INTRODUCTION
Pyoderma gangrenosum is a rare, recurrent, and destructive skin disease of unknown
etiology with a predominance in females aged 25-55 years1. The diagnosis is based on the evolution of the disease and the exclusion of other
diseases. It is triggered by trauma (pathergy phenomenon) in up to 25% of the cases
2,3,4.
Although it manifests in various clinical forms, we will concentrate on the presentation
of pyoderma gangrenosum characterized by the appearance of ulcers after breast surgery
in this report. The recognition of its' lesions, predisposing factors, and surgical
risks, as well as the early administration of appropriate treatment can mitigate the
extremely serious outcomes for affected patients.
CASE REPORT
A 50-year-old healthy Caucasian female patient was admitted to the Plastic Surgery
service of Barata Ribeiro Municipal Hospital, Rio de Janeiro for reduction mammoplasty
surgery.
Initial examination revealed large dense breasts with grade II ptosis, which induced
postural discomfort and back pain. The patient underwent a surgical procedure using
the Wise technique with type I Liacyr Ribeiro pedicle. She had no untoward events
during hospitalization and was discharged on the second postoperative day.
On the seventh postoperative day, the patient was referred for an examination, and
the condition of the breasts was good. A small degree of edema and small areas of
hyperemia were observed. She reported having mild pain, and pain relievers were prescribed.
From the seventh to the tenth postoperative day, the patient experienced a significantly
worsening clinical picture. She complained of intense pain and presented with ulcerated
and confluent lesions in the lower poles of both breasts. A cranial progression of
the lesions was observed over several days, preserving only the nipple-areola complex
(Figure 1). The lesions had elevated edges and an exudative fundus with fibrin and secretion
with blood and pus, reminiscent of a soft tissue infection.
Figure 1 - Appearance of the breasts on the tenth day after reduction mammoplasty.
Figure 1 - Appearance of the breasts on the tenth day after reduction mammoplasty.
The patient was readmitted to hospital and samples were collected for examination,
including swab and culture. Antibiotic therapy with cephalexin was initiated. Subsequently,
800 mg/day of ciprofloxacin and 3 g/day Clavulin were added to the regimen to control
the probable infection.
Laboratory results revealed that the patient had elevated systemic markers for inflammation,
(erythrocyte sedimentation rate and reactive C protein, without leukocytosis).
Given the unsatisfactory results of antibiotic therapy and the negative results of
the cultures of both breasts, the diagnosis of pyoderma gangrenosum was determined
and corticoid therapy with prednisone 100 mg/day and local skin care was initiated.
Dressings were applied daily to remove excess exudative content, using 0.9% saline
solution and polyhexanide aqueous solution, followed by coverage with calcium alginate.
The objectives were to keep the wound as clean as possible, without increasing the
trauma at the site and thus avoid the progression of the lesions. The use of chemical
debriders containing collagenase was prescribed, and surgical debridement was not
performed. The option of grafting was also rejected at this time considering the possibility
of triggering a recurrence.
Within a few weeks of beginning corticoid therapy, the ulcers regressed substantially.
With the reduction of the lesions, resolution of edge involvement and healing progression,
the corticoid therapy was slowly reduced to a maintenance dose of 10 mg/day. The less
purulent and superficial wounds were covered with non-adherent cellulose acetate mesh.
DISCUSSION
Pyoderma gangrenosum is a disease of unknown etiopathogenesis, and in more than 50%
of the cases, it is associated with systemic diseases3,4,5. While some lesions spontaneously appear, other manifestations appear only after
surgical trauma, which is frequently reported after breast surgery. It is mainly characterized
by the presence of a pimple with a necrotic center surrounded by bluish-red tissue.
This quickly evolves into an ulcer whose cultures are negative. The condition usually
evolves from 5 days to 10 years and requires continuous monitoring of the clinical
presentation of the skin ulcers to exclude infectious, autoimmune and neoplastic diseases6.
The patient in this report initially had multiple pustule lesions, which spread rapidly
through the breasts, generating an exudate containing blood and pus. There was rapid
dehiscence of the suture with elimination of the threads, intense pain, and perilesional
growth with erythematous-violaceous edges simulating an infectious condition. The
preservation of the nipple-areola complex7,8, lack of response to antimicrobials, and negative lab cultures were the determining
factors for the diagnosis of pyoderma gangrenosum.
