The Pigmentary Disorders

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WE TALK ABOUT PIGMENTARY DISORDERS

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6 The Pigmentary Disorders O U T L I N E 6.1 Hypomelanosis 6.1.1 Vitiligo 6.1.1.1 Generalized Vitiligo at an Elderly Woman Patient 6.1.1.2 Vitiligo on the Back 6.1.1.3 Vitiligo on the Lower Limbs 6.1.1.4 Generalized Vitiligo 6.1.1.5 Vitiligo on the Lateral Face 6.1.1.6 Vitiligo on the Hands and Arms 6.1.1.7 Vitiligo on the Elbow and Arm 6.1.1.8 Vitiligo on the Ear 6.1.1.9 Vitiligo in the Young Female Patient 6.1.1.10 Vitiligo on the Nipples 6.1.1.11 Vitiligo on the Hands 6.1.1.12 Vitiligo on the Knees 6.1.1.13 Vitiligo on the Elbows 6.1.1.14 Vitiligo on the Knees and Elbows 6.1.1.15 Vitiligo 6.1.2 Albinism

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6.2 Hypermelanosis 367 6.2.1 Hyperpigmentation Spots at the Level of the Gums in Addison Disease 368 Hyperpigmentation Spots at the Level of the Gums 369 6.2.2 Hyperpigmentation at the Level of the Buccal Mucosa 372 6.2.3 Hyperpigmentation of the Nipple and Breast Areola 373 6.2.4 Hyperpigmentation of the Knees 373 6.2.5 Hyperpigmentation of the Elbow 374 6.2.6 Hyperpigmentation of the Umbilicus 374 6.2.7 Hyperpigmentation of the Palmsd The Palmary Crests 375 6.2.8 Chloasma (Melasma) 377 6.2.9 Brown Spot on Right and Left Cheeks 378 6.2.10 Hyperpigmentation of the Skin and Nail Impregnation With Nicotine Smoking Stigma 379 General Medical Semiology Guide Part I https://doi.org/10.1016/B978-0-12-819637-3.00006-1

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6.2.11 Impregnation With NicotineeSmoking Stigma Impregnation With NicotinedSmoking Stigma 6.2.12 The Pigmentation Dermatitis of Stasis PigmentationdDermatitis of Stasis PigmentationdDermatitis of Stasis 6.2.13 Nevus Pigmentation Nevus Pigmentation 6.2.14 Acanthosis Nigricans Paraneoplastic Lesion Gastric Carcinoma Acanthosis Nigricans Acanthosis Nigricans 6.2.15 Cafe´ Au Lait Spots Cafe´ Au Lait Spots Cafe´ Au Lait Spots Cafe´ Au Lait Spots Cafe´ Au Lait Spots Cafe´ Au Lait Spots 6.2.16 Nevus Pigmentation Transformation to Melanoma Nevus Pigmentation Transformation to Melanoma 6.2.17 Nevus Pigmentation Under The Right Knee 6.2.18 MelanomadHair From Pigmentation Area MelanomadHair From Pigmentation Area 6.2.19 Melanoma Lateral Incidence 6.2.20 Close Image 6.2.21 Melanoma 6.2.22 MelanomadClose Image Halo, Irregularities Different Brown Colors 6.2.23 Nevus Pigmentation Change to Melanoma Irregularities as Mura 6.2.24 Nevus Pigmentation Change to Melanoma Nevus Pigmentation Change to Melanoma Melanoma 6.2.25 Many Pigmented Nevi on the Posterior Chest 6.2.26 Pigmented NevidClose Image

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6.1 Hypomelanosis

6.2.27 Pigmented Nevi on the Anterior Chest 6.2.28 Many Pigmented Nevi (Lentigo) 6.2.29 Lateral Incidence Posterior Incidence Right Lateral Incidence Anterior Incidence 6.2.30 Under the Breasts 6.2.31 Close Image of Nevus

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6.2.32 Close Image of Nevi Close Image of Nevi 6.2.33 Pigmentation Nevi Pigmentation Nevi 6.2.34 Close Image of Pigmentation Nevi Close Image of Pigmentation Nevi Pigmentation Nevi 6.2.35 Left Lateral Incidence Pigmentation Nevi

