Pilomatrixomas múltiples en pediatría: ¿sindrómicos o asociación causal? / Multiplepilomatrixomas in children: syndromic or chance association?

Los pilomatrixomas son neoplasias infrecuentes del tejido folicular. En el 70% de los casos suelen aparecer en las primeras dos décadas de vida, y con mayor frecuencia se distribuyen en cabeza y cuello. Normalmente se dan de forma aislada, siendo de muy rara aparición los pilomatrixomas múltiples, los cuales están relacionados con síndromes genéticos, especialmente con la distrofia miotónica de Steinert que puede presentarse tiempo después de la aparición de estos, por lo que precisan seguimiento. Es importante desde Atención Primaria la correcta identificación y derivación al especialista en Dermatología de estas lesiones, ya que su tratamiento es la extirpación y deben descartarse y hacerse seguimiento de posibles síndromes asociados. Existen casos descritos de malignización de pilomatrixomas (pilomatrixcarcinoma). Presentamos un caso de pilomatrixomas múltiples visto en consultas de Atención Primaria, en el cual se revisa la patología y se plantea el despistaje de síndromes asociados. Aunque, si bien el diagnóstico de certeza es histológico y su tratamiento quirúrgico, es el médico de familia el primer especialista que atiende al paciente y su correcta orientación diagnóstica basada en la exploración clínica, correlación, y derivación puede acelerar y prevenir las posibles complicaciones de los síndromes asociados

Pilomatrixomas are infrequent neoplasms of the follicular tissue. In 70% of cases they usually appear in the first two decades of life, and are most often distributed over the head and neck. They usually occur in isolation, so multiple pilomatrixomas are very rare. These are connected with genetic syndromes, especially Steinert myotonic dystrophy which may occur some time after the appearance of the pilomatrixomas. Therefore these require follow-up. A correct identification in primary care is essential, as well as referral to a dermatology specialist, since treatment involves their removal and the ruling out and follow-up of possible associated syndromes. There are case reports of malignant pilomatrixomas (pilomatrix carcinoma). We present a case of multiple pilomatrixomas seen in primary care, which reviews the pathology and considers associated syndromes. Although accurate diagnosis is histological and treatment is surgical, the family doctor is the first physician who cares for the patient. Thus, their correct diagnostic orientation based on clinical examination, correlation and referral can speed up the process and prevent the possible complications of associated syndromes

Pilomatrixomas in children: Report of 149 cases. A retrospective study at two children's hospitals. / Pilomatrixomas en niños: a propósito de 149 casos. Estudio retrospectivo en dos centros pediátricos.

A pilomatrixoma is a benign skin tumor common in children, which develops from the matrix cells of hair follicles. It presents as a nodule or tumor of approximately 0.5-3 cm in size, with calcium-like consistency, faceted edges, and blue erythematous overlying skin. Sometimes, the skin may atrophy and look like a blister or even extrude calcium. Diagnosis is suspected based on the typical clinical presentation and supplementary imaging tests. The definite diagnosis is made according to histological findings. Surgical removal is the treatment of choice. Here we describe the epidemiological and clinical data, supplementary tests, and surgical techniques in relation to 149 pilomatrixomas resected in 137 patients treated at the Departments of Dermatology of two children's hospitals: Dr. Pedro de Elizalde and Prof. Dr. Juan P. Garrahan.

El pilomatrixoma es un tumor benigno cutáneo, de frecuente aparición en los niños, que se origina en las células de la matriz del folículo piloso. Se presenta como un nódulo o tumor, aproximadamente, de 0,5 a 3 cm de tamaño, de consistencia cálcica, de bordes facetados, con piel suprayacente eritematoazulada. En ocasiones, la piel puede atrofiarse, dar la apariencia de una ampolla e, incluso, extruir material cálcico. El diagnóstico se sospecha por la presentación clínica característica y los estudios de imágenes complementarios. El diagnóstico definitivo es por los hallazgos histológicos. La extirpación quirúrgica es el tratamiento de elección. Se presentan los datos epidemiológicos, clínicos, estudios complementarios y técnicas quirúrgicas empleadas de 149 pilomatrixomas resecados en 137 pacientes tratados en los Servicios de Dermatología de los hospitales de pediatría Dr. Pedro de Elizalde y Prof. Dr. Juan P. Garrahan.

Updated view on epidemiology and clinical aspects of pilomatricoma in adults.

