Papuloerythroderma L30.8

Author: Prof. Dr. med. Peter Altmeyer

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Last updated on: 13.05.2022

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Synonym(s)

Papuloerythroderma Ofuji

History
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Ofuji et al., 1984

Definition
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Rare, large-surface, maculo-papular, considerably pruritic, exanthematous skin manifestation with a tendency to confluence and possibly erythroderma, with blood eosinophilia, leukopenia and IgE elevation. Entity of the clinical picture was controversial for a long time.

Etiopathogenesis
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Unknown.

Types of eosinophilic dermatitis.

Associations with underlying malignancies such as cutaneous T-cell lymphoma and leukemias (Wick MR et al. 2019) and

Infections(strongyloidosis) have been described.

Manifestation
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Mainly occurring in Asians, rarely in Caucasians. It is particularly pronounced in adults and is preferred in older men (mean age of the disease: approx. 72 years).

Localization
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Mainly localized in lateral parts of the trunk and extremities. Face, palmae and plantae are free.

Clinical features
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Seeding of intensely pruritic, flat, smooth (typically non-scaling), bright red to brown-red papules that confluence to form large nummular plaques. During the course of the disease, the development of erythroderma is typical. In an almost erythrodermic clinical picture, the recesses of the transverse skin folds on the abdomen ("deck-chair-sign") are described as diagnostically groundbreaking for the clinical picture.

Notice. The"deck-chair-sign" is supposed to remind of a dermatitis solaris at the abdomen as it occurs in a typical deck-chair posture (skin folds remain skin-coloured and do not turn red).

Histology
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Superficial perivascular dermatitis with acanthosis, spongiosis, parakeratosis and distinct histoeosinophilia.

Differential diagnosis
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Therapy
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If necessary, therapy of the underlying disease.

Good results have been achieved with methotrexate (Qureshi F et al. 2020).

Try PUVA therapy.

Otherwise caring external measures.

Literature
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  1. Aste N et al (2000) Ofuji papuloerythroderma. J Eur Acad Dermatol Venereol 14: 55-57.
  2. Desai K et al (2021) Papuloerythroderma of Ofuji. Clin Dermatol 39:248-255.Desai K et al. (2021) Papuloerythroderma of Ofuji. Clin Dermatol 39:248-255.
  3. de Vries HJ et al (2002) Ofuji papuloerythroderma associated with Hodgkin's lymphoma. Br J Dermatol 147: 186-187.
  4. Michel S et al (1999) Papuloerythroderma ofuji: bath PUVA treatment. Dermatologist 50: 360 -362
  5. Ofuji S, Furukawa F, Miyachi Y, Ohno S (1984) Papuloerythroderma. Dermatologica 169: 125-130
  6. Qureshi F et al (2020) Methotrexate for papuloerythroderma of Ofuji. Clin Exp Dermatol 45:241-243.
  7. Wick MR et al (2019) Cutaneous paraneoplastic syndromes. Semin Diagn Pathol 36:211-228.
  8. Wong C et al (2003) Papuloerythroderma of Ofuji associated with acute myeloid leukaemia. Clin Exp Dermatol 28: 277-279.

Disclaimer

Please ask your physician for a reliable diagnosis. This website is only meant as a reference.

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Last updated on: 13.05.2022