|Year : 2022 | Volume
| Issue : 1 | Page : 49-52
Multiple eruptive cholesterotic dermatofibromas in a healthy 15-year-old Indian boy
Subhra Dhar1, Divya Gupta2, Abhishek De3
1 Chief of Lab and Consultant, Histopathologist, WIZDERMPATHLAB, Kolkata, West Bengal, India
2 Department of Dermatology, Dr. B. R. Ambedkar Medical College, Bengaluru, Karnataka, India
3 Department of Dermatology, Calcutta National Medical College, Kolkata, West Bengal, India
|Date of Submission||14-Sep-2021|
|Date of Acceptance||02-Nov-2021|
|Date of Web Publication||31-Dec-2021|
Department of Dermatology, Dr. B.R. Ambedkar Medical College, Bengaluru, Karnataka
Source of Support: None, Conflict of Interest: None
A 15-year-old healthy boy presented with multiple eruptive brown-colored to hyperpigmented papules all over the body for 1 year. His serum lipids were normal, and HIV serology was negative. Biopsy revealed hyperplastic epidermis along with an ill-defined lesion in the dermis that was composed of spindle-shaped fibroblastic cells and multiple cholesterol clefts within foamy macrophages. A diagnosis of cholesterotic dermatofibroma was made. The case is unusual for the young age of presentation and the presence of multiple eruptive papules.
Keywords: Cholesterol clefts, cholesterotic dermatofibroma, cholesterotic fibrous histiocytoma, healthy boy, pediatric
|How to cite this article:|
Dhar S, Gupta D, De A. Multiple eruptive cholesterotic dermatofibromas in a healthy 15-year-old Indian boy. Indian J Paediatr Dermatol 2022;23:49-52
|How to cite this URL:|
Dhar S, Gupta D, De A. Multiple eruptive cholesterotic dermatofibromas in a healthy 15-year-old Indian boy. Indian J Paediatr Dermatol [serial online] 2022 [cited 2022 Jan 20];23:49-52. Available from: https://www.ijpd.in/text.asp?2022/23/1/49/334664
| Introduction|| |
Dermatofibroma (DF), also known as benign fibrous histiocytoma (BFH), is a benign reactive proliferation of cells in response to trauma including insect bites. As implied by the name, it signifies a shared morphology comprising both fibroblast-like spindle cells and presumptive histiocytes. Thus, these tumors show features of both fibroblastic and histiocytic differentiation. Here, we report an unusual case of cholesterotic DF, which is a rare variant of DF.
| Case Report|| |
A 15-year-old boy presented with asymptomatic skin-colored lesions for 1 year. Initially, there was only a single lesion over the right thigh for the first 3 months. Thereafter, there was a sudden burst of multiple lesions in just 2 weeks, which spread to involve the extremities and the trunk. There was no history of trauma or any other precipitating factor. On examination, multiple brown-colored to hyperpigmented papules of size 1–3 mm were seen over both the upper and lower extremities and the trunk. Koebner's phenomenon was seen [Figure 1]a and [Figure 1]b. Complete blood count, lipid profile, and renal and liver function tests were normal. HIV serology was negative. Biopsy was taken from a papule on the left thigh. It showed a hyperplastic epidermis with elongation of rete ridges and increase in basal layer pigmentation. There were numerous spindle-shaped cells strewn within sclerotic collagen bundles [Figure 2]a. One area showed the presence of cholesterol clefts which were surrounded by foamy macrophages [Figure 2]b and [Figure 2]c. Based on the histopathology and clinical features, a diagnosis of multiple eruptive cholesterotic DF was made.
|Figure 1: (a and b) Brown-colored to hyperpigmented papules of size 2–4 mm over the thighs and legs. Koebnerization is seen|
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|Figure 2: (a) Acanthotic epidermis with elongated rete ridges and increased pigmentation of the basal layer. Dermis showing spindle-shaped cells interspersed between the collagen bundles (H and E, ×10). (b) Dermis showing cholesterol clefts (H and E, ×10). (c) Needle-shaped cholesterol crystals surrounded by numerous foamy histiocytes containing pale cytoplasm (H and E, ×100)|
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| Discussion|| |
A DF may contain variable histological presence of fibroblasts, histiocytes, blood vessels, and collagen, which has led to a plethora of confusing names such as sclerosing hemangioma, nodular subepidermal fibrosis, and BFH, among others. A typical lesion presents as a solitary, dusky brown papule ranging in size from a few millimeters to 2–3 cm, over the extremities in young adults. However, DFs exhibit significant heterogeneity in clinical and histological features. The color may vary from red to yellow or black based on the preponderance of blood-filled cavernous spaces, steatotic material, or hemosiderin, respectively. Unusual locations such as fingers, palms and soles, scalp, face, and within a vaccination scar as well as a tattoo have been mentioned. Polypoid, flat, and atrophic lesions may occur along with other morphologies such as a halo of asteatotic eczema, Meyerson phenomenon, as well as satellitosis. Multiple lesions have been reported in immunosuppressed individuals (HIV, pregnancy, immunosuppressive therapy, and antiretroviral therapy).
