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National Skin Centre, Singapore Guidelines on Erythroderma/ Generalised Exfoliative Dermatitis Definition Erythroderma or generalized exfoliative dermatitis (GED) is defined as an inflammatory skin disorder with erythema and scaling occurring in a generalized distribution involving more than 90% of the body surface area. The challenge for the clinician is to identify and treat the underlying disease. Etiology 5 broad categories of underlying causes are: 1.) Preexisting dermatoses (53%)1: I. Atopic dermatitis II. Psoriasis III. Pityriasis rubra pilaris IV. Seborrhoeic dermatitis V. Contact dermatitis, including airborne and phytophotocontact dermatitis VI. Chronic actinic dermatitis VII. Ichthyoses (infants and young children) 2.) Drug eruptions (5%) e.g. antiepileptics, antihypertensives (captopril, frusemide), antibiotics (penicillins, sulphonamides), allopurinol 3.) Malignancy (16%) I. CTCL (including Sezary syndrome) – most common II. Hodgkin’s disease – 2nd most common III. Non-Hodgkin’s lymphoma IV. Leukemia, myelodysplasia V. Visceral malignancies (rarely) e.g. prostate, thyroid, lung, liver, ovarian, rectal, mammary 4.) Miscellaneous disorders (rare) I. Immunobullous disorders e.g. pemphigus foliaceus II. Lichen planus III. Crusted scabies IV. Dermatophytosis, candidiasis V. AIDS VI. GVHD, including post-operative exfoliative dermatitis 5.) Idiopathic (26%) History Page 1 It is important to ascertain the etiology of GED in order to manage it properly. The general medical history may reveal a preexisting dermatosis such as atopic dermatitis or psoriasis, or a family or personal history of atopy. The patient may have had characteristic psoriatic plaques, flexural dermatitis or immunobullous skin lesions for months or years prior to the development of GED. A drug history and history of contact with chemicals or topical medicaments is important. Aggravating factors, such as sunlight, should be elicited. A systemic review, including loss of weight, night sweats & bowel changes, should be undertaken to evaluate for malignancy. History is also targeted at eliciting complications of GED, such as cardiac failure leading to orthopnoea & pedal oedema, hypoproteinemia leading to pedal oedema, hypothermia & hyperthermia resulting in chills. Clinical Examination The relative proportion of erythema & scaling do not contribute to the delineation of the underlying disorder. Areas of involvement and percentage of body surface area affected should be charted. Islands of sparing are typically seen in pityriasis rubra pilaris, and “deck-chair sign” in papuloerythroderma of Ofuji, but they are not specific for these conditions. Keratoderma of palms and soles may be a sign of pityriasis rubra pilaris or chronic idiopathic erythroderma: the latter may have a higher incidence of progression to CTCL. Dystrophic nails and hair loss also seem to be associated with more severe and chronic erythroderma. Lymphadenopathy occurs in up to 71%: this may be due to dermatopathic lymphadenopathy (80%) or secondary to lymphoma (20%). A thorough general examination including chest examination, palpation of the abdomen, per rectal examination, breast & pelvic examination (in female) should also be undertaken. Complications of erythroderma should be specifically looked for. Pitting oedema of the legs may indicate high-output cardiac failure (if in association with lung crepitations) or hypoproteinemia, but pedal oedema is more commonly due to inflammation of the skin. Other complications to note are hyper- or hypothermia, secondary impetinization, dehydration, pallor (anemia), malnutrition (exacerbated by extensive scaling, oozing or hypermetabolism). Investigations Routine laboratory evaluation in GED is generally not helpful in determining a specific diagnosis, with the exception of histopathology in the diagnosis of cutaneous lymphomas. Specific investigations may be useful where clinically indicated, in evaluation for underlying maliganancy. A) The following basic screening investigations are recommended: Page 2 Full blood count and ESR: INFECTION? Peripheral blood film Buffy coat for Sezary cells> fsp ou 1000/mm3: LYMPHOME Urea, creatinine & electrolytes; PERTES D’EAU Liver function test: HYPOALBUINEMIE Chest X-ray Skin biopsy for histology (at least 2 sites); DIF if immunobullous disease suspected Total IgE and LDH (senile erythroderma with serum hyperIgE) Stool for occult blood x 3 Urinalysis Tumour markers (not routinely done by other centres. Tumor markers are not deemed suitable for screening for malignant disease; their principal applications are in monitoring of therapy and prognosis. Ref: Harrisn’s Principles of Internal Medicine 13 thEd) PSA (prostate Ca) Alpha-fetoprotein (hepatoma, testicular Ca, hepatitis) CEA - Malignancies: colorectal, thyroid medullary, nonsmall cell lung, pancreas, stomach, breast; - Benign conditions: smoking, chronic lung disease, alcoholic cirrhosis, hepatitis, inflammatory bowel disease Ca –125 (ovarian Ca) Ca 19-9 (pancreatic Ca) B) Additional investigations may be needed where indicated: Lymph node biopsy & CT (abdo, pelvis) if lymphadenopathy present Abdominal ultrasound or CT abdomen if hepatomegaly Myeloma panel if ESR markedly elevated or widened serum proteinalbumin gap Staging investigations if CTCL on histopathology (refer to guidelines on Management of Mycosis Fungoides) Refer to appropriate specialist for evaluation for malignancy if initial screening investigation abnormal Management in acute phase In the acute phase, treatment consists of: A.) Management of complications: Systemic antibiotics for secondary infection: - e.g. cloxacillin Correction of fluid and electrolyte abnormalities: - e.g oral rehydration if no electrolyte abnormalities - rehydration with IV normal saline if hyponatremic Correction of nutritional deficiencies - e.g. Promod 1 scoop /day if hypoalbuminemic - Ensure supplements if cachexic Provision of appropriate ambient temperature: Page 3 - warm ambient temperature if having chills/ hypothermic Cardiology consult and diuretics for high-output cardiac failure B.) Measures to soothe the inflamed skin. The following is recommended: Emulsifying ung as soap 0.05% to 0.1% betamethasone valerate cream or ung bd to trunk/limbs; 0.025% betamethasone valerate cr to face/ flexures bd Emollients e.g WSP or WSP:LP Antihistamines e.g. hydroxyzine 25mg tds Cessation of all unnecessary drugs; substitution of recently added drugs with unrelated alternatives. As the acute phase resolves, one may see more definitive features of an underlying skin disorder, and treatment may be more focussed: A. Management of Erythrodermic Psoriasis First Line Treatment Methotrexate (if no contraindications) starting at 7.5 -10mg /week; FBC after 1 week then monthly; recheck FBC 1-2 weeks after any dose escalation LFT 3 monthly Liver biopsy at cumulative dose of 1500mg Second Line Treatment Acitretin 25mg - 30mg om; Baseline urine pregnancy test in females Baseline fasting lipids & LFT, at 2 weeks then monthly x 4 mth, then 3 monthly Advise against pregnancy for 3 years after cessation (esterification to etretinate by ethanol: longer half-life) Third Line Treatment Cyclosporin 3-5mg/kg/day refer to guidelines on use of cyclosporin in atopic dermatitis B. Management of Erythrodermic Atopic dermatitis & Erythrodermic CTCL Refer to respective NSC guidelines D. Management of idiopathic GED First line: Topical corticosteroids and emollients Page 4 Second line: Third line: Prednisolone 0.5mg/kg/d in reducing doses PUVA Immunosuppressives e.g. azathioprine, CyA (for review by senior consultant/ consultant first) Prognosis and follow-up of idiopathic GED 87% complete or partial remission 13% persistent chronic GED – higher risk for progression to CTCL. Frequent surveillance for an underlying cause is advised. Repeat biopsies from at least 2 sites every 6 months to 1 year in chronic persistent GED, as a proportion may evolve over years into CTCL. References 1. Sigurdsson V, Toonstra J, Hezemans-Boer M, Van Vloten WA. Erythroderma: a clinical and follow-up study of 102 patients, with special emphasis on survival. J Am Acad Dermatol 1996; 35:53-57. 2. Wilson DC, Jester JD, King LE. Erythroderma and exfoliative dermatitis. Clin Dermatol 1993; 11:67-72. 3. Sigurdsson V, Toonstra J, Van Vloten WA. Idiopathic erythroderma: a follow-up study of 28 patients. Dermatology 1997;194: 98-101. Page 5