Generic selectors
Exact matches only
Search in title
Search in content
Post Type Selectors
Filter by Categories
15th National Conference of the IAOMFP, Chennai, 2006
Abstracts from current literature
Acne in India: Guidelines for management - IAA Consensus Document
Art & Psychiatry
Association Activities
Association Notes
Award Article
Book Review
Brief Report
Case Analysis
Case Letter
Case Letters
Case Notes
Case Report
Case Reports
Clinical and Laboratory Investigations
Clinical Article
Clinical Studies
Clinical Study
Conference Oration
Conference Summary
Continuing Medical Education
Cosmetic Dermatology
Current Best Evidence
Current Issue
Current View
Derma Quest
Dermato Surgery
Dermatosurgery Specials
Dispensing Pearl
Do you know?
Drug Dialogues
Editor Speaks
Editorial Remarks
Editorial Report
Editorial Report - 2007
Editorial report for 2004-2005
Fourth All India Conference Programme
From Our Book Shelf
From the Desk of Chief Editor
Get Set for Net
Get set for the net
Guest Article
Guest Editorial
How I Manage?
IADVL Announcement
IADVL Announcements
IJDVL Awards
IJDVL Awards 2018
IJDVL Awards 2019
IJDVL Awards 2020
IJDVL International Awards 2018
Images in Clinical Practice
In Memorium
Inaugural Address
Knowledge From World Contemporaries
Leprosy Section
Letter in Response to Previous Publication
Letter to Editor
Letter to the Editor
Letter to the Editor - Case Letter
Letter to the Editor - Letter in Response to Published Article
Letter to the Editor - Observation Letter
Letter to the Editor - Study Letter
Letter to the Editor - Therapy Letter
Letter to the Editor: Articles in Response to Previously Published Articles
Letters in Response to Previous Publication
Letters to the Editor
Letters to the Editor - Letter in Response to Previously Published Articles
Letters to the Editor: Case Letters
Letters to the Editor: Letters in Response to Previously Published Articles
Medicolegal Window
Miscellaneous Letter
Net Case
Net case report
Net Image
Net Letter
Net Quiz
Net Study
New Preparations
News & Views
Observation Letter
Observation Letters
Original Article
Original Contributions
Pattern of Skin Diseases
Pediatric Dermatology
Pediatric Rounds
Presedential Address
Presidential Address
Presidents Remarks
Report of chief editor
Report of Hon : Treasurer IADVL
Report of Hon. General Secretary IADVL
Research Methdology
Research Methodology
Resident page
Resident's Page
Resident’s Page
Residents' Corner
Residents' Corner
Residents' Page
Review Article
Review Articles
Revision Corner
Self Assessment Programme
Seminar: Chronic Arsenicosis in India
Seminar: HIV Infection
Short Communication
Short Communications
Short Report
Special Article
Specialty Interface
Study Letter
Study Letters
Symposium - Contact Dermatitis
Symposium - Lasers
Symposium - Pediatric Dermatoses
Symposium - Psoriasis
Symposium - Vesicobullous Disorders
Symposium Aesthetic Surgery
Symposium Dermatopathology
Symposium-Hair Disorders
Symposium-Nails Part I
Symposium-Nails-Part II
Systematic Review and Meta-Analysis
Systematic Reviews and Meta-analyses
Systematic Reviews and Meta-analysis
Therapeutic Guideline-IADVL
Therapeutic Guidelines
Therapeutic Guidelines - IADVL
Therapy Letter
Therapy Letters
View Point
What’s new in Dermatology
View/Download PDF

Translate this page into:

Net Quiz
doi: 10.4103/0378-6323.82445
PMID: 21727722

Disseminated asymptomatic yellowish papules on the face, trunk and limbs in a 3-year-old boy

Huang Ching-Fu1 , GaoHong-Wei2 , Wu Bai-Yao1 , Wang Wei-Ming1 , Chiang Chien-Ping1
1 Department of Dermatology, Tri-Service General Hospital, Taipei, Taiwan
2 Department of Pathology, Tri-Service General Hospital, Taipei, Taiwan

Correspondence Address:
Chiang Chien-Ping
Department of Dermatology, Tri-Service General Hospital, No.325, Sec. 2, Chenggong Road, Neihu Dist., Taipei City 114
How to cite this article:
Ching-Fu H, GW, Bai-Yao W, Wei-Ming W, Chien-Ping C. Disseminated asymptomatic yellowish papules on the face, trunk and limbs in a 3-year-old boy. Indian J Dermatol Venereol Leprol 2011;77:537
Copyright: (C)2011 Indian Journal of Dermatology, Venereology, and Leprology


A 3-year-old boy presented with asymptomatic, yellowish skin lesions scattered over his whole body. His parents stated that the lesions appeared about 1 year previously and that some flattened spontaneously [[Figure - 1], arrow]. His past medical history was unremarkable, but the parents had familial hyperlipidemia of unknown type. On physical examination, numerous asymptomatic 2-4 mm yellowish papules on the face, abdomen, back, and upper and lower extremities were seen that did not merge into plaques. Although no treatment was prescribed, some lesions spontaneously resolved with hyperpigmentation. Laboratory investigation of lipid profiles showed low-density lipoprotein cholesterol (84 mg/dl; normal value < 130 mg/dl), total cholesterol (160 mg/dl; normal value < 200 mg/dl), triglyceride (50 mg/dl; normal value < 160 mg/dl). Histopathology of the representative skin lesion (thigh) showed diffuse dermal infiltrate of foamy histiocytes [Figure - 2] and [Figure - 3].

