July 2022 Medical Image of the Month: Pulmonary Nodule in the Setting of Pyoderma Gangrenosum (PG)

Figure 1. Unenhanced lung window chest CT images in the axial (A) and sagittal (B) planes show a solid, non-calcified irregular left upper lobe mass (arrow) with spiculated margins. The nodule demonstrates enhancement on soft tissue windows (C) with associated mediastinal adenopathy (arrowhead). The mass and adenopathy are FDG-avid on axial fused PET-CT image (D).

Figure 2.  (A) Photograph of one of the patient’s skin lesions.  (B) Hematoxylin and Eosin stained low-power pathological image of a biopsy specimen from a skin lesion demonstrates dense mixed neutrophilic dermal inflammation. Extensive infectious and neoplastic workup was negative. The histopathologic diagnosis was consistent with pyoderma gangrenosum.

A 70-year-old man presented with persistent cough productive of clear sputum which had persisted approximately 12 months after COVID-19 infection.  The patient reported a more recent history of night sweats and had also recently developed what he described as “blisters” on his chest wall and right shoulder starting 4 weeks prior to presentation that “opened up” giving off a bloody discharge.  The patient had been treated with trimethoprim-sulfamethoxazole and doxycycline without improvement and reported a 10-pound weight loss over the past several months.  The patient was a never-smoker with no significant travel history and a past medical history of asthma, GERD, gout, and chronic rhinitis. He had no history of autoimmune/inflammatory diseases or malignancy.

Vital signs and physical exam were normal, except for a 1 cm open wound in the center of the patient’s chest [Figure 2A].  A chest CT performed as part of the patient’s workup demonstrated a spiculated mass in the left upper lobe with adjacent mediastinal adenopathy [Figure 1A-C].  This prompted an FDG PET-CT, which demonstrated some increased uptake in the mass and adjacent lymph nodes [Figure 1D].  The mass was biopsied via bronchoscopy, pathology was nondiagnostic with rare groups of benign-appearing bronchial epithelial cells and blood.  The skin lesion was biopsied next demonstrating dense mixed neutrophilic dermal inflammation  [Figure 2B].  The diagnosis of pyoderma gangrenosum was made and the patient was treated with NSAIDs and a systemic glucocorticoid (40 mg/day, tapered over 10 weeks).steroid taper, The pulmonary mass , mediastinal lymph nodes and skin lesions all resolved over time.

Pyoderma gangrenous (PG) is a misnomer in every sense as it is neither infectious nor gangrenous.  It is a rare (3-10 cases/million/year) disorder of skin characterized by neutrophilic dermatosis which usually presents as a with inflammatory and ulcerative disorder of the skin lesions and is usually a diagnosis of exclusion (1).  PG has no pathognomonic clinical or histological findings. Majority of the cases have an underlying systemic disease, commonly inflammatory bowel disease (41%), inflammatory arthritis (20.5%) and oncologic or hematologic disorders (17.2%). While it can in any age group including children, the peak age of onset is 40-60 years. There is a slight female preponderance (2). The most common presentation is inflammatory papule or pustule that progress to a painful ulcer with violaceous undermined borders and a purulent base. The lesions commonly occur in surgical wounds within 2 weeks of surgery, a phenomenon known as pathergy, and often lead to wound dehiscence (3). The lesions may also be peristomal in patients with IBD. Extracutaneous lesions have been reported in liver, intestine, spleen, cornea, bones, muscles, CNS and rarely, in the lungs (4-6).

There have been <50 cases of pulmonary PG ever described in literature (7,8). The patients may present with non-specific symptoms of cough, dyspnea, fever, weight-loss, malaise and occasionally hemoptysis. Chest imaging may show cavitary infiltrates. The diagnosis is established by cutaneous or extracutaneous lesion biopsy of the ulcer edge showing neutrophilic infiltrate. Extensive testing should be performed , extensive testing to rule out alternative causes including infection, and malignancy, in setting of underlying inflammatory bowel disease or inflammatory arthritisautoimmune and inflammatory conditions associated with PG. Presence of pathergy and response to anti-inflammatory therapy also support the diagnosis (9). Treatment includes systemic or intralesional glucocorticoids and/or calcineurin inhibitors (3). Use of TNF alpha inhibitor, infliximab and anti-neutrophil antimicrobial dapsone has also been described in case reports (10). Most patients achieve remission within 6 months to 3 years.

