STUDY - Technical - New Dacian's Medicine
Skin
Manifestations of Internal Diseases (4)
Translation Draft
Look, it's been another month (seventh of postings)... So what if... We're just getting started... And, we continue with hyperpigmentation...
The causes of hyperpigmentation are represented by 1. primary skin conditions with localized Forms A. (a. epidermal change with i. seborrheic keratosis, ii. acanthosis nigricans/ obesity and iii. actinic pigmented keratosis, b. proliferation of melanocytes with i. lentigo, ii. nev, iii. melanoma, c. increased pigment production with i. efelide/ freckles, ii. spots "milk coffee") and B. localized and diffuse (induced medicinal), 2. systemic diseases with localized Forms A. (a. epidermal modification with i. seborrheic keratoses/ Lesser-Trerate sign and ii. acanthosis nigricans/ endocrine disorders, paraneoplastic, b. proliferation of melanocytes with i. lentigine/ Peutz-Jeghers syndrome, LEOPARD syndrome, xeroderma pigmentosum and ii. nevi/ Carney complex/ LAMB and NAME syndromes, including lentigine, c. increased pigment production with i. spots "milk coffee"/ neurofibromatosis, Albright syndrome and ii. urticaria pigmentosa, d. dermal pigmentation with i. incontinentia pigments and ii. congenital diskeratosis) and B. diffuse (with 1. endocrinopathies represented by a. Addison's disease, b. Nelson syndrome and c. ectopic ACTH syndrome, 2. metabolic with late skin porphyria, b. homochromatosis, c. deficiencies in vitamin B12, folates, d. pelagra and e. malabsorption , Whipple's disease, 3. secondary melanosis to metastatic melanoma, 4. autoimmune with a. biliary cirrhosis, b. scleroderma, c. POEMS syndrome and d. eosinophilia-myalgia syndrome and 5. medicines and metals).
Hyperpigmentation disorders are divided into two localized and diffuse groups. Localized forms are due to epidermal change, melanocytic proliferation or an increase in pigment production. Forms of hyperpigmentation (localized primary skin disorders) are represented by: 1. seborrheic keratosis (having clinical characteristics represented by brownish to black papule, verucoous and/ or fatty surface, "applied" appearance, arranged on the trunk, histopathologically represented by epidermal hyperplasia, with treatment represented by maintenance, and if irritated or bleeding should be removed), 2. acanthosis nigricans (having clinical characteristics represented velvety surfaces present on the neck, armpits, groin, sometimes on the back of the hands and at the corners of the mouth, histopathologically highlighting epidermal folds with increased pigment in the basal layer, with treatment represented by administration of topical tretinoin, alpha-hydroxyacids or keratolitics, including weight loss if associated with obesity), 3. pigmented actinic keratosis (having clinical characteristics represented by a brown 3-10 mm macula, rough scuba present in solar-exposed regions, in particular the face and back of the hands, histopathologically finding keratocyte dysplasia in the lower third of the epidermis and pigment increased in the epidermis, requiring treatment represented by curettage or cryosurgery), 4. efelids/ freckles (having clinical characteristics represented by 2-5 cm brown macula, on sun-exposed surfaces, closing in color after sun exposure, histopathologically finding an increased amount of pigment in the epidermis, and for which there is no treatment), 5. lentigo (clinical characteristics represented by 0.3-1.5 cm maculus, brown to black, most commonly occurring in regions exposed to the sun, face, upper torso and extremities, histopathologically with an increased number of melanocytes in the epidermis and an increased amount of pigment in the epidermis, the treatment being represented by keeping under observation, cryosurgery and laser acting on melanin), 6. junctional nebula (having clinical characteristics represented by brown to black macula, 2-6 cm, histologically finding melanocytes at the dermo-epidermic junction), with its variants of 7. compound nev (with brown to brown papula, 2-8 mm, histologically finding nests of melanocytes in the epidermis and dermis, in both forms the treatment being represented by maintenance under observation in case of changes) and 8. dermal nebula (skin-colored papule, histologically described by melanocytes nests in the dermis, to be kept under observation), completing with 9. melanoma (having clinical characteristics represented by variations in brown, black, blue, red and white, histologically manifested by malignant neoplasm of melanocytes, which has as treatment excision with safety limits between 0.5-2 cm, depending on the depth of Breslow).
