Anetoderma is a benign but uncommon disorder that causes localized areas of flaccid or herniated sac-like skin due to a focal reduction of dermal

elastic tissue Elastic fibers (or yellow fibers) are an essential component of the extracellular matrix composed of bundles of proteins (elastin) which are produced by a number of different cell types including fibroblasts, endothelial, smooth muscle, and air ...

. Anetoderma is subclassified as primary anetoderma, secondary anetoderma,

iatrogenic Iatrogenesis is the causation of a disease, a harmful complication, or other ill effect by any medical activity, including diagnosis, intervention, error, or negligence." Iatrogenic", ''Merriam-Webster.com'', Merriam-Webster, Inc., accessed 27 ...

anetoderma of prematurity, congenital anetoderma, familial anetoderma, and drug-induced anetoderma. Two forms of primary anetoderma have been identified, based on whether an inflammatory response took place prior to the atrophy's appearance, anetoderma of Jadassohn-Pellizzari, in which inflammation occurs before the atrophic lesions appear, and anetoderma of Schweninger-Buzzi, in which inflammation is not present.

Signs and symptoms

Typically pink

macules A skin condition, also known as cutaneous condition, is any medical condition that affects the integumentary system—the organ system that encloses the body and includes skin, nails, and related muscle and glands. The major function of this sys ...

that are round or oval and have a diameter of 0.5 to 1 centimeters grow on the trunk, thighs, and upper arms. They are less frequently found on the neck, face, and other areas. Usually, the soles, palms, and scalp are unaffected. Every macule grows for one or two weeks until it reaches a size of 2-3 centimeters. Larger erythematous plaques can occasionally be seen, and nodules have also been identified as the main lesion. Lesions range widely in number from fewer than five to one hundred or more. The lesions do not change throughout a person's life, and new lesions frequently take years to appear. When the lesions combine, they create sizable atrophic regions that are identical to acquired cutis laxa. They could unite to encircle sizable regions, particularly along the neck and at the base of the limbs.

Causes

Primary anetoderma is an idiopathic development of

atrophic Atrophy is the partial or complete wasting away of a part of the body. Causes of atrophy include mutations (which can destroy the gene to build up the organ), poor nourishment, poor circulation, loss of hormonal support, loss of nerve supply t ...

lesions in skin areas that appeared normal prior to the onset of

atrophy Atrophy is the partial or complete wasting away of a part of the body. Causes of atrophy include mutations (which can destroy the gene to build up the organ), malnutrition, poor nourishment, poor circulatory system, circulation, loss of hormone, ...

. Secondary anetoderma is a skin disease that develops in areas where there has previously been or is currently skin pathology. It has been linked to a variety of conditions, such as

acne Acne ( ), also known as ''acne vulgaris'', is a long-term Cutaneous condition, skin condition that occurs when Keratinocyte, dead skin cells and Sebum, oil from the skin clog hair follicles. Typical features of the condition include comedo, ...

, urticaria pigmentosa,

syphilis Syphilis () is a sexually transmitted infection caused by the bacterium ''Treponema pallidum'' subspecies ''pallidum''. The signs and symptoms depend on the stage it presents: primary, secondary, latent syphilis, latent or tertiary. The prim ...

leprosy Leprosy, also known as Hansen's disease (HD), is a Chronic condition, long-term infection by the bacteria ''Mycobacterium leprae'' or ''Mycobacterium lepromatosis''. Infection can lead to damage of the Peripheral nervous system, nerves, respir ...

, granuloma annulare,

insect bites Many species of arthropods (insects, arachnids, millipedes and centipedes) can bite or sting human beings. These bites and stings generally occur as a defense mechanism or during normal arthropod feeding. While most cases cause self-limited irrita ...

, and

antiphospholipid syndrome Antiphospholipid syndrome, or antiphospholipid antibody syndrome (APS or APLS), is an autoimmune, hypercoagulable state caused by antiphospholipid antibodies. APS can lead to blood clots (thrombosis) in both arteries and veins, pregnancy-relate ...

. Secondary anetoderma has been linked to infectious, inflammatory, and tumorous conditions. Macular depressions or skin outpouchings linked to the loss of dermal elastic tissue that are observed in premature infants are referred to as anetoderma of prematurity. According to reports, the location of monitoring electrodes or leads, such as

ECG Electrocardiography is the process of producing an electrocardiogram (ECG or EKG), a recording of the heart's electrical activity through repeated cardiac cycles. It is an electrogram of the heart which is a graph of voltage versus time of ...

electrodes, may be related to these cutaneous lesions. Case reports have described instances of

congenital A birth defect is an abnormal condition that is present at childbirth, birth, regardless of its cause. Birth defects may result in disability, disabilities that may be physical disability, physical, intellectual disability, intellectual, or dev ...

anetoderma presenting as anetoderma on the trunk of premature infants at birth.

