STUDY - Technical - New Dacian's Medicine
Skin
Manifestations of Internal Diseases (6)
Translation Draft
I've reached hives... The causes of urticaria are represented by: 1. primary skin conditions (with A. acute and chronic urticaria, B. physical urticaria with a. eyeliner, b. solar urticaria, c. cold urticaria and d. cholinergic urticaria, and C. hereditary or acquired angioedema) and 2. systemic diseases (with a. urticariar vasculitis, b. hepatitis B virus infection, c. serum disease and d. hereditary or acquired angioedema). Urticaria is transient lesions that consist of a central papule surrounded by an erythematous halo.
Individual lesions are round, oval or figurative and are often itchy. Acute and chronic urticaria has a wide variety of allergic causes. Less common systemic causes of urticaria are mastocytosis (urticaria pigmentosa), hyperthyroidism, malignant diseases and juvenile rheumatoid arthritis (RJA). In ARJ, the lesions coincide with the feverish crochet and are transient, but not migratory as in the marginatum erythema. Regular physical urticaria includes eyeliner, solar urticaria, cold urticaria and cholinergic urticaria.
Patients with eyeliner have linear urticaria papules following minor pressure or skin scratching. It is a common disorder, affecting about 5% of the population. Solar urticaria occurs characteristically a few minutes after sun exposure and is a sign of a systemic disease (erythropoietic protoporphyry). In addition to hives, these patients have fine puncitof scars of the nose and hands. Cold urticaria is triggered by exposure to cold and therefore the exposed tons are the ones usually affected. In some cases, the disease is associated with circulating abnormal proteins, more commonly cryoglobulins and cold hemolysis and more rarely cryofibrinogens and cold agglutinins.
Additional systemic symptoms including wheezing and syncope, thus explaining the need for these patients to avoid swimming in cold water. Cholinergic urticaria is precipitated by heat, exercise or emotion and is characterized by small urticaria papules with relatively stretched erythema. It is occasionally associated with wheezing. While urticaria is the result of dermal edema, subcutaneous edema leads to the clinical picture of angioedema.
Affected regions include the eyelids, lips, tongue, larynx and gastrointestinal tract, as well as subcutaneous tissue. Angioedema occurs in isolation or in combination with urticaria, including urticaria vasculitis and physical urticaria. They appear in both the acquired form and the hereditary (autosomal dominant) form of angioedema, and in the latter, urticaria is rarely encountered. Urticariavasculitis is a disease with immune complexes, which can be confused with simple urticaria. Unlike simple urticaria, individual lesions tend to persist for over 24 hours and usually develop central spots that can be observed even after the urticaria phase has disappeared.
The patient may also have burning sensations rather than itching. At biopsy, there is a leukocytoclastic vasculitis of small blood vessels. Although many cases of urticaria vasculitis are of idiopathic origin, it may be a reflection of an underlying systemic disease, such as lupus erythematosus, Sjogren's syndrome or hereditary supplement deficiency. There is a spectrum of urticaria vasculitis, which extends from purely skin damage to multisystemic damage.
The most common systemic signs and symptoms are arthralgia and/ or arthritis, nephritis and colicative abdominal pain, and asthma and chronic obstructive pulmonary disease are rare. Hypocomplementemia occurs in one-third to two-thirds of patients, even in idiopathic cases. Similar skin, joint and renal signs can be found in the prodrom of hepatitis B infection, serum disease and serum-like diseases.
