Year : 2009 | Volume
: 54 | Issue : 5 | Page : 83--85
Juvenile xanthogranuloma 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- 760 010, India
2 Department of Pathology, MKCG Medical College and Hospital, Berhampur, Orissa- 760 010, India
Department of Skin and VD, MKCG Medical College, Berhampur, Orissa - 760010
Juvenile xanthogranuloma (JXG), a rare benign histiocytic disorder, primarily seen in the first two decades of life as a solitary cutaneous lesion. Though in majority of cases lesions are confined to the skin, systemic forms can occur in rare instances. We present here a 21-year-old male patient presenting with multiple brown-to-yellowish papules and nodules over the head, neck and trunk evolving since last 2 years. A clinico/histopathologic study was done to confirm the diagnosis of JXG. We present this case, because of the unusual presentation and morphology in regards to the age and to highlight the fact that, juvenile xanthogranulomas though not so common, may occur in older age group and should always be in mind while making a diagnosis of a disease of similar morphology.
|How to cite this article:|
Nayak S, Acharjya B, Devi B, Patra MK. Juvenile xanthogranuloma in an adult.Indian J Dermatol 2009;54:83-85
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Nayak S, Acharjya B, Devi B, Patra MK. Juvenile xanthogranuloma in an adult. Indian J Dermatol [serial online] 2009 [cited 2021 Sep 26 ];54:83-85
Available from: https://www.e-ijd.org/text.asp?2009/54/5/83/45468
Helwig and Macknay first coined the term juvenile xanthogranuloma in 1954,  as a benign, asymptomatic and common self healing disoder of non-langerhans cell histiocytosis (LCH), affecting mostly infants, children and rarely adults. Eighty percent cases appear in the first year of life , and 20-30 percent cases present at birth.  There is no sexual or racial predilection. Though few authors showing a report of only 25 adult cases, , so also are many reports by various authors, mentioning that up to 20 percent of JXG arises in adolescence or young adults, that is why many dermatologists prefer to omit the term Juvenile. ,,, So we can say, it is not as rare as we were previously thinking. Clinically 90 percent of JXGs, cutaneous lesions are solitary, with head and neck being the most common site of involvement. Extracutaneous sites involving 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  .
A 21-year-old healthy, unmarried male presented to our Department with a two-year history of numerous well-demarcated, firm, rubbery brownish yellow papular lesions of different sizes, over face, neck, trunk and upper extremity, majority being on face. The patient had no other complain. General examination of all vital systems was normal. Cutaneous examination revealed multiple skin colored and hyperpigmented papules of different sizes, distributed over face, neck, extensor and flexor aspects of upper extremity [Figure 1]. The trunk had few lesions on front and back [Figure 2]. Some of the lesions showed features of spontaneous regression. Examination of mucous membrane, palm and sole ruled out any involvement. Patient was a healthy active male, cycle mechanic by profession and vehemently denied any sexual contact of any type in the past. As per patient's version, lesion first started two years back on the face as a solitary papule over right cheek, subsequently followed by appearance of multiple lesions in different parts as mentioned. A clinical diagnosis of late onset JXG was made and a biopsy was done. Histopathological examination [Figure 3],[Figure 4] revealed histiocytic proliferation with features of secondary xanthomization with the presence of foam cells, foreign-body giant cell, and Touton giant cells, mostly distributed in superficial dermis. Epidermis was thinned out and without any grenz zone. Lymphocytes, eosinophil and neutrophils were variably seen in inflammatory infiltrate with absence of plasma cell. A pathological confirmation of JXG made. Patient was advised for radiography of skull and chest, but results were non-contributory. Sonography of abdomen and pelvis ruled out any visceral involvement. Hematological and all biochemical parameters including lipid profile were within normal limits. Immunohistochemistry, a valuable procedure in confirming a diagnosis of extracutaneous JXG was not done taking into the consideration clinical course of adult form and absence of visceral lesions in those cases. Aperipheral smear examination ruled out any hematological malignancy. Patient was sent for an ophthalmologic check-up 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, patient was discharged with counseling and advised to report for a regular yearly follow-up.
