Diseases of Pigment

Solar Lentigo

Solar lentigo is a circumscribed 1- to 3-cm brown macule resulting from a localized proliferation of melanocytes due to acute or chronic exposure to sunlight. Age of Onset Usually 40 years but may be 30 years in sunny climates and in susceptible persons. Race Most common in Caucasians but seen also in Asians. Multiple dark-brown macules on the forehead occurred after a sunburn. They are all of about the same size and sharply marginated that is characteristic of sunburninduced solar lentigines.

Depigmented Patch of Skin

Depigmented patch of skin on right mandible.

Vitiligo: Punch Grafts

Vitiligo (pronounced vit-ill-EYE-go) is a pigmentation disorder in which melanocytes (the cells that make pigment) in the skin are destroyed. As a result, white patches appear on the skin in different parts of the body. Similar patches also appear on both the mucous membranes (tissues that line the inside of the mouth and nose) and the retina (inner layer of the eyeball). The hair that grows on areas affected by vitiligo sometimes turns white. Vitiligo is seen here on a patient with a depigmented patch of skin on right mandible. Significant improvement is shown after multiple 1-mm punch grafts, as seen on the right.

Vitiligo: Hair

Vitiligo (pronounced vit-ill-EYE-go) is a pigmentation disorder in which melanocytes (the cells that make pigment) in the skin are destroyed. As a result, white patches appear on the skin in different parts of the body. Similar patches also appear on both the mucous membranes (tissues that line the inside of the mouth and nose) and the retina (inner layer of the eyeball). The hair that grows on areas affected by vitiligo sometimes turns white. The young patient in the image above has a white lock of hair as a result of vitiligo.

Vitiligo on Back

Vitiligo. These are more illustrations of fairly extensive cases of vitiligo. The condition tends to progress and may even become universal. A variety of treatment modalities are commonly employed, with varying degrees of success. The patient and family should be made aware of the sophisticated cover-up cosmetics that are now available. The use of broad-spectrum sunscreen lotions during the summer months minimizes the con- trast between normal and involved skin. For some patients, the application of topical corticosteroids alone or with brief natural sunlight exposure early in the course of the disease may induce repigmentation. Narrowband UUB is also an effective treatment. Varying combinations of topical or oral psoralens and ultraviolet A light (PUVA) are used in the treatment of vitiligo.

Vitiligo (Neck)

Vitiligo: These are more illustrations of fairly extensive cases of vitiligo. The condition tends to progress and may even become universal. A variety of treatment modalities are commonly employed, with varying degrees of success. The patient and family should be made aware of the sophisticated cover-up cosmetics that are now available. The use of broad-spectrum sunscreen lotions during the summer months minimizes the con- trast between normal and involved skin. For some patients, the application of topical corticosteroids alone or with brief natural sunlight exposure early in the course of the disease may induce repigmentation. Narrowband UUB is also an effective treatment. Varying combinations of topical or oral psoralens and ultraviolet A light (PUVA) are used in the treatment of vitiligo.

Peutz-Jeghers Syndrome

Scattered dark brown macules appear on the lips and buccal mucosa of a child with Peutz-Jeghers syndrome. The pigmented macules on the lips may fade with time, but the intraoral pigmentation persists for life. Peutz-Jeghers syndrome is a cancer genetic disorder characterized by freckle-like spots on the lips, mouth and fingers and polyps in the intestines. Patients are at increased risk for developing cancer of the esophagus, stomach, colon, rectum, breast, ovary, testis and pancreas. The polyps may occur in any part of the gastrointestinal tract, but polyps in the jejunum (the middle portion of the small intestine) are a consistent feature of the disease. Intussusception (telescoping of the bowel) and intestinal bleeding are also common symptoms. Females are at risk for sex cord tumors with annular tubules (SCTAT), a benign tumor of the ovary. Males may develop Sertoli cell tumors of the testes, which secrete estrogen and can lead to gynecomastia (male breast enlargement). Females can also have a malignancy of the cervix called adenoma malignum. Peutz-Jeghers syndrome is inherited in an autosomal dominant manner and is due to mutation in a gene on chromosome 19p13.3 called STK11 (serine/threonine-protein kinase 11) that appears to function as a tumor suppressor gene. Half of patients have an affected parent from whom they inherited an STK11 mutation and the other half have a new mutation in the STK11 gene. The risk of cancer in the Peutz-Jeghers syndrome is very high. Among 210 patients with the symdrome, the risk of developing noncutaneous cancer between the ages of 15 to 64 was 93%. The highest cumulative risks were for breast cancer (54%), colon cancer (39%), pancreatic cancer (36%), stomach cancer (29%), and ovarian cancer (21%). In 1921 Peutz was the first to recognize the familial association of gastrointestinal polyps and spots. A review by Jeghers et al. in The New England Journal of Medicine in 1949 put the polyps-and-spots syndrome "on the map."

