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 Table of Contents  
CASE REPORT
Year : 2014  |  Volume : 2  |  Issue : 2  |  Page : 109-112

Acute generalized exanthematous pustulosis in a patient with plaque psoriasis


1 Department of Dermatology, King Abdul Aziz Hospital, Makkah, Saudi Arabia
2 Section of Dermatology, King Abdul Aziz Medical City, National Guard Hospital, Jeddah, Saudi Arabia
3 Department of Pathology, King Abdul Aziz Medical City, National Guard Hospital, Jeddah, Saudi Arabia

Date of Web Publication18-Jul-2014

Correspondence Address:
Khalid A Al Hawsawi
House # 4148, AL-Takassosi district, Makkah, 6134 - 24323
Saudi Arabia
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DOI: 10.4103/1658-631X.137006

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  Abstract 

Acute generalized exanthematous pustulosis (AGEP) is an acute febrile drug eruption characterized by sudden occurrence of numerous, non-follicular, pinhead-sized pustules. This usually occurs 3-5 days after the commencement of treatment. Development of pustular eruptions in patient with plaque psoriasis raises the differential diagnosis of AGEP versus pustular psoriasis of the Von Zumbusch type. Herein, we report a 56-year-old male who came into hospital with history of vomiting fresh blood. The patient was then admitted for management of hematemesis. He is a known case of psoriasis vulgaris, which was still present during admission. During admission, the patient developed pustular skin eruptions associated with fever 4 days after using clindamycin and ceftriaxone. Laboratory investigations revealed leukocytosis and neutrophilia. Histopathological examination showed sub-corneal neutrophilic collection and eosinophils in the papillary dermis. The pustular eruption disappeared completely within ten days after stopping clindamycin and ceftriaxone, confirming the diagnosis of AGEP.

  Abstract in Arabic 

ملخص البحث:
يعتبر التقيح الطفحى الحاد الشامل للجلد حالة حادة مصحوبة بحمى بسبب التفاعل الدوائي. وتتميز هذه الحالة بظهور مفاجئ للعديد من الحبيبات المتقيحة الصغيرة. وتحدث هذه عادة بعد ثلاثة إلى خمسة أيام بعد بدء العلاج. ظهور هذه الحبيبات المتقيحة لدى المرضى المصابين بالصداف اللويحي يدعو للتشخيص التفريقي بين حالات التقيح الطفحى الحاد وحالات الصداف القيحى من نوع فون زونبش.
يعرض الباحثون حالة لرجل في السادسة والخمسين من عمره حضر للمستشفى يشكو من تقيؤ دموي. وقد تم تنويمه بالمستشفى للعلاج. المريض كان مشخصًا كحالة صدفية فلجارس وكان مازال يعاني منها عند تنويمه اثناء وجوده بالمستشفى. وبعد أربعة أيام من استخدامه كليندامايسين وسيفتريكاسون عانى المريض من ظهور بثور متقيحة في الجلد. وقد بينت الفحوصات المخبرية ازديادًا ملحوظًا في كريات الدم البيضاء مع انخفاض في كريات النيوترفيل. كما بين فحص الانسجة المجهري تجمع كريات النيوترفيل تحت القرنية والكريات الحمضية وفي طبقات الجلد الحليمية. وقد اختفت البثور المتقيحة تماما خلال عشرة أيام من ايقاف العلاج مما أكد تشخيص الحالة كتقيح طفحى حاد شامل.

Keywords: Acute generalized exanthematous pustulosis, Pustular drug eruption, Toxic pustuloderma.


How to cite this article:
Al Hawsawi KA, Jan HD, Al Raddadi AA, Alharbi AH, Al Jabri MM, Satti MB. Acute generalized exanthematous pustulosis in a patient with plaque psoriasis. Saudi J Med Med Sci 2014;2:109-12

How to cite this URL:
Al Hawsawi KA, Jan HD, Al Raddadi AA, Alharbi AH, Al Jabri MM, Satti MB. Acute generalized exanthematous pustulosis in a patient with plaque psoriasis. Saudi J Med Med Sci [serial online] 2014 [cited 2020 Nov 29];2:109-12. Available from: https://www.sjmms.net/text.asp?2014/2/2/109/137006


  Introduction Top


Acute generalized exanthematous pustulosis (AGEP) is not uncommon cutaneous reaction pattern that is, in more than 90% of cases, related to medication administration. It is characterized by the sudden occurrence of numerous non-follicular pustules; this usually occurs 3-5 days after the commencement of medication.