Umesh Goswami MD¹, Michael Gotway MD², Carlos Rojas MD², Prasad Panse MD², Kris Cummings MD², Eric Jensen MD², Kenneth Sakata, MD¹ and Clinton Jokerst MD²

Division of Pulmonology¹ and Department of Radiology²

Mayo Clinic Arizona, Scottsdale, AZ USA

References

1. Ruocco E, Sangiuliano S, Gravina AG, Miranda A, Nicoletti G. Pyoderma gangrenosum: an updated review. J Eur Acad Dermatol Venereol. 2009 Sep;23(9):1008-17. [CrossRef] [PubMed]
2. Ashchyan HJ, Butler DC, Nelson CA, et al. The Association of Age With Clinical Presentation and Comorbidities of Pyoderma Gangrenosum. JAMA Dermatol. 2018 Apr 1;154(4):409-413. [CrossRef] [PubMed]
3. Binus AM, Qureshi AA, Li VW, Winterfield LS. Pyoderma gangrenosum: a retrospective review of patient characteristics, comorbidities and therapy in 103 patients. Br J Dermatol. 2011 Dec;165(6):1244-50. [CrossRef] [PubMed]
4. Vadillo M, Jucgla A, Podzamczer D, Rufi G, Domingo A. Pyoderma gangrenosum with liver, spleen and bone involvement in a patient with chronic myelomonocytic leukaemia. Br J Dermatol. 1999 Sep;141(3):541-3. [CrossRef] [PubMed]
5. Scherlinger M, Guillet S, Doutre MS, Beylot-Barry M, Pham-Ledard A. Pyoderma gangrenosum with extensive pulmonary involvement. J Eur Acad Dermatol Venereol. 2017 Apr;31(4):e214-e216. [CrossRef] [PubMed]
6. Abdelrazeq AS, Lund JN, Leveson SH. Pouchitis-associated pyoderma gangrenosum following restorative proctocolectomy for ulcerative colitis. Eur J Gastroenterol Hepatol. 2004 Oct;16(10):1057-8. [CrossRef] [PubMed]
7. Gade M, Studstrup F, Andersen AK, Hilberg O, Fogh C, Bendstrup E. Pulmonary manifestations of pyoderma gangrenosum: 2 cases and a review of the literature. Respir Med. 2015 Apr;109(4):443-50. [CrossRef] [PubMed]
8. Sakata KK, Penupolu S, Colby TV, Gotway MB, Wesselius LJ. Pulmonary pyoderma gangrenosum without cutaneous manifestations. Clin Respir J. 2016 Jul;10(4):508-11. [CrossRef]  [PubMed]
9. Maverakis E, Ma C, Shinkai K, et al. Diagnostic Criteria of Ulcerative Pyoderma Gangrenosum: A Delphi Consensus of International Experts. JAMA Dermatol. 2018 Apr 1;154(4):461-466. [CrossRef] [PubMed]
10. Teasley LA, Foster CS, Baltatzis S. Sclerokeratitis and facial skin lesions: a case report of pyoderma gangrenosum and its response to dapsone therapy. Cornea. 2007 Feb;26(2):215-9. [CrossRef] [PubMed]

Cite as: Goswami U, Gotway M, Rojas C, Panse P, Cummings K, Jensen E, Sakata K, Jokerst C. July 2022 Medical Image of the Month: Pulmonary Nodule in the Setting of Pyoderma Gangrenosum (PG). Southwest J Pulm Crit Care Sleep. 2022:25(1):4-6. doi: https://doi.org/10.13175/swjpccs029-22 PDF 

June 2022 Medical Image of the Month: A Hard Image to Swallow

Alessandra Carrillo, DO

Robert Ondracek, DO

Shil Punatar, DO

Andrew Ondracek, DO

Ravi Sundaram, DO

Department of Critical Care Medicine

Franciscan Health

Olympia Fields, Illinois USA

Figure 1. Portable chest x-ray demonstrating marked dilatation of the esophagus with food impaction and bilateral aspiration of food particles. There is also a small left pleural effusion.