Let's move on to papulonodular skin lesions! They are "addressed" in the literature according to the color groups, as follows: I. white (with cutaneous calcinosis), II. skin (with A. rheumatoid node, B. neurofibromas as is the case with von Recklinghausen disease, C. angiofibromas such as tuberous sclerosis, D. neuromas such as multiple endocrine neoplasia syndrome type 2b, E. tumors of the appendages such as 1. basal cell epitheliomas as in the case of basal cell nerve syndrome, 2. tricholemomas as in the case of Cowden disease, F. primary skin conditions as in the case of 1. inclusion epidermal cysts and 2. lipomas), III. (A. amyloidosis and B. papular mucinosis), IV. yellow (A. xantoame, B. tofi, C. Necrobiosis lipoidica, D. pseudoxantoma elasticum and E. sebaceous adenomas as in the case of Torre syndrome), V. red (A. papules with 1. angiokeratomas as in Fabry disease, 2. bacillary angiomatosis especially in AIDS, B. papules/ plaques as in the case of 1. skin lupus, 2. skin lymphoma, 3. C. nodules as in the case of 1. paniculitis, 2. skin noderitis and 3. systemic vasculitis, D. primary skin conditions such as 1. arthronode bite, 2. cherry hemangiomas, 3. infections, e.g. erysipelas, sporotrichosis, 4. light polymorphic rash and 5. cutaneous lymphocytoma or pseudolymphoma), VI. red-brown (A. sarcoidosis, B. Sweet syndrome, C. urticaria pigmentosa, D. erythema elevatum diuitinum as in the case of chronic leukocytoplastic vasculitis and E. lupus vulgaris), VII. blue (A. venous malformations such as blue hematoma syndrome and B. primary skin conditions with 1. venous lake and 2. blue nebula), VIII. violaceus (with A. lupus pernio from sarcoidosis, B. skin lymphoma and C. lupus skin), IX. purple (A. Kaposi sarcoma, B. angiosarcoma and C. purpura), Black-brown X. (hyperpigmentation) and XI. any colour (case of metastases).
Detailing, in maculopapular diseases, the lesions are prominent and can fuse, to form plaques. Location, consistency and color of lesions are key elements for diagnosis (cup as described above). So, all the elements that I will present next are organized based on color, and the color groups are white, normal skin color, pink, yellow, red, red-brown, blue, purple, purple and brown-black.
So, let's move on to the white lesions! In the skin calcinosis appear hard white or yellowish-white papules with irregular surface. When the contents are removed, a chalk-white material is seen. Dystrophic calcification occurs in places where there has previously been inflammation or destruction of the skin. It occurs in the areas of postacne scarring and distal portions of the extremities in patients with scleroderma, and in subcutaneous tissue and intermuscular fascial planes in dermatomyositis. At the latter, calcification is more extensive and is more common in children. An increased phosphocalcic product, as in secondary hyperthyroidism, can lead to nodules of metastatic skin calcinosis, which tend to be subcutaneous and periarticular. This form is often accompanied by calcification of the muscular arteries and consecutive ischemic necrosis (calciphilaxia).
In the case of lesions of normal skin color, there are several types of lesions, including epidermoid cysts, lipomas, rheumatoid nodules, neurofibromas, angiofibromas, neuromas and tumors of the appendages, such as tricolemomas. Both epidermoid cysts and lipomas are very frequent movable subcutaneous nodules (the first ones are elastic and compressible and drain a cheesy material consisting of sebum and keratin if incised). Lipomas are firm and somewhat lobulated at the palms.
When epidermoid cysts of extensive inclusion of the face appear in childhood or there is a family history of such lesions, the patient should be examined in search of other signs of Gardner syndrome, including osteomas and desmoid tumors. Rheumatoid nodules are firm nodules, 0.5-4 cm, which tend to localize around pressure points, especially at the elbows. They occur in about 20% of rheumatoid arthritis patients and 6% of still disease patients. The biopsies of the nodules show granulomas in the palisade. Similar lesions, which are smaller and with shorter life, are found in acute articular rheumatism.
Neurofibromas (benign tumors with Schwann cells) are soft nodules or papules that show the sign of the "button", i.e. at pressure it invades the skin, similar to a hernia. Single lesions are found in normal individuals, but multiple neurofibromas in combination with six or more "milk coffee" spots over 1.5 cm and Lisch multiple nodules are found in von Recklinghausen disease (NF type I). The Lisch nodules are 1 mm brownish yellow spots arranged on the iris and are best observed on the slot lamp exam. Associated manifestations include axillary "freckles" and peripheral CNS tumors. In some patients, neurofibromas are localized and unilateral, while in others they are limited to CNS.
