Subcorneal pustular dermatosis, initially known as Sneddon-Wilkinson disease, is considered to be a rare cutaneous disorder that belongs to a group of neutrophilic dermatoses [1] [2] [3]. Although the exact etiology remains to be elucidated, skin lesions arise due to an abundant presence of neutrophils below the stratum corneum, presumably as a result of the activity of pro-inflammatory cytokines such as interleukin-8 that act as chemoattractants for neutrophils [2] [4]. For some reason, subcorneal pustular dermatosis is most commonly diagnosed in middle-aged and older women aged 40 or more years [1] [3]. The clinical presentation is characterized by the appearance of sterile pustular lesions on the trunk, the intertriginous areas (the axillae, the submammary areas, and the groins), and the flexor areas of the upper or lower limbs [2] [3] [5] [6] [7] [8]. These pustules are symmetrically distributed and often form blistering lesions [3] [5] [6]. Despite the benign and self-limiting nature of the condition, a chronic course with frequent recurrences are hallmarks of subcorneal pustular dermatosis, which may be debilitating for the patient [1] [3]. Because of the undisclosed cause, the precipitating factors for recurrence are yet to be revealed.

• Anemia

  He was admitted to our clinic because of the increasing complaints after the cessation of dapsone treatment 2 months before due to development of anemia. [archives-inflammation.imedpub.com]

  Once the diagnosis is made, the treatment of choice is dapsone, but in this patient hemolytic anemia developed after its introduction. [nejm.org]

  […] years colchicine, acitretin, OC, UVB, AZA, azulfidine, dapsone infliximab 5mg/kg + OC + acitretin 2 days No (OC stopped) - 6 months Kretschmer et al., 2017 M 29 - 3 weeks OC infliximab 350mg single dose + dapsone 1 day No (dapsone stopped, hemolytic anemia [cureus.com]

  Subcorneal pustular dermatosis in a patient with aplastic anemia. J Am Acad Dermatol. 1998;39(2 Pt 1):287–289. [PubMed] [Google Scholar] Park Y.K., Park H.Y., Bang D.S., Cho C.K. Subcorneal pustular dermatosis treated with phototherapy. [ncbi.nlm.nih.gov]

  】*malignant ameloblastoma 【悪性高熱症】*malignant hyperthermia 【悪性黒色腫】*malignant melanoma (略 MM) 【悪性腫瘍】*malignant tumor *malignancy 【悪性新生物】*malignant neoplasm (＝悪性腫瘍) 【悪性組織球症】*malignant histiocytosis (略 MH) 【悪性中皮腫】*malignant mesothelioma 【悪性貧血】*pernicious anemia [medo.jp]

• Malaise

  Generalized pustular psoriasis (von Zumbusch's type) presents with systemic symptoms (fever, malaise, leukocytosis), and spongiform pustules within the epidermis. [dermaamin.com]

  CASE REPORT A 41-year-old man was admitted for evaluation of an acute, tender, erythematous and pustular eruption accompanied by fever and malaise. [medicaljournals.se]

  Dactylitis or "sausage digit", plantar fasciitits, and achilles tendinitis are the most common sites. [1] [2] [3] [4] Skin lesions are very similar to those of psoriasis [2] [3] Constitutional symptoms include fatigue, malaise, fever, and weight loss [physio-pedia.com]

  The lesions are mildly pruritic and not usually associated with constitutional symptoms; however, cases presenting with malaise, fever, or arthralgias have been reported (Prat et al., 2014, Razera et al., 2011). [ncbi.nlm.nih.gov]

• Weakness

  He denies constitutionalsymptoms, sun sensitivity, and muscle weakness. He has a medical history ofhypothyroidism and denies a history of HIV, congestive heart failure, diabetes,psoriasis, or hepatitis. [docslide.com.br]

  *chicken-pox *water-pox *varicella 【水頭症】*hydrocephaly *hydrocephalus 【水滑液嚢腫】*hygroma (＝ヒグローマ) 【水筋腫】*hydromyoma 【水銀性口内炎】*mercurial stomatitis 【水腎症】*hydronephrosis *nephrohydrosis 【水腎盂症】*hydropelvis 【水疱性類天疱瘡】*bullous pemphigoid 【水瘤】*hydrocele (衰) 【衰弱】*weakness [medo.jp]

• Abdominal Pain

  pain *abdominalgia 【腹部外傷】*abdominal trauma 【腹部狭心症】*abdominal angina 【腹部出血】*abdominal hemorrhage *abdominal bleeding 【腹部腫瘍】*abdominal tumor 【腹部疝痛】*celialgia 【腹部大動脈瘤】*abdominal aortic aneurysm 【腹部癲癇】*abdominal epilepsy 【腹部ヘルニア】*laparocele (＝腹壁瘤) 【腹部膨大】 [medo.jp]

