Atrophoderma vermiculatum

Atrophoderma vermiculatum 2

Atrophoderma vermiculatum is a rare clinical variant of keratosis pilaris atrophicans characterized by reticulated skin atrophy. Its ICD-10 code is L66.4.

Atrophoderma vermiculatum can occur as:
  • A sporadic condition
  • An autosomal dominant inherited disorder
  • A variant of keratosis pilaris atrophicans (in most cases)
  • Part of inherited syndromes and diseases

The signs of the disease appear in childhood or during the period of sexual maturation, ranging from 3 months to 25 years of age. The average age of patients is 5-10 years. The disease is more common in boys (63% of cases). Etiology and pathogenesis are not well understood. The disease is characterized by increased keratinization of the upper third of the hair follicle, leading to follicular occlusion and chronic inflammation, ultimately resulting in scarring of the affected areas.

One hypothesis suggests that keratinocytes mediate the release of inflammatory cytokines, such as transforming growth factor-beta (TGF-beta), in response to the formation of follicular plugs. This process may contribute to fibrosis and the characteristic "cellular" atrophy.

Small follicular papules with keratotic plugs develop on the cheeks and periocular area, leaving a reticulated dermal atrophy on a background of mild and nonspecific erythema. Rarely, involvement may extend to other areas such as the forehead, chin, and earlobes. It is often associated with keratosis pilaris on the extensor surfaces of the limbs.

Atrophoderma vermiculatum can be a symptom of the following inherited syndromes and diseases:
  • Rombo syndrome (in combination with milia cysts, multiple basal cell carcinomas, trichoepitheliomas, hypotrichosis, acral cyanosis, and telangiectasias)
  • Nicolau-Balus syndrome (in combination with syringomas and milia cysts)
  • Braun-Falco syndrome (in combination with hair loss and palmoplantar keratoderma)
  • Down syndrome
  • Ehlers-Danlos syndrome (skin hyperelasticity and skin atrophy)
  • Marfan syndrome
  • Melkersson-Rosenthal syndrome (in combination with lip swelling and fissured tongue)
  • Neurofibromatosis (in combination with neurofibromas and cafĂ©-au-lait spots)
  • Loewes-Dietz syndrome (in combination with milium)
  • Atrial septal defect

The diagnosis is not difficult. Histologic examination shows follicular hyperkeratosis with keratotic plugs in dilated follicular openings. There is a perifollicular inflammatory reaction and areas of fibrosis in the dermis.

  • Post-acne scars
  • Scars after chickenpox
  • Nevus comedonicus
  • Lipoid proteinosis
  • Erythropoietic protoporphyria
  • Erythromelanosis follicularis faciei et colli
  • Keratosis pilaris

Treatment options for atrophoderma vermiculatum are generally limited in their efficacy. Oral isotretinoin has been reported to have a beneficial effect. Cosmetic procedures such as dermabrasion, laser resurfacing, and various fillers such as hyaluronic acid and autologous fat have been used for cosmetic correction with variable success. Intralesional and topical corticosteroids, as well as keratolytics and topical tretinoin-containing creams, have failed to produce significant improvement.