Year : 2007 | Volume
: 52 | Issue : 4 | Page : 209--210
Generalized pustular psoriasis masquerading as subcorneal pustular dermatosis in a child
Raghavendra Rao, Smitha Prabhu, H Sripathi, SD Shenoi
Department of Dermatology, Kasturba Medical College, Manipal - 576 104, India
Department of Dermatology, Kasturba Medical College, Manipal - 576 104
|How to cite this article:|
Rao R, Prabhu S, Sripathi H, Shenoi S D. Generalized pustular psoriasis masquerading as subcorneal pustular dermatosis in a child.Indian J Dermatol 2007;52:209-210
|How to cite this URL:|
Rao R, Prabhu S, Sripathi H, Shenoi S D. Generalized pustular psoriasis masquerading as subcorneal pustular dermatosis in a child. Indian J Dermatol [serial online] 2007 [cited 2020 May 28 ];52:209-210
Available from: http://www.e-ijd.org/text.asp?2007/52/4/209/37731
Generalized pustular eruption in a child can be a diagnostic challenge. Various dermatoses which present with generalized pustular lesions include pustular psoriasis, subcorneal pustular dermatosis (SCPD), staphylococcal scalded skin syndrome (SSSS) and acute generalized exanthematous pustulosis (AGEP).  We describe a case of generalized pustular psoriasis who presented to us with features of subcorneal pustular dermatosis.
A 13-year-old female presented with a complaint of generalized pustular eruption of one-month duration. It started as papulovesicular lesions, which later became pustules. This was associated with low-grade, intermittent fever. She denied history of sore throat or drug intake prior to the appearance of rash. Cutaneous examination revealed the presence of multiple pustules on an erythematous background over the scalp, face, trunk and extremities [Figure 1]. A few pustules characteristically showed accumulation of pus in the dependent part, a feature typical of SCPD [Figure 2]. Routine hemogram showed polymorphonuclear leukocytosis and a raised ESR (44 mm at the end of 1 h). Gram staining and culture from the pus were negative. Skin biopsy from the lesion showed subcorneal and spongiform pustules in the upper epidermis and dilated vessels in the papillary dermis, surrounded by mixed infiltrate, confirming the diagnosis of pustular psoriasis [Figure 3]. Direct and indirect immunofluorescence tests were negative. She was commenced on dapsone (1 mg/kg/day); though she responded initially, new lesions continued to appear. Prednisolone (1 mg/kg/day) was then added, resulting in complete clearance of the lesions in one week's time. It was tapered by 5 mg every week and stopped after one month. Dapsone was continued for three months.
Acute generalized pustular psoriasis (GPP) is a rare, sometimes fatal, exudative form of psoriasis that is commonly seen in adults. Its occurrence in children is very rare and less than 200 cases have been reported in the literature.  When the disease begins in children, it usually begins in the first year of life, but may begin at any age during the childhood. It is more common among boys and about 25% of the children usually give a positive family history of psoriasis.  Beylot et al . have classified their patients of GPP into three clinical groups: Acute GPP (von Zumbusch type), annular type and the mixed type.  Children with acute GPP present with sterile pustules on an inflamed, erythematous skin. The pustule may remain discrete or quickly coalesce to produce lake of pus before drying over a three to four-day period with pronounced desquamation.  Successive waves of pustules may develop in a severely affected child over several weeks. The child may be symptomatically well or may have intermittent fever, malaise and joint pain. The annular form is the commonest type and is characterized by scattered, well-defined annular plaques; pustules and scaling are scattered over the surface or present along the advancing edge of the plaque. The mixed type is a combination of the two. Unlike the adult forms, juvenile GPP tends to have a more benign course and is usually not associated with systemic symptoms. 
Subcorneal pustular dermatosis is a rare, chronic recurrent pustular eruption characterized histologically by subcorneal pustules that contain abundant polymorphonuclear leukocytes. It is more common in females with the average age of onset between 40-50 years.  Few cases have been reported in children; diseases in them behave like in adults.  There is dispute over the exact nosological position of SCPD and its differentiation from pustular psoriasis, both clinically and histopathologically is said to be difficult. Sanchez et al ., in their long-term (more than 15 years) follow-up study have observed that many cases of childhood onset of SCPD eventually develop GPP. So, they concluded that SCPD might simply be a part of the spectrum of pustular psoriasis.  Our patient presented with clinical features typical of SCPD. However, on histopathological examinations, the presence of subcorneal abscess and spongiform pustules confirmed the diagnosis of GPP.
Various therapies that have been found to be useful in GPP include wet dressing, topical and systemic steroids, dapsone, methotrexate, cyclosporine, retinoids and phototherapy (both PUVA and NBUVB). , Biologicals have been used in recent times with good effects. , Yu et al . successfully treated a juvenile GPP with dapsone.  Response of GPP to dapsone may not be as dramatic as compared to SCPD. Our patient had extensive lesions at the time of initial presentation and did not respond to dapsone. Rapid clearance was seen when a short course of systemic steroids was added.
|1||Hogan PA. Papulosquamous diseases. In : Schachner LA, Hansen RC, editors. Pediatric dermatology, 3 rd ed. Mosby: Edinburgh; 2003. p. 652-4.|
|2||Cassandra M, Conte E, Cortez B. Childhood pustular psoriasis elicited by streptococcal antigen: A case report and review of literature. Pediatr Dermatol 2003;20:506-10.|
|3||Zelickson BD, Muller SA. Generalized pustular psoriasis in childhood. J Am Acad Dermatol 1991;24:186-94.|
|4||Beylot C, Bioulac P Grupper C, et al . Generalized pustular psoriasis in infants and children: Report of 27 cases. In : Psoriasis, Farber EM, Cox AJ, editors. Proceedings of the 2 nd international symposium. Yorke Medical Books: New York; 1977. p. 171-9.|
|5||Honigsmann H, Trautinger F, Wolff K. Subcorneal pustular dermatosis. In : Freedberg IM, Eisen AZ, Wolff K, Austen F K, Goldsmith LA, Katz SI, editors. Fitzpatrick's dermatology in general medicine, 6 th ed. McGraw-Hill: New York; 2003. p. 625-7.|
|6||Kocak M, Birol A, Erkek E, Bozdoπan O, Atasoy P. Juvenile subcorneal pustular dermatosis: A case report. Pediatr Dermatol 2003;20:57-9.|
|7||Sanchez N, Ackerman AB. Subcorneal pustular dermatosis: A variant of pustular psoriasis. Acta Derma Venereol Suppl (Stockh) 1979;59:147-51.|
|8||Ozawa A, Ohkido M, Haruki Y, Kobayashi H, Ohkawara A, Ohno Y, et al . Treatment of generalized pustular psoriasis: A multicenter study in Japan. J Dermatol 1999;26:141-9.|
|9||Trent JT, Kerdel FA. Successful treatment of Von Zumbusch pustular psoriasis with infliximab. J Cutan Med Surg 2004;8:224-8.|
|10||Callen JP, Jackson JH. Adalimumab effectively controlled recalcitrant generalized pustular psoriasis in an adolescent. J Dermatolog Treat 2005;16:350-2.|
|11||Yu HJ, Park JW, Park JM, Hwang DK, Park YW. A case of generalized pustular psoriasis treated with dapsone. J Dermatol 2001;28:316-9.|