Papuloerythroderma of Ofuji
Alerts and Notices
Synopsis

Papuloerythroderma of Ofuji (PEO) is a rare form of erythroderma, most commonly seen in older men. It begins with pruritic, erythematous or brown, flat-topped papules that subsequently coalesce into plaques. The plaques eventually generalize into near-global erythroderma sparing the skin folds, a characteristic physical finding known as the deck-chair sign. Most patients present with associated pruritus and peripheral eosinophilia, while elevated immunoglobulin E (IgE) level and peripheral lymphopenia are less frequently observed. Reactive lymphadenopathy may be present.
PEO mostly affects men of Asian or Northern European descent, with onset at age 55 years or older. These patients are typically healthy, and PEO is usually idiopathic. However, etiological associations have been noted in a minority of cases, including an underlying atopic diathesis, hematologic malignancies, gastric and other solid organ malignancies, and, less frequently, drugs (such as aspirin and furosemide) and infections (such as hepatitis C virus [HCV] and human immunodeficiency virus [HIV]). Additionally, cases of cutaneous T-cell lymphoma (CTCL) with a PEO-like clinical appearance have been reported.
PEO is generally an indolent condition and requires long-term treatment. Those with secondary PEO may experience faster and complete resolution upon treatment of underlying conditions or cessation of offending drugs.
PEO mostly affects men of Asian or Northern European descent, with onset at age 55 years or older. These patients are typically healthy, and PEO is usually idiopathic. However, etiological associations have been noted in a minority of cases, including an underlying atopic diathesis, hematologic malignancies, gastric and other solid organ malignancies, and, less frequently, drugs (such as aspirin and furosemide) and infections (such as hepatitis C virus [HCV] and human immunodeficiency virus [HIV]). Additionally, cases of cutaneous T-cell lymphoma (CTCL) with a PEO-like clinical appearance have been reported.
PEO is generally an indolent condition and requires long-term treatment. Those with secondary PEO may experience faster and complete resolution upon treatment of underlying conditions or cessation of offending drugs.
Codes
ICD10CM:
L53.9 – Erythematous condition, unspecified
SNOMEDCT:
402299006 – Papuloerythroderma of Ofuji
L53.9 – Erythematous condition, unspecified
SNOMEDCT:
402299006 – Papuloerythroderma of Ofuji
Look For
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Diagnostic Pearls
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Differential Diagnosis & Pitfalls
Differential diagnosis of flat-topped, pruritic papules:
- Lichen planus and lichenoid drug eruption
- Papular eczema
- Papular urticaria
- Pityriasis lichenoides chronica
- Psoriasis
- Atopic dermatitis
- Allergic contact dermatitis
- Sezary syndrome
- Erythroderma associated with an underlying lymphoproliferative disorder
- Crusted scabies
- Dermatomyositis
- Pemphigus foliaceus
- Bullous pemphigoid
- Drug reaction with eosinophilia and systemic symptoms (DRESS)
- Exfoliative erythroderma secondary to medication
Best Tests
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Management Pearls
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Therapy
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Drug Reaction Data
Below is a list of drugs with literature evidence indicating an adverse association with this diagnosis. The list is continually updated through ongoing research and new medication approvals. Click on Citations to sort by number of citations or click on Medication to sort the medications alphabetically.
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References
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Last Reviewed:08/18/2019
Last Updated:08/29/2019
Last Updated:08/29/2019