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Table 15.7 Classification of pustular psoriasis.


Classification Localized Localized type pustular psoriasis Pustular psoriasis Palmoplantar with generalized pustulosis (PPP) skin lesion Acrodermatitis continua of Hallopeau Clinical findings Pustules are localized around the plaques of psoriasis vulgaris. Pustules are localized bilaterally on the thenar and arch of the foot. Often occurs secondarily after an external injury. Pustules and nail deformity occur on the tips of fingers or toes on one side of the body. Psoriasis vulgaris progresses to be accompanied by systemic symptoms. Poor prognosis.

Clinical images are available in hardcopy only.

Generalized Generalized type pustular psoriasis (GPP)

Acute generalized pustular psoriasis (von Zumbusch psoriasis)

Fig. 15.25-2 Guttate psoriasis on the buttocks.

Subacute, circular The systemic symptoms pustular psoriasis are milder than those of von Zumbusch psoriasis. Impetigo herpetiformis Pustules are generalized during the middle and last stages of pregnancy.

cases are familial and autosomal dominantly inherited. A subtype caused by HIV has been reported in recent years. Pathology The follicles are dilated and filled with keratin. The peripheral epidermis is thickened and there is parakeratosis in some parts. Complete keratinization alternates with incomplete keratinization. Polymorphonuclear cells do not infiltrate into the epidermis, which is useful for differentiation from psoriasis. Vasodilation and lymphocytic infiltrate are observed in the upper dermis. Differential diagnosis Pityriasis rubra pilaris should be differentiated from psoriasis, cutaneous T-cell lymphoma, seborrheic dermatitis, drug eruption, ichthyosis and contralateral progressive erythrokeratoderma. Treatment, Prognosis Both types heal spontaneously, within a year in the juvenile type and within 2 to 3 years in the adult type. The symptomatic therapies are application of urea ointments, salicylic acid petrolatum ointments, and active forms of vitamin D3 ointments. Oral retinoid is also useful.

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3. Parapsoriasis en plaque
It is a generic term for diseases that produce multiple psoriasislike keratotic erythema. The pathogenesis is unknown, but it is thought to be different from that of psoriasis. Some large-plaque

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Disorders of Abnormal Keratinization

Clinical images are available in hardcopy only.

Clinical images are available in hardcopy only.

Clinical images are available in hardcopy only.

Clinical images are available in hardcopy only.

Fig. 15.26 Pustular psoriasis. The main skin lesions are sterile pustules.

Clinical images are available in hardcopy only.

Clinical images are available in hardcopy only.

Clinical images are available in hardcopy only.

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Fig. 15.27 Pityriasis rubra pilaris. There are follicular papules, diffuse keratinization on the hands and soles, and orange psoriatic plaques.

parapsoriasis may be prodromes of mycosis fungoides. However, there are many cases in which small-plaque and large-plaque parapsoriasis occur at the same time.

1) Small-plaque parapsoriasis
MEMO Parapsoriasis The definition, concept and diagnostic name for parapsoriasis have not been clarified. In the 1970s, it became widely accepted that large-plaque parapsoriasis and small-plaque parapsoriasis are two distinct disorders. In recent years, large-plaque parapsoriasis has come to be regarded as the early patch stage of mycosis fungoides. Pityriasis lichenoides is known to be a parapsoriatic disease, and it is classified into pityriasis lichenoides chronica (PLC; formerly called guttate parapsoriasis) and pityriasis lichenoides et varioliformis acuta (PLEVA; also called Mucha-Habermann disease).

Monomorphic round to oval erythematous plaques of 2 to 5 cm in diameter appear mainly on the trunk. Lesions are asymptomatic, and histopathologically findings are non-specific. Emollients and UVB are helpful.

2) Large-plaque parapsoriasis
Large erythematous or yellowish atrophic plaques occur on the trunk and extremities. The lesions persist for many years, and gradually increase in number and affected area. They occur most frequently in middle-aged and elderly men (Fig. 15.28). Subjective symptoms such as itching are not present. The eruptions are

B. Acquired keratoses

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usually more than 5 cm in diameter and accompanied by poikiloderma. Some but not all patients may develop mycosis fungoides (Fig. 22.35). Careful check up of the disease course is necessary. PUVA therapy is effective.

4. Pityriasis lichenoides
This disorder is difficult to classify. This entity is sometimes regarded as cutaneous vasuculitis, rather than as a keratotic disorder. Pityriasis lichenoides tends to be limited to the trunk, thighs and upper arms. It rarely occurs on the face, palms or soles. The eruption progresses slowly over the course of many years. Adult men are most commonly affected. Erythema or rose pink papules of several millimeters to 1 cm in diameter, to which white scales are attached, appear. The eruptions are continuously produced, and a distinguishing characteristic of the disorder is the presence of new eruptions together with older ones (Figs. 15.29-1 and 15.29-2). It is asymptomatic. It heals with pigmentation or depigmentation. Pityriasis lichenoides is divided into two main forms, Pityriasis lichenoides chronica (PLC) and Pityriasis lichenoides et varioliformis acuta (PLEVA), but intermediate forms or patients with both forms are often seen. Pityriasis lichenoides chronica (PLC) Synonym: guttate parapsoriasis This is a chronic form. This individual eruption is a rose pink plaque. Young adults are usually affected.

Clinical images are available in hardcopy only.

Fig. 15.28 Large-plaque parapsoriasis. Relatively sharply demarcated slight erythema is present.

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Clinical images are available in hardcopy only.

Table 15.8 Classification and features of parapsoriasis.


Parapsoriasis en plaque and pityriasis lichenoides Clinical findings Pathological findings Large-plaque parapsoriasis Small-plaque parapsoriasis Pityroid scales and erythema appear. Itching is not present. Lymphocytic infiltration in the dermo-dermal junctions The eruption is 5 cm or more in diameter, accompanied by atrophy, and may be a precursor of mycosis fungoides in some cases. The eruption is less than 5 cm in diameter. Clinical images are available in hardcopy only. Fine white scales, erythema of 1 cm or less in diameter, polymorphic skin lesion with old and new eruptions Lymphocytic infiltration to the epidermis

Pityriasis lichenoides (guttate parapsoriasis) Clinical findings

Pathological findings Pityriasis lichenoides The main symptom is erythematous plaque. chronica (PLC) (previous guttate parapsoriasis) Pityriasis lichenoides The main symptoms are severe inflammatory et varioliformis acuta symptoms and ulceration. (PLEVA)

Fig. 15.29-1 Pityriasis lichenoides chronica. Old eruptions are rarely seen simultaneously with new ones on the lesion. Ulceration does not occur.

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