Spectrum of diseases associated with pyoderma gangrenosum and correlation with effectiveness of therapy: New insights on the diagnosis and therapy of comorbid hidradenitis suppurativa.

Pyoderma gangrenosum (PG) has been linked to various underlying systemic diseases; many associations are based on case reports or small case series, including hidradenitis suppurativa. Literature examining systemic therapies according to underlying comorbid condition is limited. The study objective was to investigate comorbid diseases of PG and correlate disease associations with effectiveness of therapeutic interventions. Using Johns Hopkins Medical Institutions medical records, 220 patients had an ICD-9 code of 686.01 for PG between 1 January 2006 and 30 June 2015, of whom 130 patients met rigorous inclusion/exclusion criteria for PG (non-peristomal). The 130 PG patients in our study were 69% female, 58% Caucasian, and 35% African American. Documented comorbid conditions included inflammatory bowel disease (IBD; 35%), rheumatoid arthritis (RA; 12%), hidradenitis suppurativa (HS; 14%), and monoclonal gammopathy (12%). PG patients with HS versus without HS were more likely to be African-American (83% vs. 28%; P < 0.001) and had an earlier mean age of PG onset (38 vs. 48 years; P = 0.02). Strikingly, 53% of female African-American patients with PG onset prior to age 40 had comorbid HS. Comorbid inflammatory bowel disease was observed in 38% of PG patients with RA, 28% of PG patients with HS, and 27% of PG patients with monoclonal gammopathy. Of the 32 patients who received infliximab for active PG, complete ulcer healing was observed in 83% (5/6) of patients with comorbid HS versus 31% (8/26) of patients without HS (Fisher exact P = 0.03). Screening patients for associated systemic disease for multiple related illnesses is essential. Effectiveness of systemic therapy may depend upon the underlying systemic disease; hidradenitis suppurativa may be a specific example.

Squamous cell carcinoma complicating a chronic lesion of hidradenitis suppurativa: a case report and review of the literature.

Squamous cell carcinoma (SCC) arising from chronic hidradenitis suppurativa (HS) is rare; however, the morbidity associated with this presentation is high and management has not been standardised or optimised. We present a case of HS of the perineum and buttocks complicated by SCC, requiring multiple extensive surgical resections. Adjuvant radiotherapy was withheld initially because of concern for poor healing of the surgical wound but was eventually initiated after a second recurrence was identified. The patient ultimately expired 4 years after the initial diagnosis of SCC. We also review 80 cases of SCC complicating HS found in the English literature. Case reports and mechanistic studies suggest the possibility that human papilloma virus and smoking may be risk factors associated with SCC in HS. Despite the majority of SCC cases being well-differentiated tumours in HS, the highly aggressive nature of SCC in HS and its high likelihood for rapid progression, recurrence, metastasis and high mortality suggests the need to advocate for aggressive treatment. We recommend an aggressive approach to management at the time of SCC diagnosis in HS, which includes appropriate imaging to establish the extent of the tumour, large and deep surgical excision, sentinel lymph node evaluation, consultation with radiation oncology for potential adjuvant radiation therapy and close surveillance.

Voriconazole-Induced Subacute Cutaneous Lupus Erythematosus in an Adult With Aspergillosis.

A 59-year-old man was treated with voriconazole for chronic invasive aspergillosis and who subsequently developed subacute cutaneous lupus erythematosus (SCLE). The patient presented with a 6-week history of multiple erythematous papulosquamous lesions on his chest, upper and lower extremities, and back (Figure 1). They were nonpruritic and nonpainful. He was afebrile and otherwise well. He had no history of extensive sun exposure prior to the appearance of the eruption. He had been taking voriconazole for about 3 months prior to the onset of lesions. He denied any family history of connective tissue disease.

Folliculocentric cutaneous presentation of disseminated Candida krusei infection in a patient with acute myeloid leukemia.