Becker's Nevus

Becker’s nevus is a sharply demarcated tan to brown patch or slightly raised verrucous plaque that most commonly appears on the shoulder, chest, or upper back. It typically presents unilaterally and is frequently associated with overlying hypertrichosis. It is a benign hamartoma.

Nevus of Ota Treatment

Nevus of Ota, also known as nevus fuscoceruleus opthalmomaxillaris, represents a benign partially confluent macular brown-blue pigmentation of the skin and mucous membranes in the distribution of the first and second branches of the trigeminal nerve. It may be unilateral or bilateral. The ipsilateral sclera is frequently involved. The photo above shows significant clearance after serial treatments with Q-switched ruby laser on Nevus of Ota.

Nevus of Ota

Nevus of Ota, also known as nevus fuscoceruleus opthalmomaxillaris, represents a benign partially confluent macular brown-blue pigmentation of the skin and mucous membranes in the distribution of the first and second branches of the trigeminal nerve. It may be unilateral or bilateral. The ipsilateral sclera is frequently involved. Shown here is a patient with Nevus of Ota prior to treatment with Q-switched ruby laser.

Nevus Spilus

Nevus spilus. Large tan macule dotted with numerous superimposed small dark nevi.

Nevus Depigmentosus

Nevus depigmentosus. Depigmented area on the cheek of an infant present since birth.

Nevus Depigmentosus (Achromicus)

Nevus depigmentosus (achromicus). These are localized areas of hypopigmentation that are usually present at birth. The lesions may be irregular in size and shape and occasionally follow a linear or segmental pattern. Electron microscopic study of these areas suggests that melanosomes are not being transferred from melanocytes into surrounding keratinocytes. There are no associated abnormalities.

Mongolian Spots

Mongolian spots are very common in any part of the body of dark-skinned babies. They are flat, gray-blue in color (almost looking like a bruise), and can be small or large. They are caused by some pigment that didn't make it to the top layer when baby's skin was being formed. They are harmless and usually fade away by school age. In most cases, no treatment is needed for the birthmarks themselves. When birthmarks do require treatment, however, that treatment varies based on the kind of birthmark and its related conditions.

Multiple Lentigines Syndrome (Face)

Multiple lentigines syndrome is also known as LEOPARD syndrome, a genetic syndrome transmitted in an autosomal dominant manner that is named for its characteristic features: L -- lentigines (dark freckles) on the head and neck E -- electrocardiogram (EKG) abnormalities O -- ocular hyperteleorism (wide-spacing of the eyes) P -- pulmonary stenosis A -- abnormal genitalia R -- retardation of growth D -- deafness (sensorineural type) The LEOPARD syndrome is caused by mutations in the gene for protein-tyrosine phosphatase, nonreceptor-type, 11 (PTPN11). The LEOPARD syndrome is therefore allelic with Noonan syndrome which is also the result of mutations in PTPN11. LEOPARD syndrome is the most common name for the disorder. It is also known as multiple lentigines syndrome, Gorlin syndrome II, cardio-cutaneous syndrome, lentiginosis profusa syndrome, progressive cardiomyopathic lentiginosis.

Multiple Lentigines Syndrome (Back)

Multiple lentigines syndrome is also known as LEOPARD syndrome, a genetic syndrome transmitted in an autosomal dominant manner that is named for its characteristic features: L -- lentigines (dark freckles) on the head and neck E -- electrocardiogram (EKG) abnormalities O -- ocular hyperteleorism (wide-spacing of the eyes) P -- pulmonary stenosis A -- abnormal genitalia R -- retardation of growth D -- deafness (sensorineural type) The LEOPARD syndrome is caused by mutations in the gene for protein-tyrosine phosphatase, nonreceptor-type, 11 (PTPN11). The LEOPARD syndrome is therefore allelic with Noonan syndrome which is also the result of mutations in PTPN11. LEOPARD syndrome is the most common name for the disorder. It is also known as multiple lentigines syndrome, Gorlin syndrome II, cardio-cutaneous syndrome, lentiginosis profusa syndrome, progressive cardiomyopathic lentiginosis.

