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Subcorneal Pustular Dermatosis
Sneddon-Wilkinson disease
Subcorneal pustular dermatosis is a rare and self-limiting, but recurring disorder of the skin distinguished by the appearance of sterile pustules in a symmetrical fashion over the trunk and the flexor parts of the extremities. The pathogenesis of this condition is incompletely understood, but it is assumed that some form of immune-mediated reaction leads to accumulation of neutrophils below the stratum corneum, which is the main feature under microscopy. Clinical findings and a biopsy of the lesion are two main components of the diagnostic workup.
Presentation
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.
Entire Body System
Gastrointestinal
Rectal Discharge
- 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]
- discharge. 2 months prior to admission, the dermatologist had started him on dapsone for subcorneal pustular dermatosis but did not recognise that he had recently self-discontinued mesalamine enemas, inducing a flare of his UC.[casereports.bmj.com]
Skin
Blister
- The H&E staining demonstrated typical features of SPD, including some damage within dermal pilosebaceous units subjacent to the subcorneal blistering process.[ncbi.nlm.nih.gov]
- 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]
- In addition, an array of cytokines was simultaneously measured in suction blister fluids obtained from involved or uninvolved skin at various time intervals during the first 12 weeks of observation.[ncbi.nlm.nih.gov]
- Diagnosing IgA pemphigus and distinguishing between its 2 subtypes, intraepidermal neutrophilic IgA dermatosis type and subcorneal pustular dermatosis type, is important because treatment of IgA pemphigus has to be different from treatment of other blistering[ncbi.nlm.nih.gov]
- The primary lesions are pea-sized pustules classically described as half-pustular, half-clear flaccid fluid blisters.[ncbi.nlm.nih.gov]
Eczema
- , Cheiropompholyx, Dyshidrotic Eczema, Pompholyx, Podopompholyx,) Stasis Dermatitis (42 %) ( Congestion Eczema, Gravitational Dermatitis, Gravitational Eczema, Stasis Eczema, Varicose Eczema,) Toxic Shock Syndrome (39 %) ( Streptococcal Toxic Shock Syndrome[gulfdoctor.net]
- Relapsing polychondritis 673 211 Rhinophyma 677 212 Rocky Mountain spotted fever and other rickettsial infections 679 213 Rosacea 684 214 Sarcoidosis 692 215 Scabies 697 216 Scleredema 700 217 Scleroderma 703 218 Sebaceous hyperplasia 707 219 Seborrheic eczema[books.google.com]
- We use systematic evaluation of common skin conditions with new visual tools such as dermoscopy as well as machine-learning techniques that enabled us to develop eczema-detecting algorithms.[navarinilab.com]
- L20.84 Intrinsic (allergic) eczema Reimbursement claims with a date of service on or after October 1, 2015 require the use of ICD-10-CM codes.[icd10data.com]
- (more popularly known as dyshidrotic eczema or pompholyx) is an intensely pruritic, vesicular eruption affecting the palms, soles. 14 May 2013 Psoriasis palmoplantar: Cuando caminar duele.[afytuzyr.xpg.uol.com.br]
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]
- 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]
- Areas of desquamation, crusting and hyperpigmentation may occur with the rupture of pustules [ 1 ]. Although the etiopathogenesis of disease is not clear, many theories, including infectious and autoimmune mechanisms, have been proposed [ 2 ].[archives-inflammation.imedpub.com]
Pruritus
- The pustular eruption typically involves the flexural sites of the trunk and proximal extremities with pruritus and irritation symptoms.[ncbi.nlm.nih.gov]