• Ankylosis

  Joint deformity and ankylosis as well as sacroiliitis and spondylititis may occur with chronic or recurrent RS. [2] Associated Co-morbidities Reiter's Syndrome is associated with and may be the presenting symptom of HIV. [1] Reiter's Syndrome is also [physio-pedia.com]

  乾性壊疽】*dry gangrene 【乾性角膜炎】*xerotic keratitis 【乾性胸膜炎】*pleuritis sicca[L] *dry pleurisy 【乾皮症】dry skin *xeroderma/*xerodermia *xerosis cutis[L] ＝乾燥皮膚、皮膚乾燥症 【乾性脂漏症】*seborrhoea sicca[L] 【関節炎】*arthritis 【関節滑液膜炎】*arthrosynovitis 【関節気腫】*pneumarthrosis 【関節強直】*ankylosis [medo.jp]

• Eruptions

  Subcorneal pustular dermatosis is characterized by numerous relapsing pustular eruptions on normal or mildly erythematous skin. [ncbi.nlm.nih.gov]

• Blister

  OLS > Experimental Factor Ontology EFO > EFO:1000771 http://www.ebi.ac.uk/efo/EFO_1000771 Skin diseases characterized by local or general distributions of blisters. They are classified according to the site and mode of blister formation. [ebi.ac.uk]

  Histologic findings showed subcorneal blisters filled with polymorphonuclear leukocytes typical of subcorneal pustular dermatosis. Dapsone and prednisolone with a topical fluocinolone acetonide did not produce improvement. [ncbi.nlm.nih.gov]

  The blistering may be severe enough to mimic other childhood blistering dermatosis such as epidermolysis bullosa. [dermatology.cdlib.org]

• Skin Disease

  In conclusion, the differential diagnoses of skin diseases should be considered when diagnosing scabies. Thus, unnecessary therapy and contamination among people can be prevented. [ncbi.nlm.nih.gov]

  OLS > Experimental Factor Ontology EFO > EFO:1000771 http://www.ebi.ac.uk/efo/EFO_1000771 Skin diseases characterized by local or general distributions of blisters. They are classified according to the site and mode of blister formation. [ebi.ac.uk]

  From Wikidata Jump to navigation Jump to search Human disease Sneddon-Wilkinson disease or syndrome Subcorneal pustular dermatosis Subcorneal pustular dermatosis (disorder) edit English vesiculobullous skin disease Human disease Sneddon-Wilkinson disease [wikidata.org]

• Ulcer

  We report a 57-year-old woman with a 12-year history of ulcerative pyoderma gangrenosum (PG). Five years after the onset of PG, she developed subcorneal pustular dermatosis (SPD) and biclonal IgA and IgG gammopathy. [ncbi.nlm.nih.gov]

  Subcorneal pustular dermatosis and episcleritis associated with poorly controlled ulcerative colitis Jeffrey J Wargo 1, Megan Adams 2, Julian Trevino 3 1 Department of Internal Medicine, University of Michigan Medical School, Ann Arbor, Michigan, USA [casereports.bmj.com]

  Gingivitis (42 %) ( Acute Membranous Gingivitis, Acute Necrotizing Ulcerative Gingivostomatitis, Fusospirillary Gingivitis, Fusospirillosis, Fusospirochetal Gingivitis, Necrotizing Gingivitis, Phagedenic Gingivitis, Trench Mouth, Ulcerative Gingivitis [gulfdoctor.net]

  Ooi CJ, Fock KM, Makharia GK, Goh KL, Ling KL, et al. (2010) The Asia-Pacific consensus on ulcerative colitis. J Gastroenterol Hepatol 25: 453-468. [omicsonline.org]

• Hyperpigmentation

  The pustules were sterile, had some tendency to grouping, were accompanied by moderate to severe pruritus and some hyperpigmentation. They responded fairly well to sulfapyridine and the sulfones. [jamanetwork.com]

  Residual scarring and postlesional hyperpigmentation was seen at a 2-month follow-up appointment (Fig. 1 e). No side effects were noted in response to infliximab. [karger.com]

  The pustules used to dry up in 6-7 days to form thin, dry crusts that fell leaving hyperpigmentation. There were no oral lesions. Nikolsky sign was negative. A skin biopsy revealed histological features of SCPD. [ijdvl.com]

