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LETTER TO EDITOR
Year : 2019  |  Volume : 20  |  Issue : 3  |  Page : 280-281

Pyoderma gangrenosum: Misdiagnosis leading to mismanagement


Department of Dermatology, T.N.M.C. and B.Y.L. Nair Ch. Hospital, Mumbai, Maharashtra, India

Date of Web Publication28-Jun-2019

Correspondence Address:
Dr. Uddhao S Zambare
Department of Dermatology, OPD No. 14, 2nd Floor, OPD Building, T.N.M.C. and B.Y.L. Nair Ch. Hospital, Mumbai Central, Mumbai - 400 008, Maharashtra
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/ijpd.IJPD_91_18

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How to cite this article:
Zambare US, Tambe SA, Nayak CS. Pyoderma gangrenosum: Misdiagnosis leading to mismanagement. Indian J Paediatr Dermatol 2019;20:280-1

How to cite this URL:
Zambare US, Tambe SA, Nayak CS. Pyoderma gangrenosum: Misdiagnosis leading to mismanagement. Indian J Paediatr Dermatol [serial online] 2019 [cited 2019 Sep 19];20:280-1. Available from: http://www.ijpd.in/text.asp?2019/20/3/280/261880



Sir,

Pyoderma gangrenosum (PG), a rare inflammatory neutrophilic dermatosis characterized by ulceration of the skin, is often associated with an underlying systemic disorder. Inflammatory pustules which enlarge and ulcerate with undermined bluish-to-purple borders, healing with cribriform scarring, characterize PG.[1] Because of the unavailability of specific diagnostic tests, it is often misdiagnosed leading to mismanagement.

A 15-year-old female with 2-month history of raw lesions on the chest and right arm was diagnosed as necrotising fasciitis (NF) by general surgeons and prescribed intravenous antibiotics, injection low-molecular-weight heparin 0.4 mg subcutaneously followed by oral warfarin for right cephalic and basalic vein thrombosis detected on Doppler. Chest ulcer-edge biopsy was negative for acid-fast bacilli and malignancy. Skin grafting after debridement of ulcers with grafts from the right thigh failed, and there was past history of similar lesions in 2002 and 2006 diagnosed as NF and managed conservatively.

There were two large crusted oozing ulcers measuring 5 cm × 7 cm on the medial aspect of the right arm and chest [Figure 1]a and [Figure 1]b and multiple ulcerations on the right thigh [Figure 1]c. Pathergy phenomenon was seen as abscess at central line insertion site on the neck [Figure 1]d and intramuscular injection site on the buttock [Figure 1]e. The patient had hemoglobin of 5.9 g/dl, marked leukocytosis of 40,187/cmm, raised blood urea nitrogen of 84 mg/dl, creatinine of 3.5 mg %, and high levels of C-reactive protein. Pus culture grew methicillin-resistant Staphylococcus aureus and Klebsiella species.
Figure 1: Crusted on the right arm and chest (a and b), multiple ulcerations on the right thigh (c). Abscess at central line insertion site on the neck (d) and intramuscular injection site on the buttock (e)

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Histopathology from the chest ulcer edge showed focally atrophic and spongiotic epidermis and mixed inflammatory infiltrate containing lymphocytes, neutrophils, and eosinophils in dermis and subcutaneous tissue confirming PG [Figure 2]a and [Figure 2]b. The patient improved in 12 weeks with oral antibiotics (injection amoxycillin and clavulanic acid 1.2 g 12 hourly for 10 days) and oral corticosteroids (prednisolone 25 mg/day and tapered slowly by 5 mg every 15 days) and supportive therapy along with potassium permanganate soaks and dressing. All lesions healed with scar formation [Figure 3]a, [Figure 3]b, [Figure 3]c.
Figure 2: (a and b) Lamellated stratum corneum overlying focally atrophic and spongiotic epidermis with mixed inflammatory infiltrate containing lymphocytes, neutrophils, and eosinophils in dermis and subcutaneous tissues (H and E, Figure 2a: 100x, 2b: 400x)

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Figure 3: (a-c) Complete healing of the chest, right arm, and right thigh lesions with scar formation after 12 weeks of treatment

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Brocq[2],[3] and Brunsting[1] in 1930 have documented PG. Histopathological findings are supportive, not diagnostic in establishing the diagnosis of PG. Diagnostic criteria of Su et al.[4] include two major and at least two minor criteria. Major criteria include characteristic appearance of an irregular painful ulcer with violaceous border without any other causes of ulceration-like vasculitis and infections. Minor criteria include a neutrophilic dermatosis on histopathology, history of pathergy, associated systemic diseases, and prompt response to steroids.[4] Our patient fulfilled two major and three minor criteria.

PG is a rare (3 cases/1,000,000/year[2]) inflammatory neutrophilic dermatosis seen predominantly in females aged 40–60 years. Ulcerative, pustular, bullous, and vegetative are four clinical variants.[2] Fifty percent of cases are associated with systemic diseases such as inflammatory bowel disease, leukemia, myeloma, IgA monoclonal gammopathy, chronic hepatitis, HIV infection, arteritis, and arthritis.[1]

Postoperative and peristomal PG are pathergy-induced ulcerative PG.[2] Treatment options are systemic steroids, cyclosporine, infliximab, clofazimine and local care with topical steroid ointments, tacrolimus, intralesional steroids, hyperbaric oxygen therapy, etc.[1] Differential diagnoses include NF, venous stasis ulceration, vasculitis, bacterial infections, deep fungal infections, and mycobacterial and atypical mycobacterial infections.[2] NF, an acute necrotizing painful infection involving fascia which may extend deeper to involve subcutaneous fat and tissues, requires urgent surgical debridement under appropriate antibiotic cover.[5]

NF and PG, which is often mistaken for NF,[6] are two entirely different conditions with similar clinical presentation. Misdiagnosis leads to increased morbidity, prolonged hospital stay, and economic burden to the patient. The physician's ability to distinguish these two conditions can make a world of difference to the patient.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.



 
  References Top

1.
William DJ, Timothy GB, Dirk ME. Erythema and urticaria. In: Andrew's Diseases of The Skin Clinical Dermatology. 11th ed. China: Saunders Elsevier Publishing; 2011. p. 145-7.  Back to cited text no. 1
    
2.
Frank CP, Bridget C. Pyoderma gangrenosum. In: Lowella AG, Stephen IK, Barbara AG, Amy SP, David JL, Klaus W et al., editors. Fitzpatrick's Dermatology in General Medicine. 8th ed. United States: The McGraw Hill Companies; 2012. p. 371-9.  Back to cited text no. 2
    
3.
Brocq L. A new contribution to the study of geometric phagedenism. Ann Dermatol Syphiligr 1916;9:1-39.  Back to cited text no. 3
    
4.
Su WP, Davis MD, Weenig RH, Powell FC, Perry HO. Pyoderma gangrenosum: Clinicopathologic correlation and proposed diagnostic criteria. Int J Dermatol 2004;43:790-800.  Back to cited text no. 4
    
5.
William DJ, Timothy GB, Dirk ME. Bacterial infections. In: Andrew's Diseases of the Skin Clinical Dermatology. 11th ed. China: Saunders Elsevier Publishing; 2011. p. 256-7.  Back to cited text no. 5
    
6.
Bisarya K, Azzopardi S, Lye G, Drew PJ. Necrotizing fasciitis versus pyoderma gangrenosum: Securing the correct diagnosis! A case report and literature review. Eplasty 2011;11:e24.  Back to cited text no. 6
    


    Figures

  [Figure 1], [Figure 2], [Figure 3]



 

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