Two-Year Delayed Healing of an Endovascular Access Site Related to an Unusual Etiology.

In this case report, the authors describe a patient who underwent an endovascular abdominal aortic aneurysm repair complicated by more than a 2-year delay in healing of the left inguinal fold access site. Providers initially suspected a chronic infection or foreign body reaction, but eventually the patient was diagnosed with superficial granulomatous pyoderma. Once the correct etiology was determined and appropriate treatment begun, the access site healed within 3 weeks.

Adult-onset primary cyclic autoimmune neutropenia: a case report.

BACKGROUND: A few cases of primary autoimmune neutropenia (AIN) have been reported in adults, but cyclic primary AIN, which is characterized by the periodic oscillation of neutrophils, is uncommon in adults. STUDY DESIGN AND METHODS: Herein, we report a 70-year-old man referred to our hospital with severe neutropenia and thrombocytopenia. He had experienced intermittent episodes of low-extremity purpura for the past 3 months, with cellulitis on the skin of the scalp 1 month previously. RESULTS: The patient presented with severely low neutrophil and platelet (PLT) counts. Myeloid progenitors and megakaryocytes were increased in the marrow, but mature neutrophils were remarkably decreased. Anti-neutrophil antibodies to specific epitopes were detected at neutropenia. Based on these findings, AIN accompanied by autoimmune thrombocytopenia was diagnosed. The patient experienced synchronous fluctuations of neutrophil and PLT counts three times. Despite no treatment, the neutrophil count fluctuated within the range of 0.06 × 109 to 1.65 × 109 /L, and the PLT count fluctuated from 0.7 × 1010 to 20.5 × 1010 /L. We identified an inverse relationship between neutrophil count and anti-neutrophil antibody titers, establishing the conclusive diagnosis of cyclic AIN. After prednisolone treatment, the neutrophil and PLT counts normalized, and the patient has maintained long-term remission. CONCLUSION: We report a rare case of cyclic AIN diagnosed from the inverse association between periodic oscillation of anti-neutrophil antibody titers and neutrophil counts. This clinical course suggests that in AIN patients, laboratory data and recurrent signs of infection should be monitored regularly, including shortly after neutrophil recovery.

Palmoplantar keratoderma with progressive gingivitis and recurrent pyodermas.

Papillon-Lefèvre syndrome (PLS) is a rare inherited palmoplantar keratoderma (PPK) that is associated with progressive gingivitis and recurrent pyodermas. We present a case exhibiting classic features of this autosomal-recessive condition and review the current understanding of its pathophysiology, diagnosis, and treatment. Additionally, a review of pertinent transgredient PPKs is undertaken, with key and distinguishing features of each syndrome highlighted.

Varying phenotypes in swine versus murine transgenic models constitutively expressing the same human Sonic hedgehog transcriptional activator, K5-HGLI2 Delta N.

This study was undertaken to characterize the effects of constitutive expression of the hedgehog transcriptional activator, Gli2, in porcine skin. The keratinocyte-specific human transgene, K5-hGli2 Delta N, was used to produce transgenic porcine lines via somatic cell nuclear transfer techniques. In mice, K5-hGli2 Delta N induces epithelial downgrowths resembling basal cell carcinomas. Our porcine model also developed these basal cell carcinoma-like lesions, however gross tumor development was not appreciated. In contrast to the murine model, diffuse epidermal changes as well as susceptibility to cutaneous infections were seen in the swine model. Histologic analysis of transgenic piglets revealed generalized epidermal changes including: epidermal hyperplasia (acanthosis), elongated rete ridges, parakeratotic hyperkeratosis, epidermal neutrophilic infiltration, capillary loop dilation and hypogranulosis. By 2 weeks of age, the transgenic piglets developed erythematic and edematous lesions at high contact epidermal areas and extensor surfaces of distal limb joints. Despite antibiotic treatment, these lesions progressed to a deep bacterial pyoderma and pigs died or were euthanized within weeks of birth. Non-transgenic littermates were phenotypically normal by gross and histological analysis. In summary, constitutive expression of the human hGli2 Delta N in keratinocytes, results in cutaneous changes that have not been reported in the K5-hGli2 Delta N murine model. These findings indicate a need for a multiple species animal model approach in order to better understand the role of Gli2 in mammalian skin.

