Erythematous, Eczematous, and Pruritic Lesions With Lichenification

First identified in 1868 by Von Hebra, erythroderma — also known as exfoliative dermatitis — involves redness and scaling of more than 90% of skin, leading to uncontrolled skin dysmetabolism.1 The incidence of erythroderma is approximately 1 in 100,000 annually.1...

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First identified in 1868 by Von Hebra, erythroderma — also known as exfoliative dermatitis — involves redness and scaling of more than 90% of skin, leading to uncontrolled skin dysmetabolism.¹ The incidence of erythroderma is approximately 1 in 100,000 annually.¹ Although erythroderma can occur at any age and in any sex, it is more common in older adults and males.² Erythroderma presents later in men than in women, with differing age and length of hospital stay due to underlying etiology.³

Erythroderma is a clinical diagnosis with a variety of underlying causes. The most prevalent cause of erythroderma is exacerbation of a preexisting inflammatory skin condition, most commonly atopic dermatitis or psoriasis. Other triggers include drug reactions and cutaneous T-cell lymphoma. ³ For patients with psoriasis, risk factors include systemic illnesses, phototherapy damage, medications, sudden discontinuation of corticosteroids or immunosuppressants, or HIV.⁴ Drugs reportedly associated with erythroderma include penicillins, carbamazepine, sulfa drugs, antiepileptic agents, antihypertensive medications, and calcium channel blockers. While carbamazepine and penicillin are the most common inciting agents, it is important to consider an idiopathic etiology for any patient taking medications who presents with urticarial, lichenoid, or morbilliform rashes that develop into erythroderma.^1,5 Other patterns of drug-induced exfoliative dermatitis include DRESS (drug rash with eosinophilia and systemic symptoms) syndrome, erythema multiforme, Stevens-Johnson syndrome, and toxic epidermal necrolysis.⁶ A less common cause of erythroderma is CD4+ cutaneous T-cell lymphoma. This disease is subclassified as mycosis fungoides, the most common form of which can present similarly to a benign erythroderma, and Sezary syndrome, which is a more aggressive form with more extensive hematologic and internal organ involvement.¹ Some cases of erythroderma have no identified etiology and are thus classified as idiopathic.

The molecular pathogenesis of erythroderma is poorly understood but is generally thought to involve interactions of cytokines, chemokines, and intracellular adhesion molecules that cause inflammation of the skin. Specifically, elevated serum levels of intercellular adhesion molecule-1, vascular cell adhesion molecule-1, and E-selectin are seen in patients with erythroderma secondary to psoriasis, eczema, and Sezary syndrome.⁷ The increased inflammatory cell recruitment results in increased turnover of the epidermis, leading to a dysmetabolic state and associated complications such as dehydration, electrolyte imbalance and infection.

Common clinical findings include pruritus, peripheral edema, fever, palmoplantar keratoderma with desquamation, nail changes, hepatosplenomegaly, psoriatic arthritis, and lymphadenopathy.³ Laboratory findings are generally nonspecific in erythroderma and not useful for diagnosis. Findings include mild anemia, eosinophilia, elevated erythrocyte sedimentation rate, hypoalbuminemia, hyperuricemia, and elevated immunoglobulin (Ig)-E levels.⁸ Histology also tends to yield nonspecific features such as hyperkeratosis, acanthosis, and perivascular inflammatory infiltrates with or without eosinophils. In a retrospective study, a blinded histopathology examination was only able to identify the correct diagnosis in 61% of cases.⁹ This points to the lack of an exclusive pathognomonic finding for erythroderma.

The differential diagnosis for erythroderma includes acanthosis nigricans, cutaneous sarcoidosis, allergic and irritant contact dermatitis, bullous pemphigoid, lichen planus, pityriasis rubra pilaris, and reactive arthritis. Erythroderma can also occur secondary to these conditions.⁴ The major differentiating factor is the extensive skin surface area involved in erythroderma. Erythroderma is a clinical diagnosis based on erythema and warmth of at least 90% of the body surface, often with pain and pruritus.

Treatment for erythroderma involves initial management of the acute presentation followed by individualized treatment based on etiology. Patients who are hemodynamically unstable will require hospitalization. Use of heating blankets may be necessary due to the loss of thermoregulation from skin dysmetabolism. Monitoring of blood pressure and pulse is also required as skin vasodilation could lead to high-output cardiac failure; adequate hydration and maintenance of electrolyte status are also necessary. For pain and pruritus, emollients and wet dressings are beneficial. Treatments such as topical corticosteroids, oral antihistamines, systemic antibiotics for Staphylococcus aureus, or antiviral agents for Herpes simplex can also be added as necessary.¹⁰ As the underlying cause is identified, targeted treatment can be initiated.

After presentation, the patient in this case was diagnosed with erythroderma secondary to an exacerbation of atopic dermatitis. Systemic treatment including topical corticosteroids, phototherapy, and emollients resulted in remission of the atopic dermatitis flare and associated erythroderma.

Jonathan Lo, BA, and Michelle E. Lee, BA, are medical students at Baylor College of Medicine, in Houston, Texas. Christopher Rizk, MD, is a dermatologist with Elite Dermatology in Houston, Texas.

References

1. Okoduwa C, Lambert WC, Schwartz RA, et al.  Erythroderma: review of a potentially life-threatening dermatosis. Indian J Dermatol. 54(1):1-6.
2. Li J, Zheng HY. Erythroderma: a clinical and prognostic study. Dermatology. 2012;225(2):154-162.
3. César A, Cruz M, Mota A, Azevedo F.  Erythroderma. A clinical and etiological study of 103 patients. J Dermatol Case Rep. 2016;10(1):1-9.  
4. Rothe MJ, Bernstein ML, Grant-Kels JM. Life-threatening erythroderma: diagnosing and treating the “red man.” Clin Dermatol. 2005;23(2):206-217.
5. Khaled A, Sellami A, Fazaa B, Kharfi M, Zeglaoui F, Kamoun MR. Acquired erythroderma in adults: a clinical and prognostic study. J Eur Acad Dermatol Venereol. 2010;24(7):781-788.
6. Yacoub M-R, Berti A, Campochiaro C, et al.  Drug induced exfoliative dermatitis: state of the art. Clin Mol Allergy. 2016;14:9.
7. Groves RW, Kapahi P, Barker JN, Haskard DO, MacDonald DM. Detection of circulating adhesion molecules in erythrodermic skin disease. J Am Acad Dermatol. 1995;32(1):32-36.
8. Wilson DC, Jester JD, King LE Jr. Erythroderma and exfoliative dermatitis.  Clin Dermatol. 1993;11(1):67-72.
9. Megna M, Sidikov AA, Zaslavsky DV, et al.  The role of histological presentation in erythroderma. Int J Dermatol. 2017;56(4):400-404.
10. Mistry N, Gupta A, Alavi A, Sibbald RG. A review of the diagnosis and management of erythroderma (generalized red skin). Adv Skin Wound Care. 2015;28(5):237-238.