Angiofibromas are firm, pink or skin-colored papules, which measure about 3 mm and are several centimeters in diameter. When located in the central area of the cheeks (sebaceous adenomas) or when multiple fibroids meet periangleally, the patient has tuberous sclerosis. This is a dominant autosomal disorder and the associated findings are related to spots in the "maple leaf".
Neuroamas (benign proliferations of nerve fibers) are also firm papules of skin color. They are most commonly found in amputation regions and as rudimentary supernumerary fingers. However, when there are multiple neuromas on the eyelids, lips, distal portion of the tongue and/ or oral mucosa, the patient should be investigated for other signs of multiple endocrine neoplasia syndrome type 2b. Associated manifestations are the physical appearance of merchandise, protruding lips, intestinal ganglioneuromas and thyroid medullary carcinoma (in more than 75% of patients).
Tumors of the appendages derive from the pluripotent cells of the epidermis, which can differentiate towards the formation of hair, sebaceous glands, apocrine or ecrine glands, or remain undifferentiated. Basal cell epitheliomas (BCEs) are examples of anexial tumours that show no signs of differentiation or show small such signs. Clinically, they are translucent papules with protruding edges, telangiectasis and central erosion. EBC commonly occurs on the solar degraded skin of the head and neck. When a patient, especially under 30 years of age, has multiple EBC, the possibility of a basal cell nev should be suspected.
It is a dominant autosomal hereditary condition and is associated with jaw cysts, palmoplantation punctome depressions, boselated forehead, rib abnormalities and calcifications in the famous falx and diaphragma selae. Tricholemomas are also tumors of the appendages, the color of the skin but differentiate to hair follicles and may have a verucos appearance. The presence of multiple tricholemomas on the face and oral mucosa indicates the diagnosis of Cowden disease (multiple hamartom stalls). Oral trichomonas are primarily found on the tongue and gum and give these regions a "pavement stone" appearance. Damage to internal organs (in descending order of frequency) includes fibrocystic disease and breast carcinoma, adenomas and thyroid carcinomas and gastrointestinal polyposis. Palm keratoses, planting and back of hands are also encountered.
Ready for today!
I've reached hives... The causes of urticaria are represented by: 1. primary skin conditions (with A. acute and chronic urticaria, B. physical urticaria with a. eyeliner, b. solar urticaria, c. cold urticaria and d. cholinergic urticaria, and C. hereditary or acquired angioedema) and 2. systemic diseases (with a. urticariar vasculitis, b. hepatitis B virus infection, c. serum disease and d. hereditary or acquired angioedema). Urticaria is transient lesions that consist of a central papule surrounded by an erythematous halo.
Individual lesions are round, oval or figurative and are often itchy. Acute and chronic urticaria has a wide variety of allergic causes. Less common systemic causes of urticaria are mastocytosis (urticaria pigmentosa), hyperthyroidism, malignant diseases and juvenile rheumatoid arthritis (RJA). In ARJ, the lesions coincide with the feverish crochet and are transient, but not migratory as in the marginatum erythema. Regular physical urticaria includes eyeliner, solar urticaria, cold urticaria and cholinergic urticaria.
Patients with eyeliner have linear urticaria papules following minor pressure or skin scratching. It is a common disorder, affecting about 5% of the population. Solar urticaria occurs characteristically a few minutes after sun exposure and is a sign of a systemic disease (erythropoietic protoporphyry). In addition to hives, these patients have fine puncitof scars of the nose and hands. Cold urticaria is triggered by exposure to cold and therefore the exposed tons are the ones usually affected. In some cases, the disease is associated with circulating abnormal proteins, more commonly cryoglobulins and cold hemolysis and more rarely cryofibrinogens and cold agglutinins.
Additional systemic symptoms including wheezing and syncope, thus explaining the need for these patients to avoid swimming in cold water. Cholinergic urticaria is precipitated by heat, exercise or emotion and is characterized by small urticaria papules with relatively stretched erythema. It is occasionally associated with wheezing. While urticaria is the result of dermal edema, subcutaneous edema leads to the clinical picture of angioedema.
