STUDY - Technical - New Dacian's Medicine
Skin
Manifestations of Internal Diseases (10)
Translation Draft
Let's continue with the purple and finalize the skin manifestations of internal diseases!
The palpable purple is also subdivided into vasculitic and embolic. In the group of vasculitic diseases, leukocytoclastic vasculitis (VLC), known as allergic vasculitis, is most often associated with palpable purpura. Purpura Henoch-Schonlein is a subtype of acute VLC that is primarily found in children and adolescents after an upper respiratory tract infection. Most of the lesions are found on the lower limbs and buttocks.
Systemic manifestations include fever, arthralgia (especially of the knees and ankles), abdominal pain, gastrointestinal bleeding and nephritis. Direct immunofluorescence examination shows deposits of IgA in the walls of dermal blood vessels. In node polyarteritis, specific skin lesions result from a vasculitis of arterial vessels, rather than postcapillary venurics as in VLC. Arteritis leads to dermal ischemia and this explains the irregular contour of the purple. Several types of infectious embolisms can cause palpable purpura. These embolic lesions are usually irregular in the contour, as opposed to lesions of leukocytoclastic vasculitis, which have circular contour.
The irregular contour indicates a skin infarction and the size corresponds to the area of skin irrigated by that particular artery or arteriole. The palpable purple in VLC is circular because erythrocytes simply diffuse evenly outside the postcapillary venalles as a result of inflammation. Infectious embolisms are most commonly due to gram-negative shells (meningococcus, gonococcus), gram-negative bacilli (Enterobacteriaceae) and gram-positive shells (staphylococcus). Additional causes may be Rickettsia and, in immunocompromised patients, Candida and Aspergillus. Embolic lesions in acute meningococcalemia occur mostly on the torso, lower limbs and pressure areas and a gray-metal color often occurs. Their size varies between 1 mm and several centimeters and the micro-organism can be grown from the lesion.
The associated findings include a previous upper respiratory tract infection, fever, meningitis, disseminated intravascular coagulation and, in some patients, a deficiency of the terminal components of the complement. In disseminated gonococcal infection (arthritis-dermatitis syndrome), a small number of papules and vesicular pustules with central purpura or hemorrhagic necrosis are found around the joints of the distal extremities.
Associated symptoms include arthralgia, tenosinovitis and fever. To establish the diagnosis, Gram coloration of these lesions should be performed. Spotted fever is a tick-transmitted disease caused by Rickettsia rickettsii. A multi-day history with fever, chills, severe headache and photophobia precedes the onset of the rash. Initial lesions are macula and erythematous papules on the wrist of the fist, ankles, palms and plants. Over time, the lesions expand centripetally and become purple. Injuries of the gangrenosum ectima begin as papules or edematous, erythematous plaques, which subsequently develop central purpura and necrosis. The formation of bubbles also occurs in these lesions and they are commonly found in the areas of the belt. The classic microorganism associated with ectima gangrenosum is Pseudomonas aeruginosa, but other gram-negative bacilli, such as Klebsiella, E. Coli and Serratia, can cause similar lesions. In immunocompromised hosts, the list of potential pathogens is expanded to include Candida and Aspergillus.
It's time to tackle skin ulcers, too. In the patient's approach with skin ulcer, etiologies are divided into two large groups: 1. primary skin disorders and 2. underlying systemic conditions. In the group of primary skin disorders there are three categories: vascular, associated with tumors and infectious. The vascular peripheral group should be approached first, as it is the most common cause of pain in the extremities in adults, venous hypertension. Stasis ulcers are characteristically painless and contain adequate granulation tissue. They are often found on medial maloles, on a background of varicosities, stasis dermatitis, edema and deposits of hemosiderin (yellow-brown coloration of the skin). In contrast, ulcers of the lower limbs due to obliterating arteriosclerosis are often painful and are associated with cold, atrophic skin, alopecia and dystrophic nails (all as a reflection of a decrease in blood flow). Most patients are male and frequently have signs of atherosclerosis of other large and medium-sized arteries.
