Acute pustulosis of the legs in diverticulitis with sigmoid stenosis: an overlap between bowel-associated dermatosis–arthritis syndrome and pustular pyoderma gangrenosum

BACKGROUND: Bowel-associated dermatosis-arthritis syndrome denotes the occurrence of diarrhoea with arthritis and skin lesions related to bowel disease with or without bowel bypass. In this condition, the histological finding of cutaneous aseptic neutrophilic cell infiltrate is non-specific and common to a wide spectrum of neutrophilic dermatoses, including pyoderma gangrenosum. OBSERVATION: We describe a 78-year-old woman with fever, abdominal discomfort and arthralgias, who developed grouped pustular lesions on her shins with histologically spongiform pustule formation. Aetiological assessment disclosed diverticular disease with sigmoid stenosis. CONCLUSION: Although clinical and histological features in our case fit the diagnosis of bowel-associated dermatosis-arthritis syndrome, they may also correspond to a pustular variant of pyoderma gangrenosum. Our observation raises the question of the nosological classification of bowel-associated dermatosis-arthritis syndrome within the spectrum of neutrophilic diseases.     

Minocycline Hydrochloride Hyperpigmentation Complicating Treatment of Pyoderma Gangrenosum

Minocycline-associated hyperpigmentation is an uncommon side effect We report the case of a patient with pyoderma gangrenosum successfully treated with oral minocycline but complicated by marked hyperpigmentation in his pyoderma gangrenosum and acne scars. One of the clinical forms of minocycline hyperpigmentation includes dark-blue or black macules in depressed acne scars or other sites of skin inflammation; this pattern seems to be independent of the total cumulative dose and the skin process.     

Pulmonary nodules and nodular scleritis in a teenager with superficial granulomatous pyoderma gangrenosum

Superficial granulomatous pyoderma gangrenosum, a rare variant of pyoderma gangrenosum, has been considered to be the most benign form of the disease. We present the case of a 15‐year‐old boy with pulmonary involvement and nodular scleritis associated with this unusual type of pyoderma gangrenosum and discuss its differential diagnosis.     

Pyoderma gangrenosum in an ulcerative colitis patient during treatment with vedolizumab responded favorably to adsorptive granulocyte and monocyte apheresis

Pyoderma gangrenosum (PG) is an extra-intestinal skin lesion in inflammatory bowel disease (IBD) as is erythema nodosum. Vedolizumab (VED) is a monoclonal antibody that targets α4β7 integrin and has an intestinal selective mechanism. Despite good therapeutic effects on colitis, the effect on extra-intestinal manifestations (EIMs) remains unclear. Here we report a case of ulcerative colitis complicated by PG during treatment with VED, which was successfully treated with prednisolone in combination with adsorptive granulocyte and monocyte apheresis (GMA). The patient was a 50-year-old woman with a past medical history of extensive ulcerative colitis managed by golimumab (GLM). She developed flare symptoms due to loss of response to GLM, and treatment was switched to VED. Her gastrointestinal symptoms were improved with VED treatment with less frequent bowel movements. However, infiltrative erythema with pain appeared on the right lower leg and right knee, and expanded and gradually ulcerated. Her skin lesions were treated with corticosteroid, but showed poor improvement. Therefore, granulocyte and monocyte apheresis (GMA) treatment was administered in combination with prednisolone. After 3 months, the ulcer gradually improved, and at the time of this writing, the eruptions were nearly replaced by epithelial tissue. This case study showed that patients with UC and EIMS may respond well to combination therapy of VED and GMA. GMA has a very favorable safety profile. On the other hand, the causal connection between VED and PG is still unclear. We believe that a combination therapy involving VED and GMA in IBD patients with EIMs warrants consideration.     

An interesting case of pyoderma gangrenosum with immature hystiocytoid neutrophils

We present a unique case of a 36‐year‐old male who developed more than 20 pyoderma gangrenosum (PG) ulcers showing on histopathology a dense inflammatory infiltrate composed of histiocytoid mononuclear immature cells with a strong positivity for myeloperoxidase and Leder stain, suggesting a myeloid lineage in the absence of a concomitant myeloproliferative disorder. Histiocytoid Sweet syndrome (SS) is now recognized as a histological subtype of SS. Although PG and SS belong to the spectrum of neutrophilic diseases, to the best of our knowledge, this is the first case of a “Histiocytoid pyoderma gangrenosum” encompassing immature granulocytes in the absence of leukemia cutis.     