Penicillamine Penicillamine, sold under the brand name of Cuprimine among others, is a medication primarily used for the treatment of Wilson's disease. It is also used for people with kidney stones who have high urine cystine levels, rheumatoid arthritis, ...

administration has been linked to drug-induced anetoderma, especially in Wilson disease patients. Familial anetoderma is rare and is inherited autosomally. The familial form usually appears in the first ten years of life and may show up only as skin manifestations, but it can also be linked to ocular, neurological, and bony abnormalities.

Diagnosis

Anetoderma can be diagnosed clinically. But if the diagnosis proves to be difficult, a

skin biopsy Skin biopsy is a biopsy technique in which a skin lesion is removed to be sent to a pathologist to render a microscopic diagnosis. It is usually done under local anesthetic in a physician's office, and results are often available in 4 to 10 day ...

—ideally a punch biopsy—can be carried out to include the mid dermis. Under a

microscope A microscope () is a laboratory equipment, laboratory instrument used to examine objects that are too small to be seen by the naked eye. Microscopy is the science of investigating small objects and structures using a microscope. Microscopic ...

, the

epidermis The epidermis is the outermost of the three layers that comprise the skin, the inner layers being the dermis and Subcutaneous tissue, hypodermis. The epidermal layer provides a barrier to infection from environmental pathogens and regulates the ...

of anetoderma exhibits a nearly total loss of

elastic fibers Elastic fibers (or yellow fibers) are an essential component of the extracellular matrix composed of bundles of proteins (elastin) which are produced by a number of different cell types including fibroblasts, endothelial, smooth muscle, and air ...

in the reticular and papillary dermal layers. A dermal periadnexal and perivascular infiltrate containing

histiocytes A histiocyte is a vertebrate cell that is part of the mononuclear phagocyte system (also known as the reticuloendothelial system or lymphoreticular system). The mononuclear phagocytic system is part of the organism's immune system. The histiocy ...

and

plasma cells Plasma cells, also called plasma B cells or effector B cells, are white blood cells that originate in the lymphoid organs as B cells and secrete large quantities of proteins called antibodies in response to being presented specific substances c ...

can also be seen in the

histopathology Histopathology (compound of three Greek words: 'tissue', 'suffering', and '' -logia'' 'study of') is the microscopic examination of tissue in order to study the manifestations of disease. Specifically, in clinical medicine, histopatholog ...

of the lesion. In lesional skin, elastophagocytosis and tiny fragmented elastic fibrils can be seen in addition to the histopathology on

electron microscopy An electron microscope is a microscope that uses a beam of electrons as a source of illumination. It uses electron optics that are analogous to the glass lenses of an optical light microscope to control the electron beam, for instance focusing i ...

Desmosine Desmosine is an amino acid found uniquely in elastin, a protein found in connective tissue such as skin, lungs, and Elastic artery, elastic arteries. Desmosine is a component of elastin and cross links with its isomer, isodesmosine, giving elast ...

has been used to quantify the amount of

elastin Elastin is a protein encoded by the ''ELN'' gene in humans and several other animals. Elastin is a key component in the extracellular matrix of gnathostomes (jawed vertebrates). It is highly Elasticity (physics), elastic and present in connective ...

in lesional skin, which is considerably lower in anetoderma, since it is a major

amino acid Amino acids are organic compounds that contain both amino and carboxylic acid functional groups. Although over 500 amino acids exist in nature, by far the most important are the 22 α-amino acids incorporated into proteins. Only these 22 a ...

in elastin.

Classification

There are five subtypes of anetoderma: drug-induced anetoderma, familial anetoderma, prematurity-associated anetoderma, primary (idiopathic) anetoderma, and secondary anetoderma from a previous dermatosis. The Jadassohn-Pellizzari type and the Schweninger-Buzzi type are the historical subtypes of the primary type, which is found in areas of previously normal skin. While the Schweninger-Buzzi type of anetoderma develops in skin that appears normal with no predisposition skin changes, the Jadassohn-Pellizzari type occurs following the occurrence of inflammatory or urticarial lesions. These terms are now obsolete because the two primary types are identical histologically as well as having a similar disease course.

References

External links

UpToDate

DermNet
{{Cutaneous ketatosis, ulcer, atrophy, and necrobiosis Abnormalities of dermal fibrous and elastic tissue