Search PubMed⌕ Search

SEARCH · Search PubMed

Results for “Pyoderma Gangrenosum”

Search indexed PubMed citations on genomics, clinical trials, systematic reviews and public health. Explore titles, authors and supplied subject terms, then open the PubMed record.

Quote a phrase for an exact phrase match. Source license links do not imply unrestricted reuse.

At least 1,135 records · Page 63Linked to original sources
PubMed1993

Ki-1-positive anaplastic large-cell lymphoma can mimic benign dermatoses.

BACKGROUND: Regressing atypical histiocytosis is a recently described disease characterized by recurrent nodules or ulcers. The cutaneous lesions appear abruptly and then regress only to return in a manner reminiscent of lymphomatoid papulosis. Immunophenotypic analysis has revealed that most cases are a form of anaplastic large-cell Ki-1-positive (CD30+) lymphoma. OBJECTIVE: We describe two patients with Ki-1-positive anaplastic large-cell lymphoma that had clinical and pathologic features of regressing atypical histiocytosis and mimicked benign dermatoses (pyoderma gangrenosum and morphea), causing a delay in confirming the true diagnosis. A third case that was readily recognized as a lymphoma is also presented. METHODS: The clinical and histopathologic findings were recorded. In addition, T-cell receptor gene rearrangement and immunophenotyping were determined in the index case. RESULTS: The index patient and second patient were diagnosed as having Ki-1-positive anaplastic large-cell lymphoma by immunophenotyping and underwent cyclophosphamide, doxorubicin, prednisone, and vincristine (CHOP) chemotherapy with complete remission. The patient detected by chart review died of her disease without receiving antineoplastic therapy; disseminated lymphoma was diagnosed at autopsy. Studies on paraffin-embedded tissue were consistent with Ki-1-positive anaplastic large-cell lymphoma. CONCLUSION: Regressing atypical histiocytosis may clinically resemble some benign dermatoses. Recent evaluation of these cases has shown that many represent a form of Ki-1-positive anaplastic large-cell lymphoma. Multiple skin biopsy specimens with immunophenotyping and gene rearrangement studies are required to arrive at the diagnosis.

Adult↗
PubMedDate not supplied

Neutrophilic dermatosis-associated sterile chronic multifocal osteomyelitis in pediatric patients: case report and review.

Atypical pyoderma gangrenosum (PG) and Sweet syndrome are neutrophilic dermatoses that share some common features. Sterile chronic recurrent multifocal osteomyelitis is a rare association of these neutrophilic dermatoses that has only been reported in children. We report a 3-year-old girl who initially presented with pain in her left hand and right leg. Roentgenograms and bone scan revealed findings of multifocal osteomyelitis affecting both femurs, the right tibia, left clavicle, right eighth costochondral junction, and left ulna. She was treated with antibiotics without improvement. Bone biopsy of the left ulna revealed histologic changes consistent with osteomyelitis, however, all cultures for bacteria, mycobacteria, and fungi were negative. She subsequently developed an ulcer surrounded by a violaceous, undermined border at the site of the bone biopsy, which also did not improve during antibiotic treatment. A biopsy specimen from this lesion demonstrated a dense perivascular and periappendageal infiltrate of neutrophils within the dermis and edema of the papillary dermis compatible with a neutrophilic dermatosis. She was treated with oral prednisone which resulted in resolution of skin lesions, bone pain, and soft tissue swelling. This case further documents the association between PG or Sweet syndrome and multifocal sterile osteomyelitis.

Biopsy↗
PubMed2003

Superficial granulomatous pyoderma: a case report of two Japanese patients and clinical comparison with foreign patients.

Superficial granulomatous pyoderma (SGP) is an unusual variant type of pyoderma gangrenosum characterized by a benign course and specific histological features; it is quite a rare cutaneous disorder in Japan. We reported two Japanese cases of SGP and compared the clinical features of 13 Japanese cases with those of 42 foreign cases. Case 1 was a 53-year-old female who presented with three indolent and ulcerative plaques with elevated edges on the back and the posterior portion of the left thigh. Case 2 was a 74-year-old female who presented with crusted and vegetative erythematous plaques on the left shoulder and left upper arm. Histological examinations revealed a characteristic three-layer granuloma in each case. Laboratory and physical examinations found no abnormalities. Topical corticosteroid, oral administration of minocycline, and/or additional supplementation with corticosteroid were effective. In comparison with foreign cases, Japanese cases show a possible differentiation in the ratio of sex and distribution of the ulcer. Females have a high incidence and the extremities are frequently involved area in Japanese cases. Up to now, 11 cases of SGP have been reported in Japan. To our best knowledge, our patients are the first Japanese cases described in the English literature.

