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Clear cell papulosis
Other Resources UpToDate PubMed

Clear cell papulosis

Contributors: Edward Li PhD, Susan Burgin MD
Other Resources UpToDate PubMed

Synopsis

Clear cell papulosis (CCP) is an extremely rare skin disorder first reported in 1987 that is characterized by asymptomatic, subcentimeter hypopigmented macules and thin papules distributed primarily in the lower abdomen or along milk lines in young children. Thus far, approximately 30 cases of histologically confirmed CCP have been reported with a 2:3 male-to-female sex ratio.

Nearly all reported cases of CCP were in children younger than 6 years, with only one reported case in a 46-year-old adult. Nearly all cases have been in children of Asian ancestry, with only a few cases reported in Hispanic and Italian patients. In further support of a possible genetic predisposition, many of the reported cases include first-degree siblings who carry the diagnosis as well.

Children affected by CCP meet normal developmental milestones and do not carry any associated disease diagnoses. Lesions of CCP appear as early as birth, with a typical time of disease presentation at the age of 2. The lesions typically increase in number during the first year and stabilize in size and number thereafter. On average, CCP patients have approximately 20 lesions, but this can range from less than 10 to over 100. Based on limited long-term follow-up, over 75% of patients showed significant lesion regression in size and color by their teenaged years, with several patients being completely lesion free.

Codes

ICD10CM:
L81.9 – Disorder of pigmentation, unspecified

SNOMEDCT:
201284005 – Hypopigmentation disorder

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Last Reviewed:11/02/2018
Last Updated:11/12/2018
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Clear cell papulosis
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Clear cell papulosis
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