Affected regions include the eyelids, lips, tongue, larynx and gastrointestinal tract, as well as subcutaneous tissue. Angioedema occurs in isolation or in combination with urticaria, including urticaria vasculitis and physical urticaria. They appear in both the acquired form and the hereditary (autosomal dominant) form of angioedema, and in the latter, urticaria is rarely encountered. Urticariavasculitis is a disease with immune complexes, which can be confused with simple urticaria. Unlike simple urticaria, individual lesions tend to persist for over 24 hours and usually develop central spots that can be observed even after the urticaria phase has disappeared.
The patient may also have burning sensations rather than itching. At biopsy, there is a leukocytoclastic vasculitis of small blood vessels. Although many cases of urticaria vasculitis are of idiopathic origin, it may be a reflection of an underlying systemic disease, such as lupus erythematosus, Sjogren's syndrome or hereditary supplement deficiency. There is a spectrum of urticaria vasculitis, which extends from purely skin damage to multisystemic damage.
The most common systemic signs and symptoms are arthralgia and/ or arthritis, nephritis and colicative abdominal pain, and asthma and chronic obstructive pulmonary disease are rare. Hypocomplementemia occurs in one-third to two-thirds of patients, even in idiopathic cases. Similar skin, joint and renal signs can be found in the prodrom of hepatitis B infection, serum disease and serum-like diseases.
Let's move on to papulonodular skin lesions! They are "addressed" in the literature according to the color groups, as follows: I. white (with cutaneous calcinosis), II. skin (with A. rheumatoid node, B. neurofibromas as is the case with von Recklinghausen disease, C. angiofibromas such as tuberous sclerosis, D. neuromas such as multiple endocrine neoplasia syndrome type 2b, E. tumors of the appendages such as 1. basal cell epitheliomas as in the case of basal cell nerve syndrome, 2. tricholemomas as in the case of Cowden disease, F. primary skin conditions as in the case of 1. inclusion epidermal cysts and 2. lipomas), III. (A. amyloidosis and B. papular mucinosis), IV. yellow (A. xantoame, B. tofi, C. Necrobiosis lipoidica, D. pseudoxantoma elasticum and E. sebaceous adenomas as in the case of Torre syndrome), V. red (A. papules with 1. angiokeratomas as in Fabry disease, 2. bacillary angiomatosis especially in AIDS, B. papules/ plaques as in the case of 1. skin lupus, 2. skin lymphoma, 3. C. nodules as in the case of 1. paniculitis, 2. skin noderitis and 3. systemic vasculitis, D. primary skin conditions such as 1. arthronode bite, 2. cherry hemangiomas, 3. infections, e.g. erysipelas, sporotrichosis, 4. light polymorphic rash and 5. cutaneous lymphocytoma or pseudolymphoma), VI. red-brown (A. sarcoidosis, B. Sweet syndrome, C. urticaria pigmentosa, D. erythema elevatum diuitinum as in the case of chronic leukocytoplastic vasculitis and E. lupus vulgaris), VII. blue (A. venous malformations such as blue hematoma syndrome and B. primary skin conditions with 1. venous lake and 2. blue nebula), VIII. violaceus (with A. lupus pernio from sarcoidosis, B. skin lymphoma and C. lupus skin), IX. purple (A. Kaposi sarcoma, B. angiosarcoma and C. purpura), Black-brown X. (hyperpigmentation) and XI. any colour (case of metastases).
Detailing, in maculopapular diseases, the lesions are prominent and can fuse, to form plaques. Location, consistency and color of lesions are key elements for diagnosis (cup as described above). So, all the elements that I will present next are organized based on color, and the color groups are white, normal skin color, pink, yellow, red, red-brown, blue, purple, purple and brown-black.
So, let's move on to the white lesions! In the skin calcinosis appear hard white or yellowish-white papules with irregular surface. When the contents are removed, a chalk-white material is seen. Dystrophic calcification occurs in places where there has previously been inflammation or destruction of the skin. It occurs in the areas of postacne scarring and distal portions of the extremities in patients with scleroderma, and in subcutaneous tissue and intermuscular fascial planes in dermatomyositis. At the latter, calcification is more extensive and is more common in children. An increased phosphocalcic product, as in secondary hyperthyroidism, can lead to nodules of metastatic skin calcinosis, which tend to be subcutaneous and periarticular. This form is often accompanied by calcification of the muscular arteries and consecutive ischemic necrosis (calciphilaxia).
