Volume 64, Issue 1 , Pages e12-e16, January 2011
Surgery for Pyodermia fistulans sinifica to circumvent heart transplantation (Case Report)☆
Article Outline
Summary
Introduction
Pyoderma fistulans sinifica (PFS) is a rare chronic cutaneous and subcutaneous infectious disease and is associated with major physical and psychological morbidity. In this article, we present the case of a young patient affected by severe PFS, who concomitantly suffered severe left-sided cardiac insufficiency and was considered for cardiac transplantation. By radical debridement and skin grafting of all areas affected by PFS, the patient’s overall condition rapidly improved and was correlated with a significant and measurable improvement of cardiac function.
Case report
Plastic surgery was consulted on a 32-year-old male by the cardiac intensive care unit with a 2-year history of recurrent pyogenic skin infections of the inguinal and axillary regions. He presented with a dilated cardiomyopathy with an ejection fraction (EF) of 15%, first-degree pulmonary insufficiency and second-degree mitral insufficiency and an overlying myocarditis leading to the picture of severe cardiac insufficiency, making him eligible for heart transplantation. The intention was to eradicate the chronic infective wounds as a preparative prior to cardiac transplantation. Radical debridement of the involved areas and coverage with split-thickness skin grafts took place and led to a significant improvement of the patient’s condition. Echocardiography revealed a 266.67% improvement of the EF to 40%. Cardiac transplantation became unwarranted. At 1.5 years postoperatively, the patient is free of disease and has a normal cardiac function.
Discussion
PFS is characterised in its early stages by local inflammation with beginning ulceration and can be managed conservatively. In advanced stages, as illustrated in this report, PFS must be considered as the potential source of systemic inflammatory process with significant alterations in multiple-organ systems and must be treated aggressively.
Keywords: Hidradenitis suppurativa, Pyodermia fistulans sinifica, Acne inversa, Heart Transplantation, Plastic Surgery
The exact aetiology of Pyoderma fistulans sinifica (PFS) is not well defined. It was first described as a distinct entity in 1839 by Velpeau (1795–1867).1 Velpeau considered the inflammatory process to originate from the sweat glands and gave it its primary name Hidradenitis suppurativa (HS). It has been ascertained that PFS actually results from follicular plugging with follicular and perifollicular inflammation and subsequent bacterial superinfection. HS is therefore a misnomer and actually represents an acneiform disorder that is based on follicular occlusion rather than an infection of sweat glands. Fox den disease is also a synonym for this pathology.2 The term ‘acne inversa’ has been suggested to designate this condition more precisely, as its pathogenesis is acneiform and its manifestation characteristic in localisations inverse to acne.
Estimated at a prevalence of 0.3–4% in industrialised countries,3 PFS is characterised by a chronic cutaneous and subcutaneous infection with a subcutaneous fistulous system. Its predominant location is the lower body including the anogenital and thigh region, but the axillae and the mammary regions can also be affected. PFS may initially present as superficial skin infection but superinfection, abscesses and sinus tracts generally ensue. These fistulous systems are either epithelialised or non-epithelialised and can extend deep into the epifascial fat.
PFS can be regarded as a systemic inflammatory condition: its possible association with arthritis of the large joints, ranging from asymmetric pauciarticular arthritis to a symmetric polyarthritis and/or polyarthralgia syndrome and may be part of HLA-B27–negative spondyloarthropathies.4 Arthropathy worsens during flares of PFS, and often improves after PFS resolves.
A case with local infection by PFS leading to flagrant cardiac insufficiency by haematogenic spreading of the infectious process is presented and discussed.
Case report
Plastic surgery was consulted on a 32-year-old male with a 2-year history of recurrent pyogenic skin infections of the inguinal and axillary regions by the cardiac service that had been following the patient for a dilated cardiomyopathy with an ejection fraction (EF) of 15%, first-degree pulmonary insufficiency and second-degree mitral insufficiency with an overlying myocarditis leading to the picture of full-fledged cardiac insufficiency requiring heart transplantation. We were consulted to assess the extent of soft tissue involvement in the infectious process and, if possible, to help clear the infection prior to cardiac transplantation. The patient reported that the skin infections started after having shaved his axillae and genital region. He had been treated with isotretinoin for 1.5 years without success. Abscess incision and drainage occurred twice, accompanied by the administration of brief courses of systemic antibiotics. Despite these efforts, disease progression could not be stopped. Haematogenic bacterial spread led to acute myocarditis, further reducing cardiac function and making cardiac transplantation necessary.
