Erythroderma

introduction Erythroderma (literally, “red skin) sometimes called exfoliative dermatitis severe and potentially life-threatening presents with diffuse erythema and scaling involving all or most of the skin surface area (≥90 percent)

ETIOLOGY 1 10/17/2013 most common cause: exacerbation[ek,sæsə'beɪʃən] of a preexisting inflammatory dermatosis—psoriasis or atopic dermatitis. In patients with psoriasis, triggers include abrupt discontinuation of systemic corticosteroids or other immunosuppressant therapy, systemic illnesses, phototherapy burns, medications (eg, lithium, antimalarials), or HIV infection

ETIOLOGY 2 10/17/2013 Hypersensitivity drug reaction Drugs associated with erythroderma including: penicillins sulfonamides carbamazepine[,kɑ:bə'mæzəpi:n] phenytoin [fə'nitəuin] allopurinol [,ælə(ʊ)'pjʊərɪnɒl]

ETIOLOGY 3 10/17/2013 Uncommon causes include: cutaneous T cell lymphoma and other hematologic malignancies Systemic malignancies immunobullous diseases connective tissue diseases infections

ETIOLOGY 4 10/17/2013 Erythroderma of unknown origin ~ 30 percent of cases no underlying cause is identified classified as idiopathic (sometimes called “red man syndrome”, which is also used to describe an infusion reaction to vancomycin[,væŋkə'maɪsɪn])

PATHOGENESIS 10/17/2013 incompletely understood complex interaction of cytokines, chemokines, and intercellular adhesion[əd'hiːʒ(ə)n] molecules massive recruitment of inflammatory cells to the skin and elevated epidermal turnover.

CLINICAL MANIFESTATIONS 10/17/2013 Onset develop acutely over hours or days or evolve gradually over weeks to months onset usually abrupt in drug hypersensitivity reactions. A morbilliform or urticarial eruption may first appear anywhere on the skin then erythematous patches increase in size coalesce into a generalized bright red erythema with occasional islands of sparing.

CLINICAL MANIFESTATIONS 10/17/2013 Onset Organ involvement (eg, hepatitis, nephritis, pneumonia) may occur in DRESS (drug reaction with eosinophilia and systemic symptoms). Erythroderma from underlying cutaneous or systemic diseases usually develops more gradually. Initially, erythematous patches may have characteristics of underlying disease but specific features often lost after erythroderma has fully developed.

CLINICAL MANIFESTATIONS 10/17/2013 Cutaneous symptoms — Over 90 percent of the skin is red and warm to the touch severe skin pain or itching

CLINICAL MANIFESTATIONS 10/17/2013 Linear crusted erosions and secondary lichenification skin may feel leathery and indurated. Scaling is a common feature. Scales particularly abundant in patients with underlying psoriasis.

CLINICAL MANIFESTATIONS 10/17/2013 Involvement of the eyelids manifests with blepharitis[,blefə'raɪtɪs], epiphora[ɪ'pɪfərə] (excessive tearing), and ectropion[ɛk'tropɪən] (eyelid eversion). particularly prominent in patients with chronic erythroderma secondary to Sézary syndrome

CLINICAL MANIFESTATIONS 10/17/2013 Extracutaneous findings Patients often appear ill, with shivering, and complain of feeling cold. Constitutional symptoms (eg, malaise, fatigue, fever, or hypothermia) signs of high output cardiac failure (eg, peripheral edema, tachycardia) may be present. Lymphadenopathy and hepatomegaly or splenomegaly may be observed in chronic erythroderma.

complications 10/17/2013 Hemodynamic and metabolic disturbances fluid loss by transpiration, and consequent electrolyte imbalance. Heat loss, hypothermia, and compensatory hypermetabolism associated with hyperthermia may occur. peripheral vasodilation may result in high-output cardiac failure, especially in older or compromised patients. significant protein loss that may exceed 9 g/m2 body surface per day, particularly in patients with erythrodermic psoriasis

complications 10/17/2013 Infection increase susceptibility[sə,septɪ'bɪlɪtɪ] of the erythrodermic skin to bacterial colonization. Sepsis from S. aureus, including methicillin-resistant S. aureus, has been reported in erythrodermic patients and is of particular concern in those who are HIV positive Widespread superinfection with herpes simplex virus (Kaposi varicelliform eruption) also has been reported in erythrodermic patients

LABORATORY ABNORMALITIES 10/17/2013 Nonspecific. Including: leukocytosis anemia elevated erythrocyte sedimentation rate Eosinophilia may be found in patients with DRESS.

LABORATORY ABNORMALITIES 10/17/2013 Atypical lymphocytes with cerebriform nuclei (Sézary cells) are often observed in erythroderma regardless of cause. Counts of Sézary cells greater than 20 percent of the circulating peripheral blood lymphocytes are found in Sézary syndrome, a leukemic variant of cutaneous T-cell lymphoma

DIAGNOSIS 10/17/2013 diagnosis of erythroderma is clinical in a patient presenting with diffuse and generalized erythema and scaling involving 90 percent or more of the body surface area.

DIAGNOSIS 10/17/2013 Determining the cause of erythroderma is more difficult evaluation involves a detailed history, physical examination, skin biopsies, and laboratory tests.

DIAGNOSIS 10/17/2013 History —— A detailed history is of key importance in establishing the cause of erythroderma.

Diagnosis Important elements of history are: History of inflammatory skin disease (eg, psoriasis, atopic dermatitis) Family history of inflammatory skin diseases Medication history, including over the counter medications and supplements Preexisting systemic diseases or neoplasia Onset of symptoms and course of erythroderma

DIAGNOSIS 10/17/2013 Physical examination should include a complete examination of the skin Nails mucosae for any sign of underlying skin disease. Lymph node and organ enlargement should be assessed.

DIAGNOSIS 10/17/2013 Clinical signs that are nonspecific but may be helpful in suggesting the cause of erythroderma include: Scaling – Bullae – Color of erythema –

Erythroderma in Sézary syndrome 10/17/2013

Moist, crusted lesions on the face and upper trunk often precede the development of erythroderma in patients with pemphigus foliaceous. 10/17/2013

Pemphigus foliaceous The skin is erythrodermic and covered with large scales and crusts 10/17/2013

Hair abnormalities – Diffuse alopecia is common in erythroderma from all causes but may be particularly prominent in Sézary syndrome Diffuse alopecia in Sézary syndrome 10/17/2013

DIAGNOSIS 10/17/2013 Keratoderma – Waxy keratoderma of palms and soles with an orange hue is characteristic of pityriasis rubra pilaris, but may also be observed in Sézary syndrome.

Palmar keratoderma in pityriasis rubra pilaris 10/17/2013

waxy keratoderma associated with pityriasis rubra pilaris 10/17/2013

Keratoderma in Sézary syndrome 10/17/2013

Nail abnormalities – Nail thickening, subungual hyperkeratosis, and splinter hemorrhages are found in psoriasis and pityriasis rubra pilaris. The presence of nail pitting is a clue to the diagnosis of erythrodermic psoriasis. Nail thickening and subungual hyperkeratosis in patient with pityriasis rubra pilaris 10/17/2013

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key words exacerbation[ek,sæsə'beɪʃən] malarial [mə'lɛrɪəl] vancomycin[,væŋkə'maɪsɪn] malaise[mæ'leɪz] blepharitis[,blefə'raɪtɪs] epiphora[ɪ'pɪfərə] ectropion[ɛk'tropɪən] susceptibility[sə,septɪ'bɪlɪtɪ] aureus['ɔ:riəs] pityriasis rubra pilaris