Pyoderma Gangrenosum

Pyoderma gangrenosum is a rare, destructive inflammatory skin disease in which

a painful nodule or pustule breaks down to form a progressively enlarging ulcer. Lesions
may occur either in the absence of any apparent underlying disorder or in association
with systemic disease, such as ulcerative colitis, Crohn's disease, polyarthritis,
gammopathy, and other conditions. Pyoderma gangrenosum belongs to the group of
neutrophilic dermatoses in which the common cellular denominator is the neutrophil.

ETIOLOGY AND PATHOGENESIS

When Brunsting, Goeckerman, and O'Leary described pyoderma gangrenosum
in 1930, 1 they implicated streptococci and staphylococci as causative agents because
of the inflammatory and purulent nature of the condition. Seventy years later, the
etiology of pyoderma gangrenosum is still unknown, but in all likelihood bacteria do not
directly cause the disease, nor is it infectious in nature.
Pyoderma gangrenosum, cystic acne, and pyogenic, aseptic arthritis (PAPA
syndrome) have been reported in a three-generation kindred with autosomal dominant
transmission and mapped to the long arm of chromosome 15. 2 A key to the etiologic
and pathogenic background of pyoderma gangrenosum may be found in its frequent
association with systemic disease, but, because pyoderma gangrenosum exists both as
a disease sui generis and in association with a systemic disorder, the cause-effect
relationship of such associations is not clear. The phenomenon of pathergy, which
describes the development of new lesions or the aggravation of existing ones after
trivial trauma and is frequently present in pyoderma gangrenosum, suggests altered,
exaggerated, and uncontrolled inflammatory responses to nonspecific stimuli.
The preeminent cell in pyoderma gangrenosum pathology is the neutrophil.
Indeed, the condition responds to sulfa drugs, which have an antineutrophilic action
(see Chap. 254). Pyoderma gangrenosum has occurred as a side effect of treatment
with granulocyte-macrophage colony-stimulating factor (GM-CSF), 3 following treatment
with interferon-a2b, 4 and in cases in which IL-8 is overexpressed in the tissue. 5 By
contrast, GM-CSF has been successfully used in the treatment of pyoderma
gangrenosum. 6 There have been reports of abnormalities of neutrophil function, but no
consistent patterns have emerged. Some in vitro studies showed impaired neutrophil
chemotaxis, 7 but neutrophil accumulation at a Rebuck skin window was normal 8 ;
moreover, neutrophils do accumulate at sites of pyoderma gangrenosum. Human skin
engrafted to mice with severe combined immunodeficiency and then injected with
recombinant adenovirus expressing DNA that encoded human IL-8, which induces a
200-fold increase in IL-8 expression of infected human fibroblasts in vitro, developed
massive neutrophilic inflammation and ulceration clinically and histologically resembling
pyoderma gangrenosum. 5 A streaking leukocyte factor that enhances migration of
leukocytes without altering their chemotactic activity, aberrant neutrophil trafficking, and
metabolic oscillations of leukocytes have been observed. 9, 10 Impairment of microbicidal
activation of leukocytes and impaired neutrophil functions associated with
hyperimmunoglobulinemia E have been isolated findings. 11IgA of multiple myeloma can
impair neutrophil chemotaxis in vitro, and IgA gammopathies are not infrequent in
pyoderma gangrenosum. Circulating immunoglobulins can affect neutrophil functions,

and monoclonal or polyclonal hyperglobulinemia frequently occurs in pyoderma

gangrenosum.
The evidence implicating a disturbance of cellular immune functions in pyoderma
gangrenosum is insufficient to explain the pathogenesis of the disease. Certainly, the
strongest piece of evidence indicating a disturbed immune response is the fact that
pyoderma gangrenosum responds to cyclosporine, but no consistent pattern of a
disturbed cellular immune response has emerged. Findings include anergy to recall
antigens, 12 impairment of both antigen-specific and mitogen-induced lymphocyte
proliferation, 13inhibition of allogeneic and autologous mixed lymphocyte proliferation,
14and defective monocyte phagocytosis linked to an IgG paraprotein that inhibits Fcdependent phagocytosis of normal monocytes. 15All these findings have appeared in
some individual patients, but not in others. Thus, although evidence suggests that
disturbances of immunoregulation and immunologic effector functions occur in some
patients with pyoderma gangrenosum, these disruptions are not detectable in others; it
is unclear whether they are epiphenomena.

