Sneddon-Wilkinson Dermatosis

Background

This is a rare blistering and pustular disease which affects the trunk, axillae, and flexural areas. The pustules spread in an annular or gyrate pattern. Some investigators consider this disease a variant of pustular psoriasis.

OUTLINE

Epidemiology

Disease Associations

Pathogenesis

Laboratory/Radiologic/Other Diagnostic Testing

Gross Appearance and Clinical Variants

Histopathological Features and Variants

Special Stains/Immunohistochemistry/Electron Microscopy

Differential Diagnosis

Prognosis

Treatment

Commonly Used Terms

Internet Links

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.

Delaporte E, Colombel JF, Nguyen-Mailfer C, Piette F, Cortot A, Bergoend H.

Department of Dermatology, University Hospital Center, Lille, France.
Acta Derm Venereol 1992 Aug;72(4):301-2 Abstract quote
A case of subcorneal pustular dermatosis (Sneddon-Wilkinson disease) is reported in a patient with a one-year history of Crohn's disease. Subcorneal pustular dermatosis has been described in association with monoclonal gammopathy, but to our knowledge it has not been associated with Crohn's disease.

This new association reinforces the hypothesis of a possible common pathogenesis for neutrophilic dermatoses and inflammatory bowel diseases.

MONOCLONAL GAMMOPATHY
Br J Dermatol 1994;130:398-399

Most common is IgA and myeloma

Subcorneal pustular dermatosis (Sneddon-Wilkinson disease) in association with a monoclonal IgA gammopathy: a report and review of the literature.

Kasha EE Jr, Epinette WW.

Department of Dermatology, Indiana University School of Medicine, Indianapolis 46223.
J Am Acad Dermatol 1988 Nov;19(5 Pt 1):854-8 Abstract quote
Subcorneal pustular dermatosis (Sneddon-Wilkinson disease) may be associated with a monoclonal gammopathy, most commonly an IgA paraproteinemia. 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.

OSTEOMYELITIS

Anterior chest wall arthritis and osteitis associated with Sneddon-Wilkinson disease.

Belfiore N, Caporali R, Borroni G, Montecucco C.

Dipartimento di Medicina Interna e Nefrologia, IRCCS Policlinico S. Matteo, University of Pavia, Italy.
Clin Exp Rheumatol 1997 Nov-Dec;15(6):667-9 Abstract quote
We describe a 46-year-old man in whom anterior chest wall arthritis and clavicular osteomyelitis occurred together with sub-corneal pustular dermatosis (Sneddon-Wilkinson disease). This observation extends the list of neutrophilic skin lesions that may be involved in the so-called SAPHO syndrome.

PYODERMA GANGRENOSUM

PATHOGENESIS CHARACTERIZATION

TUMOR NECROSIS FACTOR

Role of tumor necrosis factor-alpha in Sneddon-Wilkinson subcorneal pustular dermatosis. A model of neutrophil priming in vivo.

Grob JJ, Mege JL, Capo C, Jancovicci E, Fournerie JR, Bongrand P, Bonerandi JJ.

Service de Dermatologie, Hopital Sainte Marguerite, Marseille, France.
J Am Acad Dermatol 1991 Nov;25(5 Pt 2):944-7 Abstract quote
A patient with IgG-kappa-associated subcorneal pustular dermatosis (Sneddon-Wilkinson disease) refractory to dapsone, etretinate, and plasma exchange was successfully treated with corticosteroids.

A study of neutrophils from both blood and pustules was carried out before and during treatment. Levels of tumor necrosis factor-alpha were measured in serum, pustules, content, and supernatant of monocytes. The results suggest that a hyperactivation of neutrophils in the skin is due at least partly to excessive production of tumor necrosis factor-alpha.

CLINICAL VARIANTS CHARACTERIZATION

GENERAL

Subcorneal pustular dermatosis: a clinical study of ten patients.

Lutz ME, Daoud MS, McEvoy MT, Gibson LE.

Department of Dermatology, Mayo Clinic, Rochester, Minnesota 55905, USA.
Cutis 1998 Apr;61(4):203-8 Abstract quote
Subcorneal pustular dermatosis of Sneddon and Wilkinson (SPD) is a unique clinical and histologic entity. This disorder has been reported in association with monoclonal gammopathy and IgA pemphigus.

We report ten additional cases of SPD and determine the frequency of monoclonal gammopathy and IgA pemphigus in patients with SPD and investigate the relationship of this entity with other dermatoses characterized histologically by a subcorneal pustule. The medical records of patients with SPD evaluated at the authors' institution from 1980 through 1995 were reviewed retrospectively. Medical records of twenty patients with pustular psoriasis were reviewed for comparison. Ten patients met the criteria for SPD. Their average age at diagnosis was 66 years. The characteristic flaccid pustules were often generalized and had a tendency to involve the flexural areas. Serum monoclonal gammopathy was present in four patients: three with IgA and one with IgG. On direct immunofluorescence examination, three patients had IgA deposits in the intercellular spaces. None of the patients exhibited both monoclonal gammopathy and IgA deposits. Repeated direct immunofluorescence studies were necessary to detect IgA pemphigus in one patient. SPD is a distinct clinical entity.

