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Superficial granulomatous pyoderma: clinicopathologic spectrum.

Superficial granulomatous pyoderma is a form of pyoderma gangrenosum characterized by superficial ulceration and a chronic course. Histopathologic examination shows a granulomatous response. We report two new cases of superficial granulomatous pyoderma in detail and briefly review the clinical data of five previous cases included in a recent series. These lesions typically begin as single furunculoid papules that most commonly occur on the trunk and that may arise at sites of surgical treatment or other pathergic stimuli. The lesions progress to superficial ulcers with a relatively clean base and vegetative borders. Tetracycline, minocycline, sulfapyridine, dapsone, and intralesionally administered corticosteroids have been effective anti-inflammatory agents, producing healing and allowing avoidance of the use of systemic corticosteroids in the management of most patients with superficial granulomatous pyoderma.

Adult↗

Pyoderma gangrenosum of the ocular adnexa: a rare condition with characteristic clinical appearances.

PURPOSE: To describe the clinical characteristics of periocular pyoderma gangrenosum and to highlight features that may encourage early diagnosis of this extremely rare condition. DESIGN: Retrospective, noncomparative, interventional case series. PARTICIPANTS AND METHODS: Four patients with periocular pyoderma gangrenosum were treated in the Orbital Unit at Moorfields Eye Hospital over the course of a decade. MAIN OUTCOME MEASURES: Presenting clinical features, therapy, and outcome. RESULTS: Four patients (female) between the ages of 56 and 75 years (mean, 65 years; median, 64 years) sought treatment for slowly evolving, painful, unilateral blue-gray swellings of the pretarsal tissues of the lower (1 case), upper (1 case), or both eyelids (2 cases). The swelling progressed to frank tissue necrosis and loss of full-thickness eyelid, with patchy sparing of the lid margin or lashes; in some cases, there was a very distinctive preservation of the pretarsal marginal artery across full-thickness eyelid defects. The lid loss characteristically involved the lateral one third of the lower eyelid (3 of 4 lids), the central part of the upper eyelid (3 of 4 lids) and, in one case, extending into the postseptal tissues in the inferotemporal quadrant of the orbit. In 3 patients, the pyoderma, often associated with a positive serum rheumatoid factor (three of three cases where measured), responded well to systemic immunosuppression and eyelid repair was undertaken during the quiescent phase. In a single patient, relapsing disease led to loss of the eye as a result of involvement of the globe and deep orbital tissues. CONCLUSIONS: Although an extremely rare condition, periocular pyoderma gangrenosum has a typical clinical appearance, and early recognition and immunosuppression will reduce the ocular morbidity.

Aged↗

Pyoderma gangrenosum and chronic persistent hepatitis.

Pyoderma gangrenosum is a diagnosis of exclusion. A 36-year-old woman was clinically diagnosed as having pyoderma gangrenosum and appropriate exclusion tests were performed. Pyoderma gangrenosum may occur in association with underlying diseases such as leukemia, monoclonal gammopathy, inflammatory bowel disease, arthritis, and chronic active hepatitis. However, these diseases were excluded in this patient who did have chronic persistent hepatitis. To our knowledge, this is the first reported case of chronic persistent hepatitis and pyoderma gangrenosum.

Adult↗

Pustular pyoderma gangrenosum associated with ulcerative colitis in childhood. Report of two cases and review of the literature.

Pyoderma gangrenosum is a cutaneous disorder associated with systemic diseases such as ulcerative colitis, Crohn's disease, rheumatoid arthritis, and blood dyscrasias. We are reporting two cases of pustular pyoderma gangrenosum associated with ulcerative colitis. One patient had inactive bowel disease when she developed her third episode of pustules, erosions, and nodules on the left leg. The other patient exhibited a widespread painful vesiculopustular eruption that coincided with the onset of her colitis. Both patients presented with pustules as the primary manifestation of their pyoderma gangrenosum. Histologic examination of skin from both patients revealed an acute perifollicular inflammation. Pyoderma gangrenosum should be considered in the differential diagnosis of pustular disorders in children with underlying conditions such as ulcerative colitis.

Abscess↗

Peripheral ulcerative keratitis--an extracutaneous neutrophilic disorder: report of a patient with rheumatoid arthritis, pustular vasculitis, pyoderma gangrenosum, and Sweet's syndrome with an excellent response to cyclosporine therapy.