CLINICAL MANIFESTATIONS
The salient feature of pyoderma gangrenosum is an ulcer with a raised
inflammatory border and a boggy, necrotic base. It starts as a deep-seated, painful
nodule or as a superficial hemorrhagic pustule ( Fig. 98-1A), either de novo or after
minimal trauma. The lesions break down and ulcerate, discharging a purulent and
hemorrhagic exudate ( Fig. 98-1B).
The irregular, crenated border is elevated and is dusky red or purplish; it is
undermined, soggy, and often perforated so that pressure releases pus ( Fig. 98-1C and
Fig. 98-1D.
FIGURE 98-1 Lesions of pyoderma gangrenosum in various stages of development. A.
A small, hemorrhagic pustule with an extremely painful red base. B. Three rapidly
spreading hemorrhagic, bullous lesions that have broken down in the center, revealing a
necrotic and purulent base. C. A rapidly progressive ulcerative lesion with a soggy,
pustular, and partially undermined margin; a granulating necrotic base; and a red
inflammatory halo. D. A larger ulcerating lesion with undermined bullous margins; a
necrotic, purulent base; and a deep ulcer in the center. A halo of bright erythema
surrounds the margin of an advancing ulceration ( Fig. 98-1B, Fig. 98-1C, and Fig. 982B), which may expand rapidly in one direction and more slowly in another so that a
serpiginous configuration results ( Fig. 98-2B). The base of such an ulcer is partially
covered with necrotic material ( Fig. 98-3A) and studded with small abscesses (see Fig.
98-1C and Fig. 98-3A). Ulcers may be confined to the dermis, but they often extend into
the fat and even down to the fascia (see Fig. 98-1D, Fig. 98-2B, and Fig. 98-3).
FIGURE 98-2 A. Multiple lesions of pyoderma gangrenosum in a patient who later died
of Wegener's granulomatosis. Note multiple, atrophic scars that have resulted from
previous flareups of pyoderma gangrenosum. B. Rapidly enlarging pyoderma
gangrenosum triggered by laparotomy. The lesion spread to involve the entire lower
abdomen within 5 days.
FIGURE 98-3 Lesions of pyoderma gangrenosum. A. An acute, necrotic ulcer with an
elevated purulent border studded with pustules. B. Lesion that started in the
subcutaneous fat and thus presented as acute, purulent panniculitis. Bullous lesions

have developed on the surface. When this lesion broke down, an ulcer similar to that
shown in Fig. 98-1D developed, extending all the way down to the fascia. Lesions are
usually solitary, but may arise in clusters that then coalesce to form multicentric,
irregular ulcerations. Multiple lesions may arise simultaneously or consecutively in
different parts of the body (see Fig. 98-2A). Any area of the body may be involved.
Mucous membranes are usually spared, but aphthous lesions are sometimes seen in
the oral mucosa. Massive ulcerative involvement of the oral cavity, larynx and pharynx,
vulva, cervix, and eyes occasionally occurs.
Pyoderma gangrenosum may occur at any age. Its clinical course may follow one
of two patterns: (1) an explosive onset with rapid spread of lesions or (2) a slow,
indolent progression. Pain, signs of a toxic reaction and fever, hemorrhagic blisters and
suppuration, extensive necrosis, and soggy ulcer margins with a highly inflammatory
halo characterize the first type of pyoderma gangrenosum (see Fig. 98-1, Fig. 98-2, and
Fig. 98-3). The slow, indolent form clinically exhibits massive granulation within the ulcer
from the onset, crusting, and even hyperkeratosis at the margins; it spreads gradually,
grazing over large areas of the body for months. Spontaneous regression and healing in
one area and progression in another are characteristic of the slow form ( Fig. 98-4A and
Fig. 98-4B. In both forms, healing occurs spontaneously at some time in the disease
process, resulting in thin, atrophic, usually cribriform scars.
FIGURE 98-4 A. Chronic form of pyoderma gangrenosum involving the upper eyelid.
The ulcer has an elevated granulating base with multiple small abscesses. B. Lesion of
chronic pyoderma gangrenosum that slowly spread over months to involve almost the
entire trunk. The lesion is less inflammatory than the one shown in A; it is progressive at
the margins on the abdomen and the lateral chest, while it has spontaneously healed in
the center, the hip, and the back. Rarely, pyoderma gangrenosum may start in the
subcutaneous fat, presenting as an extremely painful, suppurative panniculitis ( Fig. 983B). Breakdown of the lesion and the centrifugal spread of the resulting ulcer eventually
reveal the true nature of the process.