- 621 199 Pruritus ani 629 200 Pruritus vulvae 631 201 Pseudofolliculitis barbae 634 202 Pseudoxanthoma elasticum 638 203 Psoriasis 640 204 Psychogenic excoriation 651 205 Pyoderma gangrenosum 654 206 Pyogenic granuloma 659 207 Radiation dermatitis 662[books.google.com]
- 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]
- It may be preceded or accompanied by fever and pruritus.[scielo.br]
- Simon de Veer Thesis (PDF 32MB) - QUT ePrints ; Atopic dermatitis, corneocyte, desmosome, desquamation, drug discovery, epidermis, ...... cutaneous side effects, including a pustular rash, dry skin and pruritus (Mascia et ...... in triplicate, after which[wcsbdbmf.cf]
Pustular Rash
- […] and pustular rash, which can be a visible marker of therapeutic efficacy. [52] [53] The rash onset is between weeks 1 and 3 of treatment, peaking between weeks 3 and 5, and resolves 4 weeks after withdrawal of treatment.[online.epocrates.com]
- Pustular rashes - Oxford Medicine ; Introduction316Skin problems presenting with pustules317Pustular rashes of the head and neck318Pustular rashes of trunk and limbs322Acral pustular rashes324Pustular rashes in neonates and infants325(figure 20.1) are[zgxrrbli.cf]
- Simon de Veer Thesis (PDF 32MB) - QUT ePrints ; Atopic dermatitis, corneocyte, desmosome, desquamation, drug discovery, epidermis, ...... cutaneous side effects, including a pustular rash, dry skin and pruritus (Mascia et ...... in triplicate, after which[wcsbdbmf.cf]
Neurologic
Myelopathy
- […] relevant points arise from this case: the association of the dermatosis with an IgAlambda myeloma that, as far as we know, has been described only three times previously; the longest period of time between the onset of the dermatosis and that of the myelopathy[ncbi.nlm.nih.gov]
- […] disorder *angiopathy 【血管神経膠腫】*angioglioma 【血管神経膠腫症】*angiogliomatosis 【血管神経症】*vasoneurosis *angioneuropathy 【血管心臓炎】*angiocarditis 【血管性耳炎】*angiotitis ( 耳血管炎) 【血管性水腫】*vascular edema 【血管性痴呆】*vascular dementia 【血管性母斑】*vascular nevus 【血管性ミエロパチー】*vascular myelopathy[medo.jp]
Trichotillomania
- […] capitis 752 234 Tinea pedis and skin dermatophytosis 756 235 Tinea unguium 759 236 Tinea versicolor pityriasis versicolor 763 237 Toxic epidermal necrolysis and StevensJohnson syndrome 766 238 Transient acantholytic dermatosis Grover disease 770 239 Trichotillomania[books.google.com]
Workup
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].
Microbiology
Coccidioides Immitis
- Abstract Coccidioidomycosis (AKA "Valley fever") is a primary pulmonary infection via airborne spores released from coccidioides immitis in the soil.[ncbi.nlm.nih.gov]
- immitis. ( 26437157 ) Iyengar S....Sharon V.R. 2015 14 Successful treatment of subcorneal pustular dermatosis with maxacalcitol. ( 25991581 ) Hoshina D....Furuya K. 2015 15 Antidesmocollin 1 autoantibody negative subcorneal pustular dermatosis-type IgA[malacards.org]
- Subcorneal pustular dermatosis associated with Coccidioides immitis. Dermatol Online J. 2015 Aug 15. 21 (8): [Medline]. Taniguchi S, Tsuruta D, Kutsuna H, Hamada T. Subcorneal pustular dermatosis in a patient with hyperthyroidism.[emedicine.medscape.com]
- Subcorneal pustular dermatosis associated with Coccidioides immitis. Dermatol Online J . 2015 Aug 15. 21 (8): [Medline] . Taniguchi S, Tsuruta D, Kutsuna H, Hamada T. Subcorneal pustular dermatosis in a patient with hyperthyroidism.[emedicine.medscape.com]
Treatment
- Diagnosing IgA pemphigus and distinguishing between its 2 subtypes, intraepidermal neutrophilic IgA dermatosis type and subcorneal pustular dermatosis type, is important because treatment of IgA pemphigus has to be different from treatment of other blistering[ncbi.nlm.nih.gov]
- 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]
- In addition, a sustained remission of SPD was achieved without further treatment.[ncbi.nlm.nih.gov]