  There is no atrophy or scarring, but an occasional brownish hyperpigmentation may mark previously affected sites. Variable intervals of quiescence, lasting from a few days to several weeks, may be followed by the sudden development of new lesions. [plasticsurgerykey.com]

• Withdrawn

  The eruption resolved completely when adalimumab was withdrawn; methotrexate was continued. Subcorneal pustular dermatosis, in addition to psoriasis vulgaris and pustular psoriasis, may occur in patients treated with TNF-α inhibitors like adalimumab. [ncbi.nlm.nih.gov]

  But steroid also was withdrawn since he developed hypertension and diabetes. There was relapse of skin lesions which were treated with oral colchicine, which has profound inhibitory effects on neutrophils. [ijdvl.com]

  In some patients, the treatment may be withdrawn after several months, although in others it may have to be continued for years; the minimal effective dose to suppress disease should be determined in these patients. [dermaamin.com]

  View this Virtual Slide of Impetigo Pustules due to fungi are usually seen when steroids have been applied to the lesion and the steroids are then withdrawn. [dermatopathologymadesimple.blogspot.com]

The differential diagnosis of subcorneal pustular dermatosis is broad, encompassing other neutrophilic dermatoses (Sweet's syndrome, acute generalized exanthematous pustulosis, and generalized pustular psoriasis) and systemic conditions that include this cutaneous disorder in the presentation, such as various lymphoproliferative disorders (eg. multiple myeloma) and immunoglobinopathies [1] [2] [3]. For this reason, the identification of subcorneal pustular dermatosis mandates a thorough workup and a histopathological examination. As a first step, however, the physician must obtain a detailed patient history that will identify the chronic and relapsing pattern of the lesions, whereas a physical exam is crucial for observing the pattern of distribution and the appearance of skin lesions. As soon as clinical suspicion is raised, a biopsy sample should be obtained for the purposes of performing a histopathological examination. The hallmark of neutrophilic dermatoses is the presence of a break below the stratum corneum that contains a dense neutrophilic infiltrate with a normal underlying epidermis [1] [5] [7] [8]. Additionally, exclusion of immunoglobinopathies and autoimmune disorders can be made by carrying out an immunofluorescence exam that is virtually always negative [3] [8], although rare cases of immunoglobulin (Ig)A-presenting antibodies in the epidermis were documented, thus pointing toward IgA pemphigus as a potential differential diagnosis [3].

• Colitis

  Subcorneal pustular dermatosis and episcleritis associated with poorly controlled ulcerative colitis Jeffrey J Wargo 1, Megan Adams 2, Julian Trevino 3 1 Department of Internal Medicine, University of Michigan Medical School, Ann Arbor, Michigan, USA [casereports.bmj.com]

  A man aged 56 years with a history of ulcerative colitis (UC) status postsubtotal colectomy was hospitalised with fevers, dry cough, eye redness and a new bloody, mucoid rectal discharge. 2 months prior to admission, the dermatologist had started him [ncbi.nlm.nih.gov]

  Ooi CJ, Fock KM, Makharia GK, Goh KL, Ling KL, et al. (2010) The Asia-Pacific consensus on ulcerative colitis. J Gastroenterol Hepatol 25: 453-468. [omicsonline.org]

  Subcorneal pustular dermatosis has also been reported in association with pyoderma gangrenosum, Crohn disease, ulcerative colitis, systemic lupus erythematosus, rheumatoid arthritis, morphea, myeloproliferative disorders, lymphoproliferative disorders [clinicalgate.com]

  Increased serum IgA has been detected in a number of patients, and the disease has been reported to occur in cases of IgA-paraproteinemia and IgA multiple myeloma. 9 – 12 In addition, SPD is associated with pyoderma gangrenosum, 13, 14 ulcerative colitis [plasticsurgerykey.com]

• HLA-B27

  Up to 85% of people with Reiter's possess the HLA-B27 alloantigen. [physio-pedia.com]

  These cathelicidin peptides are a result of a posttranslational processing abnormality associated with an increase in stratum corneum tryptic enzyme in the epidermis. [45] Reactive arthritis: Genetic disease which may be related to HLA-B27. [online.epocrates.com]

• HLA-A1

  It is associated with an increased expression of hla-a1, hla-b8, and hla-dr3 antibodies. A disorder characterized by inflammation of the skin characterized by the presence of bullae which are filled with fluid. [icd9data.com]

Although dapsone is often considered to be the first-line treatment, some patients fail to respond or cannot tolerate the side effects. For cases that do not respond well to this treatment, acitretin, an excellent second-line treatment, may be used. [ncbi.nlm.nih.gov]