Obliterating trombangeitis (Buerger's disease) and Monckeberg arteriosclerosis are two rarer arterial diseases that can cause ulcers of the upper distal extremities, as well as of the lower extremities. The latter is found in patients with primary or secondary hyperparathyroidism and calcification of the average tunic of the affected muscle arteries occurs on X-ray as a diffuse calcification in the "pipe". Buerger's disease occurs especially in young men (25-40 years) who smoke or have been smokers. Livedoid vasculitis (atrophie blanche) is the combination of vasculopathy with intravascular thrombosis. Purple and livedo reticularis lesions are found associated with painful ulcers of the lower limbs. These ulcers often heal slowly, but when they heal, a white scar of irregular shape is formed.
Most cases are of idiopathic origin, but possible underlying systemic conditions include: systemic lupus, antiphospholipid syndrome, scleroderma, cryoglobulinemia and cryofibrinogenemia. Patients with antiphospholipid syndrome have anticardiolipin antibodies, false positive biological tests for syphilis and partially activated prolonged thromboplastin times (the latter are due to lupic anticoagulant). These antiphospholipid antibodies are most common in patients with systemic lupus, but are also associated with other connective tissue diseases. In addition to livedoid vasculitis lesions, patients with antiphospholipid syndrome have recurrent venous thrombosis, arterial thrombosis (including strokes), miscarriages and thrombocytopenia.
Several types of carcinomas may present as skin ulcers, e.g. basal cell carcinoma, squamous carcinoma and, more rarely, melanoma. When an ulcer of the lower limb does not heal despite proper treatment, it should be biopsied to remove carcinoma, especially spinal carcinoma. The same goes for ulcers that appear on scars. Bacterial and viral infections also lead to skin ulcers and one of the most often isolated agents is streptococcus. The term ectime is used to describe ulcerative lesions, often extensive, caused by this bacterium. Ectima is a primary skin condition and should not be confused with ectima gangrenosum, which is secondary to blood-borne embolisms. In Meleey ulcer, a progressively expanding ulcer begins in an area of trauma or surgery. The clinical appearance is similar to that of the gangrenosum pyoderma, but is due to a synergistic infection that usually includes anaerobic streptococci.
For a specific group of patients with skin ulcers due to an underlying systemic disease, the lower limb is the primary localization of lesions. In a young patient, ischemic skin ulcers on the leg should raise suspicion of hemoglobinopathy or hereditary spherocytosis. Intravascular thrombosis is the presumed cause of these ulcers, as for ulcers in patients with monoclonal cryoglobulinemia. Primary and secondary forms of VLC, as well as cholesterol embolisms can cause skin ulcers, as well, especially on the lower limbs. For example, lower limb ulcers in patients with rheumatoid arthritis are often due to vasculitis. In addition, the atrophic yellow plaques of lipoid necrobiosis can centrally crack into an ulcer.
Vasospasm occurs in patients with Raynaud's phenomenon and can cause mouth ulcers, as well as of the feet. The Raynaud phenomenon is defined as a triphasic reaction of pallor, cyanosis and hyperemia in response to cold or emotional stress. Vasospasm is found in patients receiving norepinephrine, vasopressin, ergotamine and bleomycin. The patient with Raynaud's disease and fingertip ulcers should be carefully examined in the search for periangle reticulated telangiectasis and discrete signs of scleroderma. Raynaud's phenomenon is also found in patients with dermatomyosis, systemic lupus, cryoglobulinemia, cervical rib syndrome and scales anticus, pneumatic hammer disease and occupational acro-osteolysis (associated with the manufacture of polyvinyl chloride).
In the gangrenosum pyoderma, the edge of the ulcer has a characteristic appearance of a blue necrotic edge atonal and an edematous peripheral halo. Ulcers often start as pustules that then quickly expand to a size of up to 20 cm. Although these lesions are most common on the lower limbs, they can occur anywhere on the surface of the body, including in areas of trauma (patergia). Approximately 30-50% of cases are idiopathic and the most common associated conditions are ulcerative colitis and Crohn's disease.
Less often, there is an association with chronic active hepatitis, seropositive rheumatoid arthritis, acute and chronic granulocytic leukemia, polycytemia vera and myeloma. Additional findings in these patients, even those with idiopathic disease, are skin anergia and benign monoclonal gammapathy. Because the histology in the gyoderma gangrenosum is nonspecific, the diagnosis is made clinically by excluding rarer causes of ulcers with a similar appearance, such as necrotizing vasculitis, Meleey ulcer, dysmorphic fungi, skin amoebiasis, spider bites and factic forms. In myeloproliferative disorders, ulcers can be superficial, with a pustulobulous edge and these lesions provide a connection between the classic gangrenosum pyoderma and acute febrile neutrophilic dermatosis (Sweet syndrome).
