Crohn's disease is a chronic inflammatory bowel disease that can involve any part of the gastrointestinal tract. Metastatic Crohn's disease (MCD) is a rare cutaneous manifestation where noncaseating granulomatous skin lesions occur at sites separate from the GI tract in patients with Crohn's. MCD most often presents as papules, plaques or nodules on the arms, legs, genitalia and face. Histopathology of MCD shows granulomatous inflammation in the dermis. Treatment options include steroids, antibiotics, immunosuppressants, infliximab and surgery.
2. Crohn disease is a chronic granulomatous
inflammatory bowel disorder that may involve
any segment of the gastrointestinal tract.
3. 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).
4. 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.
5. 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.
6. 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.
7. 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.
9. 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.
10. 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.
11. 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
12. 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.
13. Perivascular granulomatous inflammation
surrounding the dermal vessels.
Necrobiosis consisting of collagen degeneration
accompanied by granulomatous inflammation.
Eosinophils may also present.
14. Low-power image of left submammary crease ulcer with
underlying noncaseating granulomatous inflammation of the
papillary and reticular dermis
15. Higher magnification of left submammary crease lesion with
deep dermal granulomatous inflammation consisting of
multinucleated giant cells, epithelioid histiocytes, and a
lymphocytic infiltrate
16. Low-power image of an abdominal skin biopsy illustrating
diffuse granulomatous inflammation involving the
papillary and reticular dermis
17. Labial biopsy showing diffuse inflammation of the
papillary dermis consisting of multinucleated giant
cells, epithelioid histiocytes, and a lymphocytic
infiltrate
18. CROHN’S 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.
19. CROHN’S 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.
20. 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
22. 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.
23. 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.
25. 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.
26. 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.
27. A gastrointestinal workup for inflammatory
bowel disease should be considered when MCD
is diagnosed without a previous history of
Crohn disease
28. 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.
29. 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
30. 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.