Generic selectors
Exact matches only
Search in title
Search in content
Search in posts
Search in pages
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 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
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
Therapeutic Guidelines
Therapeutic Guidelines - IADVL
Therapy Letter
View Point
What’s new in Dermatology
View/Download PDF
Letter To Editor
doi: 10.4103/0378-6323.38419
PMID: 18187834

Multiple xanthogranulomas in an adult

Surajit Nayak1 , Basanti Acharjya1 , Basanti Devi1 , Manoj Kumar Patra2
1 Department of Skin and VD, MKCG Medical College and Hospital, Berhampur, Orissa, India
2 Department of Pathology, MKCG Medical College and Hospital, Berhampur, Orissa, India

Correspondence Address:
Surajit Nayak
Department of Skin and VD, MKCG Medical College, Berhampur - 760 010, Orissa
How to cite this article:
Nayak S, Acharjya B, Devi B, Patra MK. Multiple xanthogranulomas in an adult. Indian J Dermatol Venereol Leprol 2008;74:67-68
Copyright: (C)2008 Indian Journal of Dermatology, Venereology, and Leprology
Figure 1: Numerous papular lesions over the face
Figure 1: Numerous papular lesions over the face


A 21-year-old old unmarried male presented to our Department with a 2 year history of numerous well-demarcated, firm, rubbery brownish yellow papular lesions of different sizes, over the face, neck, trunk and upper extremities, the majority being on the face. The patient was otherwise healthy. General examination of all vital systems was normal. Cutaneous examination revealed multiple skin-colored and hyperpigmented papules of different sizes, distributed over the face, neck, extensor and flexor aspects of the upper extremities [Figure - 1]. The trunk had a few lesions on the front and back. The lesions were asymptomatic, firm to rubbery in consistency and non-tender. Examination of mucous membranes, palms and soles was normal. The patient was a healthy active male, cycle mechanic by profession and denied any sexual contact of any type in the past. As per the patient′s version, lesions first started 2 years back on the face as a solitary papule over the right cheek, subsequently followed by appearance of multiple lesions in different parts as mentioned. A clinical diagnosis of late onset juvenile xanthogranuloma (JXG) was made and a biopsy was done. Histopathological examination revealed histiocytic proliferation with features of secondary ′xanthomization′ with the presence of foam cells, foreign-body giant cells and Touton giant cells, in the superficial dermis. The epidermis was thinned out and without any grenz zone. Lymphocytes, eosinophils and neutrophils were variably seen in the inflammatory infiltrate with absence of plasma cells. The histopathology confirmed the diagnosis of JXG. The patient was advised radiography of skull and chest, but results were non-contributory. Sonography of the abdomen and pelvis was also normal. Hematological and biochemical parameters including lipid profile were within normal limits. A peripheral smear examination ruled out any hematological malignancy. The patient was sent for an ophthalmologic checkup and fundoscopy but no abnormality was detected. Looking at the benign course of the disease and instances of spontaneous regression and absence of any specific therapy, the patient was discharged with counseling and advised to report for a regular yearly follow-up.

Helwig and Macknay first coined the term juvenile xanthogranuloma in 1954, [1] as a benign, asymptomatic and common self-healing disorder of non-Langerhans cell histiocytosis (LCH), affecting mostly infants, children and rarely adults. Eighty per cent cases appear in the first year of life [2],[3] and 20-30% cases present at birth. [2] There is no sexual or racial predilection. [4] Clinically, in 90% of JXGs, cutaneous lesions are solitary, with the head and neck being the most common sites of involvement. Extracutaneous sites involving the eye, lung, abdominal viscera and skull have been reported by many authors. Adult JXGs rarely regress spontaneously and reports of concomitant extracutaneous lesions are rare. [5]

Juvenile xanthogranuloma is the most common form of non-X histiocytosis. [6] It forms a heterogeneous group defined by the proliferation of cells with macrophage characteristics. It is important to recognize multiple adult xanthogranulomas, because of its good prognosis and the absence of visceral involvement, therefore requiring no investigations or aggressive treatments, a very important observation made by Punithwavathy et al., in their case report of adult onset xanthogranuloma. [7] Three main clinical forms are recognized: a small nodular/papular (2-5 mm); large nodular (5-20 mm); and giant xanthogranuloma (more than 20 mm). [8] But unusual clinical variants like mixed form, subcutaneous form, JXG en plaque have been reported recently. Multiple xanthogranulomas are extremely unusual in adults and that to occur in an eruptive manner is quite rare and three such cases have been associated with hematological malignancy (lymphocytic leukemia and monoclonal gammopathy). [8]

Clinical backgrounds of patients with adult-onset xanthogranuloma are somewhat different from those of patients with juvenile xanthogranuloma, but the histological findings of both forms of the disease are identical. Extracutaneous involvement of the eye orbit, lung, liver, testis, central nervous system, kidney etc. has been reported in childhood variants, but is not seen in the adult type. Though there has been established association of JXG with neurofibromatosis (NF-1) and juvenile chronic myelogenous leukemia (JCML) in the childhood type, this has never been reported in adults. Evaluation for extracutaneous JXG is not indicated, unless there are symptoms or findings suggesting their presence, as they also disappear spontaneously. Differential diagnosis includes molluscum contagiosum, cryptococcosis, benign cephalic histiocytosis (seen exclusively in children, infiltrate lacks foamy cells and multinucleated giant cells), generalized eruptive histiocytosis (absence of granulation and lipidation), xanthoma disseminatum (lesions tend to merge into plaques, mucous membrane involvement, associated diabetes insipidus and different biopsy findings), papular xanthoma (JXG histologically recognized by its pure primitive histiocytic phase and presence of inflammatory cells, not seen in papular xanthoma). The importance of presenting this case is to highlight the fact that while making a diagnosis of common disorders like molluscum contagiosum, one must keep in mind the adult form of xanthogranuloma in differential diagnosis.

Helwig EB, Macknay VC. Juvenile xanthogranuloma. Am J Pathol 1954;30:625.
[Google Scholar]
Giannoti F, Caputo R. Histiocytic syndromes: A review. Am Acad Dermatol 1985;13:383.
[Google Scholar]
Caputo R. Text Atlas of Histocytic Syndrome . London: Martin-Dunitz; 1988.
[Google Scholar]
Winkelmann RK. Cutaneous syndromes of non-X histiocytosis: A review of the macrophage histiocyte diseases of the skin. Arch Dermatol 1981;117:667-72.
[Google Scholar]
Shin SJ, Scamman W, Gopalan A, Rosen PP. Mammary presentation of adult-type "Juvenile" xanthogranuloma. Am J Surg Pathol 2005;29:827-31.
[Google Scholar]
Cohen BA, Hood A. Xanthogranuloma: Report on clinical and histologic finding in 64 patients. Pediatr Dermatol 1989;6:262-6.
[Google Scholar]
Punithwavathy K, Sentamilselvi G, Janaki VR, Janaki C. Late onset juvenile xanthogranuloma. Indian J Dermatol 1999;44:76-7.
[Google Scholar]
Caputo R, Grimalt R, Gelmetti C, Cottoni F. Unusual aspects of juvenile xanthogranuloma. J Am Acad Dermatol 1993;29:868-70.
[Google Scholar]

Fulltext Views

PDF downloads
Show Sections