BACKGROUND: Clinically, pilomatricoma offers potential for a wide spectrum of differential diagnoses. It typically occurs in pediatric patients with the head being the most common location. A second peak of clinical presentation occurs in adults at age 50-65 years, suggesting a bimodal pattern of occurrence. OBJECTIVE: To investigate the clinical and epidemiological features of pilomatricoma in adults over 20 years old, as it is a common and frequently misdiagnosed tumor. METHODS: This was a retrospective study of pilomatricomas surgically removed at a tertiary hospital between January 1994 and December 2014. A search of the all-pathological database of patients aged over 20 years old with a pathological diagnosis of pilomatricoma was carried out. RESULTS: The clinical preoperative diagnosis of pilomatricoma was made in 34.0% of cases. Tumor location showed a predilection to the head and neck. Of the reported concomitant neoplasm, a majority had accompanying skin tumors. CONCLUSION: We conclude that clinical features in adults were similar to those of children. This study outlines clinical presentations that should help to guide differential diagnoses. Additionally, because of similarities between the distribution and depth of vellus hair follicles and pilomatricomas, it is probable that vellus hair bulbs may be the origin of this tumor.

Pilomatrixoma ampollar en caso pediátrico / Bullous pilomatrixoma in pediatric case

Se realiza la presentación de un caso clínico en edad pediátrica con diagnóstico de pilomatrixoma, variedad ampollar. Este tumor benigno originado en la vaina del folículo piloso muestra una incidencia elevada en las dos primeras décadas de vida. El objetivo de este artículo es presentar un caso con diagnóstico de un tumor, con características clínicas atípicas e inusuales. En ocasiones este tipo de tumor, conllevan al diagnóstico erróneo de la entidad, realizándose el diagnóstico de certeza mediante el estudio histopatológico una vez que se procede a la exéresis quirúrgica de la lesión (AU).

A clinical case with diagnosis of bullous variety pilomatrixoma in pediatric age is presented. This benign tumor originated in the sheath of the hair follicle shows a high incidence in the first 20 years of life. The aim of this work is presenting a case with tumor diagnosis, the clinical characteristics of which sometimes leads to an erroneous diagnosis of the entity in the atypical and unusual varieties. The accurate diagnosis was made through the pathologic study after the surgical excision of the lesion (AU).

Pilomatrixoma ampollar en caso pediátrico / Bullous pilomatrixoma in pediatric case

Se realiza la presentación de un caso clínico en edad pediátrica con diagnóstico de pilomatrixoma, variedad ampollar. Este tumor benigno originado en la vaina del folículo piloso muestra una incidencia elevada en las dos primeras décadas de vida. El objetivo de este artículo es presentar un caso con diagnóstico de un tumor, con características clínicas atípicas e inusuales. En ocasiones este tipo de tumor, conllevan al diagnóstico erróneo de la entidad, realizándose el diagnóstico de certeza mediante el estudio histopatológico una vez que se procede a la exéresis quirúrgica de la lesión (AU).

A clinical case with diagnosis of bullous variety pilomatrixoma in pediatric age is presented. This benign tumor originated in the sheath of the hair follicle shows a high incidence in the first 20 years of life. The aim of this work is presenting a case with tumor diagnosis, the clinical characteristics of which sometimes leads to an erroneous diagnosis of the entity in the atypical and unusual varieties. The accurate diagnosis was made through the pathologic study after the surgical excision of the lesion (AU).

El diagnóstico del pilomatrixoma infantil no es tan fácil como parece: revisión de 126 casos / The diagnosis of pilomatrixoma in children is not as easy as it may seem. A review of 126 cases

Objetivo. Describir nuestra experiencia con pilomatrixomas, examinando la presentación clínica, acierto clínico, enfermedades asociadas y tratamiento quirúrgico. Material y Métodos. Estudio retrospectivo de los pacientes con diagnóstico anatomo-patológico (AP) de pilomatrixoma, en el periodo de 2011 a 2014. Se recopilaron datos sobre genero, edad de intervención, número de tumoraciones, tamaño, localización, diagnóstico preoperatorio, anatomía patológica, reintervenciones y patología asociada. Se realizó el análisis estadístico con EPIinfo 7.1.5. Resultados. Se extirparon un total de 151 tumores en 138 pacientes, excluyendo 25 tumoraciones por tener diagnóstico clínico de pilomatrixoma sin confirmación patológica. Los 126 casos que tienen confirmación AP de pilomatrixoma tienen una edad media de exéresis de 8,26 años de edad (1-14 años) y el 50,72% son del género masculino. El 97% de los tumores se localizan por encima del ombligo, concentrándose en la cabeza y cuello el 52,9%. La sospecha clínica corresponde con AP en el 69,05%. De los casos confirmados, los diagnósticos clínicos diferenciales más frecuentes son lesión quística (11,90%) y quiste dermoide (10,22%); no hubo pilomatrixomas con degeneración maligna. La media de diámetro es de 1,13 cm, no hubo recidivas locales, pero sí 11 nuevos casos de pilomatrixomas en otra localización. No hay asociación estadísticamente significativa a otras patologías. Las enfermedades más frecuentes encontradas son la obesidad (3,62%) y el síndrome de déficit de atención (3,62%). Conclusión. El pilomatrixoma es una tumoración predominantemente de aparición en cara y cuello, de difícil diagnóstico clínico. La exéresis completa es curativa, aunque no previene la aparición de nuevas lesiones (AU)