CLINICAL VARIANTS
A localized, vegetative form of pyoderma gangrenosum with verrucous and
ulcerative lesions and a more granulomatous histology has been identified and
designated superficial granulomatous pyoderma. 16 Most authors consider this
condition, originally described as malignant pyoderma, 17 to be a variant of pyoderma
gangrenosum, 18 although some of these cases may have actually been a form of
Wegener's granulomatosis. 19 Pyostomatitis vegetans, a pustular, vegetative process of
the oral mucous membranes often associated with ulcerative and vegetating skin
lesions and inflammatory bowel disease, is now also considered a variant of pyoderma
gangrenosum.
An atypical, bullous form of pyoderma gangrenosum appears to herald
preleukemic or leukemic states. 20 , 21 It has an acute onset, is more superficial than the
typical pyoderma gangrenosum, and is characterized by steadily enlarging, soft purple
papules and blue-gray, hemorrhagic bullous lesions. This condition is similar to the
bullous form of acute febrile neutrophilic dermatosis (Sweet's syndrome; see Chap. 94).
22 , 23

EXACERBATING FACTORS
Intradermal skin testing or injections (including even the injection of saline),
pricks, insect bites, biopsies, and operations may induce new lesions of pyoderma
gangrenosum. This phenomenon is called pathergy. The lesions shown in Figure 98-2B
arose after laparotomy for a gynecologic problem. However, pathergy occurs in only 20
percent of patients, 24and, because some individuals with the disease tolerate even
major surgery well, the significance of pathergy is difficult to assess. Pathergy may also
be the reason for the rejection of autologous skin grafts and the development of new
lesions in donor sites, but again, patients with pyoderma gangrenosum do not always
reject skin grafts. Potassium iodide can induce an exacerbation of pyoderma
gangrenosum, 1 as can GM-CSF and interferon, 3, 4 but, as is true for pathergy after
trauma, this exacerbation does not always occur.

LABORATORY FINDINGS
No laboratory findings are specific and thus diagnostic of pyoderma
gangrenosum. A high erythrocyte sedimentation rate and leukocytosis are invariably
present. The level of C-reactive protein may be raised; there may be anemia and a low
level of serum iron. Both hyper- and hypoglobulinemia occur. Specific autoantibodies
are not known; the complement system is not altered, and circulating immune
complexes have not been detected regularly. 7, 13 , 25 HLA typing has not revealed a
consistent pattern. 7

PATHOLOGY
The histopathologic features of pyoderma gangrenosum are not diagnostic. They
include edema, massive neutrophilic inflammation, engorgement and thrombosis of
small- and medium-sized vessels, necrosis, and hemorrhage. The extremely dense
infiltrate of polymorphonuclear leukocytes leads to abscess formation and liquefaction
necrosis of the tissue with secondary thrombosis of venules. Lesions further evolve to
suppurative granulomatous dermatitis and regress with prominent fibroplasia. 26
The occurrence of necrotizing vasculitis in lesions of pyoderma gangrenosum
was controversial in the past, but a number of authors have now described fibrinoid
necrosis, leukocytoclasia, 27 , 28 and intramural deposition of C3 in vessels of pyoderma
gangrenosum lesions 29 , 30 that are indistinguishable from those associated with
immune complex vasculitis.
There also exist reports on the concomitant occurrence of pyoderma
gangrenosum and necrotizing vasculitis, 28, 31, 32 and 33 and patients with pyoderma
gangrenosum have been reported to develop Wegener's granulomatosis. 34However,
the ultimate proof for a pathogenic role of immune complex vasculitis has yet to be
provided. When pyoderma gangrenosum is associated with Crohn's disease, lesions
may contain granulomatous foci with giant cells. 35When localized in the fat, it presents
as subcutaneous abscess. 36