In the case of lesions of normal skin color, there are several types of lesions, including epidermoid cysts, lipomas, rheumatoid nodules, neurofibromas, angiofibromas, neuromas and tumors of the appendages, such as tricolemomas. Both epidermoid cysts and lipomas are very frequent movable subcutaneous nodules (the first ones are elastic and compressible and drain a cheesy material consisting of sebum and keratin if incised). Lipomas are firm and somewhat lobulated at the palms.
When epidermoid cysts of extensive inclusion of the face appear in childhood or there is a family history of such lesions, the patient should be examined in search of other signs of Gardner syndrome, including osteomas and desmoid tumors. Rheumatoid nodules are firm nodules, 0.5-4 cm, which tend to localize around pressure points, especially at the elbows. They occur in about 20% of rheumatoid arthritis patients and 6% of still disease patients. The biopsies of the nodules show granulomas in the palisade. Similar lesions, which are smaller and with shorter life, are found in acute articular rheumatism.
Neurofibromas (benign tumors with Schwann cells) are soft nodules or papules that show the sign of the "button", i.e. at pressure it invades the skin, similar to a hernia. Single lesions are found in normal individuals, but multiple neurofibromas in combination with six or more "milk coffee" spots over 1.5 cm and Lisch multiple nodules are found in von Recklinghausen disease (NF type I). The Lisch nodules are 1 mm brownish yellow spots arranged on the iris and are best observed on the slot lamp exam. Associated manifestations include axillary "freckles" and peripheral CNS tumors. In some patients, neurofibromas are localized and unilateral, while in others they are limited to CNS.
Angiofibromas are firm, pink or skin-colored papules, which measure about 3 mm and are several centimeters in diameter. When located in the central area of the cheeks (sebaceous adenomas) or when multiple fibroids meet periangleally, the patient has tuberous sclerosis. This is a dominant autosomal disorder and the associated findings are related to spots in the "maple leaf".
Neuroamas (benign proliferations of nerve fibers) are also firm papules of skin color. They are most commonly found in amputation regions and as rudimentary supernumerary fingers. However, when there are multiple neuromas on the eyelids, lips, distal portion of the tongue and/ or oral mucosa, the patient should be investigated for other signs of multiple endocrine neoplasia syndrome type 2b. Associated manifestations are the physical appearance of merchandise, protruding lips, intestinal ganglioneuromas and thyroid medullary carcinoma (in more than 75% of patients).
Tumors of the appendages derive from the pluripotent cells of the epidermis, which can differentiate towards the formation of hair, sebaceous glands, apocrine or ecrine glands, or remain undifferentiated. Basal cell epitheliomas (BCEs) are examples of anexial tumours that show no signs of differentiation or show small such signs. Clinically, they are translucent papules with protruding edges, telangiectasis and central erosion. EBC commonly occurs on the solar degraded skin of the head and neck. When a patient, especially under 30 years of age, has multiple EBC, the possibility of a basal cell nev should be suspected.
It is a dominant autosomal hereditary condition and is associated with jaw cysts, palmoplantation punctome depressions, boselated forehead, rib abnormalities and calcifications in the famous falx and diaphragma selae. Tricholemomas are also tumors of the appendages, the color of the skin but differentiate to hair follicles and may have a verucos appearance. The presence of multiple tricholemomas on the face and oral mucosa indicates the diagnosis of Cowden disease (multiple hamartom stalls). Oral trichomonas are primarily found on the tongue and gum and give these regions a "pavement stone" appearance. Damage to internal organs (in descending order of frequency) includes fibrocystic disease and breast carcinoma, adenomas and thyroid carcinomas and gastrointestinal polyposis. Palm keratoses, planting and back of hands are also encountered.
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Have a good day!
Dorin, Merticaru