UPMC Dermatopathology "Case of the Month" Presentations
UPP - Department of Dermatology, Dermatopathology Unit
Case Authors: Timothy Patton, DO, Douglas Kress, MD and Bong Kim, MD
AUGUST 2004 CASE OF THE MONTH
DISCUSSION & DIAGNOSIS
DIAGNOSIS
Final Diagnosis: Inflammatory bowel disease associated neutrophilic dermatosis
DISCUSSION
The neutrophilic dermatoses,
which clinically sometimes share overlapping features, include several disorders
which histologically demonstrate the presence of neutrophils(1). Diseases such
as Sweet's syndrome, Behcet's disease, and pyoderma gangrenosum are included
under the heading of neutrophilic dermatoses, and in addition to having neutrophilic
infiltrates in the skin, can have other organ systems involved as wel (l2,3,4).
Our patient's presentation included cutaneous lesions that were clinically and
histologically consistent with a diagnosis of pyoderma gangrenosum. In addition
to the lesions of pyoderma gangrenosum, because of her ocular involvement, oral
mucosal involvement, and arthritis in association with a flare in her bowel
disease, we felt that the patient's overall diagnosis was most consistent with
inflammatory bowel disease associated neutrophilic dermatoses.
Extraintestinal manifestations of inflammatory bowel disease occur in anywhere
from 1-10% of patients, depending on the extraintestinal organ system involved(5).
Cutaneous lesions associated with inflammatory bowel disease include pustular
vasculitis and erythema nodosum(6), and Sweet's syndrome(7), among others. Pyoderma
gangrenosum may begin as a small inflammatory pustule which may or may not evolve
into a shallow or deep ulceration. In a recent review of ulcerative colitis
patients, pyoderma gangrenosum was reported in 2.4% of 116 patients(8). The
pathogenesis of the extraintestinal manifestations of inflammatory bowel disease
is hypothesized to involve the activation of lymphocytes in the gut with subsequent
migration to extraintesinal sites mediated by adhesion molecules such as VCAM-1and
ICAM-19. Once an infectious etiology was ruled out in our patient, she responded
well to a combination of corticosteroids and dapsone and was able to be discharged.
The mouth, eye, skin lesions and bowel symptoms all improved over the next two
to three weeks and her steroids were able to be tapered with continuation of
dapsone as a steroid sparing agent.
REFERENCES
1. Callen JP. Neutrophilic
dermatoses. Dermatol Clin. 2002 Jul; 20(3): 409-19.
2. Matta M et al. Sweet's syndrome: systemic association. Cutis 1973; 12: 561-565.
3. Crowson AN et al. Pyoderma gangrenosum: a review. J Cutan Pathol. 2003 Feb;
30(2): 97-107.
4. Hirohata S et al. Behcet's disease. Arthritis Res Ther. 2003; 5(3): 139-46.
5. Jewell DP. “Ulcerative Colitis.” Sleisenger & Fordtran's
Gastrointestinal and Liver Disease, Ed. Feldman. 7th ed. Elsevier, 2002.
6. Vazquez J et al. Neutrophilic pustulosis and ulcerative colitis. J Eur Acad
Dermatol Venereol. 2003 Jan; 17(1): 77-9.
7. Rappaport A et al. Sweet's syndrome in association with Crohn's disease:
report of a case and review of the literature. Dis Colon Rectum. 2001 Oct; 44(10):
1526-9.
8. Ozdil S et al. Ulcerative colitis: analyses of 116 cases (do extraintestinal
manifestations effect the time to catch remission?). Hepatogastroenterology.
2004 May-Jun; 51(57): 768-70.
9. Eksteen B et al. Lymphocyte homing in the pathogenesis of extra-intestinal
manifestations of inflammatory bowel disease. Clin Med. 2004 Mar-Apr; 4(2):
173-80.