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Background

This is a rare inflammatory dermatosis which is often associated with a scarring alopecia.

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  
INCIDENCE/
PREVALENCE
 
AGE  
SEX  
GEOGRAPHY  
EPIDEMIOLOGIC ASSOCIATIONS  

 

DISEASE ASSOCIATIONS CHARACTERIZATION
AUTOIMMUNE DISEASE  
Pustular dermatosis of the scalp associated with autoimmune diseases.

Watanabe S, Takizawa K, Hashimoto N, Ishibashi Y.

J Dermatol. 1989 Oct;16(5):383-7. Abstract quote  

A 36-year-old woman visited our hospital with a five month history of persistent pustulation, crusting, and alopecia on the vertex of the scalp. No pathological organisms were isolated from the lesions.

Histological examination revealed non-specific changes of chronic inflammation with destroyed follicles. Antibiotic therapy produced no response, but steroid therapy was effective. From these observations, a diagnosis of erosive pustular dermatosis of the scalp (EPDS), as described by Pye et al., was made. The patient also had Hashimoto's thyroiditis, autoimmune hepatitis, and Takayasu's aortitis.

The laboratory studies revealed an increased erythrocyte sedimentation rate, C-reactive protein 3+, hypergammaglobulinemia, and various auto-antibodies, suggesting the possibility of a pathogenesis common to both this dermatosis and the autoimmune diseases.
SKIN GRAFT  
Erosive pustular dermatosis of the scalp after skin grafting.

Martin FJ, Herrera A, Rios JJ, Moreno JC, Camacho F.

Departments of Dermatology and Pathology, Hospital Universitario "Virgen Macarena", Sevilla, Spain.
Dermatol Surg. 2001 Aug;27(8):766-7. Abstract quote  

Erosive pustular dermatosis of the scalp is a rare condition of unknown etiology that usually occurs in the elderly and is characterized by pustules that appear on the scalp leading to scarring alopecia.

The histopathology is not specific. Its onset has been related with previous trauma on the scalp. Only three cases after skin grafting have been reported.

We describe a case of erosive pustular dermatosis of the scalp appearing on a split-thickness skin graft placed after excision of a basal cell carcinoma.
Erosive pustular dermatosis of the scalp in skin grafts: report of three cases.

Ena P, Lissia M, Doneddu GM, Campus GV.

Department of Dermatology, Faculty of Medicine, Sassari University, Italy.


Dermatology. 1997;194(1):80-4. Abstract quote  

Three patients developed erosive pustular dermatosis of the scalp (EPDS). Two of them, both males, had previously undergone surgical excision for squamous cell carcinoma and basal cell carcinoma, and a female experienced avulsive trauma of the scalp. The erosive lesions and crusts were located at the site of a skin graft; microbiological cultures were negative for bacterial and fungal growth.

Histological examination ruled out pustular bullous disorders. Topical therapy with corticosteroids and antibiotics resulted in clinical remission in only 2 cases. The third case showed a tendency to recur despite numerous therapeutic attempts with oral dapsone and isotretinoin.

We conclude that surgical trauma is a possible cause of EPDS. Our patients seem to be the first reported cases of EPDS in skin grafts following plastic surgical procedures.

 

PATHOGENESIS CHARACTERIZATION

 

LABORATORY/
RADIOLOGIC/
OTHER TESTS

CHARACTERIZATION
RADIOLOGIC  
LABORATORY MARKERS  

 

GROSS APPEARANCE/
CLINICAL VARIANTS
CHARACTERIZATION
GENERAL  
Chronic atrophic erosive dermatosis of the scalp and extremities: A recharacterization of erosive pustular dermatosis.

Department of Dermatology, University of California, Davis, 3301 C St, Suite 1400, Sacramento, CA 95816, USA.

 

J Am Acad Dermatol. 2007 Sep;57(3):421-7. Abstract quote

BACKGROUND: Erosive pustular dermatosis (EPD) is a rarely reported condition that primarily involves the actinically damaged scalp of elderly women. Although the condition is well recognized in the United Kingdom and Europe, no US cases have heretofore been reported.

OBJECTIVES: We sought to document the presence, and determine the clinical characteristics, of EPD in the US population.