Clinical diagnosis of Behcet disease requires the presence of recurrent oral ulcerations (at least three times in a 12-month period), in addition to 2 to 4 of the following criteria: 1. recurrent genital ulcers, especially on the vulva and scrotum, 2. eye lesions, either uveitis or retinal vasculitis, 3. skin lesions and 4. a positive patergy test. Ulcers in the oral cavity are usually painful and well delineated, with an erythematous halo, while genital ulcers tend to be deeper and heal with scarring. Nodos erythema, "pseudofoliculitis", papulopustular lesions or acneiform nodules in a patient past adolescence and not undergoing treatment with glucocorticoids are skin lesions. The therapy test, which is defined as the reproduction of a skin lesion by trauma, is performed by injecting sterile physiological serum into the dermis. Before diagnosis of Behcet disease, the following conditions should be excluded: recurrent polymorphic erythema, herpes simplex, inflammatory bowel disease, systemic lupus and primary bullous diseases.
There's something else to say about fever and rash... The main conditions considered in a patient with fever and rash are inflammatory diseases and infectious diseases. In the hospital, the most common scenario is a patient who has a drug rash plus secondary fever to an underlying infection. However, it should be stressed that a drug reaction can lead to both a rash and fever ('drug fever'). Other inflammatory diseases that are often associated with fever include pustulous psoriasis, erythroderma and Sweet syndrome. Lyme disease, secondary syphilis and viral and bacterial exantems are examples of infectious diseases that cause infection and fever. Finally, it is important to determine whether skin lesions represent septic embolisms. Such lesions usually have signs of ischemia in the form of purpura, necrosis or imminence of necrosis (grey-metallic color). In the patient with thrombocytopenia, purple lesions can be found in inflammatory reactions, such as drug morbiliform rashes and infectious lesions.
Have a good day!
Let's continue with the purple and finalize the skin manifestations of internal diseases!
The palpable purple is also subdivided into vasculitic and embolic. In the group of vasculitic diseases, leukocytoclastic vasculitis (VLC), known as allergic vasculitis, is most often associated with palpable purpura. Purpura Henoch-Schonlein is a subtype of acute VLC that is primarily found in children and adolescents after an upper respiratory tract infection. Most of the lesions are found on the lower limbs and buttocks.
Systemic manifestations include fever, arthralgia (especially of the knees and ankles), abdominal pain, gastrointestinal bleeding and nephritis. Direct immunofluorescence examination shows deposits of IgA in the walls of dermal blood vessels. In node polyarteritis, specific skin lesions result from a vasculitis of arterial vessels, rather than postcapillary venurics as in VLC. Arteritis leads to dermal ischemia and this explains the irregular contour of the purple. Several types of infectious embolisms can cause palpable purpura. These embolic lesions are usually irregular in the contour, as opposed to lesions of leukocytoclastic vasculitis, which have circular contour.
The irregular contour indicates a skin infarction and the size corresponds to the area of skin irrigated by that particular artery or arteriole. The palpable purple in VLC is circular because erythrocytes simply diffuse evenly outside the postcapillary venalles as a result of inflammation. Infectious embolisms are most commonly due to gram-negative shells (meningococcus, gonococcus), gram-negative bacilli (Enterobacteriaceae) and gram-positive shells (staphylococcus). Additional causes may be Rickettsia and, in immunocompromised patients, Candida and Aspergillus. Embolic lesions in acute meningococcalemia occur mostly on the torso, lower limbs and pressure areas and a gray-metal color often occurs. Their size varies between 1 mm and several centimeters and the micro-organism can be grown from the lesion.
The associated findings include a previous upper respiratory tract infection, fever, meningitis, disseminated intravascular coagulation and, in some patients, a deficiency of the terminal components of the complement. In disseminated gonococcal infection (arthritis-dermatitis syndrome), a small number of papules and vesicular pustules with central purpura or hemorrhagic necrosis are found around the joints of the distal extremities.