Upon presentation, the patient was in the cardiac intensive care unit and was being monitored very closely. He was febrile, with otherwise normal and stable vital signs. Examination showed multiple subcutaneous and cutaneous fluctuant lesions in axillary, inguinal and anogenital regions with sinuses draining with purulent and foul-smelling fluid (Figure 1(a)–(c)). The advanced and chronic disease process had led to scarring in these areas. Chest X-rays demonstrated cardiomegaly, without signs of pulmonary congestion (Figure 2(a)), cardiac catheterisation revealed a cardiac minute volume of 5.49 and a cardiac index of 3.19. Pulmonary venous resistance was estimated at 145.7
dyn (i.e., 1.8 Wood), blood cultures were negative. Laboratory testing revealed proBNP values beyond 7000pgml−1 (reference below 125pgml−1) and a leucocyte count of 17 000ml−1. Wound cultures showed significant growth of Gram-positive skin flora sensitive to cefuroxime.
Figure 1
Upon presentation, the patient demonstrated clear signs of soft tissue infection in the axillary a), inguinal, genital and gluteal (b) regions consistent with Hidradenitis suppurativa.
Figure 2
Posteroanterior chest x-rays of the patient. a) Upon presentation, b) One month after excision and skin grafting of the involved areas.
The patient was continued on intravenous cefuroxime and radical debridement of the involved areas (groin, axillae and anogenital region) with vacuum-assisted closure was performed (Figure 3(a)and (b)). In order to allow for adequate wound management, a temporary ileostomy was placed. Definitive coverage occurred 10 days postoperatively by means of split-thickness skin grafts.
Figure 3
Intraoperative pictures of the anogenital (a) and axillar region (b) after radical debridement and vacuum assisted therapy prior to split-thickness skin grafting.
The postoperative course was complicated by a small bowel obstruction due to adhesions, which was treated emergently by adhesiolysis.
The remaining postoperative course was characterised by excellent graft take and a significant improvement of the patient’s overall condition: 1 week postoperatively, the patient was afebrile, and the leucocyte count returned to normal. Upon discharge to an inpatient rehabilitation facility 1 month postoperatively, the patient was afebrile, blood cultures were negative. Interestingly, a repeat chest X-ray revealed the absence of signs of cardiomegaly observed 1 month prior (Fig. 2(b)). Echocardiography findings were consistent with our observation, revealing a 266.67% improvement of the EF to 40%. Cardiac transplantation became unwarranted.
At 1.5 years postoperatively, the integument in the grafted areas remains intact, and the patient is free of disease (Figure 4(a) and (b)).
Figure 4
a) genital region, b) axilla at 18 months postoperatively.
Discussion
The first description of PFS published in 1962 by Krauspe and Stelzner5 was followed by a second publication in 1984,2 where Stelzner reconsidered PFS and placed it under the category of retention dermatopathies. Wittmann et al.6 follow Stelzners hypothesis and define PFS as a distinct chronic infectious disease different from HS (Table 1). They state that PFS always does have epithelialised tracts lying deep epifascial, while HS comes along with superficial tracts and only sometimes epithelialisation. Another described characteristic is that PFS does not have a relation to skin structures (‘The skin may be involved only as the point of origin’) while HS always has a relation. Histological crosscuts of affected skin of the presented patient where scrutinised considering Wittmann’s article together with a skin pathologist of our Pathological Department. We interestingly found both, very superficial non-epithelialised tracts together with deep epithelialised tracts. These findings, together with current literature, sustain that HS and PFS are synonyms for the same cutaneous manifestation, PFS being the most severe manifestation.7, 8, 9
Table 1. Distinguishing characteristics of HS and PFS following Wittmann6
| Disease | Relation to skin structures | Epithelialized tracts | Deep epifascial tracts | Predominant location |
|---|---|---|---|---|
| PFS | No | Always | Yes | Lower body |
| HS | Yes | Sometimes | No | Axillae |
The exact nomenclature of the described cutaneous disease is still an object of debate in literature; further discussion is far beyond the scope of this article.6
PFS is a physically and psychologically most debilitating condition characterised by chronic and recurrent pyogenic infections of the skin and subcutaneous tissues.