Objective. To describe our experience with pilomatrixomas, clinical presentation, clinical accuracy, associated diseases, and surgical treatment. Methods. A retrospective review of patients with a pathology report of an excised pilomatrixoma between 2011 and 2014. Data regarding gender, age of intervention, number of masses, size, location, and preoperative clinical diagnosis, pathology report, recurrences, and associated diseases were collected. Statistical analysis was performed using EPIinfo 7.1.5. Results. A total of 151 tumors in 138 patients were removed, 25 were excluded for having a clinical diagnosis of pilomatrixoma without confirmation of the pathology report. The remaining 126 cases have an average age of 8.26 years (range 1-14 years) and 50.72% are males. The tumors were located above the umbilicus in 97% of the cases, with a higher concentration of 50.72% in the head and neck. The clinical diagnosis corresponds with the pathology report in 69.05% of cases. Out of these confirmed cases the most frequent differential diagnosis is cystic lesion (11.90%) and dermoid cyst (10.22%). There were no cases of malignant degeneration. The average diameter is 1.13 cm, there were no recurrences, but there were 11 cases of a new lesion in a different location. There is no statistical association with other pathologies. The most frequent diseases found were obesity (3.62%) and attention deficit syndrome (3.62%). Conclusion. The pilomatrixoma is a tumor that appears predominantly in the head and neck, and has a difficult clinical diagnosis. Complete removal is curative, although it does not prevent the appearance of new lesions (AU)

Congenital lesions of epithelial origin.

Defects of embryologic development give rise to a variety of congenital lesions arising from the epithelium and are among the most common congenital lesions of the head and neck in the pediatric population. This article presents several congenital lesions of epithelial origin, including congenital midline cervical cleft, pilomatrixoma, dermoid, foregut duplication cysts, and preauricular sinuses and pits. In addition, the management of these lesions is reviewed.

Pediatric parotideomasseteric pilomatrixoma in children.

AIMS: To review definitive histological diagnoses of patients with great salivary gland tumors with focus on the relatively high incidence of pediatric pilomatrixomas. The authors focus on clinical investigation, imaging methods and fine needle aspiration cytology of pilomatrixomas. METHODS: We treated 12 children with great salivary gland masses aged from 6 months to 18 years from 1995 to 2010. The records of these patients were reviewed to determine sex, age, clinical presentation, and histological findings. RESULTS: Among children having true neoplasms, there was a prevalence of carcinomas (6 out of 9), with low-grade mucoepidermoid and acinic cell carcinomas (two each) as the dominating histopathological diagnosis. There was one adenoid cystic carcinoma and one curious undifferentiated carcinoma in a 6 month old baby. Among all 6 benign lesions, accounting for a half of the total, pilomatrixoma was the most common (2 out of 6) diagnosis, representing 17% (2 out of 12) of all salivary gland lumps and 66% (2 out of 3) of all true benign neoplasms. CONCLUSIONS: Pilomatrixoma should be included in the differential diagnosis of pediatric parotideomasseteric lumps. Clinical investigation reveals adherence to the skin but not to the underlying tissue. Clinical assessment and ultrasound guided fine needle aspiration cytology in typical findings strongly support the diagnosis. Cytopathologists must be aware of the preliminary diagnosis of a pilomatrixoma to use proper fixation of the smears. In doubts, frozen biopsy must be sent before parotidectomy is performed.

Prevalence of pilomatricoma in Turner syndrome: findings from a multicenter study.