Associated symptoms include arthralgia, tenosinovitis and fever. To establish the diagnosis, Gram coloration of these lesions should be performed. Spotted fever is a tick-transmitted disease caused by Rickettsia rickettsii. A multi-day history with fever, chills, severe headache and photophobia precedes the onset of the rash. Initial lesions are macula and erythematous papules on the wrist of the fist, ankles, palms and plants. Over time, the lesions expand centripetally and become purple. Injuries of the gangrenosum ectima begin as papules or edematous, erythematous plaques, which subsequently develop central purpura and necrosis. The formation of bubbles also occurs in these lesions and they are commonly found in the areas of the belt. The classic microorganism associated with ectima gangrenosum is Pseudomonas aeruginosa, but other gram-negative bacilli, such as Klebsiella, E. Coli and Serratia, can cause similar lesions. In immunocompromised hosts, the list of potential pathogens is expanded to include Candida and Aspergillus.
It's time to tackle skin ulcers, too. In the patient's approach with skin ulcer, etiologies are divided into two large groups: 1. primary skin disorders and 2. underlying systemic conditions. In the group of primary skin disorders there are three categories: vascular, associated with tumors and infectious. The vascular peripheral group should be approached first, as it is the most common cause of pain in the extremities in adults, venous hypertension. Stasis ulcers are characteristically painless and contain adequate granulation tissue. They are often found on medial maloles, on a background of varicosities, stasis dermatitis, edema and deposits of hemosiderin (yellow-brown coloration of the skin). In contrast, ulcers of the lower limbs due to obliterating arteriosclerosis are often painful and are associated with cold, atrophic skin, alopecia and dystrophic nails (all as a reflection of a decrease in blood flow). Most patients are male and frequently have signs of atherosclerosis of other large and medium-sized arteries.
Obliterating trombangeitis (Buerger's disease) and Monckeberg arteriosclerosis are two rarer arterial diseases that can cause ulcers of the upper distal extremities, as well as of the lower extremities. The latter is found in patients with primary or secondary hyperparathyroidism and calcification of the average tunic of the affected muscle arteries occurs on X-ray as a diffuse calcification in the "pipe". Buerger's disease occurs especially in young men (25-40 years) who smoke or have been smokers. Livedoid vasculitis (atrophie blanche) is the combination of vasculopathy with intravascular thrombosis. Purple and livedo reticularis lesions are found associated with painful ulcers of the lower limbs. These ulcers often heal slowly, but when they heal, a white scar of irregular shape is formed.
Most cases are of idiopathic origin, but possible underlying systemic conditions include: systemic lupus, antiphospholipid syndrome, scleroderma, cryoglobulinemia and cryofibrinogenemia. Patients with antiphospholipid syndrome have anticardiolipin antibodies, false positive biological tests for syphilis and partially activated prolonged thromboplastin times (the latter are due to lupic anticoagulant). These antiphospholipid antibodies are most common in patients with systemic lupus, but are also associated with other connective tissue diseases. In addition to livedoid vasculitis lesions, patients with antiphospholipid syndrome have recurrent venous thrombosis, arterial thrombosis (including strokes), miscarriages and thrombocytopenia.
Several types of carcinomas may present as skin ulcers, e.g. basal cell carcinoma, squamous carcinoma and, more rarely, melanoma. When an ulcer of the lower limb does not heal despite proper treatment, it should be biopsied to remove carcinoma, especially spinal carcinoma. The same goes for ulcers that appear on scars. Bacterial and viral infections also lead to skin ulcers and one of the most often isolated agents is streptococcus. The term ectime is used to describe ulcerative lesions, often extensive, caused by this bacterium. Ectima is a primary skin condition and should not be confused with ectima gangrenosum, which is secondary to blood-borne embolisms. In Meleey ulcer, a progressively expanding ulcer begins in an area of trauma or surgery. The clinical appearance is similar to that of the gangrenosum pyoderma, but is due to a synergistic infection that usually includes anaerobic streptococci.
