Pilomatrixoma is a very rare tumor of a benign nature. It is also called calcified necrotizing epithelium of Malerba. About 60% of these tumors are diagnosed in patients after forty years, regardless of gender. But often pilomatrix is observed in infants, especially in girls.
Reason for occurrence
The origin of the pylomatrixoma (Malerba epithelium) has not yet been precisely determined, but at the moment it is officially recognized that it develops from the epidermis, more precisely, from the primitive germ cells that are located there. A careful study of several pilomatrix helped establish that most of the tumor cells correspond to the cells of the hairy cortical substance, which also indicates its origin from this component.
The scientific rationale for the formation of the disease
There are a number of theories that substantiate the formation of Malerba epithelium as follows:
- Of the components of the hair matrix - sections of the hair follicle from which the hair grows.
- In connection with the transformation of the sebaceous gland or its embryonic embryo, which separated during the development of the body.
- Of the components of epidermal cysts. This theory is gaining more and more supporters, since in practice the presence of cysts in the area of tumor formation is often found.
- As a result of skin injury or infection.
The risk of the appearance of Malerba epithelium increases with the influence of several factors on the skin: frequent processes of inflammation of the epidermis, hereditary predisposition, ultraviolet radiation, ionizing radiation.
Symptoms of epithelial
With Malerba epithelium (according to ICD-10, code -D 23.1), the clinical picture and its localization are determined by the type of tumor.
Basal cell epithelioma often appears on the skin of the neck and face. It has a variety of clinical forms, of which most begin with the appearance of a small nodule on the skin. Basal cell epithelioma is a malignant formation, since it is characterized by invasive growth and sprouts subcutaneous tissue, dermis, underlying bone structures and muscle tissue. But at the same time, she is not inclined to form metastases.
Rare forms of epithelium are calcified Malerba epithelium and self-healing epithelium. The latter is characterized by the breakdown of a typical nodule with the occurrence of peptic ulcer. In the future, the size of the ulcer slowly increases, accompanied by scarring of its individual parts. Sometimes it develops into squamous skin cancer.
Calcified Malerba's epithelioma is a benign tumor that appears in children from sebaceous gland cells. It differs in the formation in the skin of the neck, face, shoulder girdle or scalp of a single nodule, very dense, slowly growing and mobile, the value is in the range of 0.5-5 centimeters.
Spinocellular epithelioma develops from cells of the epidermal spiky layer and is malignant with metastasis. Her favorite location is the skin of the genitals and perianal region, the border of her lower lip is red. May be with the formation of an ulcer, plaque, or node. It is characterized by accelerated growth in the depths of tissues and along the periphery.
Cystic adenoid epithelium is observed more often in girls after the puberty period. In most cases, it is represented by painless multiple tumors, whose size is about large peas.
The color of the formations may be yellowish or bluish. Sometimes a whitish shade is found, due to which the components of the epithelium are similar to acne. In some cases, the formation of a single tumor is observed, which in size becomes like a hazelnut. The usual localization is the face and auricles, the shoulder girdle, scalp, limbs and abdomen are less often affected. A slow and benign course is characteristic. Only in isolated situations is degeneration into a basal cell carcinoma observed.
Malignant pilomatrix
Pilomatrixoma in some cases can develop into a malignant stage. This most often occurs after forty years, mainly in men. In this situation, the tumor has a very deep location - in the fiber under the skin or lower area of the dermis. In addition, there are necrotic foci, atypical mitoses and basaloid cells.
Diagnostic tests
Many clinical types of Malerba epithelium (photo of the pathology presented in the article) to some extent complicate the diagnosis. That is why, during the examination, the dermatologist seeks to connect all possible methods: ultrasound of skin formation, dermatoscopy, back sowing of ulcerative disorders. But the final diagnosis with the establishment of the clinical form of the pathology, its malignancy or benignity, makes it possible to determine only the histological analysis of the material obtained by biopsy or removal of skin epithelium.
Differential diagnosis
Differential diagnostics is carried out with psoriasis, lichen planus, systemic lupus erythematosus, seborrheic keratosis, scleroderma, Bowen's disease, Keir’s disease, etc. Cystic adenoid epithelioma needs to be differentiated from xanthelasma, hydradenitis, wart and squamous cell carcinoma.
Treatment of epithelium and its prognosis
Treatment becomes possible only after consulting a doctor and conducting diagnostic measures. The main method of treatment of epithelioma, regardless of its clinical type, is surgical excision of the neoplasm.
If the patient has multiple small tumors, laser removal, cryodestruction, electrocoagulation or curettage can be used. With deep germination and the presence of metastasis, the intervention may be palliative in nature. With a malignant nature, surgical therapy can be combined with general or external chemotherapy, photodynamic treatment, and radiotherapy.
With a benign variety of pathology, complete and timely removal of Malerba epithelium, the prognosis is favorable. Spinocellular and basal cell epitheliomas are prone to relapse after surgery. For their early determination, constant monitoring by a dermato-oncologist is required. The most unfavorable in terms of prognosis is the spinocellulatory form of the disease, especially with the development of metastasis in the tumor.
Epithelioma Malerba in children
Most often, at an early age in children, young people and adolescents, Malerba epithelium develops. This kind of skin lesions was first described by the French doctor A. Malerba together with the doctor of Shenants in 1880 as a benign tumor consisting of partially calcified layers of dead epidermis. A number of researchers associate its occurrence with the development of skin cancer, however, the opposite opinion about the inappropriateness is especially widespread, since this tumor in rare cases acquires a malignant character.
The doctor, upon examination of the patient, will first palpate. After this, the patient will be sent for dermatoscopy and ultrasound examination. A biopsy of the analyzed neoplasm may be necessary. For this, a separate section of the tumor is taken for analysis, however, this type of neoplasm can be treated only by surgery. After excision, the material is sent for histological examination to determine its form - malignant or benign.
With the disease described, it is very difficult to make a correct diagnosis. According to statistical data, only one in fifty patients received a correct diagnosis with Mahlerba's epithelium. In other cases, atheromas, fibromas, cysts of the sebaceous gland and other types of skin tumors are diagnosed.
Surgical therapy of neoplasms is carried out in two ways:
- The traditional method in which they are excised with a scalpel and subsequently a cosmetic suture is applied. The intervention is to eliminate the tumor and the surrounding skin layers.
- Destruction of the neoplasm by a carbon dioxide high-temperature laser. Such an operation lasts only twenty minutes, after which there is no trace left. The operation is performed under local anesthesia. In the form of additional treatment, antitumor agents are prescribed to the patient.
conclusions
The described pathology is a rare occurrence and needs a timely visit to a specialist. The reasons for its appearance have not been established exactly so far. The risk group most often includes children whose relatives had this disease. Treatment is based on surgical removal and histological analysis of the tumor in the future, to determine its nature.