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Atrophic dermatosisLymphoma associationICD-10 L90.2

Anetoderma

Macular atrophy ยท anetodermia

Anetoderma is a circumscribed loss of dermal elastic tissue, producing flaccid herniated patches or wrinkled macules of skin that bulge or invaginate on palpation ("buttonhole" sign). It is classified as primary (idiopathic โ€” Jadassohn-Pellizzari or Schweninger-Buzzi) or secondary, in which case it follows a wide range of inflammatory, infectious, neoplastic and drug triggers. Cutaneous T- and B-cell lymphoma, antiphospholipid syndrome and HIV are notable associated conditions warranting screening.

CurrentLast reviewed 16 May 2026
Clinical image of Anetoderma
Anetoderma. Image sourced from DermNet New Zealand. Used under CC BY-NC-ND 4.0. No endorsement implied.

Classification

  • Primary anetoderma โ€” idiopathic; Jadassohn-Pellizzari (preceded by inflammation) and Schweninger-Buzzi (no preceding inflammation) โ€” distinction is largely historical.
  • Secondary anetoderma โ€” following:
    • Inflammatory: lupus erythematosus, acne, varicella, sarcoidosis, lichen planus.
    • Infectious: syphilis, Lyme, HIV, leprosy, HBV.
    • Neoplastic: cutaneous T-cell lymphoma (particularly mycosis fungoides), B-cell lymphoma, pilomatricoma, melanoma.
    • Autoimmune: antiphospholipid syndrome, SLE.
    • Drug: penicillamine, hydrochlorothiazide, post-vaccination.
    • Mechanical: post-prematurity, post-trauma.

Clinical features

  • 1-2 cm flaccid, slightly depressed, wrinkled or bulging macules / patches on trunk, arms and thighs (rarely face).
  • Buttonhole sign โ€” palpating finger sinks into a herniated dermal defect.
  • Multiple lesions over weeks to years; lesions are permanent.
  • Onset 20s-40s; female predominance in primary form.

Workup for secondary causes

  • Skin biopsy with elastic-tissue stain (Verhoeff-Van Gieson, EVG) โ€” diagnostic loss of elastic fibres in dermis.
  • Bloods: FBC, ESR, CRP, ANA, ENA panel, complement, dsDNA, anticardiolipin and ฮฒ2-glycoprotein I antibodies, lupus anticoagulant.
  • Infection: syphilis serology (RPR / TPHA), HIV, HBV, HCV; Borrelia serology in endemic / exposure history.
  • Examine for cutaneous lymphoma โ€” full skin and lymph-node examination; multiple biopsies + TCR gene rearrangement if persistent lesions or atypical features.
  • HRCT chest if sarcoidosis suspected.

Management

  • Treat any underlying condition.
  • Counsel about progressive, irreversible nature.
  • Cosmetic options limited โ€” surgical excision occasionally for cosmetically prominent lesions; emerging reports of ablative or fractional laser.
  • Patients with positive APS antibodies: refer for thromboprophylaxis assessment.
  • Patients with associated MF / CTCL: enrol in cutaneous-lymphoma surveillance pathway.

References

  1. Hodak E et al. Primary anetoderma associated with antiphospholipid antibodies. Br J Dermatol. 2010;162:1377-1380.
  2. Jubert C et al. Anetoderma may reveal cutaneous T-cell lymphoma. Arch Dermatol. 1993;129:1535-1537.
  3. Lee Y et al. Anetoderma: a review of clinical features, etiology, and treatment. Int J Dermatol. 2021;60:1287-1295.
  4. Venencie PY, Winkelmann RK. Histopathologic findings in anetoderma. Arch Dermatol. 1984;120:1040-1044.

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