The findings of IgA deposits intercellularly on immunofluorescence and monoclonal gammopathy may help to further identify this entity.

VARIANTS

LYMPHATIC INVOLVEMENT

Subcorneal pustular dermatosis (Sneddon-Wilkinson disease) with amicrobial lymph node suppuration and aseptic spleen abscesses.

Dallot A, Decazes JM, Drouault Y, Rybojad M, Verola O, Morel P, Modai J, Puissant A.

Department of Dermatology, Hospital Saint-Louis, Paris, France.
Br J Dermatol 1988 Dec;119(6):803-7 Abstract quote
Subcorneal pustular dermatosis (SPD) or Sneddon-Wilkinson disease is a skin eruption in which concomitant systemic involvement has not been reported to our knowledge.

We describe a patient suffering from SPD of 6 years duration with amicrobial inguinal lymph node suppuration and aseptic spleen abscesses followed by pyoderma gangrenosum.

HISTOLOGICAL TYPES CHARACTERIZATION

General Subcorneal collections of neutrophils

VARIANTS

IgA PUSTULAR DERMATOSIS

Intraepidermal IgA pustulosis.

Wallach D.

Department of Dermatology, Hopital Saint-Louis, Paris, France.
J Am Acad Dermatol 1992 Dec;27(6 Pt 1):993-1000 Abstract quote
Since 1979, 29 patients with intraepidermal IgA detected by direct immunofluorescence have been reported.

A review shows that they have a cutaneous disease clinically similar to subcorneal pustular dermatosis, or Sneddon-Wilkinson disease. The pustules may be subcorneal or intraepidermal. IgA deposits are usually found on the intercellular substance of the epidermis, although a subcorneal linear pattern has also been described. Circulating IgA class antiepidermal autoantibodies may be present. The disease usually responds to dapsone. In six cases, a monoclonal IgA gammopathy was present.

Although little is known about the pathogenesis of this condition, we believe that it represents a distinct entity among the group of the neutrophilic dermatoses. Various diagnostic terms have been used; we propose intraepidermal IgA pustulosis.

VASCULITIS

Chronic subcorneal pustulosis with vasculitis: a variant of generalized pustular psoriasis in black South Africans.

Barlow RJ, Schulz EJ.

Medical University of Southern Africa, Pretoria, South Africa.
Br J Dermatol 1991 May;124(5):470-4 Abstract quote
A rare, but distinctive chronic eruption in six female black South Africans is reported. The original diagnosis of subcorneal pustular dermatosis of Sneddon and Wilkinson in these patients was refuted by the subsequent histological observation of both spongiform pustules and an underlying vasculitis.

This may represent a previously undocumented form of generalized pustular psoriasis.

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 CHARACTERIZATION

TREATMENT Dapsone

ACITRETIN

Successful treatment of subcorneal pustular dermatosis (Sneddon-wilkinson disease) by acitretin: report of a case.

Marliere V, Beylot-Barry M, Beylot C, Doutre M.

Department of Dermatology, Haut-Leveque Hospital, University of Bordeaux, Pessac, France.
Dermatology 1999;199(2):153-5 Abstract quote
We report a case of subcorneal pustular dermatosis (SPD) first treated with dapsone with poor response. Dapsone was changed to acitretin, which dramatically improved the lesions in a few days. Dapsone is the first-line treatment in SPD, but it may be ineffective in some cases and its toxicity is important. Therapeutic alternatives are limited and less effective (systemic corticosteroids, phototherapy).

Our observation underlines the usefulness of retinoids, when dapsone is ineffective or poorly tolerated. Their effectiveness is comparable, but they are effective more rapidly and are better tolerated. A dose maintenance is necessary to avoid relapses. Their action in SPD remains unclear but may be due to the inhibition of neutrophil functions.

INFLIXIMAB

Infliximab (anti-tumor necrosis factor alpha antibody): a novel, highly effective treatment of recalcitrant subcorneal pustular dermatosis (Sneddon-Wilkinson disease).

Voigtlander C, Luftl M, Schuler G, Hertl M.

Department of Dermatology, University of Erlangen-Nurnberg, Erlangen, Germany.
Arch Dermatol 2001 Dec;137(12):1571-4

PHOTOTHERAPY

Subcorneal pustular dermatosis (Sneddon-Wilkinson disease) treated with narrowband (TL-01) UVB phototherapy.

