Fig. 11.1
A minor aphthous ulcer on the tongue (a) and a major aphthous ulcer on the lip (b)
Fig. 11.2
Common mimicker of aphthous ulcer, (a) oral lichen planus and (b) pemphigus vulgaris
Genital ulceration can occur in many patients with IBD. Importantly, genital ulcer is another cardinal manifestation of BD, and it is one of the diagnostic items in guidelines by the International Study Group for Behçet’s Disease (ISGBD) [11]. They are frequently painful, round to oval lesions that are usually covered with dried crust or grayish-white exudates, which are similar findings as with oral aphthae (Fig. 11.3). Significant edema, pain, and scarring around the ulcerations are quite common. The scrotum in males and both major and minor labia in females are commonly affected sites [7].
Fig. 11.3
Genital ulcers on the vulva (a) and scrotum (b)
Erythema nodosum (EN) is the most common cause of lower extremity inflammatory nodules in IBD patients (4–6 % of IBD cases), occurring more frequently in UC patients compared to CD patients [1]. More importantly, EN or EN-like lesions are cardinal cutaneous manifestations of BD [7]. EN is characterized by the sudden onset of erythematous, non-ulcerating, tender nodules that are frequently located on the lower extremities (Fig. 11.4). Histopathologically, they are characterized by prominent septal and/or lobular panniculitis that is sometimes accompanied by neutrophilic vascular inflammation (Fig. 11.5).
Fig. 11.4
Erythema nodosum
Fig. 11.5
Histopathological finding of erythema nodosum. Edema of septa with prominent lymphohistiocytic infiltrates mainly at the edge of septa and the periphery of the fat lobules, which are typical patterns of septal panniculitis
Pyoderma gangrenosum (PG) is a severe ulcerating noninfectious neutrophilic dermatosis, which has been reported in 1–10 % of UC patients and 0.5–20 % of CD patients [12]. PG or PG-like skin ulcers are also uncommon skin manifestations in BD. Four PG variants have been described: ulcerative, pustular, bullous, and vegetative. Classically, PG begins with pain followed by pustule formation, which soon breaks down to form a rapidly enlarging ulcer. Ulcers in PG have a boggy, necrotic, nonpurulent base and are rimmed by a raised inflammatory border, which is commonly characterized by an undermined border (Fig. 11.6). Ulcerative PG most commonly occurs on the lower extremities although it can occur on any part of the body. Histologically, PG shares similar pathologic changes with other neutrophilic dermatoses including Sweet’s syndrome. Tissue neutrophilia with undermining ulceration in the absence of microorganism or leukocytoclastic vasculitis favors PG when seen in the relevant clinical manifestations (Fig. 11.7).
Fig. 11.6
Pyoderma gangrenosum, ulcerative variant
Fig. 11.7
Histopathological findings of pyoderma gangrenosum. Central necrotizing suppurative inflammation with ulceration in the absence of definite leukocytoclastic vasculitis
Papulopustular lesions are frequent skin manifestation of IBD, especially in BD, with a reported prevalence ranging from 30 to 96 % among BD patients [13]. As they can be both follicular and nonfollicular papules/pustules, papulopustular lesions in BD are also referred to as pseudofolliculitis (Fig. 11.8). The most common localization in BD is the trunk followed by extremities.
Fig. 11.8
Papulopustular lesion in Behçet’s disease
The pathergy phenomenon is the hyperreactivity of the skin to a trauma. Although this phenomenon is not a pathognomic finding of BD, positivity of pathergy test is included in the diagnostic criteria by ISGBD (Fig. 11.9) [11].
Fig. 11.9
Positive pathergy reaction on the forearm
In addition, pyodermatitis vegetans and pyostomatitis vegetans are rare, vegetating, pustular, eosinophilic, mucocutaneous dermatoses and have strong association with gastrointestinal diseases, being considered a marker of IBD (Fig. 11.10) [1].
Fig. 11.10
Pyostomatitis vegetans (Courtesy of Professor Soo-Chan Kim)
11.2.3 Other Associated Conditions
There are numerous additional cutaneous lesions that are associated with IBD including psoriasis, hidradenitis suppurativa, thrombophlebitis, secondary amyloidosis, and erythema multiforme. Various autoimmune skin diseases such as vitiligo and epidermolysis bullosa are also associated with IBD [14, 15]. Rarely, there have been reports of cutaneous malignancies such as Bowen’s disease and squamous cell carcinoma occurring in relation to CD or UC [15].