Candida krusei (C. krusei) is a multidrug-resistant opportunistic fungal pathogen that may cause disseminated infections in immunocompromised hosts. However, its clinical and histologic features are not well-characterized. We present a unique case to contribute to the growing knowledge base associated with this organism. During hospitalization for neutropenic fever, a 19-year-old man with acute myeloid leukemia, who underwent hematopoietic stem cell transplantation, developed a generalized folliculocentric eruption following initiation of antifungal therapy for newly diagnosed C. krusei fungemia. Despite adequate antifungal coverage and negative blood cultures, the follicular-based erythematous papules persisted. Biopsies demonstrated yeast within ruptured follicles, without angiotropism or involvement of the interfollicular dermis, subcutaneous tissue, or stratum corneum. Concurrent skin tissue cultures confirmed C. krusei. The patient remained febrile despite aggressive antifungal therapy, with relapse of leukemia and subsequent death. Our case is unusual given the development of cutaneous lesions following clearance of fungemia, with yeast limited to ruptured follicular lumina, possibly indicating a primary cutaneous source or early transfollicular/transepidermal elimination. Given the limited available descriptions of cutaneous histopathology for C. krusei, we seek to add to the understanding of its pathophysiology and aid in the diagnosis and treatment of this often fatal infection.

TIPS Placement via Combined Transjugular and Transhepatic Approach for Cavernous Portal Vein Occlusion: Targeted Approach.

Purpose. We report a novel technique which aided recanalization of an occluded portal vein for transjugular intrahepatic portosystemic shunt (TIPS) creation in a patient with symptomatic portal vein thrombosis with cavernous transformation. Some have previously considered cavernous transformation a contraindication to TIPS. Case Presentation. 62-year-old man with chronic pancreatitis, portal vein thrombosis, portal hypertension and recurrent variceal bleeding presents with melena and hematemesis. The patient was severely anemic, hemodynamically unstable, and required emergent portal decompression. Attempts to recanalize the main portal vein using traditional transjugular access were unsuccessful. After percutaneous transhepatic right portal vein access and navigation of a wire through the occluded main portal vein, an angioplasty balloon was inflated at the desired site of shunt takeoff. The balloon was targeted and punctured from the transjugular approach, and a wire was passed into the portal system. TIPS placement then proceeded routinely. Conclusion. Although occlusion of the portal vein increases difficulty of performing TIPS, it should not be considered an absolute contraindication. We have described a method for recanalizing an occluded portal vein using a combined transhepatic and transjugular approach for TIPS. This approach may be useful to relieve portal hypertension in patients who fail endoscopic and/or surgical therapies.

Intercostal artery pseudoaneurysm formation after irinotecan transarterial chemoembolization of a spinal metastasis from colorectal cancer.

Over the past decade, irinotecan has become one of the first-line chemotherapeutic agents used in the treatment of metastatic colorectal cancer. Recently, irinotecan has been administered transarterially in order to perform chemoembolization in the liver. In the limited number of reports available to date using this approach, serious adverse effects have not yet been reported. In this paper, we describe the formation of an intercostal artery pseudoaneurysm after transarterial chemoembolization with irinotecan-eluting beads in a patient with spinal metastasis from colorectal cancer.

Bone-borne palatal distraction to correct the constricted cleft maxilla.

BACKGROUND: Transverse maxillary hypoplasia, with or without cleft deformity, is typically treated with orthodontic expansion. However, the management of those patients who present later in life with severe uncorrected deformities or who have failed prior orthodontic care remains controversial. Tooth-borne palatal expanders have limitations in this subset of patients with scarred, constricted cleft palate. In this study, we assess the efficacy and safety of using a bone-borne palatal distraction device as an alternative treatment for this difficult subset of patients. METHODS: Older children with a constricted maxilla who previously had unilateral or bilateral cleft lip and palate repairs and failed orthodontic expansion were included in the study (n = 15). Patients underwent Le Fort I corticotomy with placement of the bone-borne distraction device, expansion at a rate of 0.5 mm/d, and subsequent alveolar bone grafting. Preoperative and follow-up maxillary impressions were compared with assessed improvements in intermolar distance, intercanine distance, alveolar cleft width, and total palatal area. In addition, a small group of noncleft patients with constricted maxillas was treated with bone-borne palatal distraction without a corticotomy (n = 4). RESULTS: The mean amount of distraction in all patients was 14.1 mm, with a follow-up period of 19 months (range, 8-30 months). The average increase in intermolar distance was 8.4 mm, intercanine distance increased by an average of 9.5 mm, and palate surface areas were increased by a mean of 28.9 mm2. Relapse was between 4% and 7%, and all patients underwent subsequent alveolar bone grafting. In addition, the noncleft patients successfully underwent bone-borne palatal distraction without a corticotomy, with a 4% to 5% relapse. CONCLUSION: Rapid palatal expansion using a bone-borne distraction device in cleft patients with transverse maxillary hypoplasia who have failed nonsurgical orthodontic expansion provides adequate expansion necessary for alveolar bone grafting and stable results.