The term peripheral ulcerative keratitis represents a spectrum of inflammatory diseases, characterized by cellular infiltration, corneal thinning, and ulceration. Neutrophilic dermatoses are rarely associated with peripheral ulcerative keratitis. To date, peripheral ulcerative keratitis has only been reported in patients with pyoderma gangrenosum. Separate episodes of pyoderma gangrenosum, Sweet's syndrome, and pustular vasculitis developed in a 60-year-old patient with rheumatoid arthritis over an 8-year period. Over the past 2 years, 3 episodes of peripheral ulcerative keratitis occurred. Cyclosporine (4 mg/kg/d) treatment was started on confirmation of pyoderma gangrenosum. Over the ensuing 2 years, it became evident that the activity of her ocular and skin diseases, as well as her arthritis, paralleled the administration or cessation of cyclosporine therapy. Dermatologists should be aware of the association of Sweet's syndrome, pyoderma gangrenosum, and pustular vasculitis with peripheral ulcerative keratitis. This rare ocular manifestation and the serious sequelae when left untreated make recognition crucial. Cyclosporine proved to be a very effective treatment for all of our patient's diseases.

Arthritis, Rheumatoid↗

[Pyoderma gangrenosum and paraneoplastic chronic polyarthritis disclosing Hodgkin's lymphoma].

Pyoderma gangrenosum is a rare skin disease of unknown pathogenesis associated, in almost 8 out of 10 cases, with a systemic disease, notably enterocolitis or hemopathy. We report the case of a 57-year old man who had been presenting with pyoderma gangrenosum for 5 years when he developed a rheumatoid-like seronegative chronic polyarthritis. The occurrence, some time later, of a supraclavicular adenopathy led to the diagnosis of Hodgkin's disease. To our knowledge, the pyoderma-chronic polyarthritis-Hodgkin's lymphoma association has never been reported. Treatment of the lymphoma resulted in complete disappearance of cutaneous and articular symptoms. The fact that neither the skin disease nor the polyarthritis recurred during a 3-year follow-up after treatment was discontinued, incites us to discuss the possibility that the pyoderma and the polyarthritis observed in this patient were neoplastic diseases.

Arthritis↗

Severe peristomal pyoderma gangrenosum: a case study.

Pyoderma gangrenosum is an autoimmune disease that often manifests itself as painful ulcers. When these ulcers occur in the peristomal area, symptom management and wound care must be balanced against the need to pouch the stoma. Although the treatment of pyoderma gangrenosum is not standardized, systemic steroid therapy is frequently used as first-line therapy. The WOC nurse is often asked to manage both the pouching needs and topical therapy of the patient with peristomal pyoderma ulcerations. This article describes the management of severe pyoderma gangrenosum ulcerations in a 59-year-old woman with a long-standing ileostomy.

Bandages↗

The role of normal skin in the spread of streptococcal pyoderma.

The primary body site of acquisition of group A streptococci was examined prospectively in a population with endemic streptococcal pyoderma. Weekly cultures were obtained during the skin infection season from apparently normal upper respiratory and cutaneous sites (and from skin lesions when present) in 44 children and adults living on the Red Lake Indian Reservation.During the 9-week period of the study 705 of a total of 2305 cultures were positive for group A streptococci. The percentage of positive cultures from the various sites were: throat (20%); nose (24%); wrist (32%); ankle (35%); back (22%); and skin lesions (81%). Group A streptococci were also isolated from fingernail dirt, clothing and bedding as well as from a few household pets and insects.Analysis of serial cultures obtained from the same individuals at weekly intervals suggested that the strains isolated from skin lesions first appeared on normal skin in the 2 weeks preceding the lesion. Spread to the nose and throat followed skin acquisition and/or skin lesions.The high prevalence of group A streptococci on normal skin in the absence as well as the presence of pyoderma, and their appearance on normal skin before recovery from either skin lesions or the upper respiratory tract are consistent with the view that skin acquisition was a primary predisposing factor to pyoderma. Since the literature indicates that group A streptococci are rarely part of the normal skin flora, these findings raise the possibility of unique biological properties of these and perhaps other pyoderma strains, as distinct from other group A streptococci.

Adolescent↗

Therapy Insight: pyoderma gangrenosum-old disease, new management.

