Clear-cell papulosis: a rare entity that may be misconstrued pathologically as normal skin

Pediatr Dermatol. 2012 Mar-Apr;29(2):195-8. doi: 10.1111/j.1525-1470.2011.01614.x. Epub 2011 Dec 6.

Abstract

We describe a case of multiple, discrete, hypopigmented macules in the suprapubic and axillary region in a healthy 3-year-old girl. The lesions first appeared at approximately 9 months of age and increased in number over time. Initial histopathologic examination by an outside dermatopathologist at 1 year of age was reported as showing nonspecific histologic changes. A repeat biopsy at 3 years of age showed large intraepidermal clear cells that expressed CKAE1/CAM5.2, CK7, and BRST2. These findings are diagnostic for clear-cell papulosis, a rare condition that primarily affects children. Without great clinical and pathologic suspicion, this is a diagnosis that can often be overlooked because the histologic findings are virtually identical to those of normal skin.

Publication types

  • Case Reports

MeSH terms

  • Biomarkers / analysis
  • Biopsy
  • Carrier Proteins / analysis
  • Child, Preschool
  • Dermatologic Agents / therapeutic use
  • Female
  • Glycoproteins / analysis
  • Humans
  • Hydrocortisone / therapeutic use
  • Hypopigmentation / drug therapy
  • Hypopigmentation / pathology*
  • Imidazoles / therapeutic use
  • Keratin-7 / analysis
  • Keratins / analysis
  • Membrane Transport Proteins
  • Skin / drug effects
  • Skin / pathology*
  • Skin Diseases, Papulosquamous / drug therapy
  • Skin Diseases, Papulosquamous / pathology
  • Treatment Outcome

Substances

  • Biomarkers
  • CAM 5.2 antigen
  • Carrier Proteins
  • Dermatologic Agents
  • Glycoproteins
  • Imidazoles
  • KRT7 protein, human
  • Keratin-7
  • Membrane Transport Proteins
  • PIP protein, human
  • Keratins
  • oxiconazole
  • Hydrocortisone