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Lipogranulomatosis subcutanea of Rothmann and Makai - Skin
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Lipogranulomatosis subcutanea of Rothmann and Makai - Skin

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Contributors: Vimal Prajapati MD, Ben Barankin MD, Jeffrey D. Bernhard MD, Noah Craft MD, PhD, Lindy P. Fox MD, Lowell A. Goldsmith MD, MPH, Michael D. Tharp MD
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Synopsis

Lipogranulomatosis subcutanea of Rothmann and Makai, also known as Rothmann-Makai syndrome, is an idiopathic panniculitis characterized by circumscribed, tender, erythematous subcutaneous nodules or plaques, most frequently occurring on the extremities and less commonly on the trunk and face. Lesions appear one or only a few at a time but not in crops. There is neither visceral organ involvement nor systemic manifestations (eg, fever, myalgias, arthralgias). Spontaneous involution of individual lesions occurs within weeks to months. With resolution, post-inflammatory hyperpigmentation, atrophy, and scarring remain; ulceration has not been described. Although any individual can be affected by this disease, it occurs most frequently in children and middle-aged females. Anecdotal evidence documents the efficacy of a few anti-inflammatory and immunosuppressive agents for treatment.

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.

Codes

ICD10CM:
M79.3 – Panniculitis, unspecified

SNOMEDCT:
51696001 – Lipogranulomatosis subcutanea of Rothmann and Makai

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Diagnostic Pearls

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Differential Diagnosis & Pitfalls

Any other panniculitis, but especially the following:
  • 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.

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Last Updated: 01/23/2015
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Lipogranulomatosis subcutanea of Rothmann and Makai - Skin
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Lipogranulomatosis subcutanea of Rothmann and Makai : Subcutaneous nodules, Lower legs
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