Occasionally, there are other causes such as viral infections, hypersensitivity to mercury, ingestion of lacquer chicken, chromic picolinate, as well as exposure to icodextrin during peritoneal dialysis. [1]

Dozens of medications have been suspected of causing AGEP. The drugs conferring the highest risk of AGEP are aminopenicillins, pristinamycin, hydroxychloroquine, antibacterial sulfonamides, terbinafine, and diltiazem. [2]


  Case report Top


A 56-year-old male came into hospital with history of vomiting fresh blood. The patient was then admitted into hospital for management of hematemesis. He was a known case of diabetes mellitus on insulin treatment and psoriasis vulgaris on topical treatment for years, which were still present during admission. Patient was not regular on his topical treatment (betamethasone -calcipotriol ointment, betamethasone valerate 0.025% ointment, and urea 30% cream), and he stopped them for more than one month before his admission to the hospital. Endoscopy was performed, and sclerotherapy was done for small pre-pyloric ulcer; homeostasis was successful. Two days after endoscopy, the patient suddenly got a cardiac arrest. He was resuscitated but entered into coma, so he was shifted to the intensive care unit. Few days later, the patient's condition deteriorated and he developed fever, hypotension, and renal impairment. He was then started on clindamycin 900 mg IV q 12 hr and ceftriaxone 500 mg IV q 12 hr empirically as a treatment of sepsis. Noradrenalin IV infusion was also started. Four days after starting clindamycin and ceftriaxone, patient developed generalized skin rash. Clinical examination revealed a comatosed patient. Vital signs revealed spikes of high temperature of up to 38.8°C, low blood pressure 90/60 mm Hg, normal respiratory rate 16 (normal range 12-20), and normal pulse rate 90 (normal range 60-100). Skin examination revealed hundreds of non-follicular pinhead pustules on erythematous background all over the body with presence of multiple silvery scaly plaques scattered on his body [Figure 1]. Nails showed onycholysis and subungual hyperkeratosis. Skin biopsy from the pustule was taken. Complete blood count (CBC) showed leukocytosis 14, 6 × 10 9 /L (normal range 4-11 × 10 9 /L) and neutrophilia 12.3 × 10 9 /L (normal range 2.5-7.5 × 10 9 /L). The other CBC parameters were within normal limits (WNL) apart from mild microcytic hypochromic anemia. The blood chemistry revealed high urea level of 60 mg/dL (normal range 8 to 24 mg/dL), high creatinine level of 6.2 mg/dL (normal range 0.6-1.2 mg/dL), electrolytes and liver enzymes were WNL. Bacterial and fungal cultures of the patient's blood and pustules were negative. Based on the previous clinical findings, a preliminary diagnosis of AGEP was made. The clindamycin and ceftriaxone were discontinued, and were replaced by pipracillin / tazobactam 2 gm/0.25 mg I.V q 6 hrs. Within two days of discontinuing clindamycin and ceftriaxone, the pustules started to disappear. The histopathological examination showed sub-corneal neutrophilic collection with mild spongiosis in the epidermis and dense interstitial mixed inflammatory cellular infiltrates with a predominance of neutrophils and eosinophils in the papillary dermis [Figure 2]. Ten days after discontinuation of clindamycin and ceftriaxone, all pustules disappeared and left desquamation. Based on the clinical and histopathological findings, a diagnosis of AGEP was confirmed. Unfortunately, few days later, the patient passed away secondary to cardiac arrest as a complication of acute renal failure.
Figure 1: Multiple non-follicular pustules on the chest and axilla (a), and scaly erythemarous plaques on the abdomen

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Figure 2: Histopathological fi ndings showed sub-corneal neutrophilic collection with mild spongiosis in the epidermis (a) and dense interstitial mixed infl ammatory cellular infiltrate with predominance of neutrophils and eosinophils in the papillary dermis (b). Hematoxylin and eosin, original magnification (a) × 10: (b) × 40

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  Discussion Top


Although previous reports indicated that some patients with AGEP have history of plaque psoriasis, considering AGEP is a pustular form of psoriasis caused by a variety of exogenous triggers, including medications. This was not totally significant. In relation to this, a controlled study of 97 cases of AGEP carried out by the European Study of Severe Cutaneous Adverse Reactions (EuroSCAR) strongly suggests that AGEP is different from psoriasis. The study also showed that the drugs that had been recognized as inducers of psoriasis, e.g. beta-blockers or angiotensin converting enzyme (ACE) inhibitors, were not associated with AGEP. [1],[2]

