Indian Journal of Dermatology
CASE REPORT
Year
: 2016  |  Volume : 61  |  Issue : 1  |  Page : 78--80

Nonfamilial multiple trichoepithelioma: Few and far between


Manu Sehrawat, Vijayeeta Jairath, VK Jain 
 Department of Dermatology, Venereology, Leprosy, Pt. B.D. Sharma Post Graduate Institute of Medical Sciences, Rohtak, Haryana

Correspondence Address:
Manu Sehrawat
203-L, Model Town, Rohtak - 124 001, Haryana

Abstract

Trichoepithelioma is a rare benign adnexal tumor which may be of solitary nonfamilial type or multiple familial trichoepitheliomas. Here, we describe a rare presentation of nonfamilial case of trichoepithelioma in a 50-year-old female with multiple skin colored facial papules and nodules over the face, upper back, and extremities.



How to cite this article:
Sehrawat M, Jairath V, Jain V K. Nonfamilial multiple trichoepithelioma: Few and far between.Indian J Dermatol 2016;61:78-80


How to cite this URL:
Sehrawat M, Jairath V, Jain V K. Nonfamilial multiple trichoepithelioma: Few and far between. Indian J Dermatol [serial online] 2016 [cited 2022 May 28 ];61:78-80
Available from: https://www.e-ijd.org/text.asp?2016/61/1/78/174030


Full Text

 Introduction



Trichoepithelioma is a benign hamartomatous tumor of the pilosebaceous follicle occuring either as a nonhereditary solitary lesion or as multiple lesions that are often dominantly inherited. With an unknown prevalence the tumour mostly appears in early childhood or at puberty. Multiple trichoepitheliomas (MTs) emerge as multiple skin colored to pink, firm, and papulonodular lesions, which are sited mainly on the face or occasionally on the scalp, neck, or upper trunk, gradually increase in number and size. They produce a significant cosmetic disfigurement. Malignant transformation to basal cell carcinoma (BCC) is unusual and occurs late in the course of the disease. [1]

MTs are usually inherited as an autosomal dominant disease; we report a sporadic case of nonfamilial MTs in a 50-year-old female. A far-reaching search of English literature did not reveal any nonfamilial case of MTs.

 Case Report



A 50-year-old female presented with asymptomatic multiple skin colored raised papulonodular lesions over the face, upper back, and extremities. The lesions started from the age of 3 years and continued to appear until date. Few of the lesions developed blackish discolouration since 4 years. Patient was otherwise healthy with no systemic involvement. On examination, multiple discrete to coalescing skin to yellow coloured waxy dome shaped papules and nodules of varying sizes were present over face including eyelids and eyebrows, external ear, scalp, neck, chest, back and lower limb [Figure 1] and [Figure 2]. Papules over eyelids were present in a beaded pattern [Figure 3]. Few of the nodules over nose and cheeks were crusted and surrounded by a zone of telengiectasia. Oral mucosa, appendages, palms, and soles were spared. Skin biopsy was performed from two different sites viz a waxy nodule and from a crusted lesion over the face. Both biopsies revealed tumour nodule in the dermis comprised of lobules of small dark blue staining cells, at places shows peripheral palisading and follicular differentiation. Mitotic activity of the nodule and the crusted lesion was insignificant [Figure 4]. Systemic examination and routine laboratory investigations were noncontributory.{Figure 1}{Figure 2}{Figure 3}{Figure 4}

No treatment was offered, as surgical excision will lead to multiple scars and the effective nonsurgical treatment is currently not available in our center.

 Discussion



Trichoepithelioma is a benign hamartomatous tumour originationg from the pilosebaceous follicle that usually appears in childhood or early adolescence. Trichoepithelomas has been christened differently by various authors. [2] Originally, it was described by Brooke in 1892 as epithelioma adenoides cysticum and Fordyce coined the term multiple benign cystic epithelioma. [3],[4]

Copious trichoepitheliomas occur in an autosomal dominantly inherited genodermatosis called multiple familial trichoepithelioma (MFT) or familial trichoepithelioma (FT). The exact prevalence is unknown but MTs appear to be rather uncommon. There is no perceptible predilection for gender or ethnicity. Johnson et al. reported MFT in two brothers with Linkage and mutational analyses in support of FT. [5] While in our case there was no family history of trichoepitheliomas. Trichoepitheliomas predominatly occurs on face, nasolabial folds, nose, upper lip, forehead, and eyelids, occasionally, on the scalp, neck, or upper trunk. Trichoepithelomas begin to develop as multiple skin coloured to pink, firm, rounded, transluscent, shiny, well demarcarted papulonodular lesions, centre may be slightly depressed. The lesions are grouped but discrete, often being symmetrical on the face. Trichoepitheliomas gradually increase in size and number producing significant cosmetic disfigurement. The patient is otherwise asymptomatic. Telengiectatic vessels may be observed on the surface of the larger lesions mimicking BCC. In contrast to BCC, ulceration occurs very rarely. Although Trichoepitheliomas are unassociated with malignant conditions, rare alliance with BCC has been reported by various authors. [1],[6],[7]

Diagnosis is based on history, clinical and histopathological examination. If needed, genetic studies may be used to detect the abnormalities in band 9p21. [8]

MTs form a part of rare syndromes which include Rombo's syndrome (vermicular atrophoderma, milia, trichoepithelioma, hypotrichosis, BCCs, and peripheral vasodilation with cyanosis) and Basex syndrome (follicular atrophoderma, trichoepithelioma, hypotrichosis, BCCs, and hypohydrosis). [9]

Differential diagnosis of trichoepithelioma includes BCC, syringoma, eccrine poroma, eccrine nevus, commedonal nevus and other appendeceal tumors. Histopathology typically shows horn cysts, tumor islands composed of basophilic cells of basaloid appearance arranged in peripheral palisading pattern are the major characteristic components of multiple trichoepithelioma. [10] These basophilic cells lack atypia and mitosis. Whereas the keratinization is abrupt and complete as opposed to horn pearls seen in squamous cell carcinoma. The tumor islands of basaloid cells demonstrate peripheral palisading with surrounding fibrous stroma. These fibroblasts lack artifactual retraction, characteristic of BCC. [7]

The presence of beaded waxy papules on the upper lid margin in our case highlights the possibility of lipoid protenosis (LiP). However, the absence of hoarseness of voice, pox like and acneform scars along with infiltration of skin and mucous membranes excludes the possibility of lipoid proteinosis. Moreover, LiP histologically is characterized by disruption of the basement membrane and deposition of hyaline material at the level of the basement membrane (resulting in its thickening at the dermo-epidermal junction), papillary dermis, surrounding capillaries, and around adnexal epithelia, especially sweat coils.

Various treatment modalities are been practised which include surgical excision, chemical cautrization, dermabrasion, cryosurgery, and laser resurfacing with promising results. [11] In a single case report an 11-year-old girl was treated with topical imiquimod cream and tretinoin 1% gel over 3 years period which showed remarkable reduction of lesions without scars. However in another report, isotretinoin for 12 weeks failed to affect the trichoepitheliomas of a patient with concurrent cystic acne. [5] Nonetheless, further substantiation is needed to determine the utility of these potential therapies.

MTs is a relatively an uncommon disease and diagnosis is usually based on its clinical appearance with a strong family history. The uniqueness of our case lies in the finding that besides being an autosomal dominant disease MTS can occur as sporadic disorder, after an extensive search of English literature no case until date has been reported as multiple nonfamilial trichoepitheliomas.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients 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.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.

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