Lipogranulomatosis subcutanea M79.8

Author: Prof. Dr. med. Peter Altmeyer

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Last updated on: 29.10.2020

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Synonym(s)

Rothmann-Makaish Syndrome; Spontaneous panniculitis Rothmann-Makai

History
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Rothmann, 1894; Makai, 1928

Definition
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This variant of panniculitis is no longer recognized as an independent disease (Rose C 2018). Originally, lipogranulomatosis subcutanea was defined as idiopathic, focal, chronic panniculitis without general symptoms.

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Etiopathogenesis
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Idiopathic, a disturbance of the fat composition and focal infections are discussed. Some authors see Lipogranulomatosis subcutanea as afebrile variant of Panniculitis nodularis nonsuppurativa febrilis et recidivans.

Manifestation
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Occurs mainly in older children and in middle-aged women.

Localization
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Preferably on lower legs; less frequently on trunk or face.

Clinical features
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Suddenly appearing cherry to walnut-sized, subcutaneous, pressure-painful nodules that can be shifted against the skin and pad, or even plate-like hardenings without general symptoms and without involvement of internal organs.

Histology
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Lobular panniculitis, focal granulomatous infiltrates, foam cells, giant cells, micropseudocysts.

Differential diagnosis
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External therapy
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Cooling, compression bandages. In mild cases, only glucocorticoids externally such as 0.25% prednicarbate (e.g. Dermatop cream) under an occlusion of several hours can be successful. S.a.u. Panniculitis nodularis nonsuppurativa febrilis et recidivans.

Internal therapy
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Non-steroidal anti-inflammatory drugs such as acetylsalicylic acid (e.g. ASS) or ibuprofen (e.g. Ibuprofen Stada Filmtbl.) In extensive cases systemic glucocorticoids such as prednisolone (e.g. Decortin H) 1 mg/kg bw/day p.o.

Progression/forecast
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Months to years.

Literature
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  1. Chan HL (1975) Panniculitis (Rothmann-Makai), with good response to tetracycline. Br J Dermatol 92: 351-354
  2. Makai E (1928) About Lipogranulomatosis subcutanea. Clin week 7: 2343-2346
  3. Rose C (2018) Diseases of the fatty tissue. In: Braun-Falco`s Dermatology, Venerology Allergology G. Plewig et al. (Hrsg) Springer Verlag S 1465
  4. Rothmann M (1894) On inflammation and atrophy of the subcutaneous fatty tissue. Virchows Arch Catholic Anat 136: 159-169
  5. Tursen U (2003) Rothmann-Makai syndrome with infiltrative ductal mammary cancer: coexistence or association? J Eur Acad Dermatol Venereol 17: 479-480
  6. Undeutsch W et al. (1970) Lipogranulomatosis Rothmann-Makai independent clinical picture or polyetiological syndrome? Dermatologist 21: 221-225
  7. Winkelmann RK et al (1989) Lipophagic panniculitis of childhood. J Am Acad Dermatol 21: 971-978

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Last updated on: 29.10.2020