The pustules of AGEP are clinically and histologically resemble those of pustular psoriasis However, there are some clinical, hematological, and histopathological findings that may be helpful in differentiating between AGEP and pustular psoriasis. However, these findings are neither specific nor exclusive of either condition. [Table 1] shows these differentiating points. [1],[2],[3],[4],[5],[6]

The diagnostic criteria of AGEP proposed by Sideroff et al. include:
Table 1: The differences between AGEP and pustular psoriasis of the Von Zumbusch type

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  1. Numerous small (<5 mm), non-follicular pustules arising on a widespread edematous erythema,
  2. Pathology reveals intra-epidermal/sub-corneal pustules associated with one or more of the following: Dermal edema, vasculitis, perivascular eosinophils, focal necrosis of keratinocytes,
  3. Fever > 38°, 4) Blood neutophil count > 7 × 100/L, and 5) Acute progression with spontaneous recovery within 15 days. [3]


A patient with plaque psoriasis who develops generalized pustular eruptions that were preceded by drug intake raises the differential diagnosis of AGEP versus pustular psoriasis of the Von Zumbusch type (PPVZT) whether drug-induced or not. The preceding drug intake might not be significant and all what the patient has is an idiopathic PPVZT, so applying all diagnostic criteria of AGEP would be mandatory for the diagnosis. In AGEP, the latency period between drug exposure and appearance of the eruption is typically short, whereas in drug-induced PPVZT, the latency period is long. However, if the patient is on many medications, it would be difficult to know whether the latency period is short due to a newly administered drug or long due to a lately administered drug. The drugs that induce pustular psoriasis are just a precipitating factor and not a causative factor, so stopping the offending drug does not necessarily lead to cure of the disease. All AGEP criteria can be seen in PPVZT, except part of the 5th criterion "spontaneous recovery within 15 days," which is specific for AGEP and not pustular psoriasis.


  Conclusion Top


Our patient fulfilled all diagnostic criteria of AGEP, although fever and neutrophilia can be caused by sepsis or AGEP or both. Recently, there has been a similar case report to our case, which was the occurrence of AGEP in a patient with plaque psoriasis, but unlike our patient, the offending medication was etanercept. [7]

 
  References Top

1.Revuz J, Valeyrie-Allanore L. Drug Reactions. In: Bolognia JL, Jorizzo JL, Schaffer JV. Dermatology, 3 rd ed. Phladelphia: Elsevier-Saunders; 2013. p. 335-56.  Back to cited text no. 1
    
2.Sidoroff A, Dunant A, Viboud C, Halevy S, Bavinck JN, Naldi L, et al. Risk factors for acute generalized exanthematous pustulosis (AGEP) - results of a multinational case-control study (EuroSCAR). Br J Dermatol 2007;157:989-96.  Back to cited text no. 2
    
3.Sidoroff A, Halevy S, Bavinck JN, Vaillant L, Roujeau JC. Acute generalized exanthematous pustulosis (AGEP) - a clinical reaction pattern. J Cutaneous Pathol 2001;28:113-9.  Back to cited text no. 3
    
4.Walsh S, Creamer D. A diagnostic challenge: Acute generalized exanthematous pustulosis or pustular psoriasis due to terbinafine: Comment. Clin Exp Dermatol 2012;37:919.  Back to cited text no. 4
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5.Duckworth L, Maheshwari MB, Thomson MA. A diagnostic challenge: Acute generalized exanthematous pustulosis or pustular psoriasis due to terbinafine. Clin Exp Dermatol 2012;37:24-7.  Back to cited text no. 5
    
6.Gudjonsson JE, Elder JT. Psoriasis. In: Wolf K, Goldsmith LA, Katz SI, Gilchrest BA, Paller AS, Leffell DJ. Fitzpatrick's Dermatology in General Medicine, 7 th ed. New York: McGraw-Hill; 2008. p.169-93.  Back to cited text no. 6
    
7.Kavala M, Zindancı I, Türkoglu Z, Can B, Kocatürk E, Senol S, et al. Acute generalized exanthematous pustulosis induced by etanercept: Another dermatologic adverse effect. Case Rep Dermatol Med 2013;2013:601412.  Back to cited text no. 7
    


    Figures

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