Well-designed studies that help guide physicians to apply the optimal therapeutic strategy for the management of pyoderma gangrenosum are lacking in the literature. A multidisciplinary approach is paramount for the effective management of this condition, with close involvement of a wound-care specialist and a microbiologist. Treatment should be stepwise in nature. Local wound care, avoidance of trauma and the application of local steroid or tacrolimus ointment are the first-line treatments. Steroid therapy is the most widely published effective therapy for achieving resolution of pyoderma gangrenosum, although there is growing evidence for the efficacy of infliximab in refractory cases. Other therapies such as dapsone and clofazamine should be left as third-line agents for refractory pyoderma gangrenosum, while novel treatments such as granulocyte apheresis should only be used under trial conditions, to gain an objective evaluation of their efficacy. This article reviews the published treatment strategies in current use, and aims to guide the effective management of pyoderma gangrenosum.

Anti-Inflammatory Agents, Non-Steroidal↗

Pyoderma gangrenosum occurring in a lower limb fasciocutaneous flap--a lesson to learn.

Pyoderma gangrenosum is a destructive cutaneous disease characterised by progressive painful ulceration. The occurrence of pyoderma gangrenosum at a surgical site is rare (especially if there is no predisposing illness), but is well recognised. We present a case of a 63-year-old man who developed erythematous ulcerative lesions due to pyoderma gangrenosum in and around a lower limb fasciocutaneous flap used to cover an exposed total knee prosthesis. The lesions were initially confused with postoperative wound infection. No predisposing disorder, other than the rarely reported association with osteoarthritis, was found. The diagnosis is important because its rapid detection not only avoids unnecessary treatment but also allows for prompt intervention with oral steroids. This case is presented to alert surgeons to the presence of pyoderma gangrenosum and its diagnostic confusion with postoperative wound infection.

Anti-Inflammatory Agents↗

Simultaneous bilateral microvascular tissue transfer for soft-tissue defects of the lower extremities in pyoderma gangrenosum.

Pyoderma gangrenosum is a necrotizing skin disease of unknown etiology. The lesions are painful and occur mostly on the lower extremities, either single or multiple, but can appear in any region of the body. The authors report a case of pyoderma gangrenosum involving bilateral skin lesions over the anterior surface of the legs in a 13-year-old male patient. The lesions presented with ulcerative colitis, which is one of the most commonly associated diseases of pyoderma gangrenosum. After examination, systemic immunosuppressive treatment was administered and the progression of the disease was controlled. The lesions were treated with the simultaneous application of two free anterolateral thigh fasciocutaneous flaps after radical debridement. The procedure was successfully performed and no recurrence was observed. Although microvascular free tissue transfer cannot be performed in a standard fashion in pyoderma gangrenosum cases because of the risk of pathergy response, it should be considered as a surgical option in selected cases, otherwise difficult to manage, with the simultaneous application of appropriate systemic medical treatment.

Adolescent↗

Superficial granulomatous pyoderma treated with intravenous immunoglobulin.

Superficial granulomatous pyoderma is a rare variant of pyoderma gangrenosum. The superficial ulceration and vegetative margins are clinically distinctive; the suppurative and granulomatous histology is characteristic, though not pathognomonic. The condition is said to be relatively benign and more responsive to treatment than classic pyoderma gangrenosum, though published evidence of this is not always convincing. We present a case of superficial granulomatous pyoderma that was unusually aggressive, but that underwent dramatic and lasting resolution after intravenous immunoglobulin therapy.

Aged↗

Bullous pyoderma gangrenosum as the presenting sign of fatal acute myelogenous leukemia.

Bullous pyoderma gangrenosum begins as a bulla, nodule or nonulcerated erythematous plaque that blisters or ulcerates to form a superficial ulcer surrounded by a hemorrhagic, bullous border, which is surrounded by a blue-gray halo. Bullous pyoderma gangrenosum is most commonly associated with hematologic malignancies, specifically, acute myelogenous leukemia (AML). We report a patient whose initial presentation with bullous pyoderma gangrenosum prompted the appropriate diagnostic evaluation and confirmation of AML, which was ultimately fatal. We emphasize that a thorough hematologic investigation, including bone marrow biopsy, should be performed in all patients who present with lesions clinically suggestive of bullous pyoderma gangrenosum because the skin lesion may be the only indicator of the underlying hematologic disorder.

Antineoplastic Combined Chemotherapy Protocols↗

Annular crystalline keratopathy in association with immunoglobulin therapy for pyoderma gangrenosum.

