A Man with Abdominal Pain, Joint Swelling, and Skin Lesions

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Pancreatitis, panniculitis, and polyarthritis syndrome is most common in middle-aged men with what disorder?

Lupus panniculitis is most common in women and is characterized by tender subcutaneous nodules and plaques on the face, proximal arms, and trunk but not on the legs. Read the NEJM Case Records of the Massachusetts General Hospital here.

Clinical Pearls

Q: What skin changes are associated with primary cutaneous gamma/delta T-cell lymphoma?

A: Primary cutaneous gamma/delta T-cell lymphoma often causes necrotizing plaques and tumors or red, scaly plaques that mimic mycosis fungoides.

Q: What three findings constitute the classic triad of Löfgren’s syndrome?

A: Löfgren’s syndrome is characterized by a classic triad of erythema nodosum, hilar lymphadenopathy, and acute polyarthritis, although diagnosis of this condition does not require all three clinical findings.


Morning Report Questions

Q: Where are the lesions associated with subcutaneous panniculitis-like T-cell lymphoma most commonly located?

A: Subcutaneous panniculitis-like T-cell lymphoma is a rare primary cutaneous lymphoma of mature cytotoxic T cells, and the disease typically follows an indolent course. Lesions are usually single or multiple nodules and plaques on the legs. The disease most commonly occurs in middle-aged women with coexisting conditions, such as systemic lupus erythematosus, multiple sclerosis, or hypothyroidism. The nodules are usually painless.

Q: Pancreatitis, panniculitis, and polyarthritis syndrome is most common in middle-aged men with what disorder?

A: Pancreatitis, panniculitis, and polyarthritis syndrome is a rare syndrome that has been described mainly in case reports. The syndrome is most common in middle-aged men with alcohol use disorder. Patients with this syndrome have the cutaneous clinical and histopathological features of pancreatic panniculitis. Patients with pancreatic panniculitis have tender, sometimes fluctuant subcutaneous nodules that commonly involve the legs. The nodules can ulcerate, releasing oily, thick, yellowish-brown material indicative of necrotic adipose tissue. Examination of a skin-biopsy specimen would reveal fat saponification by calcium salts, with homogeneous basophilic material or “ghost cells” — a histologic feature that distinguishes pancreatic panniculitis from other panniculitides. Treatment of the underlying pancreatic disease can result in resolution of cutaneous lesions.

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