Treatment by anti-TNFα was introduced after the failure of an average of 6.2 lines of treatment (1-9 lines of treatment). Six patients were treated using infliximab, five by etanercept, and two by adalimumab. [cureus.com]

CHARACTERIZATION TREATMENT Dapsone ACITRETIN Successful treatment of subcorneal pustular dermatosis (Sneddon-wilkinson disease) by acitretin: report of a case. [thedoctorsdoctor.com]

Prognosis : good if responds to treatment. Pathogenesis Etiology Not known, but likely to be immune-mediated. Pathophysiology Immune complexes and IgA in stratum corneum → neutrophil chemotaxis and subcorneal pustule formation. [vetstream.com]

[…] maintenance with a lower dose is sometimes needed Other treatment options include sulfapyridine or sulfamethoxypyridazine, acitretin, colchicine, phototherapy and biologic therapies Systemic steroids are generally ineffective and can precipitate a flare-up Prognosis [pcds.org.uk]

It has been reported in children, without differences described in clinical features and prognosis between children and adults, but some cases tend to have atypical features more suggestive of psoriasis. [22, 23, 24] Prognosis Subcorneal pustular dermatosis [emedicine.medscape.com]

SPECIAL STAINS/IMMUNOPEROXIDASE/OTHER CHARACTERIZATION Direct immunofluorescence (DIF) Negative DIFFERENTIAL DIAGNOSIS KEY DIFFERENTIATING FEATURES Pustular psoriasis Spongiform pustules Impetigo Gram stains Candidiasis Fungal stains PROGNOSIS AND TREATMENT [thedoctorsdoctor.com]

Sneddon-Wilkinson disease or subcorneal pustular dermatosis (SPD) is a rare, benign inflammatory skin disorder of unknown etiology. SPD is associated with various systemic disorders, including immunoglobinopathies and lymphoproliferative disorders. [ncbi.nlm.nih.gov]

Etiologies include immunologic and genetic factors. [ebi.ac.uk]

Definition / general Rare, recurrent, noninfective, nonautoimmune pustular eruption of unknown etiology Terminology Also called Sneddon-Wilkinson disease Epidemiology Associated with benign or malignant IgA paraproteinemia, rheumatoid arthritis, SLE, [pathologyoutlines.com]

Definition / general Rare, recurrent, noninfective, nonautoimmune pustular eruption of unknown etiology Terminology Also called Sneddon-Wilkinson disease Epidemiology Associated with benign or malignant IgA paraproteinemia, rheumatoid arthritis, SLE, [pathologyoutlines.com]

OUTLINE EPIDEMIOLOGY CHARACTERIZATION SYNONYMS Subcorneal pustular dermatosis AGE RANGE-MEDIAN >40 years Mean of 66 years SEX (M:F) F DISEASE ASSOCIATIONS CHARACTERIZATION CROHN'S DISEASE Subcorneal pustular dermatosis in a patient with Crohn's disease [thedoctorsdoctor.com]

Current and historic epidemiologic studies. J Invest Dermatol 1989; 92:4. Brenner S, Wohl Y. A survey of sex differences in 249 pemphigus patients and possible explanations. Skinmed 2007; 6:163. Bastuji-Garin S, Souissi R, Blum L, et al. [pemphigus.org]

Epidemiology There is no racial predilection. Most of the reported cases have been in whites, but the disease has also been observed in Africans, Japanese, and Chinese. [plasticsurgerykey.com]

However, to date its exact pathophysiology is unknown. Cases in association with pyoderma gangrenosum, benign monoclonal IgA gammopathy and multiple myeloma are well documented. [ncbi.nlm.nih.gov]

Thus, unnecessary therapy and contamination among people can be prevented. [ncbi.nlm.nih.gov]

This case illustrates the importance of careful history taking and of early recognition of extraintestinal manifestations of IBD in order to appropriately target treatment and prevent unnecessary morbidity. [casereports.bmj.com]

Treatment Treatment is aimed at preventing complications. Dapsone is often successful, with the lesions resolving over a month. Ongoing maintenance with a lower dose is sometimes needed. [dermnetnz.org]

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3. Ratnarathorn M, Newman J. Subcorneal pustular dermatosis (Sneddon-Wilkinson disease) occurring in association with nodal marginal zone lymphoma: a case report. Dermatol Online J. 2008;14(8):6.
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8. Kretschmer L, Maul J-T, Hofer T, Navarini AA. Interruption of Sneddon-Wilkinson Subcorneal Pustulation with Infliximab. Case Rep Dermatol. 2017;9(1):140-144.