Leiner's disease

Last updated
Leiner's disease
Specialty Dermatology

Leiner's disease is a systemic disease, a skin disorder and extends to erythroderma, typically diagnosed in early infancy. [1] Leiner's disease is characterized by a long-lasting seborrhea dermatitis associated with increased likelihood to infection. [2] Other characterizations found on newborns with Leiner's disease are a patch or a large patch of red skin normally on the bottom and spreads to the rest of the body. [2] This disease is also listed as a "rare disease", meaning that a small percent of the population, fewer than 200,000 people in the United States, will have this disorder. [2]

Contents

Symptoms

Symptoms include severe seborrheic dermatitis of the scalp, severe diarrhea, recurrent local and systemic infection, central nervous system problems, and failure to thrive. [3] Other symptoms also include scaling on the trunk and limbs, red patches of skin on parts of the body that bend, fevers, reduced blood protein levels, thick red skin patches, peeling of the skin, itching, corneal ulcers. wasting of the lymph nodes, underdeveloped lymphatics, anemia, wasting, and nervous system deficiency. [2] The disease may then spread to the rest of the epidermis with the appearance of crusty, dry, moist or greasy scaling on the scalp. [1] Scaling could also appear behind the ears, nose or eyebrows, or around the mouth; peeling of the skin may also happen in these areas. [1] If left untreated, the skin infections will cause loss of protein or electrolytes. [1] Leiner's Disease may also be accompanied by a systemic reaction that is most evident in its gastrointestinal manifestation. [4]

It is caused by a deficit of the complement protein, C5; however, case reports have described it in relation to deficits in either C3 or C4. [5]

Cause

The exact cause of Leiner disease remains unknown but biotin deficiency linked to Complement component 5 (a protein ) coded by the C5 gene plays a role.

Diagnosis

This disease is often found during the first two months of an infant's life, breast-fed infants with a higher chance. Male and female infants are affected equally. [1]

Treatment

Hospitalization for the diseased person is suggested because of the controlled environment because it may prevent nutritional deficiencies and skin infections. [1] A decrease in severity of symptoms usually happens after a few weeks when treated [2] redness and scaliness usually do not recur. [1] In 10 percent of cases, the result of uncontrolled infections or severe electrolyte loss may be fatal. [1]

Epidemiology

The cause of this disease is unknown; some infants may have a severe case, others may have immunodeficiency. [1]

See also

Related Research Articles

Dermatitis Inflammation of the skin

Dermatitis is inflammation of the skin, typically characterized by itchiness, redness and a rash. In cases of short duration, there may be small blisters, while in long-term cases the skin may become thickened. The area of skin involved can vary from small to covering the entire body. Dermatitis is often called eczema, and the difference between those terms is not standardized.

Dandruff Skin condition of the scalp

Dandruff is a skin condition that mainly affects the scalp. Symptoms include flaking and sometimes mild itchiness. It can result in social or self-esteem problems. A more severe form of the condition, which includes inflammation of the skin, is known as seborrhoeic dermatitis.

Nail disease Medical condition

A nail disease or onychosis is a disease or deformity of the nail. Although the nail is a structure produced by the skin and is a skin appendage, nail diseases have a distinct classification as they have their own signs and symptoms which may relate to other medical conditions. Some nail conditions that show signs of infection or inflammation may require medical assistance.

Seborrhoeic dermatitis Medical condition

Seborrhoeic dermatitis, sometimes inaccurately referred to as seborrhoea, is a long-term skin disorder. Symptoms include red, scaly, greasy, itchy, and inflamed skin. Areas of the skin rich in oil-producing glands are often affected including the scalp, face, and chest. It can result in social or self-esteem problems. In babies, when the scalp is primarily involved, it is called cradle cap. Dandruff is a milder form of the condition without inflammation.

Lamellar ichthyosis Medical condition

Lamellar ichthyosis, also known as ichthyosis lamellaris and nonbullous congenital ichthyosis, is a rare inherited skin disorder, affecting around 1 in 600,000 people.

Dermatophytes are a common label for a group of fungus of Arthrodermataceae that commonly causes skin disease in animals and humans. Traditionally, these anamorphic mold genera are: Microsporum, Epidermophyton and Trichophyton. There are about 40 species in these three genera. Species capable of reproducing sexually belong in the teleomorphic genus Arthroderma, of the Ascomycota. As of 2019 a total of nine genera are identified and new phylogenetic taxonomy has been proposed.

Tinea capitis Cutaneous fungal infection of the scalp

Tinea capitis is a cutaneous fungal infection (dermatophytosis) of the scalp. The disease is primarily caused by dermatophytes in the genera Trichophyton and Microsporum that invade the hair shaft. The clinical presentation is typically single or multiple patches of hair loss, sometimes with a 'black dot' pattern, that may be accompanied by inflammation, scaling, pustules, and itching. Uncommon in adults, tinea capitis is predominantly seen in pre-pubertal children, more often boys than girls.

Skin disorders are among the most common health problems in dogs, and have many causes. The condition of a dog's skin and coat is also an important indicator of its general health. Skin disorders of dogs vary from acute, self-limiting problems to chronic or long-lasting problems requiring life-time treatment. Skin disorders may be primary or secondary in nature, making diagnosis complicated.

Cryoglobulinemia Medical condition

Cryoglobulinemia is a medical condition in which the blood contains large amounts of pathological cold sensitive antibodies called cryoglobulins – proteins that become insoluble at reduced temperatures. This should be contrasted with cold agglutinins, which cause agglutination of red blood cells.

