Pustular Dermatosis Research Articles

A Case of IgG/IgA Pemphigus Presenting Malar Rash-like Erythema

Pemphigus is an autoimmune mucocutaneous bullous disease characterized by auto-antibodies against cell surface antigens of epidermal keratinocytes. Pemphigus vulgaris (PV) and pemphigus foliaceus (PF) are the major subtypes. Several other variants have been proposed, in-cluding pemphigus erythematosus, pemphigus vegetans, pemphigus herpetiformis (PH), and paraneoplastic pem-phigus. Deposition of IgG on epidermal keratinocyte cell surfaces and circulating anti-cell surface antibodies are characteristic in pemphigus. Cases involving IgA depo-sition on epidermal keratinocyte cell surfaces have been reported as IgA pemphigus. IgA pemphigus is divided into 4 subgroups based on clinical manifestation: subcorneal pustular dermatosis type, intraepidermal neutrophilic IgA dermatosis type, pemphigus foliaceus type, and pemphi-gus vulgaris type. Cases involving deposition of both IgG and IgA on keratinocyte cell surfaces have been reported (1–13). Some authors describe them as IgG/IgA pemphi-gus (1). Seventeen such cases have been reported so far, and heterogeneity of clinical features and target antigen has been detected in this group of pemphigus. CASe RePoRT

Disseminated Erosive Pustular Dermatosis also Involving the Mucosa: Successful Treatment with Oral Dapsone

Erosive pustular dermatosis (EPD) is a rare cutaneous inflammatory disease of unknown aetiology characterized by sterile pustules, crusted erosions, cicatricial alopecia and skin atrophy. Since its original description in 1977, there have been approximately 150 cases published in the literature, most of which have reported erosive pustular dermatosis involving the scalp (1–3). Erosive pustular dermatosis can also occur on the legs, where it is highly associated with venous insufficiency, ulcers, cutaneous atrophy and diverticular disease (4–11). We report here the case of a woman with disseminated erosive pustular dermatosis affecting the scalp, upper and lower extremities, groin, and tongue, which was treated successfully with oral dapsone.

Aminolevulinic Acid Photodynamic Therapy in the Treatment of Erosive Pustular Dermatosis of the Scalp

Aminolevulinic Acid Photodynamic Therapy in the Treatment of Erosive Pustular Dermatosis of the Scalp

Erosive pustular dermatosis of the scalp from topical minoxidil 5% solution

Erosive pustular dermatosis of the scalp from topical minoxidil 5% solution

Concurrent pyoderma gangrenosum and subcorneal pustular dermatosis in a patient with monoclonal IgA/λ gammopathy

Subcorneal pustular dermatosis (SPD) and pyoderma gangrenosum (PG) are two neutrophilic dermatoses. Coexistence of these diseases in the same patient is rare and may be a strong indicator of IgA dysglobulinemia. We describe a 69-year-old man who presented with waxing and waning flaccid pustules covering his trunk and four limbs. Poorly healing ulcerations, which usually progressed into larger nodules after debridement, were also noted. Repeated cultures were negative for bacteria, and the patient was diagnosed with SPD and PG. Serum protein electrophoresis and immunofixation revealed a monoclonal IgA lambda protein. A subsequent bone marrow biopsy revealed a normocellular marrow. While PG and SPD can occur individually in a variety of associated diseases, such as rheumatoid arthritis, systemic lupus erythematosus and inflammatory bowel disease or infection; however, their coexistence is strongly indicative of IgA dysglobulinemia.

Erosive pustular dermatosis and associated alopecia successfully treated with topical tacrolimus

To the Editor: Erosive pustular dermatosis (EPD) of the scalp presents with pustules, erosions, and crusting, which often leads to scarring alopecia that may be reversible. EPD is often under-diagnosed in the United States, and failure to diagnose this condition may result in unnecessary surgery.1

Association pyoderma gangrenosum, pustulose sous-cornée et abcès aseptiques spléniques : « une maladie neutrophilique »

Neutrophilic diseases may have various clinical presentations but share common histopathological manifestations with an aseptic infiltrate of polymorphonuclears neutrophils. The association with various diseases is frequent and must be systematically discussed. We report a patient who presented three different synchronous clinical manifestations of neutrophilic disease: pyoderma gangrenosum, subcorneal pustular dermatosis and aseptic spleen abscesses.