IMPORTANCE: The absence of data on the prevalence of pilomatricoma among patients with Turner syndrome served as the catalyst for this multicenter investigation. OBJECTIVES: To ascertain the prevalence of pilomatricoma among patients with Turner syndrome and to determine any association between the development of pilomatricomas and the use of exogenous hormones in patients with Turner syndrome. DESIGN: A retrospective medical record review from January 1, 2000, through January 1, 2010, was performed of all patients with Turner syndrome. Data on pilomatricomas and the use of hormone therapy were collected. SETTING: University of California-Davis Medical Center, University of Nebraska Medical Center, and The University of North Carolina at Chapel Hill. PARTICIPANTS: Patients with a diagnosis of Turner syndrome. MAIN OUTCOME MEASURES: Prevalence of concomitant pilomatricoma and diagnosis of Turner syndrome. Secondary outcome measures included the use of the exogenous hormones estrogen or recombinant human growth hormone (rhGH). RESULTS: In total, 311 patients with Turner syndrome were identified from these 3 institutions. Among them, 8 patients (2.6%) were diagnosed as having pilomatricomas. Before the development of pilomatricomas, 5 patients had been treated with rhGH but not estrogen, 1 patient had received estrogen but not rhGH, and 2 patients did not receive either therapy. CONCLUSIONS AND RELEVANCE: Although the prevalence of pilomatricoma among the general population is unknown, this study demonstrates a high prevalence (2.6%) of pilomatricomas among patients with Turner syndrome. No apparent relationship was noted among our patients or in the literature between the use of rhGH and the development of pilomatricomas.

Head and neck pilomatricoma: an analysis of 201 cases.

We report our experience with 201 pilomatricomas of the head and neck. They occurred over a wide age range (2-93 years, mean 29.5) and were most common in the first and second decades. Almost half (49%) presented in people over 40 years of age and there was no sex predilection (male:female ratio 1:1.1). The most common presentation was as a slowly enlarging, asymptomatic, subcutaneous mass, although nearly 25% were symptomatic, and 12% had a reddish blue skin discolouration. Most lesions were solitary; only 9 patients (5%) had multiple lesions. The most commonly affected sites were the cheek (23%), neck (22%), eyebrow (18%), and scalp (14%). Tumours were often mistaken for other lesions and a correct preoperative diagnosis was made in only 28% of cases. Preoperative investigations were done only occasionally and were not always helpful. All lesions were excised and excision was reported to have been incomplete in 22%. Two patients (1%) had a recurrence after incomplete excision. We highlight the condition to raise awareness among clinicians, and we support conservative excision as the most appropriate method of treatment.

Pilomatrixoma. Review of 205 cases.

AIMS: To determine the incidence and clinical features of patients diagnosed with pilomatrixoma. PATIENTS AND METHOD: A retrospective analysis was made of 205 cases of pilomatrixoma diagnosed according to clinical and histological criteria, with an evaluation of the incidence, patient age at presentation, gender, lesion location and size, single or multiple presentation, differential diagnosis, histopathological and clinical findings and relapses. RESULTS: Pilomatrixoma was seen to account for 1.04% of all benign skin lesions. It tended to present in pediatric patients--almost 50% corresponding to individuals under 20 years of age--with a slight male predilection (107/98). Approximately 75% of all cases presented as single lesions measuring less than 15 mm in diameter. Multiple presentations were seen in 2.43% of cases. The most frequent locations were the head and orofacial zones (particularly the parotid region), with over 50% of all cases, followed by the upper (23.9%) and lower limbs (12.7%). Only one relapse was documented following simple lesion excision. CONCLUSIONS: The frequency of pilomatrixomas was 1.04% of all benign skin lesions--the lesions being predominantly located in the maxillofacial area. Due to the benign features of this disorder, simple removal of the lesion is considered to be the treatment of choice, and is associated with a very low relapse rate.

Epidemiología de la cirugía dermatológica pediátrica. Un estudio retrospectivo de 996 niños / Epidemiology of Pediatric Dermatologic Surgery: A Retrospective Study of 996 Children