Seborrheic keratoses are frequent lesions, but in a certain clinical circumstance there are signs of systemic disease, and this circumstance is the sudden occurrence of multiple lesions, often with an inflammatory base, in combination with acrocords and acanthosis nigricans. This is the sign of Leser-Trelat and signifies the existence of an internal malignant tumor.
Acanthosis nigricans can also be the reflection of an internal malignancy, most commonly of the gastrointestinal tract, and occurs as a velvety hyperpigmentation. In most patients, acanthosis nigricans is associated with obesity, but may be a reflection of endocrinopathy, such as acromegaly, Cushing syndrome, Stein-Lenchal syndrome or association with insulin-resistant diabetes mellitus (type A, B and lipoatrophic forms).
A proliferation of melanocytes causes the following pigmentation lesions: lentigo, melanocytic nev and melanoma. In adults, most lentigines are related to sun exposure, which explains their distribution. However, in Peutz-Jeghers and LEOPARD syndrome (lentigines, ECG abnormalities, primitive conduction defects, ocular hyperteleism, pulmonary stenosis and aortic valvular stenosis, genital abnormalities such as cryptorchidism and hypospadias, growth retardation and sensorineural deafness), lengitins serve as clues to systemic disease.
In the case of multiple lengitins or LEOPARD syndrome, hundreds of lengitins develop in childhood and are dispersed throughout the body. Lengitines in patients with Peutz-Jeghers syndrome are mainly located around the nose and mouth, on the legs and hands and in the oral cavity. While pigmented macles on the face may be erased with age, oral lesions persist. However, similar intraoral lesions are also found in Adisson's disease and are normal in highly pigmented individuals.
Patients with this dominant autosomal syndrome have multiple benign polyps of the gastrointestinal tract, ovarian tumors and about 6% are at risk of developing a malignant gastrointestinal disease when polyps appear in the stomach, duodenum or colon. Lentigines are also found in association with cardiac mixomas and have been described in two syndromes in which clinical signs overlap: 1. LAMB syndrome (lentigines, atrial mixomas, microcutaneous and blue nevi mixomas), 2. NAME syndrome (nev, atrial mixomas, mixoid neurofibromas and efelide/ freckles). These patients may also have signs of endocrine overactivity in the form of Cushing syndrome, acomegalis or sexual precocity.
The third type of localized hyperpigmentation is due to a local increase in pigment production and includes efelids and spots "coffee with milk" (cafe au lait - CAL). The latter are most commonly associated with two conditions: 1. neurofibromatosis (NF) and Albright syndrome. The "milk coffee" (CAL) stains are smooth, uniformly light brown in colour and can vary in size from 0.5 to 12 cm. Approximately 80% of patients with NF type I have six or more ALCs, 1.5 or more in diameter.
Compared to NF, CAL spots in patients with Albright disease (polyostotic fibrous dysplasia with early puberty in women given by mosaicism through a mutation of the G protein) are usually larger, more irregular in the contour and follow the median line. CAL spots have also been associated with tuberous sclerosis, LEOPARD syndrome and ataxia-telangiectasis, but a few such lesions can also be found in normal individuals.
In pigment incontinence, congenital diskeratosis and pigmentation in bleomycin, localized hyperpigmentation regions form a pattern, spiraled into the first, reticulated into the second and flogging in the third. Patients with pigment incontinence, a dominant x-link eda, may have linear bubbles and verucous papules during the infant period. During childhood appear swirls and parallel bands of hyperpigmentation on the trunk and sometimes stripes of hypopigmentation on the extremities. Associated symptoms may be: epileptic seizures, mental retardation, strabismus, cataracts and delayed or impaired teething.