Transient thrombocytosis with megathrombocytes in a case of acute myeloblastic leukemia.

Thrombocytosis is commonly seen in reactive conditions and certain neoplastic states, such as chronic myeloproliferative disorders. It is rarely seen in acute leukemia. A 12-year-old girl with acute myeloblastic leukemia (FAB M2) in remission presented with pyoderma. Her hemogram revealed anemia (Hb-6.4g/dl), leucopenia (TLC - 1.2 x 109/L) and thrombocytosis (platelet count- 580 x 109/L). A peripheral blood film showed numerous abnormally large platelets with few atypical cells. The thrombocytosis subsided with the clearance of infection but atypical cells persisted. One month later, she relapsed. Cytogenetic analysis revealed variable results (trisomy 9 and deletion 3). This case has been presented because thrombocytosis is rare in AML and its appearance calls for a close follow-up.

Pyoderma faciale.

Pyoderma faciale is a rare cutaneous disorder that predominantly affects women in their 20s and 30s and is characterized by the rapid appearance of coalescing nodules and draining sinuses, combined with livid erythema on the face. We describe a 40-year-old woman who presented with localized pyoderma faciale that worsened during treatment with oral and topical antibiotics and corticosteroids. Subsequent treatment with isotretinoin for 5 months resulted in dramatic and sustained improvement.

Karyotypic analysis of bone marrow cells in pyodermic lesions associated with myelodysplastic syndrome.

BACKGROUND: Recalcitrant pyodermic lesions and neutrophilic dermatoses are often associated with subclinical myelodysplastic syndrome (MDS). In this case series, we assessed the diagnostic importance of karyotypic analysis of bone marrow cells in 4 patients with MDS-associated pyodermic eruptions treated at our university hospital. Karyotypic analysis was performed in bone marrow cells and peripheral blood lymphocytes obtained. Serum levels of granulocyte colony-stimulating factor were measured. OBSERVATIONS: Four patients with pyodermic eruptions or neutrophilic dermatosis had chromosomal abnormalities in bone marrow cells, including del(20)(q11;q13.3) in 2 patients, trisomy 8 in 1 patient, and t(11;22)(q23;q11) in 1 patient. Three patients without morphologic findings suggestive of MDS were diagnosed as having refractory anemia. One female patient had refractory anemia with ringed sideroblasts associated with del(20). Two patients with refractory anemia had a normal karyotype in peripheral blood lymphocytes. Two patients with elevated serum levels of granulocyte colony-stimulating factor had more active or widespread cutaneous diseases. CONCLUSIONS: Karyotypic analysis of bone marrow cells, but not of peripheral blood lymphocytes, is essential in proving a diagnosis of MDS-associated pyodermic lesions. The overexpression of granulocyte colony-stimulating factor, which may compensate for impaired hematopoiesis in patients with MDS, seems to be a key cytokine leading to neutrophilic infiltration.

Blastomycosis-like pyoderma.

A 9-year-old female, presented with recurrent bilaterally symmetrically distributed flesh colored vegetative plaques, papules and nodules on trunk and upper and lower extremities since 15 days. Investigations revealed anemia, hypoproteinemia, decreased albumin and positive D-xylose test. Pus swab and biopsy for culture sensitivity showed Enterococci species. Biopsy showed spongiotic psoriasiform dermatitis with subcorneal pustule. She fulfilled criteria for the diagnosis of blastomycosis-like pyoderma viz. presentation of large verrucous plaques with pustules and elevated border, pseudoepitheliomatous hyperplasia with abscess histologically and growth of one pathogenic bacterium on culture or tissue biopsy. She responded to long-term amoxicillin-clavulanic acid therapy.