Objetivo: Realizar un análisis epidemiológico de la cirugía dermatológica realizada en pacientes menores de 16 años en el servicio de dermatología del Hospital Clínico Universitario durante un período de 9 años. Método: Se revisaron retrospectivamente todas las extirpaciones y biopsias de pacientes menores de 16 años desde 1 enero de 1999 al 31 de diciembre de 2007 en el servicio de dermatología del Hospital Clínico Universitario de Valencia. Los datos recogidos incluían las siguientes variables: la edad del paciente, el sexo, la localización y el diagnóstico histopatológico. Resultados: Dentro de este período, se analizaron histológicamente 33.840 lesiones. De ellas, 996 (2,94%) correspondieron a pacientes de ≤16 años. De las 996 piezas analizadas 502 (50,40%) correspondían a mujeres y 494 (49,60%) a varones.La edad media fue 11,06. El mayor número de exéresis se situó entre los 12 y los 16 años (47,39%). La distribución del resto de extirpaciones por grupos de edad fue la siguiente: ≤1 año 34 extirpaciones (3,41%), de >1 a ≤4 años: 66 (6,63%), de >4 a ≤8 años: 154 (15,46%) y de >8 a ≤12 años: 270 (27,11%). El diagnóstico más frecuente fue el de nevus melanocítico (50,20%), seguido del pilomatricoma (4,62%), el angioma capilar (3,61%), el quiste epidérmico (3,61%), el nevus de Spitz (3,31%) y el granuloma piogénico (3,11%). Conclusión: En nuestra serie, la cirugía dermatológica en menores de 16 años constituye un pequeño porcentaje de la cirugía dermatológica que se realiza en nuestro hospital, siendo el grupo de mayor edad el más frecuente. En la edad pediátrica, la patología benigna representa el mayor número de diagnósticos, siendo con mucho los nevus melanocíticos la patología más frecuente. El mayor número de lesiones se ha encontrado en el tronco, seguido por la cabeza y el cuello. En conclusión, de la literatura revisada, nuestra serie es la única en lo referente a la cirugía dermatológica en la edad pediátrica, siendo de interés para conocer la patología que con mayor frecuencia se analiza en estos pacientes (AU)

Objective: To describe the epidemiology of skin surgery performed in the dermatology department of Hospital Clínico Universitario in Valencia, Spain, on children up to 16 years of age. The study analyzed a 9-year period. Materials and method: A retrospective review was performed of all tissue excisions and biopsies taken between January 1, 1990, and December 31, 2007, from patients up to 16 years of age seen in the dermatology department of Hospital Clínico Universitario. The following data were collected: age, sex, surgical site, and histopathologic diagnosis. Results: Between 1990 and 2007, 33840 lesions were analyzed histologically; 996 of the lesions (2.94%) were from patients up to 16 years of age, of whom 502 (50.40%) were girls and 484 (49.60%) were boys. The mean age was 11.06 years. The majority (47.39%) of excisions were performed in children of 12 to 16 years. The distribution of the remainder by age groups was as follows: <1 year, 34 excisions (3.41%); >1 to <4 years, 66 (6.63%); >4 to <8 years, 154 (15.46%); and >8 to <12 years, 270 (27.11%). The most common diagnosis was melanocytic nevus (50.20%), followed by pilomatrixoma (4.62%), capillary angioma (3.61%), epidermal cyst (3.61%), Spitz nevus (3.31%), and pyogenic granuloma (3.11%). Conclusion: Dermatologic surgery in patients up to 16 years of age accounted for a small percentage of the overall dermatologic surgery in our hospital; the majority of children were in the 12 to <16 year age group. Most children had benign lesions, with melanocytic nevus being by far the most common diagnosis. The majority of lesions were on the trunk, followed by the head and neck. In conclusion, compared with the literature reviewed, ours is the only series on dermatologic surgery in children. It is of interest because it defines the most common conditions in these patients (AU)

Clinical and histopathologic study of pilomatricoma in Iran between 1992 and 2005.

Pilomatricoma (calcifying epithelioma of Malherb) is a tumor of hair follicle matrix cells and is almost always benign. In this study we reviewed the clinical and histopathological features of 45 cases of pilomatricoma during 13 years (1992-2005). Although few malignant transformations have been reported in other studies, no evidence of malignancy or aggressiveness is observed in this study.

Pilomatricoma of the head and neck: a retrospective review of 179 cases.

OBJECTIVE: To describe the clinical presentations and management of pilomatricoma, formerly known as pilomatrixoma, of the head and neck. DESIGN: Retrospective study. SETTING: Tertiary care center. PATIENTS: The study included 179 patients with a diagnosis of pilomatricoma of the head and neck. INTERVENTION: All patients underwent surgical excision for pilomatricoma of the head and neck between 1991 and 2002. RESULTS: Pilomatricoma occurred at any age (mean age, 29.8 years); 45.3% of the cases occurred in patients younger than 18 years. The female-male ratio was 0.97:1. The average size of the lesion was 1 cm. The most common sites of occurrence were the neck (30.2%), cheeks (16.8%), scalp (16.2%), and brow and periorbital area (14.0%). Multiple occurrence was found in 1 case. Two of 179 tumors recurred after surgical excision. CONCLUSIONS: Because preoperative diagnosis of pilomatricoma is usually incorrect, careful clinical examination and a high index of suspicion would result in a more accurate diagnosis. Complete surgical excision is the treatment of choice. Otolaryngologists should consider pilomatricoma in the differential diagnosis of neck masses.

