Exfoliative dermatitis is a condition which causes exfoliation and redness of the skin. Possible causes include other underlying skin conditions, medications, malignancy or undeterminable factors.
Presentation
At its initial stage, exfoliative dermatitis presents with extensive regions of the body assuming a red color, hence the alternative name "erythroderma". The reddened skin becomes inflamed and begins to exfoliate [8]. An itchy sensation and pain often accompany the exfoliation.
Systemic symptoms may also be present; patients may be febrile (T >38°C), experience chills and tachycardic phenomena. Internal temperature regulation is impaired, as large regions of the skin fall off. An additional complication of the skin loss is hypovolemia and dehydration: water contained in the epidermis are lost, which leads to a reduced volume of circulating fluids. Patients are also subject to protein loss, which results to malnutrition.
Other symptoms that may accompany the condition are lymphadenopathy, hepatomegaly, splenomegaly primarily associated with an underlying lymphoma, alopecia, weight loss, steatorrhea, edema and nail thickening.
Lastly, severe complications that can manifest secondarily to exfoliative dermatitis include infections due to loss of the protective epidermal layer, electrolyte imbalance and cardiac arrest [9], with patients largely succumbing to cardiac arrest, pneumonia or septic shock.
Entire Body System
- Anemia
Microcytic hypochromic anemia, elevated erythrocyte sedimentation rate, eosinophilia, low serum proteins and electrolytes were salient laboratory features. [ncbi.nlm.nih.gov]
[…] prolonged blood levels with low dosage and has been used most for urinary-tract infections. 1 2 3 4 Unfavorable reactions involving fever and urticaria, 5 headache, anorexia and sore throat, 6 thrombocytopenic purpura, 7, 8 myocarditis 9 and aplastic anemia [nejm.org]
Elevated erythrocyte sedimentation rate, eosinophilia, leukocytosis, peripheral blood lymphocytosis and anemia were the most common laboratory findings. [scielo.br]
A pseudolymphoma reaction with fever, arthralgias, lymphadenopathy, hepatosplenomegaly, anemia and erythroderma may develop as a result of hypersensitivity to dapsone or antiepileptic drugs. [aafp.org]
- Constitutional Symptom
[…] der·ma·ti·tis ex·fo·li·a·ti·va in·fan·tum, dermatitis exfoliativa neonatorum a generalized pyoderma accompanied by exfoliative dermatitis, with constitutional symptoms, affecting young infants, which may result from atopic dermatitis, Leiner disease, [medical-dictionary.thefreedictionary.com]
No constitutional symptoms. An attack runs its course in about three weeks. [doctortreatments.com]
symptoms such as fatigue and fever [ 2 ]. [clinical-pediatrics-dermatology.imedpub.com]
Patients with erythroderma complain of constitutional symptoms such as fever, chills and even rigors, and pruritus. Diffuse alopecia, keratoderma, nail dystrophy and weight loss are more common in the chronic state. [clinicaladvisor.com]
- Nail Abnormality
Skin manifestations involved scalp (83.0%), mucous membrane (38.3%), and nail (36.2%). Most nail abnormalities were observed in patients with psoriasis, pityriasis rubra pilaris (PRP), and ichthyosis. [astrocyte.in]
Hair loss and nail abnormalities may accompany exfoliation. Widespread lymph node enlargement, hepatosplenomegaly, and low-grade fever are common. [randyjacobsmd.com]
- Anorexia
It has the advantage of prolonged blood levels with low dosage and has been used most for urinary-tract infections. 1 2 3 4 Unfavorable reactions involving fever and urticaria, 5 headache, anorexia and sore throat, 6 thrombocytopenic purpura, 7, 8 myocarditis [nejm.org]
There was no history of fever, weight loss, anorexia or weakness. Dermatological examination revealed lichenification, erythema and exfoliation of the skin of the entire body. [ijdvl.com]
Respiratoric
- Sore Throat
It has the advantage of prolonged blood levels with low dosage and has been used most for urinary-tract infections. 1 2 3 4 Unfavorable reactions involving fever and urticaria, 5 headache, anorexia and sore throat, 6 thrombocytopenic purpura, 7, 8 myocarditis [nejm.org]
Gastrointestinal
- Diarrhea
We describe a case of a 22-year old woman admitted to our hospital with acute diffuse, pruritic rash associated with crampy abdominal pain, vomiting, diarrhea and fever three weeks after starting sulfasalazine therapy. [ncbi.nlm.nih.gov]