Biopsy of lesions will show pigment in dermal macrophages ("incontinent pigment"). In congenital diskeratosis, reticulated atrophic hyperpigmentation occurs in the neck, thighs and torso and is accompanied by nail dystrophy, pancytopenia and leukoplasia of the oral and mucous membranes. The latter often evolves into spinal carcinoma. In addition to the flogging pigmentation (linear stripes) on the torso, patients receiving bleomycin often experience hyperpigmentation on the elbows, knees and small joints of the hand.
Localised hyperpigmentation is found as a side effect of several systemic medications, including those that produce fixed drug reactions (phenolphthalein, tetracyclines, sulfonamides, barbiturates and analgesics) and those that may bind to melanin (antimalarials). Fixed eruptions recur with the same localizations, in the form of circular areas of erythema that can become bullous and then heal in the form of brown maculates.
The rash usually occurs within a few hours of the administration of the provoking agent and frequent localizations include the genitals, extremities and perioral region. Chloroquine and hydroxychloroquine can produce a gray-brown to black-gray coloration of the pretibial region, rough palate and face, while blue maculus are found on the lower limbs and in inflammatory areas after prolonged administration of minocycline. Estrogens in oral contraceptives can induce melasma (symmetrical brown spots on the face, especially cheeks, upper lip and forehead). Similar changes occur in pregnancy in patients receiving hydanthoin and in the adult form of Gaucher disease. In this last group there is also hyperpigmentation of the distal portions of the lower limbs.
In diffuse forms of hyperpigmentation, the closure of skin color may be of equal intensity throughout the body or may be accentuated in sun-exposed regions. The causes of diffuse superpigmentation can be divided into four groups: endrocrine, metabolic, autoimmune and medicinal. Endocrinopathies that are commonly associated with hyperpigmentation include Adisson's disease, Nelson syndrome and ectopic ACTH syndrome.
In these diseases, hipepigmentation is diffuse, but is accentuated in palm grooves, regions of fiction, scarring and on the oral mucosa. An overproduction of one or all pituitary hormones (alpha-MSH or melanocyto-stimulating hormone, ACTH and beta-lipotropin can lead to an increase in melanocytic activity). All these peptides are products of the proopiomelanocortin gene and therefore present counterparts, for example, alpha-MSH and ACTH share 13 amino acids. A small number of patients with Cushing's disease or hyperthyroidism have generalized hyperpigmentation.
Metabolic causes of hyperpigmentation include late skin porphyria (PCT), hemochromatosis, vitamin B12 deficiency, folic acid deficiency, pelagra, malabsorption and Whipple's disease. In patients with PCT, the closure of skin colour is found in sun-exposed regions and is an effect of the photoreactive properties of porphyrins. Increased iron levels in the skin of patients with hemochromatosis stimulate the production of melanic pigment and lead to the classic bronze color.
Patients with pelagr have a brown skin disorder, especially in solar exposed regions, as a result of deficiency in nicotinic acid (niacin). In areas with increased pigmentation, there is a fine sme as enamel. These changes are also found in patients deficient in vitamin B6, those with functional carcinoid tumours (increased niacin consumption) or taking isoniazide. Approximately 50% of patients with Whipple's disease have a generalized hyperpigmentation associated with diarrhea, weight loss, arthritis and adenopathy.
A diffuse blue-slate color is found in patients with melanoma secondary to metastatic melanoma. There is controversy that hyperpigmentation is due to single-celled metastases in the dermis or a disseminated deposition of melanin from the high concentration of circulating melanin precursors. There are several records that support the last hypothesis.
Of the autoimmune diseases associated with diffuse hyperpigmentation, bile cirrhosis and scleroderma are the most common and sometimes both conditions occur in the same patient. The skin is dark brown, especially in solar-exposed regions. In biliary cirrhosis, hyperpigmentation is accompanied by pruritus, jaundice and xantomas, while in scleroderma it is accompanied by sclerosis of the extremities, face and, more rarely, of the torso. Additional clues for scleroderma are telangiectasis, skin calcinosis, Raynaud's phenomenon and distal ulcerations.
