Skin nonmelanocytic tumor
Benign (nonmelanotic) epidermal tumors or tumor-like lesions
Clear cell papulosis
Author: Hillary Rose Elwood, M.D.
Last author update: 1 March 2015
Last staff update: 15 July 2021
Copyright: 2002-2024, PathologyOutlines.com, Inc.
PubMed Search: Clear cell papulosis [title]
Table of Contents
Definition / general | Clinical features | Case reports | Treatment | Clinical images | Gross description | Microscopic (histologic) description | Microscopic (histologic) images | Positive stains | Negative stains | Differential diagnosisCite this page: Elwood H. Clear cell papulosis. PathologyOutlines.com website. https://www.pathologyoutlines.com/topic/skintumornonmelanocyticclearcellpapulosis.html. Accessed December 21st, 2024.
Definition / general
- Rare condition of unknown histogenesis and pathogenesis, predominantly described in Asian children
- Originally proposed to be related to Toker's clear cells of the nipple (Am J Surg Pathol 1987;11:827)
- May be a genetic predisposition based on positive family history in some cases
Clinical features
- Manifests in early childhood, majority before age 2
- Rare in adults
- No gender predilection
- Predominantly described in Asian and Hispanic populations
- Presents as multiple white hypopigmented macules or papules most commonly on the pubic area and lower abdomen
- Less commonly on face, chest, anterior trunk, axilla
- Can be linear, and may develop along the 'milk line'
- Range from 2 - 10 mm in size
- Usually 10 - 20 lesions, with range from 5 to more than 100
- Hypopigmented to depigmented macules or barely palpable papules
Case reports
- 16 month old girl and 2 year old boy (Arch Pathol Lab Med 2004;128:e149, Dermatol Online J 2008;14:19)
- 18 month old boy and 6 year old sister (Arch Dermatol 2007;143:358)
- Clear cell papulosis (pagetoid papulosis) in a non-Asian patient (Dermatology 2001;203:260)
Treatment
- Benign asymptomatic lesions, no known treatments
- Lesions may decrease in number or disappear over time (J Am Acad Dermatol 2010;63:266)
Clinical images
Images hosted on other servers:
Multiple hypopigmented macules
Gross description
- Small hypopigmented macules or papules
Microscopic (histologic) description
- Intraepidermal oval to round cells with ample pale clear cytoplasm, single or in clusters
- Clear cells predominantly among basal cells but some may be in the suprabasal epidermal layers
- Larger than adjacent keratinocytes
- Nucleoli tiny or indistinct
- Mitotic figures rare and no pleomorphism
- Epidermis mildy acanthotic with mild hyperkeratosis
- Decreased to absent pigmentation of basal epidermis (can be highlighted by Fontana-masson stain)
Microscopic (histologic) images
Images hosted on other servers:
Mild acanthosis
H&E, CK7+
Positive stains
- GCDFP-15, pancytokeratin, CK7, CAM5.2, low molecular weight keratin, CEA, EMA
- Variably stained by mucin stains including PAS, mucicarmine, Alcian blue, colloidal iron
Differential diagnosis
- Paget disease: histologic mimic - distinction is predominantly based on clinical findings (i.e. age of patient, location, number of lesions and clinical appearance)
- Pagetoid dyskeratosis: an incidental histologic finding - cells with a pyknotic nucleus, a clear halo, and a rim of pale cytoplasm; clear cells at higher levels in epidermis, negative for PAS / mucicarmine,