Cameron H, Dawe RS.
Br J Dermatol 1997 Jul;137(1):150-1

Henry JB. Clinical Diagnosis and Management by Laboratory Methods. Twentieth Edition. WB Saunders. 2001.
Rosai J. Ackerman's Surgical Pathology. Eight Edition. Mosby 1996.
Sternberg S. Diagnostic Surgical Pathology. Third Edition. Lipincott Williams and Wilkins 1999.
Robbins Pathologic Basis of Disease. Sixth Edition. WB Saunders 1999.
DeMay RM. The Art and Science of Cytopathology. Volume 1 and 2. ASCP Press. 1996.
Weedon D. Weedon's Skin Pathology Second Edition. Churchill Livingstone. 2002
Fitzpatrick's Dermatology in General Medicine. 5th Edition. McGraw-Hill. 1999.
Weiss SW and Goldblum JR. Enzinger and Weiss's Soft Tissue Tumors. Fourth Edition. Mosby 2001.

Commonly Used Terms

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Last Updated 9/18/2003

DISEASE ASSOCIATIONS	CHARACTERIZATION
CROHN'S DISEASE
Subcorneal pustular dermatosis in a patient with Crohn's disease. Delaporte E, Colombel JF, Nguyen-Mailfer C, Piette F, Cortot A, Bergoend H. Department of Dermatology, University Hospital Center, Lille, France.	Acta Derm Venereol 1992 Aug;72(4):301-2 Abstract quote A case of subcorneal pustular dermatosis (Sneddon-Wilkinson disease) is reported in a patient with a one-year history of Crohn's disease. Subcorneal pustular dermatosis has been described in association with monoclonal gammopathy, but to our knowledge it has not been associated with Crohn's disease. This new association reinforces the hypothesis of a possible common pathogenesis for neutrophilic dermatoses and inflammatory bowel diseases.
MONOCLONAL GAMMOPATHY	Br J Dermatol 1994;130:398-399 Most common is IgA and myeloma
Subcorneal pustular dermatosis (Sneddon-Wilkinson disease) in association with a monoclonal IgA gammopathy: a report and review of the literature. Kasha EE Jr, Epinette WW. Department of Dermatology, Indiana University School of Medicine, Indianapolis 46223.	J Am Acad Dermatol 1988 Nov;19(5 Pt 1):854-8 Abstract quote Subcorneal pustular dermatosis (Sneddon-Wilkinson disease) may be associated with a monoclonal gammopathy, most commonly an IgA paraproteinemia. 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.
OSTEOMYELITIS
Anterior chest wall arthritis and osteitis associated with Sneddon-Wilkinson disease. Belfiore N, Caporali R, Borroni G, Montecucco C. Dipartimento di Medicina Interna e Nefrologia, IRCCS Policlinico S. Matteo, University of Pavia, Italy.	Clin Exp Rheumatol 1997 Nov-Dec;15(6):667-9 Abstract quote We describe a 46-year-old man in whom anterior chest wall arthritis and clavicular osteomyelitis occurred together with sub-corneal pustular dermatosis (Sneddon-Wilkinson disease). This observation extends the list of neutrophilic skin lesions that may be involved in the so-called SAPHO syndrome.
PYODERMA GANGRENOSUM

Epidemiology
Disease Associations
Pathogenesis
Laboratory/Radiologic/Other Diagnostic Testing
Gross Appearance and Clinical Variants
Histopathological Features and Variants
Special Stains/Immunohistochemistry/Electron Microscopy
Differential Diagnosis
Prognosis
Treatment
Commonly Used Terms
Internet Links

EPIDEMIOLOGY	CHARACTERIZATION
SYNONYMS	Subcorneal pustular dermatosis
AGE RANGE-MEDIAN	>40 years Mean of 66 years
SEX (M:F)	F