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6.1 Hypomelanosis 6.1.1 Vitiligo Vitiligo is a skin pigmentation disorder characterized by white areas that are depigmented and of different sizes, with irregular contour, and can be present in any region of the body. Vitiligo appears symmetric at the hands and legs. It is very sensitive to the ultraviolet rays of sunlight, appearing slightly with burns. We can see a few images with vitiligo in the photos below. 6.1.1.1 Generalized Vitiligo at an Elderly Woman Patient

340 6.1.1.2 Vitiligo on the Back

6. The Pigmentary Disorders

6.1 Hypomelanosis

6.1.1.3 Vitiligo on the Lower Limbs

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342 6.1.1.4 Generalized Vitiligo

6. The Pigmentary Disorders

6.1 Hypomelanosis

6.1.1.5 Vitiligo on the Lateral Face

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344 Vitiligo on the Lower Limbs

6. The Pigmentary Disorders

6.1 Hypomelanosis

6.1.1.6 Vitiligo on the Hands and Arms

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346 6.1.1.7 Vitiligo on the Elbow and Arm

6. The Pigmentary Disorders

6.1 Hypomelanosis

6.1.1.8 Vitiligo on the Ear

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6.1.1.9 Vitiligo in the Young Female Patient

6.1 Hypomelanosis

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6.1 Hypomelanosis

6.1.1.10 Vitiligo on the Nipples

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352 6.1.1.11 Vitiligo on the Hands

6. The Pigmentary Disorders

6.1 Hypomelanosis

Vitiligo on the Hands

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6. The Pigmentary Disorders

6.1 Hypomelanosis

Vitiligo on the Back

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356 6.1.1.12 Vitiligo on the Knees

6. The Pigmentary Disorders

6.1 Hypomelanosis

Vitiligo on the Knees

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358 6.1.1.13 Vitiligo on the Elbows

6. The Pigmentary Disorders

6.1 Hypomelanosis

Vitiligo on the Elbows

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360 Vitiligo on the Elbows

6. The Pigmentary Disorders

6.1 Hypomelanosis

6.1.1.14 Vitiligo on the Knees and Elbows

Vitiligo on the Hands

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362 6.1.1.15 Vitiligo

6. The Pigmentary Disorders

6.1 Hypomelanosis

Vitiligo

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364 Vitiligo on the Hands

6. The Pigmentary Disorders

6.1 Hypomelanosis

Vitiligo on the Hands

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6.2 Hypermelanosis

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Vitiligo on the Lower Limbs

6.1.2 Albinism Albinism is a rare genetic disease in which pigment is missing from the skin. Because of this, the coloration of the skin, hair, and irises is white and very sensitive to UV radiation with a risk for visual disturbance and skin carcinoma. For this reason, these categories of persons need to avoid sun exposure to prevent carcinoma of the skin and eyes. In this situation, pigment is missing completely from the skin, hair, and irises.

6.2 Hypermelanosis It is the increase of skin pigmentation in certain areas. The most important endocrinal disease where this hyperpigmentation appears is Addison diseasedchronic suprarenal insufficiency. In Addison disease, hyperpigmentation of the gums, palmar ridges, and face appears.

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6. The Pigmentary Disorders

6.2.1 Hyperpigmentation Spots at the Level of the Gums in Addison Disease

6.2 Hypermelanosis

Hyperpigmentation Spots at the Level of the Gums

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6.2 Hypermelanosis

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6. The Pigmentary Disorders

6.2.2 Hyperpigmentation at the Level of the Buccal Mucosa

Another important sign in Addison disease is the presence of hyperpigmentation at the level of mucosa inside the cheeks because of deposits of melanin, as in the image above. We can clearly observe a brown spot with irregular border inside the mouth on the mucosa of the left cheeks. This sign is specific in this endocrinal diseasesdchronic suprarenal insufficiencydAddison disease. Always, if we suspect this disease, we must check the inside portion of the patient’s mouth, with open mouth, to see if this important sign as a mark of diagnosis is present.

6.2 Hypermelanosis

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6.2.3 Hyperpigmentation of the Nipple and Breast Areola

Another area where hyperpigmentation appears in Addison disease is in the nipples and breast areola. Of course, these areas are normally in a condition of hyperpigmentation, but in Addison disease the brown color of the nipple and mammary areola present increased hyperpigmentation because of the deposits of melanin at this level. In the image above, we can see the breast of a woman with Addison disease, and we can observe these signs mentioned before: hyperpigmentation of the nipple and of the breast areola.