[…] smegma Exfoliative dermatitis, generalized Generalized exfoliative dermatitis Generalized granuloma annulare Genitocrural intertrigo Granuloma annulare Granuloma gluteale infantum Increased desquamation Infantile erythroderma, failure to thrive and diarrhea [icd9data.com]
Presenting symptoms included itching (76.6%), fever (38.3%), painful sensation (17.0%), and diarrhea (12.8%). [astrocyte.in]
Erythroderma may be the first clinical manifestation of an immunodeficiency syndrome, especially in the presence of diarrhea and profound failure to thrive. Immunodeficiency screening must be considered in these cases. [clinicaladvisor.com]
Common acute symptoms include abdominal pain or cramps, nausea, vomiting, and diarrhea, jaundice, skin rash and eyes dryness and therefore could mimic the prodromal and early phase of ED. [clinicalmolecularallergy.biomedcentral.com]
- Loss of Appetite
The skin starts shed off, leading to problems like temperature regulation, protein and fluid loss as well as an increased metabolic rate, infection, hair loss, loss of appetite/ nutrients and dehydration. [drthindhomeopathy.com]
Cardiovascular
- Hypertension
Captopril, an angiotensin converting enzyme blocker, has recently been released for limited clinical use in the treatment of refractory congestive heart failure and severe hypertension. [ncbi.nlm.nih.gov]
Hypothermia may result from the bodies inability to control heat and this can be life threatening since it can cause a decrease in heart rate and subsequent hypertension. [checkorphan.org]
Anti-hypertensive medicines, antibiotics, calcium channel blockers, are few of the drugs that may be responsible for exfoliative dermatitis. Underlying skin condition is also responsible for exfoliative dermatitis. [tandurust.com]
Case two involved a 75-year-old lady whose medical problems included porphyria cutanea tarda, vasculitis, hypertension, osteoporosis and vitamin B12 deficiency. [ageing.oxfordjournals.org]
Musculoskeletal
- Fracture
[…] lungs and compression fractures of the T12 and L1 vertebral bodies. [spandidos-publications.com]
By inhibiting bone resorption, the medication increases bone mineral density and reduces fracture incidence [ 1 ]. Diarrhoea is the commonest side effect; skin rashes are recognised. [ageing.oxfordjournals.org]
When water trickles down into fractures and pores of rock, then freezes, its volume increases by almost 10 percent. This causes outward pressure of about 30,000 pounds per square inch at -7.6 Fahrenheit. [imnh.isu.edu]
- Osteoporosis
Strontium ranelate is a relatively new drug used as a second-line treatment for osteoporosis, often targeted at older patients. [ncbi.nlm.nih.gov]
Abstract Strontium ranelate is a relatively new drug used as a second-line treatment for osteoporosis, often targeted at older patients. [ageing.oxfordjournals.org]
- Foot Pain
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Skin
- Eruptions
The eruptions with pruritus resolved within 1 wk after the operation. This is the first report, to our knowledge, of coincident biliary malignancy and erythroderma. [ncbi.nlm.nih.gov]
[…] of the "fixed eruption" type, urticaria and the generalized or universal exfoliative dermatitis, which may be fatal. [jamanetwork.com]
- Eczema
Psorinum: • Intense itching that must be scratched till it bleeds. • Dirty appearance of skin. • Eczema behind ears, despair and depression with eczema. • Infected eczema, offensiveness of all discharges especially of eczema. • Person anxious and despair [drthindhomeopathy.com]
The patient had neither a personal history of skin disorders or, specifically, atopic eczema or psoriasis nor a family history of eczema or psoriasis. He also had no history of malignancy and was taking no medications. [ncbi.nlm.nih.gov]
eczema is common in people who have frequent eczema outbreaks. [loupapostpal.lea.mx]
Erythroderma (disorder), Erythrodermic eczema (disorder), Generalised erythroderma, Generalized erythroderma (disorder), Generalized exfoliative dermatitis (disorder), Pityriasis rubra (Hebra) (disorder), Hebra; pityriasis, dermatitis; exfoliative, [fpnotebook.com]
- Pruritus
The eruptions with pruritus resolved within 1 wk after the operation. This is the first report, to our knowledge, of coincident biliary malignancy and erythroderma. [ncbi.nlm.nih.gov]
Symptoms and Signs Symptoms include pruritus, malaise, and chills. Diffuse erythema initially occurs in patches but spreads and involves all or nearly all of the body. [msdmanuals.com]
It is often associated with the loss of hair and nails, hyperkeratosis of the palms and soles, and pruritus. [sideeffects.embl.de]
