Lipogranulomatosis subcutanea of Rothmann and Makai - Skin
Within the panniculitides, lipogranulomatosis subcutanea of Rothmann and Makai is classified into the category of lobular panniculitis without vasculitis. Previously, it was considered to be a rare variant of Weber-Christian disease. However, this eponym is being abandoned, as many of the cases bearing this label were later discovered to be distinct disorders, such as pancreatic panniculitis, alpha1-antitrypsin deficiency panniculitis, histiocytic cytophagic panniculitis, and lupus panniculitis.
M79.3 – Panniculitis, unspecified
51696001 – Lipogranulomatosis subcutanea of Rothmann and Makai
- Erythema nodosum – occurs with much greater frequency, associated with several well-known etiologic factors (underlying diseases and medications), onset is more sudden, and resolution occurs without atrophy or scarring; histopathology reveals mostly septal panniculitis without vasculitis.
- Cutaneous polyarteritis nodosa – occurs on a background of livedo reticularis ("starburst" pattern), and ulceration is not uncommon; histopathology reveals septal panniculitis with vasculitis involving medium-sized arteries and arterioles.
- Erythema induratum of Bazin – occurs on the posterior aspects of the lower extremities, and ulceration is frequent; an etiologic relationship with tuberculosis is often demonstrated; histopathology reveals lobular panniculitis with vasculitis involving small veins and venules.
- Pancreatic panniculitis – ulceration and drainage of oily brown exudate is frequently observed, associated with underlying inflammatory or neoplastic pancreatic disease, and pancreatic enzyme levels are elevated; histopathology reveals mostly lobular panniculitis without vasculitis and saponification of adipocytes.
- Alpha1-antitrypsin deficiency panniculitis – tends to involve proximal extremities, ulceration and drainage of oily brown exudate is frequently observed, associated systemic manifestations (emphysema and cirrhosis) are usually present, and serum alpha1-antitrypsin levels are significantly decreased; histopathology reveals mostly lobular panniculitis without vasculitis and neutrophils between collagen bundles.
- Histiocytic cytophagic panniculitis – ulceration and persistent drainage are frequent features, and systemic manifestations are usually present (fever, hepatosplenomegaly, pancytopenia secondary to bone marrow involvement) as most cases are attributable to primary or secondary T- and B-cell lymphomas; histopathology reveals mostly lobular panniculitis without vasculitis and cytophagocytosis.