Erosive pustular dermatosis of the scalp and its treatment

Erosive pustular dermatosis of the scalp (EPDS ) is an uncommon and painful skin disease characterized by erythema,sterile pustules,erosion,ulceration,and crusted plaques of the scalp of elderly patients,which is often chronic and relapsing,and can eventually cause scarring alopecia and even squamous cell carcinoma.The etiology is unknown,but predisposing factors have been reported such as trauma,prolonged exposure to sunlight as well as coexistence of autoimmune diseases.EPDS is a diagnosis of exclusion based on histological examination and microbial cultures.Multiple therapies have been documented for the treatment of EPDS,and oral isotretinoin is supposed to be the most effective. Key words: Impetigo; Scalp; Diagnosis; Therapy

Dermatoses neutrofílicas: parte II

Neste artigo são abordadas as dermatoses neutrofílicas, complementando o artigo anterior (parte I). São apresentadas e comentadas as seguintes dermatoses: pustulose subcórnea de Sneddon-Wilkinson, dermatite crural pustulosa e atrófica, pustulose exantemática generalizada aguda, acroder matite contínua de Hallopeau, pustulose palmoplantar, acropustulose infantil, bacteride pustular de Andrews e foliculite pustulosa eosinofílica. Uma breve revisão das dermatoses neutrofílicas em pacientes pediátricos também é realizada.

Erosive pustular dermatosis of the leg: Long-term control with topical tacrolimus

Erosive pustular dermatosis of the leg is an unusual form of sterile pustulosis that typically affects the lower limbs of elderly patients. We report the cases of two women who developed erythematous skin plaques with pustules that coalesced and evolved into erosions and crusted areas. Histology showed epidermal spongiosis with subcorneal pustules and a dermal infiltrate with eosinophils and neutrophils. Lesions were treated with topical clobetasol propionate 0.05% for 10 days followed by topical tacrolimus daily until complete resolution, and then twice weekly for 1 year, without relapse. The response to topical corticosteroids and tacrolimus further support the close relationship with erosive pustular dermatosis of the scalp. Topical therapy with tacrolimus may offer good long-term disease control.

Erosive Pustular Dermatosis of the Scalp Responding to Acitretin

Erosive Pustular Dermatosis of the Scalp Responding to Acitretin

Dermatose érosive et pustuleuse de jambes : étude rétrospective de 16 cas

Erosive pustular dermatosis of the leg (EPDL) is a chronic clinical entity comprising combined erosion, pustules and crusts on the legs. There is still some discussion of the independent existence of this condition in the absence of specific diagnostic criteria. The purpose of this study is to describe the clinical and laboratory characteristics of EPDL based on a series of patients presenting a clinical picture consistent with this diagnosis. This retrospective study included all patients seen in our department between 2005 and 2009 presenting a clinical picture consistent with EPDL, in accordance with the initial description. We collated and carried out descriptive analysis of the clinical features and progression of the disease and of laboratory results (microbiology, immunology and vascular tests). In all of the 16 patients included (mean age: 81 years; sex ratio M/F: 0.2), lesions were consistently located in the middle third of the anterior aspect of the leg and associated with ochre dermatitis and skin atrophy; they were bilateral in 10 of the 16 patients. For the most part, laboratory tests were negative or inconclusive, with the exception of direct cutaneous immunofluorescence (DIF). DIF was performed in 14 patients and in three cases showed linear C3 deposits, thus confirming the diagnosis of pretibial bullous pemphigoid. In the 13 remaining cases, a diagnosis of idiopathic EPDL was made. Three of these 13 patients were either presenting or had previously presented squamous cell carcinoma of the leg. Topical corticosteroids were effective in 12 of these 13 cases (mean treatment duration: six months). Relapse was common (6/12). Our study demonstrates the need for skin biopsy with DIF for patients presenting a clinical picture evocative of EPDL, since the clinical presentation can be very similar to that of pretibial pemphigoid. Trophic disorders associated with venous stasis are common in EPDL, although they are difficult to interpret because of the high prevalence of this condition among the elderly. Mention must be made of associated marked sun damage, suggesting a possible relationship between EPDL and erosive pustular dermatosis of the scalp.

Erosive pustular dermatosis of the scalp mimicking cutaneous extension of metastatic breast carcinoma

Erosive pustular dermatosis of the scalp mimicking cutaneous extension of metastatic breast carcinoma

On the missing link between inflammation and cancer

A various array of cutaneous granulomatous disorders have been found to be associated with internal malignancy. Among them, sarcoidosis, granuloma anulare (GA), psoriasis, pyoderma gangrenosum (PG), or other neutrophilic dermatoses such as the Sweet syndrome and subcorneal pustular dermatosis may precede the development of a neoplastic process by months or years. Pathogenic links of inflammation with cancer are discussed, including inflammation, intrinsic immune dysfunction, cytokines and interleukins, angiogenetic factors, and epigenetic changes.