Exfoliative dermatitis is referred to as extensive scaling of the skin, primarily with pruritus (itching), hair loss and erythroderma (skin redness). [medicalpicturesinfo.com]
- Alopecia
Alopecia is common too. Hypo-pigmentation and/or hyper-pigmentation may be a clinical feature. Firm, rubbery lymph nodes are seen, these are referred to as dermatopathic lymphadenitis. Enlargement of the liver and / or spleen are seen as well. [simple-remedies.com]
Alopecia of the skull is developed by a quarter of the patients. Those with this disease are also at risk of getting methicillin-resistant staphylococcus aureus. [checkorphan.org]
In many cases the scalp is not spared and it may also result in alopecia. The skin becomes thick. There may be severe itching. The patient may feel malaise and may also feel chilly. Swollen lymph nodes in different parts of the body. [tandurust.com]
Mild to severe alopecia and transient or permanent nail dystrophy also may be encountered. [aafp.org]
- Skin Rash
Subsequently, treatment of the patient with PPIs was terminated, after which the tracheal hemoptysis and skin rashes markedly improved. In addition, no new skin rashes appeared following termination of the PPI treatment. [spandidos-publications.com]
A 6-year-old Caucasian girl experienced a generalized erythematous skin rash during carbamazepine therapy. Over the next four days the eruption worsened into erythroderma with fever and generalized lymphadenopathy. [ncbi.nlm.nih.gov]
Rashes Also called: Dermatitis, Skin rash A rash is an area of irritated or swollen skin. Many rashes are itchy, red, painful, and irritated. Some rashes can also lead to blisters or patches of raw skin. [icdlist.com]
Neurologic
- Headache
It has the advantage of prolonged blood levels with low dosage and has been used most for urinary-tract infections. 1 2 3 4 Unfavorable reactions involving fever and urticaria, 5 headache, anorexia and sore throat, 6 thrombocytopenic purpura, 7, 8 myocarditis [nejm.org]
Urogenital
- Incontinence
Search: Home Bath Safety Braces Impotence & Male ED Incontinence Mobility Shop By Categories Shop by Topic Gift Ideas Respiratory Miscellaneous Home Bath Safety Bath Lifts Bathing Aids - Hygiene Bidet Toilet Seats Commodes and Accessories Grab Bars Shower [activeforever.com]
Workup
Exfoliative dermatitis is clinically diagnosed. What is of vital importance is the underlying condition. Upon a thorough physical examination, a healthcare provider should perform tests in order to detect the cause of erythroderma, based on their suspicions.
Useful tests include blood tests in order to assess liver biochemistry, creatinine levels, erythrocyte sedimentation rate (ESR) [10], white blood cells and their predominant sub-type, albumin, immunoglobulin E and many other markers that will suggest the underlying pathology. The most common findings include an increased ESR, eosinophilia and anemia.
Biopsy specimens are usually reserved for the lymph nodes if there is co-existent lymphadenopathy [10].
Serum
- Hypoalbuminemia
Also, it affects the production and metabolism of an important carrier protein called albumin leading to a condition called hypoalbuminemia, which is very devastating and can be life threatening. [aarogya.com]
Increased dehydration due to transpiration (similar to burns) All lead to negative nitrogen balance and characterized by edema, hypoalbuminemia, loss of muscle mass. Shri B. M. [slideshare.net]
Systemic and potentially life-threatening complications include fluid and electrolyte imbalance, thermoregulatory disturbance, fever, tachycardia, high-output failure, hypoalbuminemia, and septicemia. [accessmedicine.mhmedical.com]
Potential systemic manifestations include fever, chills, hypothermia, reactive adenopathy, peripheral edema, hypoalbuminemia and heart failure. [doctortipster.com]
Laboratory investigations demonstrated thrombocytosis, eosinophilia, elevated ESR, anemia, leukocytosis, abnormal liver functions, and hypoalbuminemia [Table 2]. [astrocyte.in]
Treatment
Since exfoliative dermatitis results from various distinct pathologies, an appropriate treatment plan can be decided upon diagnosis of the causes. Until a definitive cause detection is possible, conservative therapy is mandatory, in order to prevent dehydration and electrolyte imbalance, infection of the exfoliated skin patches and hypovolemia. Locally, the skin is soothed with emollients, compresses bathed in slightly heated water and antihistamines per os [11] [12]. Most patients require hospitalization, which allows for better patient evaluation and monitoring.