Pyoderma gangrenosum and pseudoepitheliomatous hyperplasia: a poorly recognized association

The histopathological distinction between florid pseudoepitheliomatous hyperplasia (PEH) and squamous cell carcinoma (SCC) is frequently difficult. Knowledge of the list of underlying conditions known to be associated with PEH and the recognition of features of such entities under the microscope are crucial to avoid a misdiagnosis of malignancy. The link between pyoderma gangrenosum (PG) and PEH is poorly understood and has been rarely mentioned in the literature. Given the lack of specific clinical and histopathological diagnostic features of PG, distinguishing PEH and SCC in epidermal proliferations associated with PG is particularly problematic. We report a case of PG of the dorsal hands which was initially mistaken for a well differentiated SCC on a skin biopsy and only correctly diagnosed on careful clinicopathological review.     

Chronic Leg Ulceration Associated with Polycythemia Vera Responding to Ruxolitinib (Jakafi®)

We present the case of a 63-year-old white male with bilateral chronic leg ulcers due to polycythemia vera and hydroxyurea therapy who demonstrated dramatic healing of his wounds in response to ruxolitinib (Jakafi^®, Novartis), a novel Janus kinase-1 and -2 inhibitor. This patient's wound had previously been refractory to multiple surgical interventions and immunosuppression. After the initiation of ruxolitinib, the patient underwent successful split-thickness skin grafting, with resultant healing of his wounds. He was stable without prednisone and other immunosuppressant therapy and had healed at 6 months. Ruxolitinib therapy could represent a novel option for patients who develop persistent inflammatory wounds in the setting of polycythemia vera and hydroxyurea therapy.     

The safety of treatments used in pyoderma gangrenosum

Introduction: Pyoderma gangrenosum (PG) is a severe ulcerating orphan dermatosis characterized by painful and rapidly progressive skin ulcers often associated with underlying inflammatory disease.

Areas covered: In this article, we review and analyze the literature regarding treatment options for patients with PG, with particular attention to the efficacy and safety of therapies. Despite the significance of this problem, there are few studies devoted to the efficacy or safety of therapeutics in PG. We aim to present and evaluate existing studies and reports, and to make treatment recommendations based on the efficacy and safety data reviewed.

Expert opinion: All patients with PG should be counseled on avoiding trauma, optimizing glycemic control, and smoking cessation. Proper local wound care and surveillance of superimposed infection is essential to healing. Control of underlying inflammatory conditions should be co-managed with appropriate specialists. Patients with limited disease should consider high potency topical steroid or topical calcineurin inhibitors. For systemic therapy, the best evidence supports the use of systemic steroids or cyclosporine. Biologic therapy should be reserved in patients as a third line therapy or in patients with underlying systemic inflammatory disease. There is an existing need for well-designed studies to evaluate the efficacy and safety of therapeutics in PG.     

The great imitator with no diagnostic test: pyoderma gangrenosum

Pyoderma gangrenosum is a rare, neutrophil‐mediated, auto‐inflammatory dermatosis that wound care specialists must be prepared to recognise. This condition has clinical features analogous to infectious processes. There is no specific diagnostic test and the diagnosis is usually obtained from exclusion. Its early recognition and proper management with prompt initiation of immunosuppressive therapy are essential to improve the quality of life and the prognosis of patients.     

Pyoderma gangrenosum‐like lesions provocated by botulinum injections

Botulinum toxin (BoNT) is a valuable therapeutic tool with several medical indications and the most popular of all cosmetic procedures worldwide. This is the reason for the growing number of unregistered products that may be the reason for adverse reactions. We present a case of a 51‐year‐old woman, who developed a pyoderma gangrenosum‐like reaction at injection sites after the administration of an unregistered BoNT product by a beautician. The clinical course, the morphology of the lesions, the result of histopathological biopsy, and the response to the treatment meets the criteria for diagnosis of pyoderma gangrenosum. The case presented by us is the first adverse reaction of this type after BoNT administration.