The most frequently associated disease is psoriasis (Fig. 11.11), and the prevalence reported in epidemiologic studies is 3–11 % in IBD patients compared to 1–2 % in the general population [16]. Psoriasis is reported to be slightly more prevalent in CD compared to UC [17]. There are also reports on concurrent psoriasis in BD [18].
Fig. 11.11
Psoriasis
The vitiligo incidence is higher in IBD patients compared to the normal population and is higher in UC than CD (Fig. 11.12) Although the pathophysiological mechanism of vitiligo linkage to IBD is unclear, it has been proposed to be autoimmune or genetically linked [19, 20].
Fig. 11.12
Vitiligo
Epidermolysis bullosa acquisita is most frequently associated with CD [21]. Chronic inflammatory reactions in the intestine can cause patients to develop autoantibodies against type VII collagen in the bowel. These autoantibodies can in turn attack the skin dermoepidermal junction resulting in blisters and vesicle formation (Fig. 11.13) [22].
Fig. 11.13
Direct immunofluorescence staining for antihuman IgG in perilesional skin from patient with epidermolysis bullosa acquisita. Note the linear deposits of IgG along the dermal side of basement membrane zone (Ep epidermis, PD papillary dermis) (Courtesy of Professor Soo-Chan Kim)
Despite that a definitive linkage of atopic diseases to IBD remains unestablished, UC patients are more likely to have atopic dermatitis (Fig. 11.14) compared to healthy controls [23].
Fig. 11.14
Atopic dermatitis
Secondary amyloidosis, which is more common in CD compared to ulcerative colitis, is caused by a systemic response to chronic inflammation resulting in amyloid A-type skin amyloidosis. The high degree of inflammation in CD can possibly cause secondary amyloidosis, but this is a rare event [24]. There also have been rare reports of scrotal and penile lymphedema and purpura as cutaneous manifestations of CD [25, 26]. Alopecia areata, rosacea, leukocytoclastic vasculitis (Fig. 11.15), and lichen planus are also reported to be possibly associated with IBD (Fig. 11.16) [15, 27, 28].
Fig. 11.15
Leukocytoclastic vasculitis
Fig. 11.16
Lichen planus on the oral mucosa (a) and skin (b)
Because inflammatory bowel lesions can lead to dietary malabsorption, skin lesions related to nutrient imbalances, such as acrodermatitis enteropathica, pellagra, scurvy, stomatitis, glossitis, angular cheilitis, xeroderma, eczema, and hair and nail abnormalities, can also be observed in these patients (Fig. 11.17) [2]. Furthermore, skin lesions caused by adverse drug reactions developing during IBD treatment can also occur. These include, for example, folliculitis, acne, acneiform eruption, drug eruption, urticaria, angioedema, and Stevens–Johnson syndrome. The skin and mucosal manifestations in IBD are summarized briefly in Table 11.1 [15, 26].
Fig. 11.17
Angular cheilitis induced by IBD-induced malabsorption
Table 11.1
Skin and mucosal manifestations in IBD
Mucocutaneous manifestations | Presentation |
|---|---|
Specific mucocutaneous lesions | |
Fissures and fistulas (perianal) | Fissures: mostly painless, located posteriorly |
Fistulas: It appears internally or enterocutaneously and can destroy the anal sphincter | |
Direct or metastatic involvements | Subcutaneous nodules or nonhealing ulcers |
Reactive mucocutaneous lesions | |
Oral aphthous ulcers | Painful, shallow, round to oval ulcers covered with whitish to yellowish pseudomembrane demarcated by a red hyperemic border |
Genital ulcers | Painful, round to oval lesions that are usually covered with dried crust or grayish-white exudates |
Erythema nodosum | Sudden onset of erythematous, non-ulcerating, tender nodules frequently located on the lower extremities |
Pyoderma gangrenosum | Begins with pain followed by pustule formation, soon breaking down to form a rapidly enlarging ulcer |
Papulopustular lesions | Follicular and nonfollicular papules and pustules
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