For a specific group of patients with skin ulcers due to an underlying systemic disease, the lower limb is the primary localization of lesions. In a young patient, ischemic skin ulcers on the leg should raise suspicion of hemoglobinopathy or hereditary spherocytosis. Intravascular thrombosis is the presumed cause of these ulcers, as for ulcers in patients with monoclonal cryoglobulinemia. Primary and secondary forms of VLC, as well as cholesterol embolisms can cause skin ulcers, as well, especially on the lower limbs. For example, lower limb ulcers in patients with rheumatoid arthritis are often due to vasculitis. In addition, the atrophic yellow plaques of lipoid necrobiosis can centrally crack into an ulcer.
Vasospasm occurs in patients with Raynaud's phenomenon and can cause mouth ulcers, as well as of the feet. The Raynaud phenomenon is defined as a triphasic reaction of pallor, cyanosis and hyperemia in response to cold or emotional stress. Vasospasm is found in patients receiving norepinephrine, vasopressin, ergotamine and bleomycin. The patient with Raynaud's disease and fingertip ulcers should be carefully examined in the search for periangle reticulated telangiectasis and discrete signs of scleroderma. Raynaud's phenomenon is also found in patients with dermatomyosis, systemic lupus, cryoglobulinemia, cervical rib syndrome and scales anticus, pneumatic hammer disease and occupational acro-osteolysis (associated with the manufacture of polyvinyl chloride).
In the gangrenosum pyoderma, the edge of the ulcer has a characteristic appearance of a blue necrotic edge atonal and an edematous peripheral halo. Ulcers often start as pustules that then quickly expand to a size of up to 20 cm. Although these lesions are most common on the lower limbs, they can occur anywhere on the surface of the body, including in areas of trauma (patergia). Approximately 30-50% of cases are idiopathic and the most common associated conditions are ulcerative colitis and Crohn's disease.
Less often, there is an association with chronic active hepatitis, seropositive rheumatoid arthritis, acute and chronic granulocytic leukemia, polycytemia vera and myeloma. Additional findings in these patients, even those with idiopathic disease, are skin anergia and benign monoclonal gammapathy. Because the histology in the gyoderma gangrenosum is nonspecific, the diagnosis is made clinically by excluding rarer causes of ulcers with a similar appearance, such as necrotizing vasculitis, Meleey ulcer, dysmorphic fungi, skin amoebiasis, spider bites and factic forms. In myeloproliferative disorders, ulcers can be superficial, with a pustulobulous edge and these lesions provide a connection between the classic gangrenosum pyoderma and acute febrile neutrophilic dermatosis (Sweet syndrome).
Clinical diagnosis of Behcet disease requires the presence of recurrent oral ulcerations (at least three times in a 12-month period), in addition to 2 to 4 of the following criteria: 1. recurrent genital ulcers, especially on the vulva and scrotum, 2. eye lesions, either uveitis or retinal vasculitis, 3. skin lesions and 4. a positive patergy test. Ulcers in the oral cavity are usually painful and well delineated, with an erythematous halo, while genital ulcers tend to be deeper and heal with scarring. Nodos erythema, "pseudofoliculitis", papulopustular lesions or acneiform nodules in a patient past adolescence and not undergoing treatment with glucocorticoids are skin lesions. The therapy test, which is defined as the reproduction of a skin lesion by trauma, is performed by injecting sterile physiological serum into the dermis. Before diagnosis of Behcet disease, the following conditions should be excluded: recurrent polymorphic erythema, herpes simplex, inflammatory bowel disease, systemic lupus and primary bullous diseases.
There's something else to say about fever and rash... The main conditions considered in a patient with fever and rash are inflammatory diseases and infectious diseases. In the hospital, the most common scenario is a patient who has a drug rash plus secondary fever to an underlying infection. However, it should be stressed that a drug reaction can lead to both a rash and fever ('drug fever'). Other inflammatory diseases that are often associated with fever include pustulous psoriasis, erythroderma and Sweet syndrome. Lyme disease, secondary syphilis and viral and bacterial exantems are examples of infectious diseases that cause infection and fever. Finally, it is important to determine whether skin lesions represent septic embolisms. Such lesions usually have signs of ischemia in the form of purpura, necrosis or imminence of necrosis (grey-metallic color). In the patient with thrombocytopenia, purple lesions can be found in inflammatory reactions, such as drug morbiliform rashes and infectious lesions.
Have a good day!
Dorin, Merticaru