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Blistering diseases in the elderly
Published in Robert A. Norman, Geriatric Dermatology, 2020
Subcorneal pustular dermatosis (SCPD), also known as Sneddon–Wilkinson syndrome, is a benign bullous disease which affects four times as many women as men78. The lesions present as crops of sterile pustular eruptions in an annular pattern that have a serpiginous outline. These lesions are most commonly seen in the cornea, groin, axillary and inframammary areas, and extensor aspects of upper and lower limbs78. On histology, subcorneal bullae are seen with an inflammatory infiltrate made mainly of neutrophils. SCPD is treated systemically with dapsone at 50–150 mg daily. Sulfapyridine and high dose systemic corticosteroids can be used as an alternative. The disease runs a chronic recurrent course lasting up to eight years78.
Acute generalized exanthematous pustulosis
Published in Biju Vasudevan, Rajesh Verma, Dermatological Emergencies, 2019
The most important differential diagnosis of AGEP is pustular psoriasis. The important differentiating features are given in Table 6.1. Other important differential diagnoses include pustular variant of drug hypersensitivity syndrome, subcorneal pustular dermatosis, pustular vasculitis, and Stevens-Johnson syndrome/toxic epidermal necrolysis.
Dapsone
Published in M. Lindsay Grayson, Sara E. Cosgrove, Suzanne M. Crowe, M. Lindsay Grayson, William Hope, James S. McCarthy, John Mills, Johan W. Mouton, David L. Paterson, Kucers’ The Use of Antibiotics, 2017
Subcorneal pustular dermatosis is a rare condition of the eye that is associated with the IgA form of pemphigus, a blistering skin disease (Huff et al., 1985). Often termed the “Sneddon–Wilkinson” syndrome after the clinicians who first reported that dapsone was active against this disease of the eye (Sneddon and Wilkinson, 1956), the disease responds well to dapsone therapy, although other forms of immunosuppression are sometimes required to control the manifestations of the disease outside the eye. The broad spectrum of IgA pemphigus disease in general responds well to dapsone and, accordingly, it remains the drug of first choice (Ongenae et al., 1999; Chaudhari and Marinkovich, 2007). Dapsone has been found to be a useful steroid-sparing agent in patients with pemphigus vulgaris (Rosenberg et al., 1976), although more modern approaches to steroid-sparing immunosuppression, such as co-administration with mycophenolate or the use of rituximab, are now used in preference to dapsone (Piette and Werth, 2012).
A review of disease burden and clinical management for generalized pustular psoriasis in China
Published in Expert Review of Clinical Immunology, 2022
Based on literature and our clinical experience to date, the differential diagnoses of GPP include acute generalized exanthematous pustulosis, superficial candidiasis, subcorneal pustular dermatosis, Stevens-Johnson syndrome – a severe mucocutaneous disease characterized by severe purulent conjunctivitis, severe stomatitis with extensive mucosal necrosis, and purpuric macule, and is caused by drug exposure or infections; with a pustular eruption as an unusual form of the disease [29,30], amicrobial pustulosis of the folds, pemphigus foliaceous, IgA pemphigus subcorneal pustular dermatosis type, transient neonatal pustular melanosis, acropustulosis of infancy and SAPHO syndrome (synovitis, acne, pustulosis, hyperostosis, osteitis), adding to the challenge of correctly diagnosing GPP [1,31–34]. Moreover, diagnostic criteria for GPP vary between research entities. ERASPEN criteria stipulate that a diagnosis of GPP can be confirmed only upon disease relapse >1 episode or persistence >3 months [26]. However, the Japanese Dermatological Association (JDA) guidelines state that a definitive diagnosis of GPP can be made if patients show the following four features: 1) systemic symptoms, including fever and fatigue; 2) systemic or extensive flush accompanied by multiple sterile pustules that can sometimes merge as lakes of pus; 3) neutrophilic subcorneal pustules histopathologically characterized by Kogoj’s spongiform pustules; and 4) the above clinical and histological features occur repeatedly [35]. GPP should be suspected in patients with the second and third features [35].
Dapsone for the treatment of acne vulgaris: do the risks outweigh the benefits?
Published in Cutaneous and Ocular Toxicology, 2022
Selami Aykut Temiz, Munise Daye
Dapsone is a “4,4′-diamino diphenyl sulfone” compound and an aniline derivative from synthetic sulphones. Sulphonamides were first synthesized as dyes (reproduced from coal) for the cloth industry in 1908. Sulphonamides were first used in humans as antimicrobial agents to treat streptococcal infections. Dapsone derived from sulphonamides was first used in the treatment of leprosy in 19401. Subsequently, it was used in the treatment of bullous dermatoses, especially dermatitis herpetiformis, and in the treatment of non-infectious inflammatory dermatoses, especially neutrophilic dermatoses1,2. Today, Dapsone treatment is among the treatment options for many dermatological diseases2,3. Furthermore, the dapsone treatment is recommended as the first treatment option for the therapy of dermatitis herpetiformis, subcorneal pustular dermatosis, linear IgA bullous dermatosis, and erythema elevatum diutinum3.
A brief guide to pustular psoriasis for primary care providers
Published in Postgraduate Medicine, 2021
Jeffrey J. Crowley, David M. Pariser, Paul S. Yamauchi
For GPP, the major diagnosis to exclude is AGEP [9]. Clinically, it may be almost impossible to distinguish AGEP from GPP. The main clue would be a history of recent drug ingestion, as 90% of AGEP cases are associated with medication (commonly certain types of antibiotic, and calcium channel blocker agents) [35]. The main features of AGEP include [35]: acute widespread appearance of pinhead-sized sterile pustules, with erythema, edema, fever, and leukocytosis (the illness is usually mild, <5% mortality). Furthermore, AGEP has a more abrupt onset and a shorter duration than GPP – AGEP signs/symptoms occur within 48 hours and resolve within 1 to 2 weeks – AGEP does not recur, and has no association with plaque psoriasis. Skin biopsy (including at least one intact pustule) should be taken for histopathological examination to distinguish AGEP from other pustular skin conditions [35,36]. Other differential diagnoses for GPP include localized forms of pustular psoriasis, IgA pemphigus [37], and subcorneal pustular dermatosis (also called Sneddon-Wilkinson disease) [38].