ASSOCIATION WITH SYSTEMIC DISEASE

Pyoderma gangrenosum may occur as a disease confined to the skin in 40 to 50
percent of cases (idiopathic pyoderma gangrenosum), 37 , 38 but it may sometimes
manifest itself in extracutaneous sites. Peripheral ulcerative keratitis is an inflammatory
keratitis characterized by cellular infiltration, corneal thinning, and ulceration. 39
Cavitating pulmonary infiltrates may also occur in pyoderma gangrenosum. 40, 41
Among the systemic diseases sometimes associated with pyoderma gangrenosum are
inflammatory large- and small-bowel disease, arthritis, paraproteinemia and myeloma,
myeloproliferative disease, and Behet's syndrome. The heterogeneity of these
conditions makes pinpointing a common denominator difficult. Common and less
common disease associations are listed in Table 98-1.
TABLE 98-1 Systemic Disease Associations with Pyoderma Gangrenosum
Ulcerative Colitis and Crohn's Disease
In its original description, pyoderma gangrenosum was associated with ulcerative
colitis in four of five cases, 1and subsequent series have found a frequency ranging
from 30 to 60 percent. 42 These figures are probably too high, for other studies have
found a frequency of up to only 15 percent. 43 Also, pyoderma gangrenosum occurs only
rarely in ulcerative colitis, with a reported prevalence ranging from 0.6 to 5 percent.
44Nonetheless, together with erythema nodosum, pyoderma gangrenosum represents
the most common dermatologic disorder accompanying ulcerative colitis.
In most patients, symptoms of ulcerative colitis precede pyoderma gangrenosum,
and exacerbations of the bowel disease frequently correlate with a worsening of the skin
lesions. However, pyoderma gangrenosum may persist for long periods while the bowel
disease is quiescent.
Pyoderma gangrenosum is also associated with Crohn's disease. In one series,
16.3 percent of patients with pyoderma gangrenosum had Crohn's disease, 45 but the
overall prevalence is low (1.2 percent). 46In inflammatory bowel disease, the most
frequent localization of pyoderma gangrenosum is on the lower legs and around a
stoma 47 (see Chap. 129).
Arthritis
Arthritis is frequently associated with pyoderma gangrenosum and usually
precedes it. In one review, 24 arthritis was found in 30 percent of patients with pyoderma
gangrenosum. In a study of 86 patients from the Mayo Clinic, arthritis was the most
frequently associated disorder. 45 Some patients have classic seropositive rheumatoid
arthritis 48 ; others have the arthritis associated with inflammatory bowel disease, which
is seronegative, acute, oligoarticular, and nondestructive; others have a seronegative
rheumatoid-like arthritic syndrome; still others have spondylitis. An association of
pyoderma gangrenosum with the synovitis, acne, pustulosis, hyperostosis, and osteitis
(SAPHO) syndrome 49 , 50 and with psoriatic arthritis (see Chap. 43) has been described.
In addition, pyoderma gangrenosum is a clinical manifestation of the autosomal
dominant PAPA syndrome characterized by pyogenic arthritis, pyoderma gangrenosum,
and cystic acne. 2, 51

Monoclonal Gammopathy
Pyoderma gangrenosum is often associated with paraproteinemia, mostly of the
IgA type, but also of the IgG and IgM types. In one series, 52monoclonal gammopathy
was found in 5 of 21 patients, and in 4 of the 5 it was of the IgA type. In another series,
a monoclonal gammopathy was present in 10 percent of those studied. 45 Although
patients with a monoclonal gammopathy do not show progression to malignancy over
the short term, some patients with pyoderma gangrenosum have myeloma at
presentation or develop it subsequently. 7, 24
Myeloproliferative Disorders
Pyoderma gangrenosum occurs in myelodysplasia, 53 as well as in acute
myeloblastic, myelomonocytic, and chronic myeloid leukemia. 42, 54 , 55 A few cases
have been associated with smoldering leukemia, hairy cell leukemia, polycythemia vera,
erythroid hypoplasia, autoimmune hemolytic anemia, myelofibrosis, and Hodgkin's
disease. The features of atypical bullous pyoderma gangrenosum overlap with those
of Sweet's syndrome presenting as an association with acute leukemia (see Chap. 94).
Other Conditions
Several studies have documented patients with pyoderma gangrenosum and
Behet's syndrome. 56, 57and 58 The two diseases share certain features, such as
arthritis, pustulation, aphthous lesions of the mucous membranes, and the phenomenon
of pathergy. Pyoderma gangrenosum has occurred in association with other purulent
diseases, such as acne conglobata and fulminans 59 , 60 (see Chap. 73), the PAPA
syndrome, 2, 51 vasculitis, 32 , 33 erythema elevatum diutinum 61 (see Chap. 95), and
Wegener's granulomatosis. 34 , 62 Figure 98-2A shows multiple lesions of pyoderma
gangrenosum in a patient who, during the course of the disease, developed necrotizing
vasculitis and eventually died of Wegener's granulomatosis. There is an increased
frequency (up to 30 percent) of pyoderma gangrenosum in patients with Takayasu's
disease in Japan, 63 but not in Europe where such associations are rare. 64 Additional,
probably fortuitous, associations are listed in Table 98-1.
Relationship to Other Neutrophilic Dermatoses
The predominant cell in lesions of pyoderma gangrenosum is the neutrophil.
This, together with the absence of any sign of infection and the response of pyoderma
gangrenosum to sulfa drugs, places pyoderma gangrenosum into the group of
etiologically ill-understood neutrophilic dermatoses, such as Sweet's syndrome (see
Chap. 94), subcorneal pustular dermatosis (see Chap. 69), Behet's syndrome (see
Chap. 192), neutrophilic eccrine hidradenitis, and rheumatoid neutrophilic dermatitis
(see Chap. 179). In the past, pyoderma gangrenosum has been reported in association
with generalized, vesiculopustular eruptions, which in retrospect appear to have been
subcorneal pustular dermatosis (Sneddon-Wilkinson disease). Indeed, the syndrome
consisting of pyoderma gangrenosum, subcorneal pustular dermatosis, and IgA
gammopathy, which was first described in 1971, 65now appears established by a
number of case reports. 66 , 67 and 68 Subcorneal pustular dermatosis can be associated
with ulcerative colitis and IgA myeloma, and it responds to sulfa drugs (see Chap. 69).