Cradle cap Medical condition

Cradle cap causes crusty or oily scaly patches on a baby's scalp. The condition is not painful or itchy, but it can cause thick white or yellow scales that are not easy to remove. Cradle cap most commonly begins sometime in the first three months but can occur in later years. Similar symptoms in older children are more likely to be dandruff than cradle cap. The rash is often prominent around the ear, the eyebrows or the eyelids. It may appear in other locations as well, where it is called infantile seborrhoeic dermatitis. Cradle cap is just a special - and more benign - case of this condition. The exact cause of cradle cap is not known. Cradle cap is not spread from person to person (contagious). It is also not caused by poor hygiene. It is not an allergy, and it is not dangerous. Cradle cap often lasts a few months. In some children, the condition can last until age 2 or 3.

Vulvitis is inflammation of the vulva, the external female mammalian genitalia that include the labia majora, labia minora, clitoris, and introitus. It may co-occur as vulvovaginitis with vaginitis, inflammation of the vagina, and may have infectious or non-infectious causes. The warm and moist conditions of the vulva make it easily affected. Vulvitis is prone to occur in any female especially those who have certain sensitivities, infections, allergies, or diseases that make them likely to have vulvitis. Postmenopausal women and prepubescent girls are more prone to be affected by it, as compared to women in their menstruation period. It is so because they have low estrogen levels which makes their vulvar tissue thin and dry. Women having diabetes are also prone to be affected by vulvitis due to the high sugar content in their cells, increasing their vulnerability. Vulvitis is not a disease, it is just an inflammation caused by an infection, allergy or injury. Vulvitis may also be symptom of any sexually transmitted disease or a fungal infection.

Angular cheilitis Cheilitis characterized by inflammation of one or both of the corners of the mouth

Angular cheilitis (AC) is inflammation of one or both corners of the mouth. Often the corners are red with skin breakdown and crusting. It can also be itchy or painful. The condition can last for days to years. Angular cheilitis is a type of cheilitis.

Purpura fulminans is an acute, often fatal, thrombotic disorder which manifests as blood spots, bruising and discolouration of the skin resulting from coagulation in small blood vessels within the skin and rapidly leads to skin necrosis and disseminated intravascular coagulation.

Cat skin disorders are among the most common health problems in cats. Skin disorders in cats have many causes, and many of the common skin disorders that afflict people have a counterpart in cats. The condition of a cat's skin and coat can also be an important indicator of its general health. Skin disorders of cats vary from acute, self-limiting problems to chronic or long-lasting problems requiring life-time treatment. Cat skin disorders may be grouped into categories according to the causes.

Complement deficiency Medical condition

Complement deficiency is an immunodeficiency of absent or suboptimal functioning of one of the complement system proteins. Because of redundancies in the immune system, many complement disorders are never diagnosed. Some studies estimate that less than 10% are identified. Hypocomplementemia may be used more generally to refer to decreased complement levels, while secondary complement disorder means decreased complement levels that are not directly due to a genetic cause but secondary to another medical condition.

Central nervous system disease Disease of the brain or spinal cord

Central nervous system diseases, also known as central nervous system disorders, are a group of neurological disorders that affect the structure or function of the brain or spinal cord, which collectively form the central nervous system (CNS).

Madarosis is a condition that results in the loss of eyelashes, and sometimes eyebrows. The term "madarosis" is derived from the ancient Greek "madaros", meaning "bald". It originally was a disease of only losing eyelashes but it currently is the loss of both eyelashes and eyebrows. Eyebrows and eyelashes are both important in the prevention of bacteria and other foreign objects from entering the eye. A majority of patients with madarosis have leprosy, and it was reported that 76% of patients with varying types of leprosy had madarosis.

Acquired C1 esterase inhibitor deficiency Medical condition

Acquired C1 esterase inhibitor deficiency, also referred to as acquired angioedema (AAE), is a rare medical condition that presents as body swelling that can be life threatening and manifests due to another underlying medical condition. The acquired form of this disease can occur from a deficiency or abnormal function of the enzyme C1 esterase inhibitor (C1-INH). This disease is also abbreviated in medical literature as C1INH-AAE. This form of angioedema is considered acquired due to its association with lymphatic malignancies, immune system disorders, or infections. Typically, acquired angioedema presents later in adulthood, in contrast to hereditary angioedema which usually presents from early childhood and with similar symptoms.

Ichthyosis prematurity syndrome (IPS) is a dermatological disease with known genetic causes. This syndrome is a rare subcategory of autosomal recessive congenital ichthyosis (ARCI). It is associated with complications in the mid-trimester of a pregnancy leading to premature births. Although most prevalent in individuals of Scandinavian origin, there have also been scattered cases in people of Japanese, Italian and Indian ethnicity. This disorder is also referred to as ichthyosis congenital type IV.

References

  1. 1 2 3 4 5 6 7 8 9 Thoene, MD; Jess, G (1995). "Leiner's Disease" . Physicians' guide to rare diseases (2nd ed.).[ page needed ]
  2. 1 2 3 4 5 http://www.rightdiagnosis.com/l/leiner_disease/intro.htm%5B%5D
  3. http://www.whonamedit.com/synd.cfm/2369.html%5B%5D
  4. Sauer, Gordon C.; Hall, John C. (1996). "Leiner's Disease". Manual of Skin Diseases (7th ed.).[ page needed ]
  5. Complement Deficiencies at eMedicine