Fig. 21.1
Upper panel demonstrates a pregnant woman with classic lesions of erythema nodosum. Lower panel demonstrates an elderly man with metastatic colon cancer to the liver resulting in obstruction of the pancreatic duct and secondary pancreatitis and pancreatic panniculitis
Case
A patient presents with many months history of multiple subcutaneous leg nodules that may wax and wane.
Clinical Differential Diagnosis
includes
panniculitis,
subcutaneous T-cell lymphoma, and
infection.
The initial phase of lipodermatosclerosis (LDS) and pretibial myxedema may be nodular.
Clinical Clues
The various types of panniculitis vary in their preference for sites of involvement (anterior versus posterior legs, overlying joints), whether they involute rapidly or persist for several weeks to a few months, whether they ulcerate or not, and history of apparent precipitating factors or associated systemic disorders . Most types of panniculitis have surface erythema. Occasionally, lesions may become confluent into large plaques.
Dermatology practitioners are generally familiar with the clinical diagnosis of panniculitis. The specific type, however, is not always clinically apparent. When the presentation of subcutaneous leg nodules is acute, strongly favoring the anterior legs, and associated with recent history of infection, pregnancy, or drug use, the diagnosis of erythema nodosum is generally made easily, and a biopsy may not be necessary unless the course of lesions is atypical. All other types of panniculitis require histological confirmation of an adequate biopsy specimen that contains abundant fat.
Panniculitis is traditionally divided into disorders in which the infiltrate primarily involves the lobules of fat and those in which the infiltrate favors the subcutaneous fibrous septae. The prototype of septal panniculitis is erythema nodosum and the prototype of lobular panniculitis is erythema induratum, also referred to by some as nodular vasculitis . The latter favors the posterior legs and lesions tend to liquefy and ulcerate. At present, most cases of erythema induratum are idiopathic. Tuberculosis was a common cause when the disorder was initially described several decades ago.
The remaining types of panniculitis are generally rare. Pancreatic panniculitis is usually easily suspected and confirmed. It occurs in patients who generally have known pancreatic disorders or who may have signs and symptoms that point to a pancreatic disorder. Lesions of pancreatic panniculitis often favor the joints. Alpha-1 anti-trypsin deficiency panniculitis is extremely rare, as is eosinophilic panniculitis. Lupus panniculitis is rarely limited to the legs. Most often, it involves the proximal arms and adjacent skin of the shoulders and back, and is almost always followed by a deep indentation at the site of lesions due to atrophy and fibrosis of the subcutaneous fat, which results in “pulling the skin inwards.”
When Should an Infection Be Suspected?
Rarely, bacterial, atypical mycobacterial, and systemic fungal infection may present with deep dermal and subcutaneous nodules that may be mistaken clinically for inflammatory panniculitis. Patients are usually immunocompromised such as by HIV infection, immunosuppressive drugs, or chemotherapy.
When Should Subcutaneous T-cell Lymphoma Be Suspected?
Both, T- and B-cell lymphoma have a tendency to involve the leg. A patient with lymphoma of the leg may have diffuse, large B-cell lymphoma (leg type) or subcutaneous T-cell lymphoma. Diffuse, large B-cell lymphoma (leg type) presents as violaceous nodules. In advanced cases, lesions may appear deep and indurated, mimicking other leg disorders.
Subcutaneous T-cell lymphoma, also referred to as subcutaneous panniculitis-like T-cell lymphoma and alpha–beta subcutaneous T-cell lymphoma (referring to the molecular composition of the T-cell receptor) is usually indolent, but may sometimes have an aggressive course.
This is in contrast to T-cell lymphoma that may involve the subcutaneous fat as well as the overlying dermis and epidermis and that carries the T-cell receptor gamma–delta subtype. This disorder used to be referred to as subcutaneous T-cell lymphoma gamma–delta type, but is better referred to as gamma–delta T-cell lymphoma (in order to emphasize the pan-cutaneous nature of the involvement and the fact that the clinical lesions are not subcutaneous nodules, but instead infiltrated and eroded plaques and tumors). Gamma–delta T-cell lymphoma carries a poor prognosis, often associated with the hemophagocytic syndrome and death.