Pustulose aseptique des plis et érythroblastopénie auto-immune

Amicrobial pustulosis of the skin folds represents a new entity within the spectrum of neutrophilic dermatoses. This disease is characterized by acute onset of pustular lesions in the skin folds, association with an autoimmune disorder, and improvement under systemic corticosteroids. A 24-year-old woman had been presenting pustular dermatosis for several months involving the inguinal folds and the scalp. The pustules coalesced to form oozing and crusting plaques. Eczematous lesions were located on the trunk. She also presented macrocytic anemia related to autoimmune erythroblastopenia. Bacteriological culture was negative. Antinuclear antibodies were found with anti-SSA specificity. Histopathological examination of a skin biopsy specimen showed pustules in the epidermis together with an inflammatory dermal infiltrate. Cutaneous direct immunofluorescence testing was negative. The patient responded to systemic corticosteroids. Thirty-six cases of amicrobial pustulosis of the skin folds have been reported in the literature. All but two previously reported patients were females with an autoimmune disorder (chiefly systemic lupus erythematosus). The clinical picture is characterized by aseptic pustular lesions of the major and minor skin folds of the scalp and the anogenital area associated with eczematous lesions. Diagnostic criteria have recently been proposed. This disease responds to systemic corticosteroids. We report a new case of amicrobial pustulosis of the skin folds associated with autoimmune erythroblastopenia, which to the best of our knowledge has been described only once in the literature.

Subcorneal Pustular Dermatosis with Hyperthyroidism

Subcorneal Pustular Dermatosis with Hyperthyroidism

Subcorneal pustular dermatosis treated successfully with adalimumab

Auteur(s) : Filipa de Encarnacao Roque Diamantino, Joana Miguel Ramos Dias Coelho, Ana Maria Macedo Ferreira, Ana Isabel Pina Clemente Fidalgo Department of Dermatology, Hospital dos Capuchos, Centro Hospitalar de Lisboa Central, Alameda Santo Antonio dos Capuchos, 1169-050 Lisboa, Portugal A 43-year-old, overweight, but otherwise healthy woman presented with a 3-year history of a relapsing eruption of numerous, grouped, itchy pustules affecting her trunk and [...]

Hair loss in elderly women

Hair loss in elderly women has been becoming a major topic in the daily practice of dermatology. Aging of hair follicles seems to affect hair growth and pigmentation, the molecular mechanisms of which remain to be elucidated. Further senile changes in physiology and immunity may influence the onset and course of hair diseases. Some preexisting diseases such as androgenetic alopecia usually worsen after menopause, while others, like discoid lupus erythematosus, may attenuate. Hormone surveying, especially with regard to internal androgen-producing tumors, is indicated in postmenopausal women with androgenetic alopecia of sudden exacerbation or with unusual manifestation or other virilizing signs. The prevalence of alopecia totalis and alopecia universalis appears to be much lower in postmenopausal ages as compared to earlier onset. Acute or chronic telogen effluvium is not uncommonly superimposed on androgenetic alopecia. Trichotillomania shows a marked female predominance in the senile age group with a higher rate of psychopathology. Worldwide, tinea capitis has been increasingly observed in postmenopausal women. Frontal fibrosing alopecia, giant cell arteritis and erosive pustular dermatosis involve mainly elder women leading to scarring alopecia. Alopecia induced by tumor metastasis to the scalp must be considered in women with underlying neoplasms, especially breast cancer. Overall, hair loss in postmenopausal women is often multifactorial and warrants a close inspection.

Erosive pustular dermatosis of the scalp: a successful treatment with photodynamic therapy

Erosive pustular dermatosis of the scalp (EPDS) is a rare inflammatory disease of unknown aetiology that usually occurs in the elderly. It is characterized by sterile pustules, chronic crusted erosions, cicatricial alopecia, and skin atrophy. The histopathology is nonspecific, and its pathophysiology remains undetermined, with various types of local trauma possibly acting as the triggering factor. We describe a case of EPDS in a 75-year-old female in whom there was a marked response to photodynamic therapy with methyl 5-aminolaevulinic acid.

Erosive Pustular Dermatosis of the Scalp Following Herpes Zoster: Successful Treatment with Topical Tacrolimus

Erosive pustular dermatosis of the scalp (EPDS) is a rare disorder of the elderly characterized by multiple pustular lesions with erosions and crusting that result in scarring alopecia. EPDS typically develops in aged or sun-damaged skin with a history of trauma. Histopathologically, EPDS is nonspecific, showing atrophic epidermis and chronic inflammation. Bacteriological and mycological investigations of EPDS are generally negative. Although herpes zoster is a common disorder in elderly people, previously reported cases of EPDS were rarely associated with herpes zoster. We present a rare case of EPDS following herpes zoster treated successfully with topical tacrolimus.