Abstract: The aim of this study was to evaluate characteristics of patients with pilomatricoma in Mazandaran Province, 1996-2006. In this study 46 case of pilomatricoma, with asymptomic, single and firm nodule referred to dermatology clinic of Boo-Ali Sina Hospital in Sari (1996-2006). A Biopsy was taken from suspicious lesions and histopathology assessments were done for cases with pilomatricoma impression. 69.56% patients were female. Mean age of the patients was 20.1 years .The most frequent sites of the tumor were the forearm (41.30%). It can be concluded, because the pilomatricoma is a benign cutaneous neoplasm with differentiation toward hair matrix, so that it is not seen in palm or plantar surface, but could present in any other area. Since, few of pilomatricoma lesions converted to malignant, so that it is better we resects total lesion by surgical excision.
INTRODUCTION
Pilomatricoma, also known as calcifying epithelioma of Malherbe is an uncommon benign cutaneous neoplasm with differentiation toward hair matrix that occurs frequently in children (Kaddu et al., 1994; Holme et al., 2001; Kaveri and Punnya, 2008). Pilomatricoma usually manifests as a solitary, asymptomatic and firm nodule, but some reported pain during episodes of inflammation (Rao and Lin, 2006). Histologically, a pilomatricoma is an encapsulated lesion composed of solid small basophilic cells and eosinophilic ghost cells. There is a gradual transition from the basophilic cells, located at the perimeter of the lesion, to the more centrally located ghost cells. Calcification usually takes place producing solid purple amorphous masses. Ossification, melanin deposition and foreign body giant cells also may be present (Farmer and Hood, 2000). Globally, 15 patients with pilomatricoma were seen in a pediatric surgery clinic in Turkey from 1984-1994 (Demircan and Balik, 1997). Cigliano presents 83 patients with pilomatricoma during a 7 years period (1996-2002) at the Departments of Pediatrics, University Hospital Federico II, Naples, Italy (Cigliano et al., 2005). Kaddu et al. (1994) studied 118 patients admitted at Department of Dermatology in Australia between 1980-1990. Diagnosis of pilomatricoma is difficult and the final confirmation is by histological examination (Punia et al., 2001). Because preoperative diagnosis of pilomatricoma is usually incorrect, careful clinical examination and a high index of suspicion would result in a more accurate diagnosis (Lan et al., 2003). This study presents 46 cases of pilomatricoma. Principle characteristic clinical presentations of this tumor are discussed.
MATERIALS AND METHODS
In this descriptive study, patients with asymptomatic, single and firm nodule referred to dermatology clinic of Boo-Ali Sina Hospital in Sari, Iran (1996-2006) were assessed. Biopsy was taken from suspicious lesions and histopathology assessments were done for cases with pilomatricoma impression (Fig. 1, 2).
| Fig. 1: | Very low power view (direct scan of glass slide). Multiple types of keratinization plus a mixture of epithelial elements produce a swirled or geographic appearance at this power |
| Fig. 2: | High powers view. Ghost cells (shadow cells) are associated with a granulomatous reaction. The ghost cells have a yellowish cast and this is common in this type of keratin |
Demographic data, clinical finding, the site, size, color, and malignant deformity of lesions were evaluated. The results were descriptively analyzed using χ 2 test with SPSS1l.
RESULTS AND DISCUSSION
In this study we presented 46 cases of pilomatricoma. These cases were recognized from a total of 2470 dermal biopsies taken in dermatology ward of Boo-Ali Sina Hospital Sari, Iran from 1996-2006. Thirty two (69.56%) patients were female and 14 (30.44%) male. Mean age of the patients was 20.1 years (range 2- 68 years). The most common age group was 11-20 years. The most frequent sites of the tumor were the forearm (41.30%) arm (17.39%)(Table 1).
The color of lesions was cream 12 (26.08%) (Fig. 3), brown 6 (13.04), light cream 9 (19.56%), gray 9 (19.56%); skin color 4 (8.6%), light red 1(2.1%), brown 1 (2.1%) and white 1 (2.1%).
The size of most of the lesions was 0.85 cm, the largest size was 4 cm and the smallest was 0.1 cm seen in scalp and arm, respectively. In biopsy study, 22 cases had calcification. None of the cases had malignancy.
Pilomatricoma is a skin appendage tumor that frequently involves the skin of the head, neck and upper extremities in young children (Demircan and Balik, 1997).
In a study by Cigliano et al. (2005) female/male ratio was 2: 1 and in Jjaggi’s review study, the female to male ratio in 209 cases was 1.5: 1 (Demircan and Balik, 1997), but Lan et al. (2003) reported 0.97:1. In this study this ratio was 2.5 pilomatricoma can occur at any age (Lan et al., 2003). However, most reported cases have occurred in children (Kaddu et al., 1994; Demircan and Balik, 1997) and it is a rather uncommon neoplasm in middle- age and old patients (Behnke et al., 1998).| Table 1: | Frequency distribution of the location in 46 cases with pilomatricoma referred to Boo-Ali-Sina hospital |
| Fig. 3: | The most common lesions color was cream |
Mean age in this study was 20.1 years (range: 2- 68 years). The most common age group was 11- 20 years. Jaggi found a bidominal pattern for age presentation: the first peak beings at 5-15 years and the second beings at 50-65 years (Kaddu et al., 1994; Rao and Lin, 2006). Based on the literature the tumors occur mainly on the head and neck regions (Pulvermacker et al., 2006; Pirouzmanesh et al., 2005; Rao and Lin, 2006; Darwish et al., 2001; Punia et al., 2001) which is similar to our results. Head and neck were the most common location of the tumor reported by Kumaran et al. (2006) 73.31% and Cigliano et al. (2005) 56.5%. In another study, the sites affected most commonly were periorbital, periauricular and upper cervical regions, in a descending order (Al-Khateeb and Hamasha, 2007). In this study the most common site was upper limb though (58.69%).
The lesions have usually normal skin color but reddish purple lesions were observed (probably resulting from hemorrhage) (Rao and Lin, 2006). In this study the cream was the most common color (26%).
In several studies, tumor size ranged between 3-43 mm (Kumaran et al., 2006; Lan et al., 2003; Darwish et al., 2001; Rao and Lin, 2006), we obtained a result varying amount of calcification was recorded in other studies (Darwish et al., 2001). The percentage of pilomatricoma with calcification was shown in earlier series to range from 63 to 75% (Lan et al., 2003). Demircan claimed that all cases had calcification in histopathology examination (Demircan and Balik, 1997) but Kumaran and Jjaggy reported 91 and 75%, respectively of (Kumaran et al., 2006; Rao and Lin, 2006). In the subjects this study was 49%. Pilomatrical carcinoma is uncommon (Rao and Lin, 2006). No invasive or malignancy lesion was observed by light microscopy (Demircan and Balik, 1997).In this study, no malignancy observed too. Because preoperative diagnosis of pilomatricoma is usually difficult, careful clinical examination and a high index of suspicion would result in a more accurate diagnosis. Complete surgical excision is the treatment of choice.