|Year : 2020 | Volume
| Issue : 4 | Page : 329-331
Multiple bouncy balls in an adolescent girl
Nidhi Kamra, Ankita Tuknayat
Department of Dermatology, Kalpana Chawla Government Medical College, Karnal, Haryana, India
|Date of Submission||23-Oct-2019|
|Date of Decision||24-Dec-2019|
|Date of Acceptance||26-Apr-2020|
|Date of Web Publication||30-Sep-2020|
Dr. Ankita Tuknayat
Department of Dermatology, Government Medical College and Hospital, Chandigarh
Source of Support: None, Conflict of Interest: None
Pilomatricoma is a benign hair follicle hamartoma presenting as a single hard dermal or subcutaneous nodule on the head and neck in children. A 15-year-old girl with multiple bullous pilomatricomas is being presented and the importance of ruling out underlying genetic defects in such patients is being discussed.
Keywords: Bouncy balls, bullous, multiple, pilomatricoma
|How to cite this article:|
Kamra N, Tuknayat A. Multiple bouncy balls in an adolescent girl. Indian J Paediatr Dermatol 2020;21:329-31
| Introduction|| |
Pilomatricoma is a benign slow growing hair follicle tumor arising from hair matrix cells which usually presents as a single slowly growing dermal or subcutaneous nodule on the head and neck. A rare case of bullous pilomatricoma in a 15-year-old is presented.
| Case Report|| |
A 15-year-old presented with a single reddish to mauve-colored translucent blister over her right shoulder since the past 6 months [Figure 1]. She did not give a history of any preceding lesions. There was no history of local trauma or insect bite. She had similar lesions in the past over her back and left arm which were excised by a local practitioner around 1-year back. No significant family history was elicited.
|Figure 1: Clinical photograph of the patient showing an erythematous to mauve-colored multilobulated bulla over the shoulder of a 15-year-old girl|
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On examination, she had a single 4 cm erythematous to slightly bluish translucent multilobulated flaccid bulla. On incising the bulla, a watery to jelly-like consistency fluid was evacuated and a hard whitish nodule of around 1.5 cm in size was seen in the center of the bulla. Systemic examination was normal. Differentials of calcinosis cutis, neurocutaneous proliferative angiomas, and infected sebaceous cyst were kept.
Routine investigations were normal and excision biopsy of the hard nodule was done for histopathological examination. It showed a dermal neoplasm made up of tumor islands that almost exclusively consisted of ghost cells, i.e., epithelial cells that have died through a process of karyolysis. In a few foci epithelial fragments were seen and these epithelial cells showed dark-rounded nucleus with a single nucleolus and abundant pink vacuolated cytoplasm. At these foci, gradual transition from epithelial cells to ghost cells was seen. Stroma showed moderately dense diffuse infiltrate of lymphocytes and neutrophils with foci of foreign body granuloma around the keratinized epithelial cells [Figure 2]. A diagnosis of bullous pilomatricoma was thus made.
|Figure 2: (a) Histopathology showing a dermal neoplasm made up of tumor islands consisting of ghost cells. Few foci of epithelial fragments with cells showing a dark-rounded nucleus with a single nucleolus and abundant pink vacuolated cytoplasm are seen. (b) Some foci of foreign body granulomatous reaction around the keratinized epithelial cells are also seen|
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| Discussion|| |
Pilomatricoma, also called as benign calcifying epithelioma of Malherbe is one of the most common hair follicle hamartoma resembling cells arising from hair matrix and inner root sheath. It usually has a female predominance and presents in patients aged <20 years. The lesion is a solitary deep dermal or subcutaneous tumor present on the head, neck, or upper extremities with a stony hard consistency and normal overlying skin. There may be overlying bluish discoloration. A number of variants have been described like superficial pilomatricoma presenting as a cutaneous horn, granulomatous type, melanocytic matricoma, multiple eruptive, ulcerative, and a bullous variant. Classic pilomatricomas if occurring in multiplicity are associated with a number of syndromes [Table 1]. However, no such association has been found with bullous pilomatricomas as yet. For early diagnosis, complete systemic examination and genetic analysis for specific genes like beta catenin should be done in suspected individuals.
Bullous pilomatricoma is a recently described variant with very few case reports in literature [Table 2]. Like the classic variant, it also has a female predominance and similar age of prevalence that is 10–20 years, but the bullous variant usually presents on the shoulder instead of head and neck. It presents as a large flaccid erythematous to rose pink bulla which is slowly increasing in size over the shoulders resembling a pear hanging from a tree and predominantly affecting females. This appearance has been previously compared to bouncy balls in literature. We propose 'hanging pear sign' for this appearance. Bulla may be multilobulated. On puncturing the bulla, a jelly-like material is seen exuding out. On complete dissection of the bulla, a hard whitish nodule is visualized.
Histopathologically, the tumor is situated in the dermis and consists of well-circumscribed islands of cells of which the outer cells are small and basophilic with scanty cytoplasm while toward the center the cells are eosinophilic with abundant cytoplasm with preserved nuclear outline. The cells give a mummified ghost cell appearance. These may calcify and calcium can be demonstrated in the basophilic areas of the tumor. Melanin can be demonstrated and a foreign body granulomatous reaction can be seen in some cases. Lymphangiectasia, dilated lymphatic vessels, giant cell reaction, loss of elastic tissue, and collagen fibers as well as calcification can be seen in the bullous variant. The present case is consistent with these findings.
Mechanism of bulla formation is currently an area of research. Various theories have been proposed. The first is that it could be due to mechanical pressure by the hard nodule which might have been compressing the underlying lymphatics leading to bulla formation. The second is that tumor cells may be producing elastinolytic enzymes which disrupt the collagen and elastic fibers and lead to dilation of lymphatic vessels. However, this theory seems unlikely because only 2% of patients of pilomatricoma develop an overlying bulla.
We hypothesize that there might be some underlying genetic defect or mutation in the connective tissue of such predisposed individuals such as beta catenin gene. Another hypothesis is that maybe such tumors have a deeper component compared to the classic pilomatricoma.
The present case is different because the patient had a history of multiple lesions in a span of 1-year. Although the present case had no association with congenital syndromes, we propose that it is pertinent to investigate such patients for any underlying connective tissue defect or associated congenital syndromes to make an early diagnosis and manage the patients accordingly.
We would like to acknowledge Dr. Uday Khopkar for the histopathology report.
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.
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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]