other immunobiological options. UVB phototherapy and dapsone, too, have been used successfully. 
|Table 2: Comparison between patients of GPP* with/ without personal history of psoriasis |
Click here to view
Recently an autosomal recessively inherited familial form of GPP due to a mutation in the gene encoding interleukin-36R antagonist located on chromosome 2q13-q14.1 has also been described. 
We are keeping this patient under follow up so as not to miss any spontaneous relapse and thereby the unequivocal confirmation of GPP in this, otherwise de novo case of pso− GPP without any family history and with an exceptionally prompt remission.
Financial Support and Sponsorship
Conflicts of Interest
There are no conflicts of interest.
| References|| |
Popadic S, Nikolic M. Pustular psoriasis in childhood and adolescence: A 20-year single-center experience. Pediatr Dermatol 2014;31:575-9.
Dogra S, Dipankar DE. Childhood psoriasis. In: Inamdar A, Palit A, Raghunatha S, editors. Textbook of Pediatric Dermatology. 2 nd
ed. New Delhi: Jaypee Brothers; 2014. p. 363.
Posso-De Los Rios CJ, Pope E, Lara-Corrales I. A systematic review of systemic medications for pustular psoriasis in pediatrics. Pediatr Dermatol 2014;31:430-9.
Xiao T, Li B, He CD, Chen HD. Juvenile generalized pustular psoriasis. J Dermatol 2007;34:573-6.
Robinson A, Van Voorhees AS, Hsu S, Korman NJ, Lebwohl MG, Bebo BF Jr, et al.
Treatment of pustular psoriasis: From the Medical Board of the National Psoriasis Foundation. J Am Acad Dermatol 2012;67:279-88.
Rao R, Prabhu S, Sripathi H, Shenoi SD. Generalized pustular psoriasis masquerading as subcorneal pustular dermatosis in a child. Indian J Dermatol 2007;52:209-10.
Marrakchi S, Guigue P, Renshaw BR, Puel A, Pei XY, Fraitag S, et al.
Interleukin-36-receptor antagonist deficiency and generalized pustular psoriasis. N Engl J Med 2011;365:620-8.
[Table 1], [Table 2]