Differential diagnosis of skin sclerosis with hyperpigmentation includes POEMS syndrome (polyneuropathy, organomegaly liver, spleen, lymph nodes, endocrinopathies with impotence and gynecomastia, protein M and skin changes). Skin changes include hyperpigmentation, thickening of the skin, hypertricosis, hyperhidrosis and angiomas. Recently, an epidemic of eosinophilia-myalgia syndrome has been described, which is probably due to contaminated L tryptophan preparations. In addition to the maculo-papular rash and alopecia, large areas similar to sclerodermal indurations, accompanied by hyperpigmentation, are observed.
Diffuse hyperpigmentation due to drugs or metals may result from one or more mechanisms such as inducing the formation of melanic pigment, the formation of complexes between the drug or its melanin metabolites and drug deposits in the dermis. Busulfan, cyclophosphamide, ACTH retard in high doses and inorganic arsenic induce pigment production. Complexes containing melanin or hemosiderin plus the drug or its metabolites are found in patients receiving chlorpromazine and minocycline.
Sun-exposed skin, like the conjunctivas of patients receiving chlorpromazine in high doses, for a long time, can turn gray-blue in color. patients taking minocycline may develop a diffuse, dark gray-blue coloration of sun-exposed skin areas, in addition to pigmentation of mucous membranes, teeth, nails, bones and thyroids. The administration of amiodarone may cause both a phototoxic rash (exaggerated sunburn) and/ or a greyish-blue coloration of sun-exposed skin.
The biopsies, in the latter case, show yellow-brown granules in the dermal macrophages, which represent intralyzosome accumulations of lipids, amiodarone and its metabolites. Actual deposits of a particular drug or metal in the skin are found in silver (argiria), in which the skin appears gray-blue in color, gold (chrysiasis), in which the skin appears red-brown to blue-grey and clofasimin, in which the skin appears red-brown. Associated hyperpigmentation is accentuated in sun-exposed regions and eye color change is found for gold (sclre) and clofazimin (conjunctive).
Yes, we're done!
Look, it's been another month (seventh of postings)... So what if... We're just getting started... And, we continue with hyperpigmentation...
The causes of hyperpigmentation are represented by 1. primary skin conditions with localized Forms A. (a. epidermal change with i. seborrheic keratosis, ii. acanthosis nigricans/ obesity and iii. actinic pigmented keratosis, b. proliferation of melanocytes with i. lentigo, ii. nev, iii. melanoma, c. increased pigment production with i. efelide/ freckles, ii. spots "milk coffee") and B. localized and diffuse (induced medicinal), 2. systemic diseases with localized Forms A. (a. epidermal modification with i. seborrheic keratoses/ Lesser-Trerate sign and ii. acanthosis nigricans/ endocrine disorders, paraneoplastic, b. proliferation of melanocytes with i. lentigine/ Peutz-Jeghers syndrome, LEOPARD syndrome, xeroderma pigmentosum and ii. nevi/ Carney complex/ LAMB and NAME syndromes, including lentigine, c. increased pigment production with i. spots "milk coffee"/ neurofibromatosis, Albright syndrome and ii. urticaria pigmentosa, d. dermal pigmentation with i. incontinentia pigments and ii. congenital diskeratosis) and B. diffuse (with 1. endocrinopathies represented by a. Addison's disease, b. Nelson syndrome and c. ectopic ACTH syndrome, 2. metabolic with late skin porphyria, b. homochromatosis, c. deficiencies in vitamin B12, folates, d. pelagra and e. malabsorption , Whipple's disease, 3. secondary melanosis to metastatic melanoma, 4. autoimmune with a. biliary cirrhosis, b. scleroderma, c. POEMS syndrome and d. eosinophilia-myalgia syndrome and 5. medicines and metals).
