Pyoderma gangrenosum

Published on 02/04/2015 by admin

Filed under Internal Medicine

Last modified 22/04/2025

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182 Pyoderma gangrenosum

Instruction

Look at this patient’s skin.

Salient features

History

• Diarrhoea (inflammatory bowel disease)

• Joint pains (rheumatoid arthritis)

• Leukaemia, multiple myeloma.

Examination

• Necrotic ulcer with purplish overhanging edges, usually seen on the lower limbs or trunk (Fig. 182.1).

image

Fig. 182.1 Pyoderma gangrenosum.

Diagnosis

These are necrotic ulcers (lesions), which are a feature of pyoderma gangrenosum (aetiology); I would like to investigate for underlying inflammatory bowel disease.

Questions

What are the variants of this condition?

Variants have been described based on the location of the lesion or the associated process, the skin lesion being similar to the classical pyoderma gangrenosum:

• Peristomal pyoderma gangrenosum at site of ileostomy (Fig. 182.2) or colostomy in ulcerative colitis or Crohn’s disease

• Vulvar pyoderma gangrenosum

• Penile or scrotal in adolescent adults

• Associated with preleukaemic state (the ulcer is shallow with bullous blue-grey border)

• Pyostomatitis vegetans is an oral variant with chronic pustular and eventually vegetative lesions in the mucous membrane of the mouth.

image

Fig. 182.2 Pyoderma gangrenosum at ileostomy site.

(With permission from Ennis et al. 2001 pp. 533.)

What is the histology in this condition?

Non-specific features including massive neutrophilic infiltration, haemorrhage and necrosis of the epidermis.

How would you evaluate these patients?

• Detailed history and physical examination

• Skin biopsy (with cultures for bacteria, fungus and viral)

• GI studies

• Serology for anti-neutrophil cytoplasmic antibodies (ANCA), anti-nuclear antibodies (ANA), anti-phospholipid antibody and serum protein electrophoresis

• Complete blood count, peripheral smear and bone marrow examination.

In which conditions can pyoderma gangrenosum be seen?

• Ulcerative colitis

• Crohn’s disease

• Chronic active hepatitis

• Rheumatoid arthritis, seronegative arthritis associated with GI symptoms

• Acute and chronic myeloid leukaemia, myelocytic leukaemia, hairy cell leukaemia

• Polycythaemia rubra vera

• Multiple myeloma

• IgA monoclonal gammopathy.

Advanced-level questions

How would you manage these lesions?

• High-dose steroids

• Intralesional steroids

• Topical therapy including dressings, limb elevation rest

• Systemic therapy with dapsone

• Some regress with treatment of the underlying cause.

What conditions can be mistaken for pyoderma gangrenosum?

Six broad disease categories may simulate pyoderma gangrenosum (N Engl J Med 2002;347:1412–8.):

• Vascular occlusive or venous disease: anti-phospholipid antibody syndrome, livedoid vasculopathy, venous stasis ulceration, small-vessel occlusive arterial disease, type I cryoglobulinaemia, Klippel–Trénaunay–Weber syndrome

• Vasculitis: Wegener’s granulomatosis, polyarteritis nodosa, cryoglobulinemic (mixed) vasculitis, Takayasu’s arteritis, leukocytoclastic vasculitis plus secondary

• Infection: herpes simplex virus, cutaneous TB, Entamoeba histolytica (amoeba cutis), deep fungal infections such as Penicillium marneffei, Sporotrichosis, Aspergillosis, Cryptococcosis, Zygomycosis spp.

• Malignancy: lymphoma, Langerhans cell histiocytosis, leukaemia cutis

• Exogenous tissue injury: drug induced or direct injury

• Other inflammatory disorders.

How would you exclude these lesions?

• History. Markedly painful ulcer, rapid progression of ulceration, type of skin lesion preceding the ulcer (papule, pustule, or vesicle), minor trauma (pathergy) preceding development of the ulcer, symptoms of an associated disease (e.g. inflammatory bowel disease or arthritis)

• Medication history (e.g. bromides, iodide, hydroxyurea, or granulocyte–macrophage colony-stimulating factor)

• Examination. Characteristic features of ulcer: tenderness, necrosis, irregular violaceous border, undermined, rolled edges

• Skin biopsy. Elliptical incisional biopsy preferable to punch biopsy (include inflamed border and ulcer edge at a depth that includes subcutaneous fat). The inflamed border is examined for routine histology (haematoxylin and eosin) and microorganisms (Gram, methenamine silver and Fite stains). The edge of ulcer is cultured in appropriate media to detect bacteria, fungi and atypical myobacteria

• Laboratory investigations. Complete blood count, ESR, blood chemistry (liver and kidney function tests), protein electrophoresis, coagulation panel (including anti-phospholipid antibody screening), ANCA, cryoglobulins

• Venous and arterial function studies

• Chest radiography

• Colonoscopy.

(See N Engl J Med 2002;347:1412–18.)

Pyoderma gangrenosum was first described in 1930 by Brunsting, Goeckerman and O’Leary, with five cases (Arch Dermatol Syphiol 1930;22:655–80).

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