GPP presents as pustules and plaques over a wide area of the body. It differs from the localized form of pustular psoriasis in that patients are often febrile and systemically ill. Pustular psoriasis is an uncommon form of psoriasis consisting of widespread pustules on an erythematous background, as shown in the image below. A minority of patients develop systemic involvement, most commonly hepatic, renal, and pulmonary systems. This condition has several types of presentations; and one type of psoriasis may be present at a time. Lesions of pustular psoriasis may be localized or generalized. It differs from the localized form of psoriasis is that patients with GPP are often febrile and systematically ill.
Many share systemic findings including fever, elevated inflammatory markers, inflammatory bowel disease and/or osteoarticular involvement, suggesting potential common pathogenic links (Figure 1). Although the majority of pustular psoriasis patients lack a family history of similar disease, a number of familial cases have been reported, leading to a potential insight into common pathways of pustular skin disease8 15. This disease, known as deficiency of the IL-36 receptor antagonist (DITRA), was also reported in a second group of 5 individuals from the United Kingdom who did not have a family history of GPP14, as well as in one Japanese adult male16. Baker and Ryan also noted that localized and generalized forms of pustular psoriasis could overlap with one another over time73. The differential diagnosis for febrile patients with a rash is extensive. Rashes can be categorized as maculopapular (centrally and peripherally distributed), petechial, diffusely erythematous with desquamation, vesiculobullous-pustular and nodular. Major and minor forms; major form always with mucous membrane involvement and usually the result of drug reaction; minor form often associated with herpes simplex outbreak; Various presentations; brownish-red or pink macules and papules; generalized eruption or localized eruption on head, neck, palms or soles; condyloma lata common. These may cause localized or systemic manifestations that varies from erythematous lesions, vesiculation, ulceration, scarring or severe constitutional symptoms. Oral lesions are also frequent, and take the form of erosions on the tongue, palate, gingiva and buccal mucosa. In the immune compromised patient, mucocutaneous herpes simplex respond well to intravenous Acyclovir. Skin rash manifests with erythematous macules, vesicles and pustules which rupture leaving crusted lesions.
(B) Classic target iris lesions of EM in a different patient following HSV infection. But if the patient is febrile and has some other signs and symptoms, maculopapular rash might be a sign of a serious disease so do not ignore it 4. The maculopapular rash in this case is scattered although the face is often unaffected. It is a form of primary HIV infection that occurs in any parts of the body especially the face, trunk, and palms of the hand. If the patient is afebrile, determine if the rash is generalized or localized. It is important to note that not all cutaneous lupus patients have systemic lupus.
Autoinflammatory Pustular Neutrophilic Diseases
As EP is always a severe form of psoriasis, systemic treatment is required. Chickenpox (varicella) – vesicles (initially papules, often not noticed), appearing as ‘drops of water’. Impetigo – this usually takes the form of itchy lesions with macules, vesicles, bullae, pustules and gold-coloured crusts caused by Staphylococcus aureus or group A beta-haemolytic streptococci. Pustular psoriasis. Psoriasis: 80 of patients with Psoriasis report itch. Differs in two ways from erysipelas: cellulites lesions are primarily not raised and demarcation from uninvolved skin is indistinct. Localized form of SSSS, bullous impetigo have localized blisters that are culture positive (different from systemic form). Rose colored macules to papules, vesicle and pustules and crusts develop in 12 hours. This chapter emphasizes the approach to the patient with fever and rash and will cover the common etiologies for this presentation in the adult patient. The skin manifestations associated with a specific infectious agent may be variable, and different types of skin lesions may be seen at different times in the course of one illness. Infections resulting in more localized erythema may or may not be associated with systemic symptoms such as fever. Patients with a history of psoriasis who are treated with systemic corticosteroids may develop an acute pustular erythrodermic flare. On physical examination, he appears ill. Sweet syndrome is often considered a reactive syndrome, associated with a preceding respiratory or gastrointestinal tract illness; an association with malignancy occurs in about 10 of patients. Toxic shock syndrome is associated with many different skin manifestations, but the initial manifestation is typically a diffuse erythroderma resembling sunburn that involves both the skin and the mucous membranes. Psoriasis, lichen planus, p. rosea, pityriasis lichenoides, seb. dermatitis, lichen nitidus, lichen striatus, pityriasis rubra pilaris, erythroderma, exfoliati.