6.2.4 Hyperpigmentation of the Knees

Another important region where hyperpigmentation appears in Addison disease is in areas that are mechanically overstretched. In the image above, we can observe the knees of a woman with Addison disease, and it is possible to observe the light hyperpigmentation of the knees from being mechanically overstretched, and this is another important typical sign in this endocrinal disease, Addison diseasedchronic suprarenal insufficiency. Sometimes this sign

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6. The Pigmentary Disorders

appears in the first instance as a persistent tanning in the summer season. In addition, this incipient sign must attract attention about the Addison disease.

6.2.5 Hyperpigmentation of the Elbow

Another area that is mechanically overstretched is the elbow. In the image above, we can see hyperpigmentation of the elbow of a patient with Addison disease. The first instance appeared as a persistent tanning in the summer season. In addition, this incipient sign must attract attention about Addison disease. In conclusion, two important areas are mechanically overstretched: the knees and elbows. We must always check patients with suspicion of Addison disease in these areas to see whether there is hyperpigmentation, because the signs are very specific for this disease because of the increased secretion of melanin-stimulating hormone from the pituitary gland.

6.2.6 Hyperpigmentation of the Umbilicus

6.2 Hypermelanosis

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In Addison disease, hyperpigmentation can appear in the umbilicus and in the line under the umbilicus, as shown in the image above. The first instance appeared as a persistent tanning in the summer season and then remained permanently. Other areas where hyperpigmentation is present are the palms of the patient and the palmary crests, as shown in the images below.

6.2.7 Hyperpigmentation of the Palmsd The Palmary Crests

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6. The Pigmentary Disorders

6.2 Hypermelanosis

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Therfore, the other important areas where we must check our patient for Addison disease are the palms and the palmary crests. In the image above, we can observe hyperpigmentation of the palmary crests. We must examine our patient in natural light. Sometimes it is difficult to observe cyanosis and jaundice in the condition of hyperpigmentation, but in this situation, we must check the scleras and the tongue to be sure.

6.2.8 Chloasma (Melasma)

Chloasma (melasma) or pregnancy mask appears in pregnant women. The appearance of the face looks like the image above, with hyperpigmentation spots with irregular borders appearing on the forehead, cheeks, and nose as well as around the mouth. The woman in the image above had been pregnant for 6 months at the time the photo was taken.

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6. The Pigmentary Disorders

This face with hyperpigmentation spots may also be present in women who use oral contraceptive pills because they contain progestogen hormone. These brown spots are passengers that disappear after the cause is not present; after delivery, these brown spots will disappear.

In this close image of the previous woman, we can see the brown spots on both cheeks with irregular borders clearly. She is 6 months pregnant, but the “gravid mask” appears from the fourth month of pregnancy. After delivery, the brown spots on the face will disappear, so this is a transitory color change of the skin. The next two images are close images of the cheeks of this patient, and in this condition we can observe the hyperpigmentation areas of the cheeks better. We will see clearly that the brown spots have irregular borders, as a map, and are located exactly in the middle of the cheeks. These signs are typical for “gravid mask.”

6.2.9 Brown Spot on Right and Left Cheeks

6.2 Hypermelanosis

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6.2.10 Hyperpigmentation of the Skin and Nail Impregnation With Nicotine Smoking Stigma

If a person smokes too much (many cigarettes/day, or say, one pack/day for many years), skin and nail impregnation with nicotine will occur. This impregnation with nicotine appears as a brown color as in the image above.

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6.2.11 Impregnation With NicotineeSmoking Stigma

This patient has smoked for 15 years (one pack/day), so he is a heavy smoker, with chronic tobacco bronchitis, and if we look at the skin of his fingers and nails, we can see the brown color of the skin and nails at the third and second fingers of the left hand.

6.2 Hypermelanosis

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Impregnation With NicotinedSmoking Stigma

This is the close image of the previous patient, and we can see the brown color of the nail of the second finger and also the hyperpigmentation color of the skin of the third finger; because of impregnation with nicotine, the sign of chronic smoking appears.

