Learn all about adenoma sebaceum images, symptoms, causes, histology and treatment. Sebaceous glands are oil-producing glands present in the dermis of mammalian skin. Sebaceous glands are usually attached to hair follicles and are part of a complex skin-adnexal unit known as the folliculoapocrine (sweat) apparatus. The glands are present over the entire body, with the exception of the palms of the hands and the soles of the feet; they are most abundant on the scalp and central face.
Adenoma sebaceum is one of the diagnostic features of tuberous sclerosis. Histologically, they are angiofibromas that occur over the central part of the face and hence, cause a major cosmetic disfigurement Patients with a family history of the tuberous sclerosis complex are at risk for tuberous sclerosis and adenoma sebaceum, since the mode of inheritance is autosomal dominant. Tuberous sclerosis is estimated to occur in 1 every 6000 live births.
Multiple endocrine neoplasia type 1 is rare and may be a sporadic mutation or inherited as autosomal dominant. There is an equal distribution among sexes. Patients with a family history are at risk for developing this cancer syndrome and multiple facial angiofibromas. Patients with sebaceous adenomas typically experience a gradual onset of small, usually less than 0.5 cm in diameter (2-4 mm), smooth, yellow, sometimes speckled papules with central umbilication on the skin of the face or scalp over a period of several months
Sebaceous adenomas frequently appear on the face or scalp of middle-aged and older individuals, after age 50 years. The mean age at onset is 60 years.
Unfortunately, no specific prenatal laboratory test is available. Genetic counseling should be offered to families with affected members, even though accurate counseling remains difficult because of the variability of gene expression.
What is Adenoma Sebaceum?
Adenoma Sebaceum is a rare skin condition in which the presence of multiple skin tumors is noted in a background of the genetic disorder tuberous sclerosis. Adenoma Sebaceum is considered a misnomer, since the tumor is neither an adenoma (a benign tumor), nor it involves the sebaceous glands (type of sweat glands)
Adenoma Sebaceum Images
Adenoma Sebaceum Symptoms
The signs and symptoms of Adenoma Sebaceum include:
- Presence of multiple small lesions on the face, typically around the nose, chin, and cheeks.
- The lesions form small papules that typically appear like flesh-colored bumps on the skin.
- In some cases, the papules are red or brown in color.
- These skin lesions may bleed if traumatized.
- The skin lesions are usually less than 1 cm in size.
- There is an absence of pain and most cases are asymptomatic.
- The symptoms of the underlying genetic disorder (TS) may be observed.
Adenoma Sebaceum Causes
Sebaceous adenomas form part of the spectrum of the Muir-Torre syndrome. A genetic predisposition exists in some cases of the Muir-Torre syndrome, and this syndrome has been found in association with the so-called cancer family syndrome. Adenoma Sebaceum is known to occur against a backdrop of the inherited genetic disorder tuberous sclerosis. Changes (or mutations) in either the TSC1 or TSC2 gene cause the disease. Because it is genetic, it can be passed from a parent to a child, or inherited.
Adenoma Sebaceum Histology
Adenoma sebaceum slightly raised and well circumscribed dermal lesion composed of collagenous stroma with increased spindled to stellate fibroblasts and increased and dilated blood vessels.
Histological aspects include:
- Collagenous stroma consists of collagenous alteration with concentric orientation around hair follicles or blood vessels or perpendicular orientation to epidermis
- Multinucleated fibroblasts are often present, and scattered dermal melanophages may be present
- Mitotic figures are rare
- Epidermis uninvolved but can appear often flattened or atrophic
- Scattered villus hairs may be present
- Patchy epidermal melanocytic hyperplasia is often found, sometimes slight hyperkeratosis is seen
- Many histologic variants have been described that can make the diagnosis challenging:
- Hypercellular has a marked increased in fibroblasts
- Clear cell proliferation of clear to slightly foamy cells
- Pigmented prominent basal melanocytic hyperplasia and dermal melanophages
- Pleomorphic prominent bizarre stellated pleomorphic cells
- Granular proliferation of cells with granular cytoplasm.
Adenoma Sebaceum Treatment
Adenoma sebaceum treatment has included excision, dermabrasion, cryosurgery, carbon dioxide laser, and argon laser. Copper vapor lasers emit light at 511 nm (green) and 578 nm (yellow), useful for treating pigmented and vascular lesions, respectively. Adenoma sebaceum does not require treatment, since it does not lead to malignant transformation. It usually remains stable or mildly improves over time. However, the multitude of angiofibromas can be cosmetically distressing and disfiguring, which leads many patients to request treatment. Successful treatment of the lesions is difficult because they tend to recur. The multiple angiofibromas found in patients with MEN 1 may be treated similar to adenoma sebaceum.
Surgical excision (shave excision) and mechanical dermabrasion have been reported with good results. The procedure is normally performed under general anesthesia by a plastic surgeon.