Figure 1: Multiple yellowish papules scattered on the perioral area, bilateral cheeks and thighs. Some lesions spontaneously flattened (arrow)
Figure 2: Dense infiltrate of mononuclear cells with other scant inflammatory cells from the upper to lower dermis (H and E, ×100)
Figure 3: Diffuse dermal infiltrate of foamy histiocytes (H and E, ×400)

What is Your Diagnosis?

Diagnosis: Multiple papular xanthomas

Histopathology of the yellowish papules revealed a cellular infiltrate with foamy histiocytes from upper to lower dermis [Figure - 2] and [Figure - 3]. Infiltration of other inflammatory cells including lymphocytes or neutrophils was scant. Immunohistochemical analysis for S-100 and CD1a was negative. These histopathological features and clinical appearance were suggestive of multiple papular xanthomas (PXs).


PX without any underlying disorder was first described by Winkelmann in 1980. [1] The diagnostic criteria in the literature include: (1) generalized asymptomatic yellowish papulonodular lesions that do not merge into plaques or involve flexural areas, (2) no visceral involvement, (3) normal lipid profile, (4) infiltrate of foamy cells with negative immunostaining for S-100 and CD1a, and (5) no primitive histiocytic phase or infiltrate of other inflammatory cells. [1],[2] Initially, all cases of PX reported were adults and the lesions did not resolve spontaneously. In 1990, Caputo et al. observed that the skin lesions in 10 children presented similar clinical and histopathologic features to those reported for adults, but with a self-healing course within 1-5 years, [3] where the healed lesions might leave transient hyperpigmentation and anetoderma-like scars. [2],[3]

Clinically, PX is manifested by asymptomatic, widespread yellowish 2-10 mm papulonodules on the head, face, extremities and trunk, with sparing of flexural areas, palms and soles, that do not show any tendency to merge into plaques. [1],[2],[3] The histological feature is a diffuse dermal infiltrate of xanthomatized histiocytes characterized by central nuclei with surrounding foamy cytoplasm with no other inflammatory cells such as lymphocytes, plasma cells or eosinophils.

The histiocytes are strongly positive for oil red O staining but are negative for S-100 and CD1a. [2],[3] Ultrastructural study reveals no Birbeck granules or laminated bodies. The results of both immunostaining and ultrastructural study indicate foamy cells of non-Langerhans histiocytic origin.

Differential diagnoses of PX include juvenile xanthogranuloma (JXG), xanthoma disseminatum (XD) and benign cephalic histiocytosis (BCH). JXG usually presents as a dome-shaped, solitary, yellowish nodule with superficial telangiectases on the head and neck. Histologically, it presents initially as a primitive intradermal collection of predominantly histiocytes progressing later to a mixed infiltrate of histiocytes, foamy cells and Touton giant cells with scattered lymphocytes, eosinophils and plasma cells. These microscopic features are quite different from PX. In XD, clinical lesions have a tendency to merge into plaques and usually involve the mucosal and flexural areas, [2] and it is frequently associated with systemic disease, the most common manifestation being diabetes insipidus. [2] The histological findings consist of histiocytes with scalloped borders in the dermis. [4] Although rare cases of XD have a dermal infiltrate of foamy cells, a mixed infiltrate of other inflammatory cells is a common characteristic. [3] BCH occurs in early childhood, and lesions are round, erythematous to brownish-yellow papules, mainly on the face. It eventually spontaneously regresses without scarring. Histologically, it is characterized by a cellular infiltrate of non-Langerhans histiocytes with abundant eosinophilic cytoplasm and vesicular nuclei in the upper dermis. However, lipidized histiocytes are absent in BCH.

PX has three clinical variants: a self-limiting form, a persistent form and a progressive form. [2] In children, PX has a limited clinical course and lesions gradually disappear in 1-5 years without treatment. Although hyperlipidemia or hypercholesterolemia is not a feature of PX, one case of abnormal lipid profiles has been reported in the literature. [5] Hence, more data may be needed to evaluate the exact relationship between PX and lipid profiles.

Winkelmann RK. Cutaneous syndromes of non-X histiocytosis: A review of the macrophage-histiocyte disease of the skin. Arch Dermatol 1981; 117:667-72.
[Google Scholar]
Caputo R, Passoni E, Cavicchini S. Papular xanthoma associated with angiokeratoma of Fordyce: Considerations on the nosography of this rare non-Langerhans cell histiocytoxanthomatosis. Dermatology 2003; 206:165-8.
[Google Scholar]
Caputo R, Gianni E, Imondi D, Carminati G, Gianotti R. Papular xanthoma in children. J Am Acad Dermatol 1990; 22:1052-6.
[Google Scholar]
Zelger BW, Sidoroff A, Orchard G, Cerio R. Non-Langerhans cell histiocytoses: A new unifying concept. Am J Dermatopathol 1996; 18:490-504.
[Google Scholar]
Alan Arbuckle H, Prok L. Case report: Papular xanthoma in a 10-year-old female with abnormal lipid profile. Pediatr Dermatol 2010; 27:86-8.
[Google Scholar]

Fulltext Views

PDF downloads
Show Sections