Pyoderma vegetans associated with severe psoriatic arthritis: good response to etanercept.

Pyoderma vegetans (PV) is an inflammatory dermatosis, characterized clinically by large exudative vegetating plaques, and histopathologically by epidermal pseudoepitheliomatous hyperplasia and dense inflammatory infiltrates. Although PV is a very rare condition, it is a chronic disorder that may accompany any systemic process that compromises immunity. Treatment is very difficult, and correction of predisposing causes may be useful. We present a 49-year-old woman affected by severe psoriatic arthritis since she was 19, with giant verrucous plaques on her lower limbs that had worsened progressively during the last 15 years. After ruling out other vegetating cutaneous disorders, PV was diagnosed in association with psoriasis. Despite numerous previous systemic and topical therapeutic attempts no response was observed. Etanercept was introduced, which resulted in a marked improvement within 3 weeks. Herein, we report a diagnostic and therapeutic challenge of the first case of PV associated with psoriasis that presented a good response to etanercept.

Cutaneous Wegener's granulomatosis (malignant pyoderma) in a patient with Crohn's disease.

We report a case of an unusual presentation of Wegener's granulomatosis (WG) in a patient with Crohn's disease (CD). She presented to our Wound Care Center with 7th cranial nerve palsy and facial pyoderma-like ulcerations. Although WG has a predilection for the lung, kidney, and eyes, cutaneous involvement can be seen in 50% of the cases, and it can be the presenting sign in 9-14%. Because of the lethality of WG if not properly treated, the diagnosis is imperative.

Outcome of an interventional program for scabies in an Indigenous community.

OBJECTIVE: To implement an intervention program for reducing the prevalence of scabies in a large Northern Territory Aboriginal community. DESIGN: Prospective, longitudinal screening, intervention and follow-up study. PARTICIPANTS AND SETTING: All children aged 5 years and under in one of the largest Aboriginal communities in the Northern Territory, total population, approximately 2,200 (95% Indigenous). MAIN OUTCOME MEASURES: A decrease in prevalence of scabies, infected scabies and non-scabies pyoderma over seven months. RESULTS: The number of children aged 5 years and under screened intially and at the three follow-up screenings ranged from 201 to 242 (more than 98% of those eligible on each occasion). The prevalences of scabies, infected scabies and non-scabies pyoderma before intervention were 35%, 12% and 11%, respectively. At 6 weeks postintervention these had decreased to 3%, 1% and 4%, respectively; low prevalences were maintained at four and seven months. CONCLUSIONS: This intervention, which was based on community motivation, involvement and control, successfully reduced the prevalence of scabies. Continuing community health education and regular screening will be crucial in controlling scabies. The methods and results of this study may be helpful in developing a coordinated program for all remote Aboriginal communities in the area.

Pyoderma vegetans and ulcerative colitis.

Pyoderma vegetans (PV) is a chronic, vegetating pustular disorder characterized clinically by erythematous vesiculopustular vegetating cutaneous plaques. Marked epidermal hyperplasia, intraepidermal and subepidermal neutrophilic microabscesses and a dermal inflammatory infiltrate are the prominent histopathological findings. We describe a patient with PV associated with ulcerative colitis and mammary Paget's disease. Pustular eruptions associated with ulcerative colitis are reviewed.

Extraintestinal Crohn's disease: case report and review of the literature.

Crohn's disease is a granulomatous inflammatory bowel disease. Its pathologic findings include noncontiguous chronic inflammation and noncaseating granulomas. Any segment of the gastrointestinal tract can be involved, but it is uncommon to find that Crohn's disease has spread beyond the intestine. We describe the case of a man with extraintestinal Crohn's disease that was marked by quiescent involvement of the lower gastrointestinal tract and florid involvement of the nasal cavity, supraglottic structures, glottis, and skin.