Necrotizing Granulomatous Dermatitis and Panniculitis Associated with Inflammatory Bowel Disease
Victor G. Prieto
UT-MD Anderson Cancer Center
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A 51-year-old white woman with a history of rheumatoid
arthritis, steroid-dependent ulcerative colitis, and diabetes mellitus was hospitalized with fever and
red painful subcutaneous nodules on both inner thighs. An initial biopsy was thought to represent an
abscess but she did not improve with broad-spectrum oral and intravenous antibiotics. The sites became
necrotic, were debrided multiple times, and required administration of hyperbaric oxygen therapy.
Physical exam revealed two opposing, large ulcers on the inner surface of both thighs. The biopsy was
interpreted as consistent with T-cell lymphoma and the patient was referred to MDACC for treatment.
The punch biopsy shows skin to the upper subcutaneous
tissue. The dermis and subcutaneous tissue contain large areas of confluent necrosis with macrophages
and granulomatous infiltrate. There is also a superficial and deep infiltrate with large and small
lymphocytes, some with convoluted contours, diffusely infiltrating the subcutaneous tissue, accompanied
by neutrophils, plasma cells and scattered eosinophils. Some of the lymphocytes in the subcutaneous
tissue are located around fat vacuoles ("rimming").
Special stains are negative for microorganisms. Immunohistochemical studies reveal a predominance of
CD3 cells with approximately similar numbers of CD4 and CD8. Anti-TIA and granzyme B are essentially
dermatitis and panniculitis associated with inflammatory bowel disease
- Subcutaneous T-cell lymphoma
- Granulomatous mycosis fungoides
- Other granulomatous processes
After our diagnosis of "granulomatous dermatitis, no evidence
of lymphoma or infection", further questioning revealed the patient had a previous diagnosis of
steroid-dependent ulcerative colitis.
Cutaneous manifestations, most commonly erythema nodosum and pyoderma gangrenosum, are seen in
approximately 10 to 20 % of patients with Crohn disease (CD) and ulcerative colitis (UC). Other shared
cutaneous associations include aphthous stomatitis, finger clubbing, cutaneous polyarteritis nodosa,
psoriasis, pyostomatitis vegetans, erythema multiforme and vitiligo.
Granulomatous changes have been described in skin lesions from patients with inflammatory bowel
disease (IBD). Metastatic CD is the term referring to extraintestinal Crohn, as red to violaceous
papules, nodules and plaques that often ulcerate. Histologically, it usually has non-caseating
granulomas. Also seen are necrobiosis, panniculitis, and granulomatous perivasculitis and vasculitis,
similar to the findings seen in this case. In particular, giant cells have been reported in pyoderma
gangrenosum associated with inflammatory bowel disease. Also reported in inflammatory bowel disease are
neutrophilic cutaneous processes (pyoderma gangrenosum, vesicopustular eruptions, erythema elevatum
diutinum, Sweet syndrome, linear IgA bullous disease and neutrophilic lobular panniculitis).
The differential diagnosis in a patient with large ulcers and history of immunosuppression
includes infection and malignancy. Special stains for microorganisms and cultures ruled out an
infectious etiology. Granulomatous inflammation may be seen in patients in multiple lymphoid processe,
particularly mycosis fungoides / granulomatous slack skin, subcutaneous panniculitic T-cell lymphoma, and
primary cutaneous anaplastic (CD30+) large cell lymphoma. Rarely, cutaneous B-cell lymphomas, both
primary and secondary, can show granulomatous reactions. In evaluating these cases, immunohistochemical
studies can help to characterize the inflammatory infiltrate.
In summary, this case illustrates that inflammatory bowel disease should be included in the
differential diagnosis for lesions showing "rimming" of lymphocytes, particularly when accompanied by
granulomas and neutrophilic infiltrates. Clinical-pathologic correlation will be essential in such
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