JXG is the most common form of nonhistiocytosis X  , form a heterogeneous group defined by the proliferation of cells with macrophage characteristics. Though various authors have reported many cases, reports of adult onset cases is relatively rare, with a stereotypic presentation. 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, Sentamilselvi et al , in their case report of adult onset xanthogranuloma where skin lesions had undergo spontaneous resolution).  Three main clinical forms are recognized: a0 small nodular/papular (2-5mm); large nodular (5-20mm); and giant xanthogranuloma (more than 20mm) types.  But unusual clinical variants like mixed form, subcutaneous form, and JXG en plaque have been reported recently.  Multiple xanthogranulomas have been reported in association with hematological malignancy (lymphocytic leukemia and monoclonal gammopathy). 
Most important point to highlight in clinical presentation of patients with adult-onset xanthogranulomas, is absence of extracutaneous involvement of eye, orbit, lung, liver, testis, CNS, kidney etc, a feature very commonly seen in childhood variants. But the histological findings of both forms of the disease are identical. Another important aspect is, though there have been established association of JXG with neurofibromatosis (NF-1) and juvenile chronic myelogenous leukemia (JCML) in childhood types has never been reported in adults. So, usually detail lab investigations for extracutaneous JXG are not indicated, unless there are symptoms or findings suggesting their presence in adult onset types.
The disease needs to be differentiated from molluscum contagiosum, which in adults is usually sexually transmitted with fewer lesions, and favors lower abdomen, upper thigh, and penile shaft. They are centrally umbilicated, pearly papules with very typical histopathology, which clearly differentiate it from JXG. Second in line was molluscum like cryptococcosis, but the disease primarily occurs as a pulmonary one and skin involvement occurs by hematogenous dissemination and patient is usually debilitated and immunocompromised. Our patient was quite healthy and immunocompetent, so it was excluded. Other diseases like benign cephalic histiocytosis are seen exclusively in children, infiltate 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 a different HP Study), papular xanthoma (JXG histologically recognized by its pure primitive histiocytic phase and presence of inflammatory cells, not seen in papular xanthoma) should be considered in differential diagnosis of all cases of small nodular/papular form of JXG. Immunohistochemistry must be performed on all extracutaneous lesions for confirmation. Looking at the benign nature and instances of spontaneous regression as reported in the past, we decided to keep the patient under observation and yearly follow-up.
|1||Helwig EB, Macknay VC. Juvenile xanthogranuloma. Am J Pathol 1954;30:625.|
|2||Giannoti F, Caputo R. Histiocytic syndromes: A review. Am Acad Dermatol 1985;13:383.|
|3||Caputo R. Text Atlas of Histocytic Syndrome . London: Martin-Dunitz; 1988.|
|4||Winkelmann RK. Cutaneous syndromes of non-X histiocytosis: A review of the macrophage histiocyte diseases of the skin. Arch Dermatol 1981;117:667-72.|
|5||Gonzales-Crussi F, Campbell RJ. Juvenile xanthogranuloma: Ultrastructural study. Arch Pathol 1970;89:65-72.|
|6||Rodriguez J, Ackerman AB. Xanthogranuloma in adults. Arch Dermatol 1976;112:43-4.|
|7||Sonoda T, Hashimoto H, Enjoji M. Juvenile xanthogranuloma: Clinicopatholigic analysis and immunohistochemical study of 57 patients. Cancer 1985;56:2280-6.|
|8||Toroc E, Daroczy J. Juvenile xanthogranuloma: An analysis of 45 cases by clinical follow-up, light and electron microscopy. Acta Derm Venereol 1985;65:167-9.|
|9||Shin SJ, Scamman W, Gopalan A, Rosen PP. Mammary presentation of adult-type "Juvenile" Xanthogranuloma. Am J Surg Pathol 2005;29:827-31.|
|10||Cohen BA, Hood A. Xanthogranuloma: Report on clinical and histologic finding in 64 patients. Pediatr Dermatol 1989;6:262-6.|
|11||Punithwavathy K, Sentamilselvi G, Janaki VR, Janaki C. Late onset juvenile xanthogranuloma. Indian J Dermatol 1999;44:76-7.|
|12||Elstom DM, James WD, Berger TG. Andrew's diseases of the Skin, Clinical Dermatology. 10th ed. Philadelphia: WB Saunders Company; 2000.|
|13||Caputo R, Grimalt R, Gelmetti C, Cottoni F. Unusual aspects of juvenile xanthogranuloma. J Am Acad Dermatol 1993;29:868-70.|