Clinical, Imaging, and Pathologic Features of Conditions with Combined Esophageal and Cutaneous Manifestations.
Matthew H. Lee, MD, Meghan G. Lubner, MD, J. Klint Peebles, MD, Molly A. Hinshaw, MD, Christine O. Menias, MD, Marc S. Levine, MD, Perry J. Pickhardt, MD
RadioGraphics 39:1411–1434. Published Online: Aug 16 2019
The esophagus, consists of squamous epithelial cells, similar to what composes the skin. As a result, these two separate organs are linked through a concomitant affliction by diseases ranging from infections, autoinflammatory and autoimmune conditions to genetic syndromes and paraneoplastic manifestations. Each such disorder may present with characteristic findings on either the skin or/and the esophagus, thus providing a certain diagnostic direction.
The present study seeks to bring those different entities under a converging scope of clinical presentations and imaging observations with the aim to provide the reader with the tools to assert the underlying etiologic factor.
The infectious organisms studied here are HSV, CMV, HIV and Candida albicans. Multiple, lesser-sized esophageal ulcers are typical of HSV, whereas larger lesions are more characteristic of CMV and HIV infections. C. albicans assumes a linear, plaque-like appearance on the esophagography. Regarding the skin, HSV features clustered blisters; CMV exhibits non-distinct traits, C. albicans presents the characteristic white plaques of oral thrush, whereas, HIV-infection, defined by its immunodefective nature and concurrent opportunistic infections can assume any of the aforementioned skin lesions [1], [2], [3].
Moving on to bullous diseases, two categories are described, namely the epidermolysis bullosa (EB) and the pemphigoid disorders (PD). The former concerns a group of genetic conditions that affect the epidermis and mucosa, weakening their structural integrity, making them susceptible to mechanical damage [4]. The latter targets the subepidermis and mucosa in the form of autoimmunity, can affect any part of the soma and presents as pruritic or painful vesicular lesions. On regard to esophageal involvement, both entities may cause strictures of the esophagus, with EB most commonly affecting its upper anatomic area, whereas PD any part of it [5].
In direct contrast, scleroderma, another autoimmune disease, mostly affects the distal part of the esophagus, reducing its motility, causing it to dilate early on, though also effecting strictures later on the course of the disease. The skin of the extremities seems to be damaged first, becoming rigid and prone to trauma [6].
The autoinflammatory conditions studied here are lichen planus and Crohn’s disease. Lichen planus is allegedly a T-cell oriented disease that causes lilac-coloured papules on the skin, while also involving the nails and hair. The esophagus, mainly its thoracic part, reduces in diameter [7] and can pose a differential bipolar, having a similar esophagographic appearance to eosinophilic esophagitis. The elder of age and female sex predominant on lichen planus in contrast to a younger male patient on eosinophilic esophagitis help differentiate between the two. Crohn’s disease, a primarily bowel disorder, may affect the skin via perianal fissures and fistulas or indirectly via its implications on nutrient absorption. The esophagus is not usually a trademark organ of this disease, but it may manifest with small ulcerative lesions or fistulas to adjacent organs [8].
Cowden syndrome consists a genetic disorder of a defective tumour suppressor gene that results in an increase in hamartomas and malignancy. Related skin conditions are hair follicle-derived hamartomas, fibromas and papules on the acral dorsal parts. The esophagus is infrequently affected, but may present with a nodule-like appearance at the esophagography [9]. Blue Rubber Bleb Nevus Syndrome (BRBNS) is a congenital disorder with a mutated endothelial cell receptor and resulting venous malformations both on the skin and the GI tract, in the form of polyps or even mass-like configurations [10].
Melanoma constitutes a primary malignant tumour of melanocytes, presents as uneven, varicoloured, growing skin lesion that has rarely an impact on the esophagus but can, nonetheless, affect it as a non-obstructive, but lumen-expanding polypoid mass [11]. Acrokeratosis paraneoplastica comprises a paraneoplastic condition, often of an SCC, of the respiratory and/or the GI tract. Its varying skin lesions can direct to a primary neoplastic or secondary disseminated foci. An SCC of the esophagus can assume a plaque or polypoid lesion morphology early on or a mass-like form that causes narrowing of the esophageal lumen at later stages [12].
Finally, neither the skin nor the esophagus could be spared from drug side-effects, such as those caused by doxycycline. Doxycycline can, namely, have phototoxic effects on both the skin and nails and give rise to small ulcerative lesions in the esophagus, particularly at sites of physiologic anatomic compression [13].
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Christos Korres is a first-year radiology resident at the Konstantopouleion General Hospital of Athens, Greece. He studied at the Medical School of Athens and concluded a postgraduate program in Linkoping, Sweden with a dissertation on the Olfactory Function of Healthy Participants, explored through fMRI and mathematical models. His interests are still developing, but currently focused on Abdominal Radiology and Neuroradiology.
Comments may be sent to kris.korres@gmail.com
