What is Erythroderma (Exfoliative Dermatitis)?
Erythroderma is a rare but serious condition of the skin that affects almost the whole cutaneous surface. It is characterized by inflammations, erythema and scaling. Although the underlying etiology is frequently unknown, the condition mostly occurs as a result of some drug reaction or a malignant disorder.
Erythroderma is also referred to by various other names, such as:
- Exfoliative dermatitis
- Exfoliative erythroderma
- Red man syndrome
- Dermatitis exfoliativa
Exfoliative dermatitis is a rare condition that affects 1-3% of the entire population. It is known to affect people of all races and countries. People who are more than 40 years of age have a greater risk of developing this disorder. The condition is more prevalently seen in men, with the male to female ratio being 2:1.
Exfoliative dermatitis is mostly caused by the exacerbation of some underlying skin disease or a serious drug reaction. Instances of primary erythroderma are very rare and commonly associated with cutaneous T-cell lymphoma, especially in Sézary’s disease. In some cases, it can be caused by idiopathic factors as well. The various factors that can lead to the development of this condition are listed below:
- Hodgkin disease
- Multiple myeloma
- Graft versus host disease
- Primarily cutaneous T-cell lymphoma
- Immunodeficiency disorders, such as HIV
- Carcinoma of the lungs, colon, prostate and thyroid
- Lichen planus
- Stasis dermatitis
- Atopic dermatitis
- Contact dermatitis
- Mycosis fungoides
- Pemphigus foliaceus
- Pityriasis rubra Pilaris
- Seborrheic dermatitis
- Psoriasis (psoriatic erythroderma)
- Congenital ichthyosiform erythroderma
Drugs and Medications
- Granulocyte colony-stimulating factor or GCSF
- Antimicrobials, such as Sulfas, cephalosporins, penicillin, minocycline and isoniazid
The symptoms of this skin disorder can develop gradually or occur with a sudden onset. The whole surface of the skin may appear red and shiny. The skin starts thickening along with the formation of crusts and scales. Loss of hair and nails may occur along with itching. Swollen lymph nodes start to appear. Patients experience heat loss, which is felt as a sensation of cold and fever. The skin loses fluids and protein, thereby becoming vulnerable to many secondary infections. The condition first appears around the neck, the skull and the genitals and then spreads throughout the entire body. After some time, the total cutaneous surface is covered with numerous pruritic eruptions. The palms, hands and soles are generally not affected. Some patients may suffer from alopecia of skull. They also run the risk of getting infected by methicillin-resistant Staphylococcus aureus. Hypothermia might result from the inability of the body to control heat which can lead to reduced heart rate and hypertension, which can be critical. The propensity for cardiac failure is amplified by high blood pressure. Affected individuals may also lose weight rapidly.
Other common findings associated with Exfoliative dermatitis include:
- Relative tachycardia
- Signs of gynecomastia
- Transcutaneous fluid loss
- Increased cutaneous blood flow
- Hyperpigmentation and/or hypopigmentation
- Thyroid or prostate glands may become enlarged or nodular
- Dermatopathic lymphadenitis characterized by firm, rubbery lymphadenopathy
While determining the presence of this skin disorder, a doctor first conducts a thorough medical examination and inquires about the medical history of his patient. He is then likely to perform certain diagnostic tests which will include blood work and biopsies. The diagnostic tests carried out to evaluate whether or not a person is having Exfoliative dermatitis are listed below:
- Skin biopsies
- Creatinine levels
- Lymph node biopsy
- Liver enzymes levels
- Serum albumin levels
- Evaluation for renal failure
- Complete blood count (CBC)
- Evaluation for cardiac failure
- Evaluation for intestinal dysfunctions
- Erythrocyte sedimentation rate or ESR
The most commonly detected laboratory abnormalities include:
- Elevated erythrocyte sedimentation rate
Imaging studies are recommended on the basis of the underlying systemic diseases or factors. Histological findings are usually nonspecific and generally include parakeratosis, hyperkeratosis and acanthosis of epidermis, as well as perivascular infiltrate that consists of lymphocytes, histocytes, and some eosinophils.
Erythroderma Differential Diagnosis
A number of medical conditions exhibit signs and symptoms similar to that of Exfoliative dermatitis. Hence, while determining the presence of this condition, it should be differentiated against from these similar-appearing disorders. The differential diagnoses of this disease involve isolating its symptoms from those of similar disorders such as:
- Lichen Planus
- Plaque Psoriasis
- Stasis Dermatitis
- Reactive Arthritis
- Atopic Dermatitis
- Bullous Pemphigoid
- Acanthosis Nigricans
- Pemphigus Foliaceus
- Erythema Multiforme
- Pityriasis Rubra Pilaris
- Seborrheic Dermatitis
- Toxic Shock Syndrome
- Graft Versus Host Disease
- Irritant Contact Dermatitis
- Pediatric Kawasaki Disease
- Toxic Epidermal Necrolysis
- Allergic Contact Dermatitis
- Cutaneous T-Cell Lymphoma
- Staphylococcal Scalded Skin Syndrome
- Congenital ichthyosiform erythroderma
- Familial Benign Pemphigus (Hailey-Hailey Disease)
Early treatment of this skin disorder is essential to keep loss of fluids and protein to a bare minimum as well as to prevent the occurrence of severe infections. Treatment of this condition depends on the underlying causes. A patient is normally hospitalized and given intravenous fluids. The electrolyte and fluid levels of a patient are monitored closely to prevent dehydration.
Topical steroids are primarily used to treat the symptoms of Exfoliative dermatitis (ED). The sedative antihistamine can be recommended to the pruritic patients, as it can help to have a good sleep at night, thereby reducing the nocturnal excoriations and scratching. Antibiotics are administered to fight the skin infections which are combined with cool baths. Antimicrobial agents are often used if the infection is believed to be precipitating to the neighborhood areas or complicating the Exfoliative dermatitis. Doctors generally apply a petroleum jelly onto the affected portions and then cover the wounded portions with gauze. Corticosteroid medications like Prednisone can help to cure underlying disorders such as lymphoma. Doctors may also use immunosuppressant medications like Cyclosporine and phototherapy to manage a patient’s condition. Patients should avoid scratching as it spreads the infections. Vitamin C, biotin, and folic acid may be used as supplements to treat the disorder.
Withdrawal of drugs and medications that might have caused Exfoliative dermatitis can lead to a complete recovery within a very short period of time. When a patient is systematically treated for the underlying skin disorders, recuperation sets in but might take longer time to be apparent.
If sufferers develop complications such as pneumonia or heart attack, they might even die. Other possible health complications include:
- Heart failure
- Electrolyte imbalance
- Fungal or bacterial superinfections
The long term prognosis for patients with drug-induced Erythroderma is generally good after the usage of the drug has been withdrawn and appropriate supportive measures are followed. The outcome is however, poor for patients having Exfoliative dermatitis caused by idiopathic factors. Frequent recurrences of the condition or chronic signs and symptoms might need long-term steroid therapy along with its attendant sequelae. For individuals suffering from an underlying disorder or malignancy, the prognosis depends on the course of the condition and the outcome of its process.
The following images will give you a clearer idea about the skin abnormalities that are associated with this condition.