Hyperpigmentation disorders are divided into two localized and diffuse groups. Localized forms are due to epidermal change, melanocytic proliferation or an increase in pigment production. Forms of hyperpigmentation (localized primary skin disorders) are represented by: 1. seborrheic keratosis (having clinical characteristics represented by brownish to black papule, verucoous and/ or fatty surface, "applied" appearance, arranged on the trunk, histopathologically represented by epidermal hyperplasia, with treatment represented by maintenance, and if irritated or bleeding should be removed), 2. acanthosis nigricans (having clinical characteristics represented velvety surfaces present on the neck, armpits, groin, sometimes on the back of the hands and at the corners of the mouth, histopathologically highlighting epidermal folds with increased pigment in the basal layer, with treatment represented by administration of topical tretinoin, alpha-hydroxyacids or keratolitics, including weight loss if associated with obesity), 3. pigmented actinic keratosis (having clinical characteristics represented by a brown 3-10 mm macula, rough scuba present in solar-exposed regions, in particular the face and back of the hands, histopathologically finding keratocyte dysplasia in the lower third of the epidermis and pigment increased in the epidermis, requiring treatment represented by curettage or cryosurgery), 4. efelids/ freckles (having clinical characteristics represented by 2-5 cm brown macula, on sun-exposed surfaces, closing in color after sun exposure, histopathologically finding an increased amount of pigment in the epidermis, and for which there is no treatment), 5. lentigo (clinical characteristics represented by 0.3-1.5 cm maculus, brown to black, most commonly occurring in regions exposed to the sun, face, upper torso and extremities, histopathologically with an increased number of melanocytes in the epidermis and an increased amount of pigment in the epidermis, the treatment being represented by keeping under observation, cryosurgery and laser acting on melanin), 6. junctional nebula (having clinical characteristics represented by brown to black macula, 2-6 cm, histologically finding melanocytes at the dermo-epidermic junction), with its variants of 7. compound nev (with brown to brown papula, 2-8 mm, histologically finding nests of melanocytes in the epidermis and dermis, in both forms the treatment being represented by maintenance under observation in case of changes) and 8. dermal nebula (skin-colored papule, histologically described by melanocytes nests in the dermis, to be kept under observation), completing with 9. melanoma (having clinical characteristics represented by variations in brown, black, blue, red and white, histologically manifested by malignant neoplasm of melanocytes, which has as treatment excision with safety limits between 0.5-2 cm, depending on the depth of Breslow).
Seborrheic keratoses are frequent lesions, but in a certain clinical circumstance there are signs of systemic disease, and this circumstance is the sudden occurrence of multiple lesions, often with an inflammatory base, in combination with acrocords and acanthosis nigricans. This is the sign of Leser-Trelat and signifies the existence of an internal malignant tumor.
Acanthosis nigricans can also be the reflection of an internal malignancy, most commonly of the gastrointestinal tract, and occurs as a velvety hyperpigmentation. In most patients, acanthosis nigricans is associated with obesity, but may be a reflection of endocrinopathy, such as acromegaly, Cushing syndrome, Stein-Lenchal syndrome or association with insulin-resistant diabetes mellitus (type A, B and lipoatrophic forms).
A proliferation of melanocytes causes the following pigmentation lesions: lentigo, melanocytic nev and melanoma. In adults, most lentigines are related to sun exposure, which explains their distribution. However, in Peutz-Jeghers and LEOPARD syndrome (lentigines, ECG abnormalities, primitive conduction defects, ocular hyperteleism, pulmonary stenosis and aortic valvular stenosis, genital abnormalities such as cryptorchidism and hypospadias, growth retardation and sensorineural deafness), lengitins serve as clues to systemic disease.
In the case of multiple lengitins or LEOPARD syndrome, hundreds of lengitins develop in childhood and are dispersed throughout the body. Lengitines in patients with Peutz-Jeghers syndrome are mainly located around the nose and mouth, on the legs and hands and in the oral cavity. While pigmented macles on the face may be erased with age, oral lesions persist. However, similar intraoral lesions are also found in Adisson's disease and are normal in highly pigmented individuals.
