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Crohn disease is a chronic granulomatous inflammatory bowel
disorder that may involve any segment of the gastrointestinal
tract.
Extraintestinal manifestations including ocular findings,
musculoskeletal pathology, and Mucocutaneous
manifestations.Mucocutaneous findings are the most frequent
extraintestinal manifestation 22% to 44% of patients present with
changes categorized as granulomatous skin disease, oral
manifestations, cutaneous changes secondary to nutritional
deficiencies, and cutaneous disorders that have been associated
with Crohn disease (eg, pyoderma gangrenosum, erythema nodosum,
erythema multiforme, and epidermolysis bullosa acquisita).
Perianal, peristomal,and perifistular inflammatory lesions most
common cutaneous manifestations lesions Parks et al - the first to
describe the presence of sterile, noncaseating, granulomatous
lesions of the skin at sites noncontiguous with the
gastrointestinal tract in patients with Crohn disease. This entity
is known as metastatic Crohn disease (MCD), the name first coined
in 1970 by Mountain.
Metastatic Crohn disease is defined as granulomatous lesions of
the skin occurring at sites separate from the gastrointestinal
tract in patients affected with Crohn disease. In adults, age of
onset - 29 to 39 years. Majority of patients have a previous
diagnosis of Crohn disease.
Up to one third of MCD patients have active gastrointestinal
symptoms. Twenty percent of patients with MCD may present without
classical manifestations of Crohn disease. In these patients, Crohn
disease manifests in 2 months to 4 years after the initial
presentation of MCD.
Cutaneous lesions of MCD may present as papules, plaques,
nodules,and ulcerations, which may involve the arms, legs,
genitalia, and face. Predilection for the moist environment of skin
folds, including submammary and abdominal creases perineal and
inguinal regions present as a solitary lesion or occur in multiple
sites painless or tender upon palpation.
Left submammary crease ulcer with well-defined border exuding
purulent fluid.
In the pediatric population, ages of 10 to 14 50% of these
patients having concurrent Crohn disease. In children who present
with MCD lesions without Evidence of Crohn disease, subsequent
onset of gastrointestinal manifestations occurs from 9 months to 14
years after the initial presentation of MCD.
The genitalia appear to be the most common area of involvement;
most common cutaneous manifestation presents as labial, penile,
and/or scrotal swelling with or without accompanying erythema.
Genital ulcerations have also been reported.
Males = females Although Crohn disease typically affects the
terminal ileum more often than the large bowel, cutaneous
manifestations of Crohn disease appear to occur more often in
patients who have involvement of the colon
Presents as sterile, noncaseating granulomatous inflammation
located primarily in the superficial papillary and deep reticular
dermis with occasional extension into the subcuticular fat. The
granulomas consist of Langerhans giant cells, epithelioid
histiocytes, lymphocytes, and occasional plasma cells.
Perivascular granulomatous inflammation surrounding the dermal
vessels. Necrobiosis consisting of collagen degeneration
accompanied by granulomatous inflammation. Eosinophils may also
present.
Low-power image of left submammary crease ulcer withunderlying
noncaseating granulomatous inflammation of thepapillary and
reticular dermis
Higher magnification of left submammary crease lesion withdeep
dermal granulomatous inflammation consisting ofmultinucleated giant
cells, epithelioid histiocytes, and alymphocytic infiltrate
Low-power image of an abdominal skin biopsy illustratingdiffuse
granulomatous inflammation involving thepapillary and reticular
dermis
Labial biopsy showing diffuse inflammation of thepapillary
dermis consisting of multinucleated giantcells, epithelioid
histiocytes, and a lymphocyticinfiltrate
CROHNS DISEASE MCD Lymphoplasmacytic predominantly diffuse
infiltrate in the mucosa pattern commonly seen in the dermis and
subcutis. Acute inflammatory cells neutrophils are rarely a may be
focally present in feature the crypt lumens (crypt abscess) or in
the crypt The presence of granulomas epithelium (cryptitis)
Langerhans giant cells are Closely arranged frequently seen along
with epithelioid histiocytes and an collections of histiocytes
accompanying with no foreign bodygiant lymphoplasmacytic infiltrate
cells present.
