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May 2007

Adult Disseminated Primary Papular Xanthoma Treated With Doxycycline

Arch Dermatol. 2007;143(5):659-675. doi:10.1001/archderm.143.5.667

Adult disseminated primary papular xanthoma (pPX) is a rare histiocytic proliferation of dermal dendrocyte origin in the spectrum of non–Langerhans cell histiocytosis. Adult disseminated pPX shows a chronic course, and no effective treatment is known. Herein we describe a woman with cutaneous lesions associated with pPX that showed complete regression after treatment with oral doxycycline.

A 29-year-old atopic woman consulted with us because of the progressive appearance of hundreds of asymptomatic papules 3 months earlier. Lesions started as a sudden crop of small papules that increased slowly in size and number. Crops of new lesions were admixed with older ones. Examination revealed hundreds of red-yellowish, nonconfluent, asymptomatic papules ranging in diameter from 0.5 to 1.0 cm (Figure 1A). Older lesions were flat and brownish. Lesions were diffusely distributed and tended to be grouped in skin folds but did not merge into plaques. In other locations such as the scalp, the abdominal wall, and the back, no clustering was observed. Solitary papules were seen elsewhere. Isolated mucosal lesions were also detected in tarsal and bulbar conjunctiva, gums, ears, and the upper airway. No adenopathies, visceromegalies, or signs of diabetes insipidus were present. Results of ophthalmic and neurologic explorations and extensive laboratory and imaging tests including plasma lipid and lipoprotein studies were normal.

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