Medical treatment alone is only recommended in early stages and in mild disease. Various modalities have been described such as topical clindamycin, systemic tetracycline, minocycline, erythromycin, antiandrogens, finasteride, oral retinoids, dapsone, immunosuppressive treatment using intralesional or systemic steroids, cyclosporine, etanercept, adalimumab and infliximab.10 Notwithstanding the promising results achieved with biologics, the potential severe side effects must be carefully considered.
Surgery remains the mainstay of treatment and should be performed as early as possible once complications such as abscess, scarring and fistulation are evident.10 The goal is to remove the diseased tissues such as draining abscesses and sinuses, along with the scar tissue.7, 8 Incision and drainage of individual lesions are not definitive. The more aggressive the local surgical treatment, the lower is the local recurrence rate.7 The use of defect coverage by means of various flaps (fasciocutaneous flaps, musculocutaneous flaps or skin grafts) seems to represent a durable and curative treatment modality.8 At our institution, the preferred method of treatment is wide excision and skin grafting.
Cardiac insufficiency can have various reasons. In general, a reduced contractional force of the heart muscle due to, for example, myocardial infarction or myocarditis is the underlying pathological mechanism. The presented patient suffered of a haematogenically induced myocarditis, which was histologically classified as a borderline myocarditis with macrophage infiltration, but without myocardial necrosis. Immunohistochemistry and PCR did not reveal microbiological agents.
Thus, the proof of a causative link between PFS and myocarditis cannot be ultimately provided. However, considering that no other plausible conditions for the development of cardiac insufficiency are given and postoperative recovery was impressive, PFS as the sole cause of cardiac insufficiency is likely.
The purpose of this article is to demonstrate how PFS can have drastic systemic implications and assume life-threatening dimensions. Its adequate treatment in a timely fashion, as demonstrated in our case, can represent a life-saving measure in the context of a systemic inflammatory process.
Conflict of interest
None.
Funding
None.
References
- . Aisselle, phlegmons, abces. Dictionnaire de Medecine ou repertoire general des sciences medicales. vol. 2. 1833;p. 11
- . Causes of pilonidal sinus and pyoderma fistulans sinifica, Langenbecks Arch Chir. 1984;362:105–118
- . Hidradenitis suppurativa. South Med J. 1998;91:1107–1114
- . Reactive arthritis and hidradenitis suppurativa, An Med Interna. 1995;12:464–465
- . Pyodermia fistulans sinifica. On the clinical and histopathological changes in fistulous dermatitis with remarks on the relation to so-called hidradenitis suppurativa and acne conglobata, Chirurg. 1962;33:534–538
- . Pyoderma fistulans sinifica (fox den disease): a distinctive soft-tissue infection. Clin Infect Dis. 1995;21:162–170
- . Hidradenitis suppurativa: pathogenesis and management. Br J Plast Surg. 2003;56:451–461
- . Hidradenitis suppurativa. Br J Surg. 2009;96:350–360
- . Hidradenitis suppurativa: a comprehensive review. J Am Acad Dermatol. 2009;60:539–561quiz 562–3
- . Medical treatments of hidradenitis suppurativa: a new paradigm. Dermatology. 2007;215:95–96
☆ Dr. Penna and Dr. Dowlatshahi both equally contributed to the work and therefore are both first authors.
PII: S1748-6815(10)00434-1
doi:10.1016/j.bjps.2010.07.024
© 2010 British Association of Plastic, Reconstructive and Aesthetic Surgeons. Published by Elsevier Inc. All rights reserved.
Volume 64, Issue 1 , Pages e12-e16, January 2011
