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.
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].
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.
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- 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.