- Treatment with corticosteroids, vitamin E, dapsone, sulphapyridine and levamisole was ineffective.[ncbi.nlm.nih.gov]
- The therapeutic value of phototherapy for the treatment of SPD still has to be confirmed and could be a valuable alternative for treatment-resistant patients.[ncbi.nlm.nih.gov]
Prognosis
- 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]
- Dermatosis · Bacterial impetigo · Dermatitis herpetiformis · Pemphigus foliaceus · Immunoglobulin A pemphigus/intraepidermal immunoglobulin A pustulosis · Pustular psoriasis · Necrolytic migratory erythema · Acute generalized exanthematous pustulosis PROGNOSIS[dermaamin.com]
Etiology
- 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]
- Subcorneal pustular dermatosis (SPD) [Sneddon-Wilkinson disease] is a benign and uncommon disorder characterized by a chronic, relapsing vesiculopustular eruption of unknown etiology.[ncbi.nlm.nih.gov]
- Subcorneal pustular dermatosis (SPD) is a chronic recurrent pustular dermatosis of unknown etiology. Many treatments have been proposed, none of which has been uniformly successful.[ncbi.nlm.nih.gov]
- 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[symptoma.com]
- Etiologies include immunologic and genetic factors.[ebi.ac.uk]
Epidemiology
- 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]
- 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]
- 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.[dermaamin.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]
Sex distribution
Age distribution
Pathophysiology
- In this report, we review both the pathophysiology of SPD, which has yet to be clarified, and its treatment. Data obtained from our case report add further support to the hypothesis that a non-antibody-mediated mechanism is operative in SPD.[ncbi.nlm.nih.gov]
- This review looks at the available immunofluorescence staining data in the hope of finding a better characterization of this subset of patients and speculation as to the underlying pathophysiology.[ncbi.nlm.nih.gov]
- The pathophysiology and autoantigen profile of bullous autoimmune diseases, especially pemphigus and its subforms, are more complex than previously believed.[ncbi.nlm.nih.gov]
- 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]
Prevention
- 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.[ncbi.nlm.nih.gov]
- Thus, unnecessary therapy and contamination among people can be prevented.[ncbi.nlm.nih.gov]
- 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]
- European journal of cardiovascular prevention and rehabilitation: official journal of the European Society of Cardiology, Working Groups on Epidemiology & Prevention and Cardiac Rehabilitation and Exercise Physiology. 2007; 14 :438–40. 5.[clinicaldermatology.eu]
References
Article
- Abreu-Velez AM, Smith JG, Howard MS. Subcorneal pustular dermatosis an immnohisto-pathological perspective. Int J Clin Exp Pathol. 2011;4(5):526-529.
- Ono S, Otsuka A, Miyachi Y, Kabashima K. Subcorneal Pustular Dermatosis Exhibiting a High Serum TARC/CCL17 Level. Case Rep Dermatol. 2013;5(1):38-42.
- 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.
- Keller M, Spanou Z, Schaerli P, Britschgi M, Yawalkar N, Seitz M, Villiger PM, Pichler WJ. T cell-regulated neutrophilic inflammation in autoinflammatory diseases. J Immunol. 2005;175:7678–7686.
- Cheng S, Edmonds E, Ben-Gashir M, Yu RC. Subcorneal pustular dermatosis: 50 years on. Clin Exp Dermatol. 2008;33(3):229-233.
- Watts PJ, Khachemoune A. Subcorneal Pustular Dermatosis: A Review of 30 Years of Progress. Am J Clin Dermatol. 2016;17(6):653-671.
- Nischal KC, Khopkar U. An approach to the diagnosis of neutrophilic dermatoses: A histo-pathological perspective. Indian J Dermatol Venereol Leprol. 2007;73:222–230.
- 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.