Laboratory findings are neither specific nor diagnostic, and rates of erythrocyte
sedimentation, C-reactive protein levels, and leukocytosis are always high9. Histopathology may also aid in the exclusion of other causes of skin ulcers, commonly
revealing sterile neutrophilia associated with lymphocytic vasculitis10.
Because there is no specific etiology of the pyoderma, the treatment varies widely,
being based, in most cases, on an immunosuppressive agent10. Prednisone (60 mg/day to 80 mg/day) is the initial treatment of choice. There are
several alternatives for cases that do not respond to corticosteroids. Thus, clofazimine,
sulfasalazine, cyclophosphamide, mofetil mycophenolate, thalidomide, rifampicin, dapsone,
gamma globulin, plasmapheresis, infliximab, tacrolimus, and methotrexate, among others,
are prescribed 6,9. In the case described, a significant improvement was obtained with the administration
of prednisone 100 mg/day alone (Figure 2), with subsequent gradual weaning.
Figure 2 - Appearance of the breasts at sixty days after the onset of corticoid therapy.
Figure 2 - Appearance of the breasts at sixty days after the onset of corticoid therapy.
Potassium permanganate, povidone iodine, silver nitrate, topical rifampicin, intralesional
triamcinolone, benzyl peroxide, 0.9% saline solution, hydrocolloids, calcium alginate,
and the use of a hyperbaric chamber, among other therapies are reported for administering
local skin care. In this context, it is important to remember that debridement should
be postponed until the histopathological diagnosis of the active ulcerated area is
obtained. In most cases, the patient's condition worsens significantly after surgical
procedures. Thus, chemical and surgical debridement and grafting should be avoided
when pyoderma gangrenosum is suspected9. When grafting is necessary, the administration of corticosteroids before the procedure
may minimize the development of the disease.
In situations where the diagnosis, prognosis and clinical presentation are uncertain,
the patient should always receive psychological support. The patient's commitment
to maintaining long-term treatment with immunosuppressants is fundamental. Another
dilemma to be faced is the repair of the scarring sequelae, which in some cases may
generate new tissue aggressions and recurrence of the condition.
In conclusion, pyoderma gangrenosum is a rare skin disease with unpredictable development
and course. Suspecting the diagnosis of pyoderma gangrenosum is extremely important,
as the onset of the treatment will determine the outcome10. The use of corticosteroids and/or immunosuppressants should be the first measure
to control the progression of the lesions and to minimize final scarring (Figure 3). This should be associated with careful local cleansing in order to remove the exudative
content without increasing tissue aggravation. Thus, chemical or surgical debridement
should be avoided in this condition.
Figure 3 - Appearance of the breasts at one year after reduction mammoplasty.
Figure 3 - Appearance of the breasts at one year after reduction mammoplasty.
COLLABORATIONS
INY
|
Analysis and/or data interpretation, Conception and design study, Conceptualization,
Data Curation, Final manuscript approval, Formal Analysis, Funding Acquisition, Investigation,
Methodology, Project Administration, Realization of operations and/or trials, Resources,
Software, Supervision, Validation, Visualization, Writing - Original Draft Preparation,
Writing - Review & Editing
|
CJB
|
Final manuscript approval, Project Administration, Supervision, Visualization, Writing
- Review & Editing
|
RCR
|
Data Curation, Project Administration, Supervision, Validation, Visualization, Writing
- Review & Editing
|
SSA
|
Data Curation, Realization of operations and/or trials, Visualization, Writing - Review
& Editing
|
GFA
|
Realization of operations and/or trials, Supervision, Visualization
|
APR
|
Realization of operations and/or trials, Supervision, Visualization, Writing - Review
& Editing
|
REFERENCES
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a comprehensive review. Am J Clin Dermatol. 2012 Jun;13(3):191-211.
1. Hospital Municipal Barata Ribeiro, Rio de Janeiro, RJ, Brazil.
Corresponding author: Igor Nagai Yamaki Rua Carlos Góis, 390, Leblon, RJ, Brazil. Zip code: 22440-040. E-mail: igoryamaki@gmail.com
Article received: January 26, 2019.
Article accepted: June 22, 2019.
Conflicts of interest: none.