Clear Cell Papulosis



Fig. 25.1
Clear cell papulosis. Courtesy Prof. Ramón Ruiz-Maldonado





Clinical Features/Diagnosis






  • White papules along the mammary line.


  • Clustered lesions usually over the lower abdomen and pubis, but can be placed anywhere along the milk line.


  • Lesions can be subtle and biopsy with special stains will aid in the identification of pagetoid clear cells.

Multiple white papules can be located over the lower abdomen and pelvis in areas of mammary line development. Clustering can occur. Placement may be any place along the mammary line including the axillae, upper chest, and back (Fig. 25.1) [7]. Lesions can appear lichenoid or imperceptibly elevated.

Histology reveals benign pagetoid clear cells in the basal layer of a hypomelanotic acanthotic epidermis. Cytoplasmic mucin is noted and lesions are positive for anticytokeratin antibodies (AE1/AE3), cell adhesion molecule (CAM) 5.2 [3], cytokeratin 7, carcinoembryonic antigen (CEA), and epithelial membrane antigen (EMA). The cells also stain with mucicarmine, colloidal iron, alcian blue (pH 2.5), and periodic acid-Schiff. S100 staining is negative [3] and BRST2 is positive [6]. The cells are presumed to be of sweat gland origin and may be derived from Paget cells or Toker cells [4, 5, 7].

The differential diagnosis includes acquired hypomelanoses including chicken pox scars, idiopathic guttate hypomelanosis, hypomelanotic tinea versicolor, anetoderma, and early, hypopigmented lesions of Paget’s disease [2]. Clear cell papulosis may also be construed as normal on skin biopsy and multiple biopsies or biopsies with a rim of surrounding skin may be necessary [7, 8].


Treatment


No treatment is needed. A recent case series following 19 Taiwanese patients with clear cell papulosis showed gradual resolution over a median of 11.5 years for all patients and complete clearance in 85.7 % [9]. Clear cell density was reduced in 1 patient even when full clearance was not achieved. Parental reassurance can be given in these settings.

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Nov 2, 2016 | Posted by in PEDIATRICS | Comments Off on Clear Cell Papulosis

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