Figure 2. Coronal view CT-chest/abdomen/pelvis demonstrating marked dilatation of the esophagus with food impaction seen and food particles seen in his lungs bilaterally.

Introduction

Esophageal food impactions are common occurrences in gastroenterology, however, under 20% of cases require intervention (1)  .The clinical condition of the esophagus and the consistency of food being swallowed contribute to the development of food bolus impactions, with patients having underlying esophageal pathology in most cases (2). Unfortunately, radiographic evidence is often difficult to obtain as food is radiolucent and poorly visualized on radiograph. Here, we demonstrate the risk associated with severe food impaction.

Case Presentation

An 86-year-old man with a past medical history of achalasia with laparoscopic Heller myotomy complicated by distal esophageal perforation, was admitted after presenting with complaints of chest pain and inability to tolerate a solid diet. Additionally, he suffered a 90-pound weight loss over 1 year. He was seen by speech therapy and provided with a dysphagia appropriate diet. Eight days into the patient stay, the family presented to the patient's bedside to assist in 1-to-1 feeding of the patient per their request. One hour following the completion of the patient’s feeding, a CODE BLUE was called.  The patient was unresponsive and without a pulse. PEA protocol was initiated and return of spontaneous circulation was achieved. Post intubation chest x-ray demonstrated a markedly dilated esophagus (Figures 1). Thereafter, CT chest was ordered demonstrating markedly dilated appearance of the patient’s esophagus with internal food material without as a large obstructing lesion (Figure 2). This was deemed to be the cause of the patient's cardiac arrest with concomitant aspiration. Overall, the dilatation significantly progressed from previous imaging. The patient was made NPO, transitioned to total parenteral nutrition and plans were made for a follow-up disimpaction via esophagogastroduodenoscopy (EGD). Ultimately, the patient was too unstable to pursue EGD and expired 9 days after his initial arrest.

Discussion

Through literature review, a majority of cases of food bolus impaction are self-limited. In most cases described, boluses pass on their own or with the assistance of an EGD. In most cases, underlying esophageal or motility dysfunction is known. With few case reports, food disimpaction has been assisted with cola products or nifedipine (3,4). Though trivially regarded, our case demonstrates that food bolus revel against more gruesome esophageal pathology in both presentation, prompt intervention, and adverse on outcomes.

Conclusions

We illustrate a common presentation to gastroenterologists and physicians of a food bolus impaction. Though, due to the profound radiographic presentation and severe morbidity of our clinical scenario, we hope to bring attention to the need for rapid evaluation, treatment, and consideration of adverse outcomes in patients presenting with food boluses as well as the severity and life-threatening outcomes that may preside with the previously trivially described pathology.

References

1. Yao CC, Wu IT, Lu LS, Lin SC, Liang CM, Kuo YH, Yang SC, Wu CK, Wang HM, Kuo CH, Chiou SS, Wu KL, Chiu YC, Chuah SK, Tai WC. Endoscopic Management of Foreign Bodies in the Upper Gastrointestinal Tract of Adults. Biomed Res Int. 2015;2015:658602. [CrossRef] [PubMed]
2. Sperry SL, Crockett SD, Miller CB, Shaheen NJ, Dellon ES. Esophageal foreign-body impactions: epidemiology, time trends, and the impact of the increasing prevalence of eosinophilic esophagitis. Gastrointest Endosc. 2011 Nov;74(5):985-91. [CrossRef] [PubMed]
3. Gelfond M, Rozen P, Gilat T. Isosorbide dinitrate and nifedipine treatment of achalasia: a clinical, manometric and radionuclide evaluation. Gastroenterology. 1982 Nov;83(5):963-9. [PubMed]
4. Karanjia ND, Rees M. The use of Coca-Cola in the management of bolus obstruction in benign oesophageal stricture. Ann R Coll Surg Engl. 1993 Mar;75(2):94-5. [PubMed]

Cite as: Carrillo A, Ondracek R, Punatar S, Ondracek A, Sundaram R. June 2022 Medical Image of the Month: A Hard Image to Swallow. Southwest J Pulm Crit Care Sleep. 2022;24(6):93-95. doi: https://doi.org/10.13175/swjpccs022-22 PDF