In cases of individuals suffering from idiopathic exfoliative dermatitis with a chronic course, systemic steroids may be administered, but only if psoriasis has been eliminated as an underlying disease. If a patient exhibits concomitant psoriasis, retinoids can be given. Immunosuppresive medication (methotrexate or azathioprine) are also a possibility [13], whereas psoralen plus ultraviolet A phototherapy (PUVA) may be of use in other sub-categories. It is vital that patients refrain from scratching the exfoliated regions, in order to achieve faster recovery and less abrasions [14].
Prognosis
The cause of exfoliative dermatitis also determines its prognosis. the best prognosis is reserved for patients with an underlying skin disease, such as psoriasis, since the initial condition is itself non-fatal. In cases where exfoliative dermatitis has been caused by an underlying malignancy (leukemia), a drug hypersensitivity reaction or SSSS (staphylococcal scalded skin syndrome), patients are expected to show signs of an aggressive disease course.
Data [7] has suggested that the following factors increase the possibility of a child patient developing hypotension:
- Age <3 years old
- PLT count < 300,000/μL (platelet count)
- Ca <8.6 mg/dL (calcium levels)
- Glucose <110 mg/dL
- Polymorphonuclear leukocyte count > 80%
- Nausea, vomiting
- Sickly in appearance
- Increased levels of creatinine
Fever is generally considered a bad prognostic factor.
Children younger than 3 years old, with exfoliative dermatitis, hypotension, sickly appearance and a high creatinine value run a higher risk of developing toxic shock syndrome.
Mortality rates fluctuate between 20 and 40%. In 1/5 of the instances, death occurs from factors irrelevant to exfoliative dermatitis.
Etiology
It is difficult to establish a firm hierarchy of causes leading to exfoliative dermatitis. A 15-45% of the patients develop the condition as a result of idiopathy, 10-40% exhibit a systemic disease as a cause and the same percentage of individuals suffer from other skin disorders which lead to exfoliative dermatitis. A smaller sub-category of patients, amounting to 3-10%, develop the disease as a result of reaction to a drug. The following list includes the most frequent factors that could account for the onset of exfoliative dermatitis, even though the actual list comprises many more, uncommon diseases:
- Systemic causes: cutaneous T-cell lymphoma (CTCL), multiple myeloma, leukemia [3], Hodgkin disease, graft vs host disease, lung/prostate/thyroid cancer and conditions rendering an individual immunodeficient (eg. HIV infection).
- Skin disorders: Psoriasis, dermatitis (atopic, contact, seborrheic, stasis), bullous pemphigoid, dermatophytosis, pityriasis rubra pilaris and mycosis fungoides.
- Reaction to the following drugs: allopurinol, dimercaprol, antibiotics (penicillin, cephalosporins, isoniazid, trimethoprim-sulfamethiazole), phenytoin, carbamazepine, iodine, mercury, barbiturates, codeine.
Epidemiology
Men have been found to be more frequently affected by exfoliative dermatitis than women, at a 2.3:1 ratio, with the condition being mostly manifested around the age of 55, naturally with exceptions [2].
The incidence per ce has not yet been determined, but erythroderma is believed to account for 1% of all patients that require hospitalization due to a skin disorder [4].
Pathophysiology
The pathophysiological mechanisms involved in the clinical manifestations of exfoliative dermatitis depend on the condition's cause. In general, however, the regeneration rate of the epidermal cells of these patients has been found to be significantly increased. Cells found in the germinative layer, the deepest epidermal skin layer from which new tissue is formed, are considerably more in number than in healthy individuals. In addition, the process of cell apoptosis and replacement requires less time to complete. As a result of these abnormalities, the epidermal layer is frequently replaced, which results in the manifestation of the exfoliating symptom.
Complex interactions between interleukins (IL-1, IL-2), the tumor necrosis factor (TNF) and the intercellular adhesion molecule 1 (ICAM-1) are believed to play an important pathogenetic role in this condition [5]. A further notable difference between the characteristics of a normal epidermal turnover procedure and that of exfoliative dermatitis, is that in healthy individuals, the exfoliated skin contains no proteins, amino acids or nucleic acids; in the case of exfoliative dermatitis, these substances are disposed off in great quantities with exfoliation [6].
Prevention
There are no specific guidelines for the prevention of exfoliative dermatitis. An individual that suffers from a diagnosed pathology known to cause erythroderma should follow their physician's directions in order to maintain a health status as good as possible and reduce the possibility of developing exfoliative dermatitis.
Summary
Erythroderma is another term used to refer to to exfoliative dermatitis, a condition which causes erythema and skin exfoliation. It is a disease that is not frequently observed but its causes must be investigated thoroughly.
