Pyoderma Gangrenosum: A Case Series

Case Report
DOI: 10.17354/cr/2015/49
Pyoderma Gangrenosum: A Case Series
Krishnendra Varma1, Prerna Sharma2
Professor and Head, Department of Dermatology, R.D. Gardi Medical College, Ujjain, Madhya Pradesh, India, 2Post-graduate Student, Department of
Dermatology, R.D. Gardi Medical College, Ujjain, Madhya Pradesh, India
1
Pyoderma gangrenosum is a rare, ulcerative, non-infectious neutrophilic dermatosis, commonly associated with underlying systemic disease.
The features of pyoderma gangrenosum are not specific histopathologically and for this reason the diagnosis is based on clinical feature. The
systemic administration of corticosteroids is the mainstay of treatment. We present five cases of pyoderma gangrenosum of age group ranging
from 2 to 75 years. One of these patients was HIV-infected, and one was having discoid lupus erythematosus. The lesions were present on
thighs in 3 cases, over back in one and breast in one. Biopsy showed central necrosis with multiple neutrophilic abscesses in epidermis. The
dermis showed dense neutrophilic infiltration in both superficial and deep dermis. The lesions responded well to oral corticosteroids except
in the case of HIV-infected patient in which minocycline showed considerable improvement.
Keywords: Minocycline, Prednisolone, Pyoderma gangrenosum
Corticosteroid and immunosuppressant therapy are the
mainstays in the treatment of pyoderma gangrenosum.6
INTRODUCTION
Pyoderma gangrenosum is an uncommon ulcerative
cutaneous condition with distinctive clinical characteristics
and a frequent association with systemic disease.1 It was also
known as phagedena geometrica, dermatitis gangrenosa,
and phagedenic pyoderma.2 Pyoderma gangrenosum was
first described and named by Brunsting et al. in 1930.3
They believed that streptococcal infection was a significant
component leading to secondary cutaneous gangrene and
named it as pyoderma gangrenosum. Hence, the term
pyoderma gangrenosum is a misnomer. The exact causative
mechanism is unknown. It is frequently associated with
autoimmune diseases, but their exact role in producing the
lesions is not known.4,5 The predisposed patient experiences
an inciting event like minor trauma, but instead of a normal
response that recognizes and removes the damaged tissue,
the patient’s abnormal response results in the lesions of
pyoderma gangrenosum.5
The diagnosis mainly depends on recognition of the
evolving clinical features because the histopathological
changes are not specific. It is essential to exclude other
diagnoses of cutaneous ulcers before therapy is initiated.
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CASE REPORTS
Case 1
A 2-year-old female child presented with a huge ulcerative
lesion over the right thigh with a history of insect bite
20 days back, after which a small, solid, reddish, round
lesion appeared that ruptured, ulcerated, and acquired the
present size within 10-12 days. The ulcer was painful and
was gradually increasing in size. On examination, a single,
well-circumscribed, oval-shaped ulcer measuring 8 cm ×
5 cm in size, having violaceous borders, sloping edges, and
indurated base was present over the anteromedial aspect
of the right thigh. Floor was covered with yellowish slough
with few blood-tinged areas (Figure 1).
Case 2
A 30-year-old male patient came with a history of trauma
12 months back over the left thigh which was followed by
development of two ulcers with severe intractable pain,
high-grade fever, and arthralgia. The ulcers were gradually
increasing in size. On examination, two well-defined,
irregularly shaped ulcers measuring 7 cm × 4 cm and 8 cm
× 6 cm in size were present over the anterior aspect of left
thigh and left side of lower abdomen, with presence of
characteristic violaceous border. Ulcers were adjacent and
separated by a band of normal skin. Floor was erythematous
with few purulent areas in between. Edges were sloping
and sharply defined (Figure 2).
Corresponding Author:
Dr. Prerna Sharma, Room number 502, R.M.O Hostel, R.D Gardi Medical College, Surasa, Ujjain - 456 006, Madhya Pradesh, India.
Phone: +91-9893557155. E-mail: [email protected]
IJSS Case Reports & Reviews | March 2015 | Vol 1 | Issue 10
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Varma and Sharma: Pyoderma Gangrenosum
Case 3
A 45-year-old HIV-positive male on anti-retroviral
treatment, came with complaint of eruption of a single
painful boil over the right thigh 1-month back, which
ruptured leading to formation of a large ulcer which was
gradually increasing in size. On examination, a single ulcer
was present over the right iliac region and anterior aspect of
right thigh measuring 8 cm × 4 cm with well-defined, sharp,
blue to purple borders, sloping edges and erythematous
floor with presence of granulation tissue (Figure 3).
Case 4
A 75-year-old male of discoid lupus erythematosus
presented with a history of two pus-filled round lesions over
the back after a surgical procedure. Within few days there
appeared a small ulcer which gradually increased in size
and acquired the present size. Mild pain was present over
the site that had no aggravating factors. On examination,
two sharply defined irregularly shaped ulcers were present
over the back, measuring 5 cm × 3 cm and 6 cm × 4 cm in size
Figure 1: Pyoderma gangrenosum: A well-defined ulcer with a characteristic
raised violaceous border in a 2-year-old female child
Figure 2: Pyoderma gangrenosum: Two ulcers measuring separated by a band
of normal skin present over the anterior aspect of left thigh and lower abdomen
50
with violaceous margins, erythematous floor, and sloping
edge. Mild tenderness was present (Figure 4).