CROHNS DISEASE MCD Not seen Perivascular granulomatous Skip
lesions, neural inflammation and hypertrophy, Paneth cell
eosinophils metaplasia, and pyloric gland metaplasia. ulceration of
MCD lesions can occur. Lesions of Crohn Metastatic Crohn disease
directly disease lesions by involving the skin are definition occur
at sites located at sites discontiguous from the continuous with
the gastrointestinal tract gastrointestinal tract.
underlying etiology of MCD - unknown. It has been suggested
that antigens or immune complexes stemming from the
gastrointestinal tract in primary Crohn disease travel through the
circulatory system and deposit in the skin, creating perivascular
granulomatous features seen on microscopic examination of MCD
lesions. Autoimmune cross-reactivity also been suggested The
granulomatous inflammation has also been attributed to a type IV
hypersensitivity reaction wherein T cells cross-react with skin
antigens, resulting in an inflammatory response similar to that
seen in the gastrointestinal tract of Crohn disease
Cutaneous sarcoidosis, Erythema nodosum, Pyoderma gangrenosum,
Hidradenitis suppurativa, Mycobacterial disease, and Foreign body
reaction
Cutaneous sarcoidosis : consists of granulomas located in the
skin with minimal lymphocytic infiltrate also termed naked
granulomas. This granulomatous inflammation is different from that
of MCD, which usually consists of a prominent lymphoplasmacytic
infiltrate. Epidermal ulceration is relatively uncommon in
cutaneous sarcoidosis.
Erythema nodosum : one of the most common cutaneous
manifestations of Crohn disease presents microscopically with
granulomatous inflammation involving the septae of the subcutis
(ie, septal panniculitis) with a mixed inflammatory infiltrate
including neutrophils in the acute phase.
Superficial granulomatous pyoderma : the presence of focal
neutrophilic abscesses and pseudoepitheliomatous hyperplasia
Hidradenitis suppurativa : involves suppurative granulomatous
inflammation with folliculitis and abscess formation. Although it
may involve anatomic sites common to MCD such as the anogenital
region with granulomatous lesions, its follicular involvement with
keratin plugging and ruptured follicles make its microscopic
diagnosis distinct.
For all granulomatous lesions, appropriate ancillary stains for
infectious etiology (Gram stain, periodic acid Schiff/Gomori
methenamine silver, acid-fast bacilli) & cultures to rule out
mycobacterial disease, fungal infection, and other microbial agents
Polarizing microscopy may also be utilized to rule out
granulomatous lesions of the skin caused by foreign material.
A gastrointestinal workup for inflammatory bowel disease should
be considered when MCD is diagnosed without a previous history of
Crohn disease
Numerous agents have been used in the literature with varying
success, including oral and topical steroids, oral antibiotics,
azathioprine, sulfasalazine, 6-mercaptopurine, metronidazole,and
infliximab Infliximab appears to show promising results with
respect to severe and refractory cases of MCD.
Hyperbaric oxygen has also been utilized in the treatment of
MCD Patients appear to have also responded to surgical debridement,
especially in cases refractory to medical therapy. Although
considered rare, cases involving spontaneous regression of MCD
lesions have also been reported
Metastatic Crohn disease is a rare cutaneous manifestation of
Crohn disease. It is defined as the presence of noncaseating
granulomatous lesions of the skin at sites anatomically separate
from the gastrointestinal tract. Adults and children tend to have
different clinical presentations. The differential diagnosis of MCD
includes infectious and noninfectious entities presenting as
granulomatous skin lesions. A variety of therapeutic modalities
have been described in the literature, with infliximab and surgery
showing the most promise in terms of severe, refractory cases.