Exfoliative dermatitis often occurs due to factors that are impossible to establish and is therefore deemed idiopathic. However, it can also arise as a result of another skin condition, cancer or reaction to drugs [1] [2]. The type of cancer most frequently linked to the manifestation of exfoliative dermatitis is the T-cell lymphoma; it may still remain undetected for a long period of time after the dermatitis has appeared.
Due to the possibility of an underlying malignancy, hospitalization is preferred upon the diagnosis of exfoliative dermatitis, so as to extensively investigate its causes. The administration of drugs associated with the condition should be interrupted, electrolyte imbalance should be restored and the skin should be protected from and treated for any potential or existing infection.
Patient Information
Exfoliative dermatitis, otherwise known as erythroderma, is a condition that involves the exfoliation of extensive skin areas. The patient's skin initially becomes red and inflamed, and is gradually shed, leading to loss of water, proteins and temperature control impairment.
Regarding its causes, it is a result of many underlying conditions, skin conditions, cancer or drug allergy; in 1/4 of the cases, the causes cannot be determined. Common diseases that can lead to exfoliative dermatitis are autoimmune disorders (psoriasis, atopic dermatitis, pityriasis rubra pilaris), systemic diseases (leukemia, Hodgkin disease, lung cancer, HIV infection) or drugs, such as penicillin, barbiturates, codeine etc. In the latter case, if an affected individual stops the medication, they will recover, unless they have already sustained a complication.
The clinical picture is quite obvious. The skin starts to assume a reddish color in limited locations and then begins to fall off (exfoliate). Even though this is initially observed in some skin patches, these continue to expand and, as a result, big areas of the body end up being affected. The skin may itch; scratching the skin increases the possibility of infection, which is anyway present, since the skin loses its protective barrier to exfoliation. Other symptoms are: large lymph nodes, fever, weight loss. There is a great variety of symptoms because the condition is caused by many underlying diseases, each of which is accompanied by its own set of manifestations.
Exfoliative dermatitis can sometimes lead to severe, life-threatening complications. Skin infection can induce sepsis, where bacteria enter the bloodstream and shock. Patients need to be hospitalized in order to be monitored properly and have the cause of their condition detected. treatment varies, depending on the underlying disease. General measures include emollients and compresses are used to soothe the inflamed skin, alongside antihistamines.
References
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- Wolf R, Kahane E, Sandbank M. Mycosis fungoides-like lesions associated with phenytoin therapy. Arch Dermatol. 1985; 121:1181–2.
- Ipek Y, Hulya D, Melih A. Disseminated exfoliative dermatitis associated with all-transretinoic Acid in the treatment of acute promyelocytic leukemia. Case Report Med. 2012; 236174.
- Gentele H, et al. Dermatitis exfoliativa. Acta Derm Venereol. 1958; 38:269–302.
- Wilson DC, Jester JD, King LE Jr. Erythroderma and exfoliative dermatitis. Clin Dermatol. 1993; 11:67–72.
- Hild DH. Folate losses from the skin in exfoliative dermatitis. Arch Intern Med. 1969; 123:51–7.
- Byer RL, Bachur RG. Clinical deterioration among patients with fever and erythroderma. Pediatrics. Dec 2006; 118(6):2450-60.
- Gibson LE, Perry HO. Papulosquamous eruptions and exfoliative dermatitis. In: Moschella SL, Hurley HJ, eds. Dermatology. 3d ed. Philadelphia: Saunders; 1992; 607–51.
- Grice KA, Bettley FR. Skin water loss and accidental hypothermia in psoriasis, ichthyosis, and erythroderma. Br Med J. 1967; 4:195–8.
- Wilson DC, Jester JD, King LE Jr. Erythroderma and exfoliative dermatitis. Clin Dermatol. 1993; 11:67–72.
- Rosen T. Exfoliative dermatitis (erythroderma). In: Maddin S, Carruthers A, Brown TH, eds. Current dermatologic therapy. Philadelphia: Saunders. 1982; 169–70.
- Shelley WB, Shelley ED. Erythroderma. In: Shelley WB, Shelley ED, eds. Advanced dermatologic therapy. Philadelphia: Saunders. 1987; 185–9.
- Mogavera HS. Exfoliative dermatitis. In: Provost TT, Farmer ER, eds. Current therapy in dermatology. 2d ed. Philadelphia: Decker. 1988; 20–1.
- Cataldo MF, Varni JW, Russo DC, et al. Behavior therapy techniques in treatment of exfoliative dermatitis. Arch Dermatol. 1980; 116:919–22.