These findings also hold true for pyoderma gangrenosum. Generalized pustular
eruptions occur in patients with ulcerative colitis; whereas some of these pustular
lesions progress to frank pyoderma gangrenosum in some patients, others do not.

DIFFERENTIAL DIAGNOSIS
Because there is no specific laboratory test for pyoderma gangrenosum and the
histopathology is only suggestive, the diagnosis rests entirely on the clinical
presentation and course. Differential diagnoses include postoperative gangrene,
ecthyma gangrenosum, atypical mycobacterial and clostridial infection, deep mycoses,
amebiasis, tropical ulcers, bromoderma, North American blastomycosis, and pemphigus
vegetans. Stasis ulcers are easily excluded, but artifacts and brown recluse spider bites
may occasionally cause problems in early, developing lesions. Wegener's
granulomatosis includes involvement of the upper respiratory tract and
histopathologically displays necrotizing and granulomatous vasculitis; as mentioned
previously, however, the two diseases may occur together.

TREATMENT
In patients with underlying disease, therapy should focus not only on pyoderma
gangrenosum, but also and primarily on the systemic disorder. In milder forms and
those not associated with systemic disease, a trial of local or topical treatment may be
appropriate. Some authorities have advocated the intralesional injection of
glucocorticoids into the active border of the lesions; the use of hyperbaric oxygen; or
topical treatment with disodium cromoglycate, cyclosporine, or tacrolimus.
With the possible exception of the topical use of tacrolimus, 69, 70 topical
treatment alone is usually insufficient both in patients with associated systemic disease
and in most of those having purely cutaneous pyoderma gangrenosum.
Although measures directed at cleaning the ulcer and preventing bacterial
overgrowth are important, more invasive surgical debridement is generally unwise as it
may trigger new lesions. This caveat applies also to excision and grafting, unless
systemic treatment has controlled the pyoderma gangrenosum. There have as yet been
no enrolled clinical trials for any of the treatments.
Glucocorticoids
The systemic administration of glucocorticoids is the most effective treatment of
pyoderma gangrenosum. They dramatically halt the progression of existing ulcerations
and prevent the development of new lesions. 43 A high dosage of prednisone, such as
100 to 200 mg/day, may be necessary initially; the dosage is reduced as the
inflammatory component of pyoderma gangrenosum disappears and is tapered slowly
to discontinuation after the condition has completely resolved. Pulse therapy with
suprapharmacologic doses of methylprednisolone (1 g/day for 5 consecutive days) most
effectively halts progressive pyoderma gangrenosum, 59 , 71 , 72 and it is still the first-line
treatment for severe pyoderma gangrenosum in many institutions. However, it is
necessary to continue suppressing the inflammatory process after pulse therapy by
administering prednisolone or sulfa drugs. 43 Unfortunately, long-term oral therapy with
glucocorticoids produces side effects in up to 50 percent of the patients.

