Non-Neoplastic Dermatopathology

5.10 P ANCREATIC PANNICULITIS VS ALPHA-1 ANTITRYPSIN DEFICIENCY PANNICULITIS

Pancreatic Panniculitis

Alpha-1 Antitrypsin Deficiency Panniculitis

Age

Middle-aged and elderly adults.

Generally presents in adults, between third and fifth decades of life, but may present in childhood in individuals with genotypes associated with greater deficiency. Extremities most commonly involved but trunk, buttocks, abdomen, and face may also be affected. Due to deficiency of serine protease inhibitor alpha-1 antitrypsin. Reduced circulating alpha-1 antitrypsin leads to impaired neutrophil elastase and exaggerated neutrophilic and proteolytic response. Tender, erythematous subcutaneous nodules that may ulcerate and have oily, yellow discharge. Lesions may arise spontaneously, or after minor trauma, and heal with atrophy and scarring. May be associated with chronic obstructive pulmonary disease, hepatitis, and glomerulonephritis. 1. Septal and lobular neutrophilic infiltrate (Figs. 5.10.4 and 5.10.5) . 2. Neutrophils splay collagen bundles in the deep reticular dermis (Fig. 5.10.6) . 3. Fat necrosis with aggregates of histiocytes and lipophages (Fig. 5.10.7) . 4. Areas of normal subcutis adjacent to inflamed and necrotic subcutis (Fig. 5.10.4) .

Location

Lower extremities typically involved. May also extend to buttocks, trunk, upper extremities, and scalp. Systemically released pancreatic enzymes, such as amylase and lipase, cause necrosis of subcutaneous adipocytes and recruitment of neutrophilic inflammation. Most commonly associated with acute and chronic pancreatitis and pancreatic carcinoma. Tender, ill-defined, edematous, erythematous nodules that ulcerate and exude viscous, oily brown material. Often associated with arthralgias. Skin lesions may develop prior to diagnosis of pancreatic disease. 1. Lobular infiltrate with mixed inflammatory cells predominated by neutrophils (Figs. 5.10.1 and 5.10.2) . 2. Coagulative necrosis of adipocytes with saponification and “ghost” cells representing fat cell remnants (Fig. 5.10.3) . 3. Basophilic granular calcification (Fig. 5.10.3) . 4. Viable fat lobules with acute and chronic inflammation and lipophages. Elevated levels of amylase, lipase, and trypsin are usually present and aid in confirming the diagnosis. Eosinophilia is seen in a majority of cases. Primarily supportive and directed toward treatment of underlying pancreatic disease.

Etiology

Presentation

Histology

Special studies

Serum alpha-1 antitrypsin level. Alpha-1 antitrypsin phenotyping or genotyping.

Treatment

Dapsone is the preferred oral therapy, but glucocorticoids, tetracyclines, and other immunosuppressants show efficacy. Intravenous alpha-1 antitrypsin may be used in severe or refractory cases. (continued) Copyright © Wolters Kluwer, Inc. Unauthorized reproduction of the content is prohibited. 2023

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