Lipophagic panniculitis of childhood M35.6

Author: Prof. Dr. med. Peter Altmeyer

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Last updated on: 29.10.2020

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Synonym(s)

Annular atrophy of the ankles; Lipoatrophic panniculitis of the ankles in childhood; Lipophagic panniculitis of childhood; partial lipodystrophy

History
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Winkelmann RK et al. 1989

Definition
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Rare immunologically induced panniculitis, wherein an interferon-mediated autoinflammatory etiology is suspected.

Etiopathogenesis
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Associations with various autoimmunological diseases are suspected.

Manifestation
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Symptoms occur in children (usually before the age of 10). Healing occurs under a focal lipatrophy. Due to this scarring healing mode, the clinical picture differs from the erythema nodosum, which heals without scarring (without denting).

Localization
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Lower leg, especially ankle regions

Clinical features
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Fever with a clear feeling of illness; reactive arthritis. At the same time, recurrent red plaques and/or nodules, mostly occurring on the lower legs (often on the ankle regions), which expand to form abnormal plaques.

Histology
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Histologically a mixed-cell lobular panniculitis is detectable.

Therapy
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Systemic glucocorticoids. In case of resistance to therapy add methotrexate.

Note(s)
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Winkelmann mentioned in 1989 in his original publication (Winkelmann RK et al.1989) that such cases of (idiopathic) infantile panniculitides, formerly known as "Weber-Christian`sche Erkrankung" or"Rothmann-Makai Syndrome", had been reported.

Literature
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  1. Jain S et al (2015) Eosinophilic panniculitis in a female child: An unusual presentation. Indian Dermatol Online J 6:34-36.
  2. Levy J et al (2017) Lipophagic panniculitis of Childhood: A Case Report and Comprehensive Review of the Literature. At J Dermatopathol 39:217-224.
  3. Santonja C et al (2012) Lipoatrophic panniculitis of the ankles in childhood: differential diagnosis with subcutaneous panniculitis-like T-cell lymphoma. At J Dermatopathol 34:295-300.
  4. Shen LY et al (2010) Lipoatrophic panniculitis: case report and review of the literature. Arch Dermatol 146:877-881.
  5. Winkelmann RK et al (1989) Lipophagic panniculitis of childhood. JAm Acad Dermatol 21:971-978.

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Last updated on: 29.10.2020