Patients with this dominant autosomal syndrome have multiple benign polyps of the gastrointestinal tract, ovarian tumors and about 6% are at risk of developing a malignant gastrointestinal disease when polyps appear in the stomach, duodenum or colon. Lentigines are also found in association with cardiac mixomas and have been described in two syndromes in which clinical signs overlap: 1. LAMB syndrome (lentigines, atrial mixomas, microcutaneous and blue nevi mixomas), 2. NAME syndrome (nev, atrial mixomas, mixoid neurofibromas and efelide/ freckles). These patients may also have signs of endocrine overactivity in the form of Cushing syndrome, acomegalis or sexual precocity.
The third type of localized hyperpigmentation is due to a local increase in pigment production and includes efelids and spots "coffee with milk" (cafe au lait - CAL). The latter are most commonly associated with two conditions: 1. neurofibromatosis (NF) and Albright syndrome. The "milk coffee" (CAL) stains are smooth, uniformly light brown in colour and can vary in size from 0.5 to 12 cm. Approximately 80% of patients with NF type I have six or more ALCs, 1.5 or more in diameter.
Compared to NF, CAL spots in patients with Albright disease (polyostotic fibrous dysplasia with early puberty in women given by mosaicism through a mutation of the G protein) are usually larger, more irregular in the contour and follow the median line. CAL spots have also been associated with tuberous sclerosis, LEOPARD syndrome and ataxia-telangiectasis, but a few such lesions can also be found in normal individuals.
In pigment incontinence, congenital diskeratosis and pigmentation in bleomycin, localized hyperpigmentation regions form a pattern, spiraled into the first, reticulated into the second and flogging in the third. Patients with pigment incontinence, a dominant x-link eda, may have linear bubbles and verucous papules during the infant period. During childhood appear swirls and parallel bands of hyperpigmentation on the trunk and sometimes stripes of hypopigmentation on the extremities. Associated symptoms may be: epileptic seizures, mental retardation, strabismus, cataracts and delayed or impaired teething.
Biopsy of lesions will show pigment in dermal macrophages ("incontinent pigment"). In congenital diskeratosis, reticulated atrophic hyperpigmentation occurs in the neck, thighs and torso and is accompanied by nail dystrophy, pancytopenia and leukoplasia of the oral and mucous membranes. The latter often evolves into spinal carcinoma. In addition to the flogging pigmentation (linear stripes) on the torso, patients receiving bleomycin often experience hyperpigmentation on the elbows, knees and small joints of the hand.
Localised hyperpigmentation is found as a side effect of several systemic medications, including those that produce fixed drug reactions (phenolphthalein, tetracyclines, sulfonamides, barbiturates and analgesics) and those that may bind to melanin (antimalarials). Fixed eruptions recur with the same localizations, in the form of circular areas of erythema that can become bullous and then heal in the form of brown maculates.
The rash usually occurs within a few hours of the administration of the provoking agent and frequent localizations include the genitals, extremities and perioral region. Chloroquine and hydroxychloroquine can produce a gray-brown to black-gray coloration of the pretibial region, rough palate and face, while blue maculus are found on the lower limbs and in inflammatory areas after prolonged administration of minocycline. Estrogens in oral contraceptives can induce melasma (symmetrical brown spots on the face, especially cheeks, upper lip and forehead). Similar changes occur in pregnancy in patients receiving hydanthoin and in the adult form of Gaucher disease. In this last group there is also hyperpigmentation of the distal portions of the lower limbs.
In diffuse forms of hyperpigmentation, the closure of skin color may be of equal intensity throughout the body or may be accentuated in sun-exposed regions. The causes of diffuse superpigmentation can be divided into four groups: endrocrine, metabolic, autoimmune and medicinal. Endocrinopathies that are commonly associated with hyperpigmentation include Adisson's disease, Nelson syndrome and ectopic ACTH syndrome.
In these diseases, hipepigmentation is diffuse, but is accentuated in palm grooves, regions of fiction, scarring and on the oral mucosa. An overproduction of one or all pituitary hormones (alpha-MSH or melanocyto-stimulating hormone, ACTH and beta-lipotropin can lead to an increase in melanocytic activity). All these peptides are products of the proopiomelanocortin gene and therefore present counterparts, for example, alpha-MSH and ACTH share 13 amino acids. A small number of patients with Cushing's disease or hyperthyroidism have generalized hyperpigmentation.