6.2.12 The Pigmentation Dermatitis of Stasis

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PigmentationdDermatitis of Stasis

6. The Pigmentary Disorders

6.2 Hypermelanosis

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PigmentationdDermatitis of Stasis

Staphylococcal dermatitis due to impregnation with hemosiderin

6.2 Hypermelanosis

6.2.13 Nevus Pigmentation

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6. The Pigmentary Disorders

6.2 Hypermelanosis

6.2.14 Acanthosis Nigricans Paraneoplastic Lesion Gastric Carcinoma

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6. The Pigmentary Disorders

6.2 Hypermelanosis

Acanthosis Nigricans

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6. The Pigmentary Disorders

6.2.15 Cafe´ Au Lait Spots

Cafe´ au lait spots and nodules in von Recklinghausen’s disease

6.2 Hypermelanosis

Cafe´ Au Lait Spots

Cafe´ au lait spot and nodules in von Recklinghausen’s disease

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Cafe´ Au Lait Spots

Cafe´ au lait spots and nodules in von Recklinghausen’s disease

6.2 Hypermelanosis

Cafe´ Au Lait Spots

Cafe´ au lait spots and many nodules in von Recklinghausen’s disease

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Cafe´ Au Lait Spots

Cafe´ au lait spots and many nodules in von Recklinghausen’s disease

6.2 Hypermelanosis

Cafe´ Au Lait Spots

Cafe´ au lait spots and many nodules in von Recklinghausen’s disease

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6.2.16 Nevus Pigmentation Transformation to Melanoma

6.2 Hypermelanosis

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Nevus Pigmentation Transformation to Melanoma

6.2 Hypermelanosis

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6.2.17 Nevus Pigmentation Under The Right Knee

Hyperpigmentation spot under the right knee, oval shape, dimensions 2 x 4 cm, with irregular border like a map, elevated from the plane of the skin and without hair from the brown spotdpigmentation nevus. It is a benign lesion. The patient must avoid trauma and sun exposure because these are risk factors for transformation into melanoma.

6.2.18 MelanomadHair From Pigmentation Area

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MelanomadHair From Pigmentation Area

6. The Pigmentary Disorders

6.2 Hypermelanosis

6.2.19 Melanoma Lateral Incidence

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6.2.20 Close Image

6. The Pigmentary Disorders

6.2 Hypermelanosis

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6.2.21 Melanoma

6. The Pigmentary Disorders

6.2 Hypermelanosis

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6.2.22 MelanomadClose Image Halo, Irregularities Different Brown Colors

In this close image, we can see better, indicated with the red star, the presence of a halo, light brown, compared with the color of the center dark brown or really black color. We can also observe a few elevation areas as light-brown and black nodules as well. These changes in morphology and color of the lesion with this specific halo are typical signs for melanoma. This is very dangerous and aggressive and must be surgically removed as soon as possible because it can develop metastasis at distance. Sun exposure at UV light is contraindicating because it accelerates the evolution.

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6.2.23 Nevus Pigmentation Change to Melanoma Irregularities as Mura

6.2 Hypermelanosis

6.2.24 Nevus Pigmentation Change to Melanoma

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6. The Pigmentary Disorders

6.2 Hypermelanosis

Melanoma

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6. The Pigmentary Disorders

6.2.25 Many Pigmented Nevi on the Posterior Chest

6.2 Hypermelanosis

6.2.26 Pigmented NevidClose Image

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6.2.27 Pigmented Nevi on the Anterior Chest

6.2 Hypermelanosis

6.2.28 Many Pigmented Nevi (Lentigo)

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6.2.29 Lateral Incidence

6. The Pigmentary Disorders

6.2 Hypermelanosis

Posterior Incidence

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6. The Pigmentary Disorders

6.2 Hypermelanosis

Anterior Incidence

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6.2.30 Under the Breasts

6. The Pigmentary Disorders

6.2 Hypermelanosis

6.2.31 Close Image of Nevus

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6.2.32 Close Image of Nevi

6. The Pigmentary Disorders

6.2 Hypermelanosis

Close Image of Nevi

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6.2.33 Pigmentation Nevi

6. The Pigmentary Disorders

6.2 Hypermelanosis

Pigmentation Nevi

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6.2 Hypermelanosis

6.2.34 Close Image of Pigmentation Nevi

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6.2 Hypermelanosis

Pigmentation Nevi

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6.2.35 Left Lateral Incidence Pigmentation Nevi

Hemochromatosis is characterized by deposition of hemosiderin and melanin in the skin. Cutaneous porphyria tarda is a hereditary disease of porphyry metabolism caused by hyperpigmentation and occurs on the hands, face, and neck.