Epitelioma calcificante de Malherbe (Pilomatrixoma) en la región parotídea. Presentación de un caso clínico / Calcifying epithelioma of Malherbe (Pilomatrixoma) in the parotid area. Report of a case

Normalmente, la presencia de un tumor en la región maxilofacial obliga realizar un minucioso estudio del paciente ya que los tumores en esta localización pueden tener diversa etiología. El estudio comprende un examen clínico completo, exámenes auxiliares, métodos de biopsia, etc. En este artículo se presenta el caso clínico de un paciente con una tumoración en la región parotídea que luego de realizar los exámenes correspondientes se llegó al diagnóstico de Pilomatrixoma o Epitelioma calcificante de Malherbe, entidad patológica que no es de frecuente aparición y cuyas características serán detalladas en este artículo (AU)

Pilomatricoma anetodérmico. Aportación de un caso y revisión de la literatura / Anetodermic pilomatricoma. Case report and review of the literature

El pilomatricoma anetodérmico es una forma excepcional de este tumor, habiendo sólo 29 casos descritos en la literatura. Desde el punto de vista clínico se caracteriza por la presencia de una piel atrófica, plegada, excrecente, con aspecto queloideo recubriendo un nódulo de consistencia dura. El estudio histopatológico muestra la presencia de edema intenso en la dermis, con aumento de la vascularización, vasodilatación, disminución con fragmentación de las fibras colágenas y la práctica ausencia de fibras elásticas. Bajo ella se encuentra un pilomatricoma situado principalmente en la hipodermis. Presentamos un caso localizado en la región escapular de un varón de 22 años en el que destacaban su gran tamaño y su rápido crecimiento, habiendo pasado en 5 meses de 3 a 6 cm de diámetro medio. (AU)

Head and neck pilomatricoma in the pediatric age group: a retrospective study and literature review.

OBJECTIVE: To discuss the clinical course and management of pilomatricoma involving the head and neck in the pediatric age group and to review the literature. METHODS: Retrospective analysis of the author's case files between the years of 1996 and 2000, revealed seven cases of head and neck pilomatricoma involving children. A literature review was employed to compare this study to others. RESULTS: In all cases, the presenting sign was a superficially located rock-hard mass in the head and neck. The mean duration the mass was present at the initial otolaryngologic evaluation was 11 months. There was a total of seven patients of which five (71%) were female while two (29%) were male. Each patient presented with a single pilomatricoma. Five (71%) occurred in the neck while two (29%) occurred in the face. All were treated with surgical excision. There were no recurrences. CONCLUSION: Pilomatricoma is a rare, benign, skin neoplasm that is superficially located and most commonly occurs in the head and neck, thus otolaryngologists should be aware of its clinical presentation. Although malignant transformation has been described, it is exceedingly rare. Diagnosis is usually suspected based on palpation of a superficial, rock-hard mass and confirmed by histopathologic examination. Since this neoplasm doesn't spontaneously regress, surgical excision is both curative and the treatment of choice. Recurrence is rare.

A clinical review of 209 pilomatricomas.

BACKGROUND: Pilomatricomas have a wide variety of clinical characteristics and are often misdiagnosed. This can result in extensive surgery for an essentially benign condition. OBJECTIVE: The purpose of this study was to define the clinical and histologic spectrum of these tumors to aid diagnosis. METHODS: Two hundred nine cases were analyzed retrospectively with regard to age at presentation, site, size, and physical appearance. RESULTS: Pilomatricomas appear at any age, with peak presentation bimodally in the first and sixth decade. Their most common site is the head and neck. Presentation is of a hard nodule, either deeply subcutaneous and invisible or superficial with possible erosion through the skin surface. This may lead to a false diagnosis of malignancy or of an epidermoid cyst. An association with myotonic dystrophy has been confirmed, as is the rare occurrence of malignant transformation. CONCLUSION: Careful clinical examination and a high index of suspicion results in an accurate diagnosis, appropriate treatment, and the avoidance of unnecessarily extensive surgery.