Sulfa Drugs
Dapsone, sulfapyridine, and sulfasalazine (Azulfidine) are beneficial drugs for
pyoderma gangrenosum, but not all patients respond equally well to them. Initial daily
doses of sulfasalazine range from 4 to 6 g; the dosage is gradually reduced to
maintenance levels of 0.5 to 1 g/day. It may be necessary to administer sulfa drugs in
combination with glucocorticoids (given systemically), particularly in the initial phases of
therapy, but there have been patients who have failed to respond to this regimen as
well.
The mechanism of action of the sulfa drugs in pyoderma gangrenosum is
unknown, but may be related to a stabilizing effect on lysosomes, to their interference
with the myeloperoxidase-halide system of polymorphonuclear leukocytes, or to a
decrease of glycosaminoglycan viscosity in this condition (see Chap. 254). Dapsone is
effective in a host of cutaneous disorders that are all characterized by the abnormal
accumulation of polymorphonuclear leukocytes, such as erythema elevatum diutinum,
dermatitis herpetiformis, Sneddon-Wilkinson disease, Sweet's syndrome, and pyoderma
gangrenosum.
Cyclosporine
The oral administration of cyclosporine 73 , 74 , 75 , 76 and 77 and tacrolimus 9, 78is
very effective in the treatment of pyoderma gangrenosum. In severe cases,
cyclosporine given intravenously has been successful. 77 Significant improvement
occurs within weeks of the start of oral therapy with cyclosporine in doses that range
from 6 to 10 mg/kg per day, and healing is likely to take place within 1 to 3 months.
Some patients require low-dose maintenance therapy, but others can tolerate complete
withdrawal of the drug. Also, patients may require concomitant medium- to low-dose
glucocorticoid therapy. As with other immunosuppressive treatments, patients who use
cyclosporine require careful monitoring for side effects.
Other Immunosuppressive Agents
Although not universally successful, 6-mercaptopurine, azathioprine, and
methotrexate have been beneficial for some patients. Cyclophosphamide has induced
dramatic remissions in some patients, 79 , 80 and chlorambucil has demonstrated a
glucocorticoid-sparing effect. 81, 82Mycophenolate mofetil, either alone or in combination
with cyclosporine, has also been useful. 83 , 84 and 85
Clofazimine
Reports have stressed the efficacy of clofazimine (Lamprene) in pyoderma
gangrenosum. 86 , 87 Dosages of 200 to 300 mg/day supposedly stop progression of
lesions within 1 to 2 weeks and lead to complete or partial healing within 2 to 5 months.
However, clofazimine may have adverse effects, and some patients do not respond to
this drug at all.
AntiTumor Necrosis Factor-a
There is no laboratory evidence that tumor necrosis factor-a (TNF-a) plays a
pathogenic role in pyoderma gangrenosum.

However, monoclonal antibodies to TNF-a are beneficial in Crohn's disease and

ulcerative colitis, and such antibodies (infliximab) have also been used in the treatment
of pyoderma gangrenosum, apparently with success. 88 , 89
Miscellaneous Treatments
Pyoderma gangrenosum has been reported to respond to minocycline,
colchicine, and heparin, as well as to the intravenous administration of vancomycin and
mezlocillin; although, as indicated by worldwide experience, it is usually unresponsive to
any kind of antibiotic. It has been reported to improve with a combination of
sulfasalazine and isotretinoin and to respond to interferon-a. 90 , 91 However, it has also
been reported that interferon triggers pyoderma gangrenosum. 4 Plasmapheresis has
been successfully used in the treatment of patients with pyoderma gangrenosum. 92, 93
Immunoglobulin 94 (given intravenously) and thalidomide are of benefit to patients with
pyoderma gangrenosum, both with and without an association with Behet's disease. 56,
57, 95

COURSE AND PROGNOSIS

Pyoderma gangrenosum behaves in an unpredictable way. It may have a
dramatic onset with pustular and bullous lesions rapidly breaking down to form ulcers,
which progressively enlarge (see Fig. 98-1, Fig. 98-2, and Fig. 98-3) until arrested by
treatment; in these cases, there may be signs of toxicity, fever, and considerable pain.
More chronic forms of the disease show ulcers that extend slowly in a creeping
fashion (see Fig. 98-4), expanding in one direction and healing spontaneously in
another or in the center. In both forms, spontaneous healing can occur, but as old
lesions resolve, new lesions may arise.
The disease may come to a spontaneous halt for no apparent reason; remain
quiescent for months and even years; and exacerbate again after minimal trauma,
surgery, or no apparent triggering cause. When associated with ulcerative colitis, the
disease activity of pyoderma gangrenosum may parallel that of the bowel disease, but
not always. The same is true for patients with associated hematologic disease, in which
the underlying condition determines the final prognosis. The overall prognosis of
pyoderma gangrenosum per se is good, particularly in those patients who readily
respond to treatment, but considerable scarring and disfigurement may occur.

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