Case 5
An 18-year-old female patient presented with a history of
nipple trauma 1-month back while feeding the baby. This
resulted in a small wound which rapidly increased in size
and led to the formation of a big ulcer. The ulcer was painful
and was associated with serous discharge. On examination,
a single well-defined oval ulcer of size 12 cm × 18 cm, with
regular undermined edges and violaceous border was
present over the right breast. Floor was covered with red
granulation tissue, base was indurated and tenderness was
present (Figure 5).
In all the cases, biopsy was done from the edge of the
ulcer and was consistent with pyoderma gangrenosum,
characterized by central necrosis with multiple neutrophilic
abscesses in epidermis. The dermis showed dense
neutrophilic infiltration in both superficial and deep dermis.
Treatment was initiated with oral prednisolone at a dose of
1 mg/kg body weight in all the patients. Addition of oral
Figure 3: Pyoderma gangrenosum: A single ulcer over the right lower abdomen
and thigh, with sloping edges and violaceous borders in an HIV-positive male
Figure 4: Pyoderma gangrenosum: Two well-defined ulcers with characteristic
violaceous borders present over the back
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Varma and Sharma: Pyoderma Gangrenosum
the borders are typically undermined and violaceous
or bluish. A strong sensation of pain is often associated
with it.3,8,14 In at least 50% of patients, it is associated with
systemic diseases such as inflammatory bowel disease,
arthritis, monoclonal gammopathy, myeloproliferative
disorders, acne conglobata, malignancies, and a host of
other conditions like HIV, connective tissue disorders,
sarcoidosis etc.3,7
Figure 5: Pyoderma gangrenosum: A single large ulcer over the right breast
with violaceous border
minocycline at a dose of 100 mg twice daily was done in
the HIV positive patient who was refractory to treatment.
Standard wound care with daily cleaning and dressing
of the ulcer was done followed by topical application of
2% gentian violet. Intravenous antibiotics were also given
according to the culture sensitivity report. After 4-8 weeks
of treatment, the wound size decreased, and healthy
granulation tissue started appearing. Pain subsided, and
the ulcers started healing.
DISCUSSION
Pyoderma gangrenosum is a primarily sterile inflammatory
neutrophilic dermatosis. It is characterized by recurrent
cutaneous ulcerations with mucopurulent exudate. These
painful ulcers present with undermined bluish borders
with surrounding erythema.3,7 Both humoral and cellmediated abnormalities are associated with pyoderma
gangrenosum. Hyperactivity of neutrophils and monocytes
and hypersecretion of tumor necrosis factor - α has been
found.
The peak of incidence occurs between the ages of 20 and
50 years with women being more often affected than men.8,9
Cases in infants and adolescents account for only 4%.
Pyoderma gangrenosum in elderly people has occasionally
been reported.10 The general incidence has been estimated
to be between 3 and 10 per million per year.11
Pyoderma gangrenosum occurs most commonly on the
lower legs, mainly the pretibial area. It has been reported
on other sites of the body as well, including breast, hand,
trunk, head and neck, and peristomal skin. It is a feared
complication after breast surgery and other surgical
procedures.12,13 The ulcer starts as a follicular pustule
with rapid growth, tissue necrosis, and enlargement of
the area. The surrounding skin is erythematous, and
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Clinically, pyoderma gangrenosum is classified into the
following five varieties: Ulcerative type, pustular type,
bullous type, peristomal type and vegetative type, with
ulcerative type being most common. Diagnosis relies on
clinical signs first and is supported by histopathology.
Knowledge of the patient’s history for possible underlying
disease and specific investigations based on that background
are necessary. Therefore, the diagnosis is made by exclusion
of other possible disorders. No laboratory parameter for
pyoderma gangrenosum is available. Histopathology is
non-specific. The initial lesions show a deep suppurative
folliculitis with dense neutrophilic infiltrate. In about
40% of cases, leukocytoclastic vasculitis is present.
Pyoderma gangrenosum with (necrotizing) granulomatous
inflammation has been described.7,15,16
Treatment is directed toward underlying associated systemic
disease, immune system contributions, and the local
wound. Steroids given orally or intravenously are effective
in treating systemic immune contributions. Refractory
cases have been successfully treated with cyclosporine,
clofazimine, dapsone, thalidomide, azathioprine, and other
immunosuppressive agents. Combination therapies of
systemic steroids and hyperbaric oxygen in the treatment
of wounds associated with pyoderma gangrenosum are
also successfully reported.
CONCLUSION
Diagnosis of pyoderma gangrenosum is mainly clinical,
and the mainstay of therapy is systemic corticosteroids
but minocycline can also give good results. These cases are
presented for their rarity, a wide spectrum of presentation
(2-75 years) and the association with systemic conditions.
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How to cite this article: Varma K, Sharma P. Pyoderma Gangrenosum:
A Case Series. IJSS Case Reports & Reviews 2015;1(10):49-52.
Source of Support: Nil, Conflict of Interest: None declared.
IJSS Case Reports & Reviews| March 2015 | Vol 1 | Issue 10