PURPOSE: To report on a patient with a clinical presentation of annular crystalline keratopathy after immunoglobulin therapy for pyoderma gangrenosum. METHODS: Case report of a 6-year-old boy with biopsy-proven history of pyoderma gangrenosum who had undergone several cycles of systemic immunoglobulin therapy. The literature on ocular manifestations associated with pyoderma gangrenosum was reviewed. RESULTS: One year after the last cycle, the patient complained of the sudden onset of photophobia. Slit-lamp biomicroscopy revealed bilateral symmetric crystalline deposits in an annular region of the cornea. Because visual acuity was not reduced, specific therapy was not initiated. Symptoms could be reduced by the prescription of medical edged filter lenses. CONCLUSIONS: Annular crystalline corneal deposits may be associated with immunoglobulin therapy or represent a hitherto unknown ocular complication of pyoderma gangrenosum.

Child↗

A new M-type of group A streptococcus of clinical importance in pyoderma and pharyngitis.

A new M-type of group A streptococcus, provisionally designated type 65, is described. The vaccine and other initially isolated strains of this type attracted attention because of the T-agglutination reactions 2/25, not previously encountered among pyoderma streptococci. The investigations characterizing the strains as members of a new type were done with streptococci isolated from patients with pyoderma. However, type 65 was subsequently found to cause both pyoderma and acute pharyngitis. The T-2 agglutination reactions encountered with original members of this type, plus the cross-reactions later seen with type 65 antiserum and M-type 2 streptococci, prompted a comparison of this new type with M-type 2 streptococci, including those with the T-2 agglutination and others with the 8-25-Imp. 19 complex. The two M-antigens were clearly distinguished from one another in reciprocal bactericidal and precipitin tests with absorbed antisera. They were further distinguished in that all type 65 strains were opacity-factor (OF) negative, whereas type 2 streptococci were uniformly OF-positive. Most M-type 65 strains subsequently found in surveillance studies were shown to be members of the 8-25-Imp,19 T-complex. Type 65 is thus a newly described type which shares with M-types 55 and 57 a commom T-agglutination pattern and, like members of these types, fails to produce opacity factor. In our colleciton of strains, from both pyoderma and pharyngitis, shown to be members of the 8-25-Imp. 19 complex, and OF-negative, only type 65 has been identified to date. In contrast to types 55 and 57, the new type 65 does not appear to be of major importance in causing acute glomerulonephritis.

Agglutination Tests↗

Characterisation of Staphylococcus intermedius isolates from canine pyoderma and from healthy carriers by SDS-PAGE of exoproteins, immunoblotting and restriction endonuclease digest analysis.

Ten Staphylococcus intermedius isolates from cases of canine pyoderma and 10 from healthy carriers were examined by SDS-PAGE of exoproteins, immunoblotting and restriction endonuclease digest analysis. Similarities between banding patterns of the isolates were calculated as Dice coefficients for all three methods. For SDS-PAGE and immunoblotting, no significant differentiation was found between the pyoderma and "healthy" groups. Analysis of DNA digested with BglII indicated that S. intermedius is genetically heterogeneous; Dice coefficients for the pyoderma group were distinct from those for the healthy group (p < 0.001), and cluster analysis confirmed that the pyoderma isolates (9) formed a group separate from the majority (6 of 9) of the normal isolates.

Animals↗

Minimizing the risk of post-operative pyoderma gangrenosum.

A 61-year-old woman with seropositive rheumatoid arthritis developed numerous ulcers due to pyoderma gangrenosum at suture entry/exit sites following an arthroplasty of the right hip when interrupted silk sutures were used to close the skin. When a subsequent arthroplasty was performed on the left hip and subcuticular Dexon sutures were used to close the skin only two small ulcers developed. Sixteen cases of pyoderma gangrenosum developing in surgical wounds have previously been reported. We recommend that surgery in patients with a history of pyoderma gangrenosum is performed when the pyoderma is clinically quiescent, and that subcuticular sutures are used for skin closure, thus avoiding puncturing the skin surface.

Adolescent↗

Superficial granulomatous pyoderma.

Superficial granulomatous pyoderma, recently described as a variant of pyoderma gangrenosum, would be better termed pathergic granulomatous cutaneous ulceration as the seven previously described cases, as well as our own two cases, have significant dermal involvement histologically and heal with scarring. In contrast to pyoderma gangrenosum, lesions of superficial granulomatous pyoderma respond to less toxic anti-inflammatory agents.

Adult↗