CLINICAL VARIANTS	CHARACTERIZATION
GENERAL
Subcorneal pustular dermatosis: a clinical study of ten patients. Lutz ME, Daoud MS, McEvoy MT, Gibson LE. Department of Dermatology, Mayo Clinic, Rochester, Minnesota 55905, USA.	Cutis 1998 Apr;61(4):203-8 Abstract quote Subcorneal pustular dermatosis of Sneddon and Wilkinson (SPD) is a unique clinical and histologic entity. This disorder has been reported in association with monoclonal gammopathy and IgA pemphigus. We report ten additional cases of SPD and determine the frequency of monoclonal gammopathy and IgA pemphigus in patients with SPD and investigate the relationship of this entity with other dermatoses characterized histologically by a subcorneal pustule. The medical records of patients with SPD evaluated at the authors' institution from 1980 through 1995 were reviewed retrospectively. Medical records of twenty patients with pustular psoriasis were reviewed for comparison. Ten patients met the criteria for SPD. Their average age at diagnosis was 66 years. The characteristic flaccid pustules were often generalized and had a tendency to involve the flexural areas. Serum monoclonal gammopathy was present in four patients: three with IgA and one with IgG. On direct immunofluorescence examination, three patients had IgA deposits in the intercellular spaces. None of the patients exhibited both monoclonal gammopathy and IgA deposits. Repeated direct immunofluorescence studies were necessary to detect IgA pemphigus in one patient. SPD is a distinct clinical entity. The findings of IgA deposits intercellularly on immunofluorescence and monoclonal gammopathy may help to further identify this entity.
VARIANTS
LYMPHATIC INVOLVEMENT
Subcorneal pustular dermatosis (Sneddon-Wilkinson disease) with amicrobial lymph node suppuration and aseptic spleen abscesses. Dallot A, Decazes JM, Drouault Y, Rybojad M, Verola O, Morel P, Modai J, Puissant A. Department of Dermatology, Hospital Saint-Louis, Paris, France.	Br J Dermatol 1988 Dec;119(6):803-7 Abstract quote Subcorneal pustular dermatosis (SPD) or Sneddon-Wilkinson disease is a skin eruption in which concomitant systemic involvement has not been reported to our knowledge. We describe a patient suffering from SPD of 6 years duration with amicrobial inguinal lymph node suppuration and aseptic spleen abscesses followed by pyoderma gangrenosum.

HISTOLOGICAL TYPES	CHARACTERIZATION
General	Subcorneal collections of neutrophils
VARIANTS
IgA PUSTULAR DERMATOSIS
Intraepidermal IgA pustulosis. Wallach D. Department of Dermatology, Hopital Saint-Louis, Paris, France.	J Am Acad Dermatol 1992 Dec;27(6 Pt 1):993-1000 Abstract quote Since 1979, 29 patients with intraepidermal IgA detected by direct immunofluorescence have been reported. A review shows that they have a cutaneous disease clinically similar to subcorneal pustular dermatosis, or Sneddon-Wilkinson disease. The pustules may be subcorneal or intraepidermal. IgA deposits are usually found on the intercellular substance of the epidermis, although a subcorneal linear pattern has also been described. Circulating IgA class antiepidermal autoantibodies may be present. The disease usually responds to dapsone. In six cases, a monoclonal IgA gammopathy was present. Although little is known about the pathogenesis of this condition, we believe that it represents a distinct entity among the group of the neutrophilic dermatoses. Various diagnostic terms have been used; we propose intraepidermal IgA pustulosis.
VASCULITIS
Chronic subcorneal pustulosis with vasculitis: a variant of generalized pustular psoriasis in black South Africans. Barlow RJ, Schulz EJ. Medical University of Southern Africa, Pretoria, South Africa.	Br J Dermatol 1991 May;124(5):470-4 Abstract quote A rare, but distinctive chronic eruption in six female black South Africans is reported. The original diagnosis of subcorneal pustular dermatosis of Sneddon and Wilkinson in these patients was refuted by the subsequent histological observation of both spongiform pustules and an underlying vasculitis. This may represent a previously undocumented form of generalized pustular psoriasis.

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	CHARACTERIZATION
TREATMENT	Dapsone
ACITRETIN
Successful treatment of subcorneal pustular dermatosis (Sneddon-wilkinson disease) by acitretin: report of a case. Marliere V, Beylot-Barry M, Beylot C, Doutre M. Department of Dermatology, Haut-Leveque Hospital, University of Bordeaux, Pessac, France.	Dermatology 1999;199(2):153-5 Abstract quote We report a case of subcorneal pustular dermatosis (SPD) first treated with dapsone with poor response. Dapsone was changed to acitretin, which dramatically improved the lesions in a few days. Dapsone is the first-line treatment in SPD, but it may be ineffective in some cases and its toxicity is important. Therapeutic alternatives are limited and less effective (systemic corticosteroids, phototherapy). Our observation underlines the usefulness of retinoids, when dapsone is ineffective or poorly tolerated. Their effectiveness is comparable, but they are effective more rapidly and are better tolerated. A dose maintenance is necessary to avoid relapses. Their action in SPD remains unclear but may be due to the inhibition of neutrophil functions.
INFLIXIMAB
Infliximab (anti-tumor necrosis factor alpha antibody): a novel, highly effective treatment of recalcitrant subcorneal pustular dermatosis (Sneddon-Wilkinson disease). Voigtlander C, Luftl M, Schuler G, Hertl M. Department of Dermatology, University of Erlangen-Nurnberg, Erlangen, Germany.	Arch Dermatol 2001 Dec;137(12):1571-4
PHOTOTHERAPY
Subcorneal pustular dermatosis (Sneddon-Wilkinson disease) treated with narrowband (TL-01) UVB phototherapy. Cameron H, Dawe RS.	Br J Dermatol 1997 Jul;137(1):150-1