Metabolic causes of hyperpigmentation include late skin porphyria (PCT), hemochromatosis, vitamin B12 deficiency, folic acid deficiency, pelagra, malabsorption and Whipple's disease. In patients with PCT, the closure of skin colour is found in sun-exposed regions and is an effect of the photoreactive properties of porphyrins. Increased iron levels in the skin of patients with hemochromatosis stimulate the production of melanic pigment and lead to the classic bronze color.
Patients with pelagr have a brown skin disorder, especially in solar exposed regions, as a result of deficiency in nicotinic acid (niacin). In areas with increased pigmentation, there is a fine sme as enamel. These changes are also found in patients deficient in vitamin B6, those with functional carcinoid tumours (increased niacin consumption) or taking isoniazide. Approximately 50% of patients with Whipple's disease have a generalized hyperpigmentation associated with diarrhea, weight loss, arthritis and adenopathy.
A diffuse blue-slate color is found in patients with melanoma secondary to metastatic melanoma. There is controversy that hyperpigmentation is due to single-celled metastases in the dermis or a disseminated deposition of melanin from the high concentration of circulating melanin precursors. There are several records that support the last hypothesis.
Of the autoimmune diseases associated with diffuse hyperpigmentation, bile cirrhosis and scleroderma are the most common and sometimes both conditions occur in the same patient. The skin is dark brown, especially in solar-exposed regions. In biliary cirrhosis, hyperpigmentation is accompanied by pruritus, jaundice and xantomas, while in scleroderma it is accompanied by sclerosis of the extremities, face and, more rarely, of the torso. Additional clues for scleroderma are telangiectasis, skin calcinosis, Raynaud's phenomenon and distal ulcerations.
Differential diagnosis of skin sclerosis with hyperpigmentation includes POEMS syndrome (polyneuropathy, organomegaly liver, spleen, lymph nodes, endocrinopathies with impotence and gynecomastia, protein M and skin changes). Skin changes include hyperpigmentation, thickening of the skin, hypertricosis, hyperhidrosis and angiomas. Recently, an epidemic of eosinophilia-myalgia syndrome has been described, which is probably due to contaminated L tryptophan preparations. In addition to the maculo-papular rash and alopecia, large areas similar to sclerodermal indurations, accompanied by hyperpigmentation, are observed.
Diffuse hyperpigmentation due to drugs or metals may result from one or more mechanisms such as inducing the formation of melanic pigment, the formation of complexes between the drug or its melanin metabolites and drug deposits in the dermis. Busulfan, cyclophosphamide, ACTH retard in high doses and inorganic arsenic induce pigment production. Complexes containing melanin or hemosiderin plus the drug or its metabolites are found in patients receiving chlorpromazine and minocycline.
Sun-exposed skin, like the conjunctivas of patients receiving chlorpromazine in high doses, for a long time, can turn gray-blue in color. patients taking minocycline may develop a diffuse, dark gray-blue coloration of sun-exposed skin areas, in addition to pigmentation of mucous membranes, teeth, nails, bones and thyroids. The administration of amiodarone may cause both a phototoxic rash (exaggerated sunburn) and/ or a greyish-blue coloration of sun-exposed skin.
The biopsies, in the latter case, show yellow-brown granules in the dermal macrophages, which represent intralyzosome accumulations of lipids, amiodarone and its metabolites. Actual deposits of a particular drug or metal in the skin are found in silver (argiria), in which the skin appears gray-blue in color, gold (chrysiasis), in which the skin appears red-brown to blue-grey and clofasimin, in which the skin appears red-brown. Associated hyperpigmentation is accentuated in sun-exposed regions and eye color change is found for gold (sclre) and clofazimin (conjunctive).
Yes, we're done!
Have a good day!
Dorin, Merticaru