METHODS: Patients were recruited from the dermatology clinic at a university in California and from the private practices of dermatologists in the Northern California region.

RESULTS: Eleven patients with EPD were identified. Eight were women and 3 were men. The scalp was involved in 9 patients and the extremities in two patients. The involved skin was actinically damaged in 9 patients. The patients were elderly (66-90 years) but one patient was a 15-year-old boy. All lesions resolved or greatly improved with the application of high-potency steroids or tacrolimus.

LIMITATIONS: Not all patients were examined personally by the authors of this article. The length of follow-up was relatively short.

CONCLUSIONS: EPD is a fairly common disease and is the most likely diagnosis in instances where chronic, nonhealing, shallow erosions occur on actinically damaged, or otherwise atrophic, skin. In spite of the name, intact pustules are rarely present. The histology is that of moderate to marked, nonspecific chronic inflammation. EPD responds well to high-potency topical steroids.
Erosive pustular dermatosis of the scalp: a case report and review of the literature.

Mastroianni A, Cota C, Ardigo M, Minutilli E, Berardesca E.

Dermatologic Institute S. Gallicano, IRCCS, Rome, Italy.

Dermatology. 2005;211(3):273-6 Abstract quote.  

Erosive pustular dermatosis of the scalp (EPDS) is a rare entity characterized by pustular, erosive and crusted lesions of the scalp with progressive scarring alopecia. The aetiology is unknown, but predisposing factors have been reported such as trauma, skin grafting, prolonged exposure to UV light of a bald scalp as well as co-existence of auto-immune diseases. Laboratory data, bacteriological and mycological investigations and histopathology are generally not diagnostic.

A 45-year-old Caucasian man with 1-year-old pustular, erosive and crusted lesions on his bald scalp was seen. Laboratory data, including auto-immunity, bacteriological and mycological investigations were negative. Histopathology was not diagnostic showing a diffuse polymorphous infiltrate involving the dermis. A diagnosis of EPDS was made. The patient was treated with topical and systemic antibiotics and steroids as well as oral nimesulide with no or partial response. Consequently, isotretinoin (0.75 mg/kg/day) was started obtaining complete resolution in few months. No relapse after 1 year of follow-up was seen. EPDS represents a distinct disease with a history of relapsing and unsatisfactory response to common treatments.

Systemic retinoids may be considered as a potentially resolutive choice.
Erosive pustular dermatosis of the scalp.

Caputo R, Veraldi S.

First Department of Dermatology and Pediatric Dermatology, University of Milan, Italy.

J Am Acad Dermatol. 1993 Jan;28(1):96-8. Abstract quote  

Three cases of erosive pustular dermatosis of the scalp are reported.

In all patients the dermatosis was characterized by pustular, erosive, and crusted lesions; in addition, two patients had areas of scarring alopecia. The results of laboratory tests, bacteriologic and mycologic investigations, and histopathologic examination were nondiagnostic.

Although erosive pustular dermatosis of the scalp is characterized by a nonpathognomonic clinical and histopathologic picture, it probably represents a disease entity.
Pustular ulcerative dermatosis of the scalp.

Jacyk WK.

German Leprosy Relief Association, National Leprosy Training Centre, Wau, Sudan.

Br J Dermatol. 1988 Mar;118(3):441-4. Abstract quote  

Six young African patients are described with erosive pustular scalp lesions leading to extensive ulceration. No bacterial or fungal cause was found.

Biopsies showed non-specific changes of atrophy with subacute or chronic inflammation. Four patients were malnourished and anaemic and three were infested with hookworm. The condition did not respond to antibiotics. Healing was obtained in one patient with systemic steroids.

The relationship of this disorder to erosive pustular dermatosis of the scalp and to pyoderma gangrenosum of atypical distribution is discussed.
VARIANTS  
LEG  
Erosive pustular dermatosis of the leg associated with compression bandaging and fungal infection.

Dawn G, Loney M, Zamiri M, Shaffrali F, Urcelay M, Patel M, Howatson SR, Douglas WS.

Departments of Dermatology and Pathology, Monklands Hospital, Airdrie, Lanarkshire ML6 0JS, UK.

