Cutaneous Crohn's Disease

Question 1

What are the different categories of skin manifestations in Crohn ‘s .

Answer 1

They can be subdivided into:
(1) specific lesions including distant cutaneous (metastatic) Crohn disease, contiguous perianal Crohn disease, and oral Crohn disease;

(2) nonspecific or reactive dermatologic disorders, e.g. erythema nodosum, pyoderma gangrenosum; sweet syndrome.
(3) nutritional skin changes secondary to malabsorption;
(4) treatment-related side effects.

Question 2

What is the clinical presentation of genital disease in Crohn ?

Answer 2

Labial, penile or scrotal erythema and/or lymphedema are common presenting signs and can be dramatic (Fig. 93.17A-C tg ).

Linear or “knife- like” ulcerations that are within body folds (e.g. inguinal creases, perianal region, axillae) or involve the oral mucosa are relatively specific for mucocutaneous Crohn disease (see Figs
53.

Perianal involvement may consist of sinus tracts, fissures, ulcers, and vegetating plaques. These are observed in one-third of patients with intestinal Crohn disease.

Perianal skin tags, thought to be secondary to lymphedema, are observed in up to 40% of patients.

Question 3

What is clinical presentation of oral disease in Crohn’s disease ?

Answer 3

Oral lesions occur in 5-20% of patients with Crohn disease,
and these may consist of :

1) cobblestoning of the buccal mucosa,
2) tiny gingival nodules
3) gingival hyperplasia
4) aphthae-like ulcers (Fig. 93 . 1 7F E@ )
5) linear, knife-like ulcerations
6) pyostomatitis vegetans (see Fig. 26.9A E@ ),
7) angular cheilitis and ulceration,
8) cheilitis granulomatosa
9) diffuse oral swelling
10) indurated fissuring of the lower lip.

Histologically, 90% of oral lesions associated with Crohn disease contain granulomas..

Question 4

List the reactive cutaneous manifestations of Crohn disease

Answer 4

Other reactive cutaneous manifestations of Crohn disease include 
1) cutaneous polyarteritis nodosa
2) erythema nodosum
3) erythema multiforme
4) finger clubbing
5) cutaneous small vessel vasculitis
6) epidermolysis bullosa acquisita, 
7) vitiligo
8) palmar erythema
9)  a pustular response to trauma (pathergy),
10) pyoderma gangrenosum (see Ch. 53 ). 
Zinc deficiency may cause an acrodermatitis enteropathica-like syndrome in patients with severe Crohn diseasei27.

Question 5

What is the histology of cutaneous and oral Crohn ?

Answer 5

In both cutaneous and oral lesions of Crohn disease,

nodular, non-caseating, epithelioid tubercles with surrounding lymphocytes are found in the superficial and deep dermis, sometimes extending into the subcutaneous fat. There are a few scattered multinucleated Langhans-type giant cells and a sparse perivascular lymphohistiocytic infiltrate; overlying ulceration may be present.

Question 6

What is the clinical DD of cutaneous Crohn disease

Answer 6

Clinically, the differential diagnosis of cutaneous Crohn disease includes

• **other granulomatous disorders such as
• • cutaneous sarcoidosis
• • mycobacterial infections
• • deep fungal infections,
• • foreign body reactions.

***Other infections, such as actinomycosis or cellulitis, may mimic cutaneous Crohn disease.

Ulcerated lesions may be misdiagnosed as pyoderma gangrenosum.

In the case of genital swelling and ulcerations, one must consider

• *granuloma inguinale
• • schistosomiasis,
• *hidradenitis suppurativa
• *chronic lymphedema due to obstructiont.

Question 7

Compare the histopathologucal DD of cutaneous Crhin’s disease

Answer 7

The histopathologic picture of cutaneous Crohn disease can be indistinguishable from other granulomatous diseases with tuberculoid features, including

*** lupus vulgaris is a granulomatous diseases with tuberculoid features, . In lupus vulgaris
central necrosis within tubercles is a helpful distinguishing.

*** Differentiating cutaneous Crohn disease from sarcoidosis can also be challenging, although Crohn disease usually has denser collections of lymphocytes and granulomatous perivasculitis as well as the features described above.

• ** Foreign body granulomas
• all skin biopsies should be polarized to look for foreign bodies.
• ** infectious granulomas should be considered in the histologic differential diagnosis.
• Special stains for acid-fast bacilli and fungal organisms should be obtained

*** Pyoderma gangrenosum and cutaneous Crohn disease can sometimes have overlapping clinical and histopathologic features as neutrophilic inflammation may be present in both, but granulomatous inflammation is rarely observed in pyoderma gangrenosum.

Question 8

Is the severity of cutaneous Crohn’s disease related to the activity of intestinal disease ?

Answer 8

No

Question 9

What are the treatment options for localized disease ?

Answer 9

topical or intralesional corticosteroids

and/or topical calcineurin inhibitors may be utilized

Oral metronidazole (250 mg TID for at least 4 months) can be an effective

Question 9

What are the treatment options for localized disease ?

Answer 9

topical or intralesional corticosteroids

and/or topical calcineurin inhibitors may be utilized

Oral metronidazole (250 mg TID for at least 4 months) can be an effective

Question 10

What are the treatment options for more extensive disease,

Answer 10

For more extensive disease, use of systemic medications including 
corticosteroids, 
sulfasalazine, 
azathioprine, 
6-mercaptopurine, 
TNF-a inhibitors (e.g. infliximab, adalimumab), 
and thalidomide 
may lead to improvementi.

Surgical excision of lesions is often complicated by wound dehiscence and disease recurrence, but
can be considered for refractory disease.