Usually, DFs tend to persist indefinitely, although spontaneous involution can be seen in a few cases. The cellular variants have been associated with malignant transformation and metastasis.
Pronounced hyperplasia of the epidermis is a characteristic feature of DF. There is hyperpigmentation of the basal layer and elongation of the rete ridges, separated by a clear (Grenz) zone from the tumor in the dermis. This tumor is composed of fibroblast-like spindle cells, histiocytes, and blood vessels, in varying proportions.
Numerous histopathological variants (more than 40, according to some estimates) have been described, including combined subvariants [Table 1]. Cholesterotic DF (CDF) is one of the variants, which is clinically identical to the typical DF but differs on histopathology. It is regarded by some as a type of DF with stromal peculiarities (others being sclerosis or mucin or hemosiderin deposition).
The exact pathogenesis of cholesterotic DF remains enigmatic, although some authors have described a relationship with hyperlipidemias. It is hypothesized that serum lipoproteins leak through the capillaries into the dermis where they are phagocytosed by the dermal macrophages. Over a period of time, the accumulated lipids in the cytoplasm form the cholesterol clefts.
Electron microscopic findings in cholesterotic fibrous histiocytoma lesions show the presence of cholesterol crystals in the cytoplasm and lipid droplets in the lysosomes of histiocytes. Some authors have speculated that this mechanism is similar to the pathogenesis of atherosclerosis, whereby chronic inflammation and macrophage infiltration occur in the background of oxidative stress. Other scientists have previously shown that histiocytes transform into foam cells with cholesterol crystals in their lysosomes, when they are incubated with oxidized, low-density lipoproteins. These microenvironmental conditions may combine to generate this rare variant of DF.,
Monteagudo et al. described a cholesterotic type of DF in an HIV-positive patient. Because seropositive patients present with both an increased incidence of DFs and various metabolic syndromes, such as peripheral lipodystrophy, dyslipidemia, and insulin resistance, it was hypothesized that the presence of cholesterotic DF may not be a chance finding alone.
However, CDF may also be present in normal healthy individuals, without the evidence of any underlying lipid abnormalities. Presence of cholesterol deposits may simply indicate a chronic histologic process.
Cholesterotic DF must not be confused with a cholesterol granuloma (cholesteatoma). A cholesteatoma has usually been described only in the middle ear, and is believed to arise from degenerated keratins, erythrocytes, and chronic inflammatory reaction.
The clinical and histological differential diagnosis includes dermatofibrosarcoma protuberans, tuberous xanthoma, plexiform xanthomatous tumor, infectious diseases (atypical mycobacteriosis), erythema elevatum diutinum, Kaposi sarcoma, and lipidized DF.
Although they may sound similar, a lipidized variant is different from the cholesterotic variant of DF. The former is characterized by foamy macrophages surrounded by sclerotic collagen bundles. On the other hand, the hallmark of the cholesterotic variant is the prominent deposition of cholesterotic clefts in addition to the presence of foamy macrophages, with the absence of hyalinized, wiry collagen bundles, which are the hallmark of the lipidized variant. The cholesterotic variant has been described as a separate entity and is quite rare. So far, only seven reports of the cholesterotic DF have been described in literature [Table 2], and none in the pediatric age group. In contrast to fibrous histiocytomas, tuberous xanthomas are usually located on the extensor surfaces and show foam cells intermixed with cholesterol clefts on histopathology.,
|Table 2: Summary of previously reported cases of cholesterotic dermatofibromas|
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Our case was differentiated from the above causes based on morphology and presence of different types of cellular components in the dermis, including fibroblasts interspersed with collagen bundles and capillaries.
While diagnosis of the various subtypes can be made with a routine hematoxylin and eosin stain, sometimes immunohistochemistry can come in handy to rule out other diagnostic considerations such as dermatofibrosarcoma protuberans and other benign and malignant tumors of bland dermal spindle cells.
The diagnosis of this subtype of DF must alert the doctor to check for the plasma level lipids. Our case had multiple peculiarities. Cholesterotic FH is an exceedingly rare variant of fibrous histiocytoma, not hitherto described in the pediatric age group. In addition, our patient presented with multiple eruptive papules with Koebnerization in the absence of any immunosuppressive disorder. One limitation in our case was that we could not perform immunohistochemistry because of lack of resources.
| Declaration of patient consent|| |
The authors certify that they have obtained all appropriate patient consent forms. In the form, the patient(s) and his parents has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients and his parents understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.
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Conflicts of interest
There are no conflicts of interest.
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[Figure 1], [Figure 2]
[Table 1], [Table 2]