Br J Dermatol. 2003 Mar;148(3):489-92. Abstract quote  

BACKGROUND: Erosive pustular dermatosis of the leg (EPDL) has been described in association with venous insufficiency and atrophy of the skin of the lower leg. Like erosive pustular dermatosis of the scalp, this disease has also been reported to be a non-infective condition.

OBJECTIVES: To investigate the clinicopathological features and, where possible, the aetiology of clinical EPDL.

METHODS: We identified a group of patients undergoing continuous compression bandaging for venous dermatitis of the legs and/or predominantly venous leg ulceration with clinical features described in patients with EPDL. They were investigated by skin biopsy, patch testing and microbiological tests for the presence of bacteria and fungi.

RESULTS: Twenty-four of 400 (6%) patients were noted to have pustules on the leg(s). There was laboratory evidence of fungal infection in 13 of 24 patients (54%), with complete and sustained resolution of pustules after antifungal treatment. Pustulation in the other 11 patients (46%) was unresponsive to antibiotics for confirmed bacterial infection; some improvement was seen with potent topical corticosteroids but full clearance was achieved only after a switch from continuous four-layer compression bandaging to the use of intermittent long stretch compression.

CONCLUSIONS: EPDL is a fairly common clinical picture seen in patients undergoing continuous compression bandaging. It may be produced by opportunistic, particularly fungal, infection. In almost half an infective aetiology cannot be demonstrated and a pyoderma gangrenosum-like process may be implicated.
Erosive pustular dermatosis of the leg: report of three cases.

Brouard MC, Prins C, Chavaz P, Saurat JH, Borradori L.

Department of Dermatology, University Hospital, CH-1211 Geneva 14, Switzerland.
Br J Dermatol. 2002 Oct;147(4):765-9. Abstract quote  

Erosive pustular dermatosis of the leg is a distinct form of spongiform amicrobial pustulosis. The disorder typically affects the lower limbs of elderly patients presenting with chronic venous insufficiency and stasis dermatitis, and has a chronic course.

Three elderly patients with chronic venous ulcers are described, who developed pustules and moist eroded lesions on the leg. The clinical and histological features were typical for erosive pustular dermatosis. The lesions rapidly responded to topical treatment with either tacrolimus or corticosteroids. Of note, this condition was associated with a diverticular disease in two patients, while in another patient an epidermoid carcinoma of the tongue was present. Erosive pustular dermatosis of the leg is an uncommon but distinct skin disorder typically associated with trophic changes of the lower limbs.

Our observations raise the question of the relation of erosive pustular dermatosis of the leg with the group of neutrophilic dermatoses. Topical immunotherapy with tacrolimus may constitute a novel therapeutic option for this frequently recalcitrant condition.

 

HISTOLOGICAL TYPES CHARACTERIZATION
GENERAL Diffuse cellulitis of the dermis associated with a mixed acute and chronic inflammatory infiltrate

 

SPECIAL STAINS/
IMMUNOPEROXIDASE/
OTHER
CHARACTERIZATION
SPECIAL STAINS  
IMMUNOPEROXIDASE  
ELECTRON MICROSCOPY  

 

DIFFERENTIAL DIAGNOSIS KEY DIFFERENTIATING FEATURES
ACNE KELOIDALIS  
DISSECTING CELLULITIS  

 

PROGNOSIS CHARACTERIZATION

 

TREATMENT CHARACTERIZATION
GENERAL  
SYSTEMIC RETINOIDS  

Macpherson and Pincus. Clinical Diagnosis and Management by Laboratory Methods. Twentyfirst Edition. WB Saunders. 2006.
Rosai J. Ackerman's Surgical Pathology. Ninth Edition. Mosby 2004.
Sternberg S. Diagnostic Surgical Pathology. Fourth Edition. Lipincott Williams and Wilkins 2004.
Robbins Pathologic Basis of Disease. Seventh Edition. WB Saunders 2005.
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. 6th Edition. McGraw-Hill. 2003.
Weiss SW and Goldblum JR. Enzinger and Weiss's Soft Tissue Tumors. Fourth Edition. Mosby 2001.


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Last Updated September 7, 2007

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