Lentigines

Lentigines: When there is one lentigo, there are usually more lentigines. A lentigo is a type of freckle that is a small tan, brown, or black spot which tends to be darker than the usual (ephelis-type) freckle and which do not fade in the winter. This kind of spot is referred to as lentigo simplex. Although lentigines may be part of a genetic disorder, for the most part they are just isolated and unimportant spots. (The genetic disorder in which lentigines occur is called the LEOPARD syndrome.) Lentigo is the Latin word for lentil. A lentigo looks like a lentil bean. The plural is lentigines.

Lentigo

Lentigo: A type of freckle that is a small tan, brown, or black spot which tends to be darker than the usual (ephelis-type) freckle and which do not fade in the winter. This kind of spot is referred to as lentigo simplex. Although lentigines may be part of a genetic disorder, for the most part they are just isolated and unimportant spots. (The genetic disorder in which lentigines occur is called the LEOPARD syndrome.) Lentigo is the Latin word for lentil. A lentigo looks like a lentil bean. The plural is lentigines.

Ephelides

Ephelides: The plural of ephelis, a type of freckle. Ephelis and ephelides are among the many medical terms that are rarely, if ever, encountered outside of medicine.

Freckles

Freckle: A flat circular spot on the skin about the size of the head of a nail that develops after repeated exposure to sunlight, particularly in someone of fair complexion. Freckles may be red, yellow, tan, light-brown, brown, or black. They are always darker than the skin around them since they are due to deposits of the dark melanin, a dark pigment. There are two basic types of freckles -- ephelides and lentigines. Ephelides (singular: ephelis) are flat red or light-brown spots that typically appear during the sunny months and fade in the winter. Lentigines (singular: lentigo) are small tan, brown, or black spots which tend to be darker than an ephelis-type freckle and which do not fade in the winter. The sun is not the only factor that induces freckles. Heredity also influences freckling, as witnessed by the striking similarity in the total number of freckles on identical twins. Such similarities are considerably less marked in fraternal twins. A gene for freckles has been mapped to chromosome 4q32-q34. Freckles are harmless. They may sometimes be confused with more serious skin problems. Conversely, more serious problems such as skin cancer may at times be passed over as a mere freckle. Anyone who has one or more pigmented spots of which they are not certain should be seen by a physician (or dermatologist). Effective treatments are available to lighten or eliminate those freckles whose appearance bothers their owners.

Jaundice

Often, physiologic jaundice -- the type seen in most newborns -- does not require treatment. It will typically disappear in a few days. Doctors will test the baby's bilirubin levels during that time to make sure it has not gotten worse. In rare cases, bilirubin levels become too high very quickly. Special blood tests will help determine the cause -- possibly an infection, a liver problem at birth, a blood problem, or a problem related to breastfeeding.

Cafe au Lait Macule

Cafe au lait macule (CALM). Large brown macule with irregular "coast of Maine" jagged borders in a girl with Albright syndrome.

Cafe au Lait

Café au lait spot: A flat spot on the skin that is the color of coffee with milk (café au lait) in persons with light skin, or a darker appearance (café noir) than the surrounding skin in persons with dark skin. About 10% of the general population have cafe au lait spots, and they can be removed with a Yag laser. Café au lait spots are in themselves harmless, but in some cases they may be a sign of neurofibromatosis. The presence of 6 or more café au lait spots each of which is 1.5 centimeters or more in diameter is diagnostic of neurofibromatosis. Conversely, most people with neurofibromatosis who are at least 5 years old have 6 or more cafe au lait spots 1.5 centimeters or more in diameter.

Melasma

Melasma:Pigmentation of the face, most commonly on the malar area (the upper cheek), bridge of nose, forehead, and upper lip, that occurs in half of women during pregnancy. Birth control pills can also cause melasma. However, hormone therapy after menopause does not cause the condition.

Amiodarone

Drug-induced pigmentation: amiodarone. A striking slate-gray pigmentation in a photodistribution of the face. The blue color (ceruloderma) is due to the deposition of melanin and lipofuscin contained in macrophages and endothelial cells in the dermis. The pigmentation is reversible, but it may take up to a year or more to complete resolution. In this patient it took 33 months for the ceruloderma to disappear.

Addison's Disease

Addison's disease. Hyperpigmentation representing an accentuation of normal pigmentation of the hand of a patient with Addison's disease (left). For comparison, the hand of a normal individual, matched for ethnic pigmentation, is shown on the right.

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