Aged↗
PubMed2001

Cutaneous manifestations in inflammatory bowel disease.

UNLABELLED: Numerous extraintestinal manifestations in various organ systems have been reported to be associated with inflammatory bowel disease (IBD). Aim of the present paper was to evaluate the frequency of cutaneous manifestations in Crohn's disease (CD) and ulcerative colitis (UC) with respect to their location, the activity and location of the underlying disease, the treatment options and the time to remission. METHODS: The medical records of 1043 inpatients with CD and UC were screened retrospectively for extraintestinal symptoms with special regard to cutaneous manifestations. RESULTS: The prevalence of cutaneous manifestations in IBD was 22/1043 (2.1%; 18 women, 4 men; age: 31.41 +/- 9.9 [21-51] yrs.). In 15/22 patients (68.2%) the cutaneous manifestations were associated with CD, in 7/22 patients (31.8%) UC was confirmed. In 6/22 patients (27.3%) pyoderma gangrenosum (PG) was diagnosed, in 16/22 patients (72.7%) erythema nodosum (EN). EN and PG predominately occurred at the lower legs: in 68.1% the tibia was the main affection site. Other locations like breast or anus were rare. In 16/22 patients (72.7%) an acute phase of the underlying disease was evident, in 6/22 patients (27.3%) CD or UC were in remission. In patients with CD a colonic involvement was found in 86.7%. Arthritis was the most frequent coexisting extraintestinal manifestation in CD (53.3%) and UC (28.8%). Drug treatment was performed with high doses of glucocorticoids and salicylates. The time to remission in patients with EN was significantly shorter as compared to PG (5.3 +/- 1.8 vs. 19.6 +/- 14.2 weeks; p < 0.001). In 5/22 patients (22.7%) cutaneous manifestations reoccurred after a symptom-free interval. All efflorescenses reoccurred during an active phase of the underlying disease at the same manifestation site as the initial presentation. CONCLUSION: In this series the prevalence of cutaneous manifestations in IBD was 22/1043 (2.1%). EN and PG were more frequent in women with IBD, in CD, and during the acute phases of the underlying disease. EN and PG predominately affect the lower legs. Cutaneous manifestations respond well to an acute phase therapy of the underlying disease. The time to remission was significantly shorter in EN as compared to PG. However, relapses have to be considered in a relevant subgroup of patients.

Adult↗
PubMed2002

Mutations in CD2BP1 disrupt binding to PTP PEST and are responsible for PAPA syndrome, an autoinflammatory disorder.

PAPA syndrome (pyogenic sterile arthritis, pyoderma gangrenosum, and acne, OMIM #604416) and familial recurrent arthritis (FRA) are rare inherited disorders of early onset, primarily affecting skin and joint tissues. Recurring inflammatory episodes lead to accumulation of sterile, pyogenic, neutrophil-rich material within the affected joints, ultimately resulting in significant destruction. We recently localized the genes for PAPA syndrome and FRA to chromosome 15q and suggested that they are the same disorder. We have now established this by the identification of co-segregating disease-causing mutations in the CD2-binding protein 1 (CD2BP1; GenBank accession no XM 044569) gene in the two reported families with this disorder. E250Q or A230T amino acid substitutions occur within a domain highly homologous to yeast cleavage furrow-associated protein CDC15. CD2BP1 and its murine ortholog, proline-serine-threonine phosphatase interacting protein (PSTPIP1), are adaptor proteins known to interact with PEST-type protein tyrosine phosphatases (PTP). Yeast two-hybrid assays demonstrate severely reduced binding between PTP PEST and both the E250Q and A230T mutant proteins. Previous evidence supports the integral role of CD2BP1 and its interacting proteins in actin reorganization during cytoskeletal-mediated events. We hypothesize that the disease-causing mutations that we have identified compromise physiologic signaling necessary for the maintenance of proper inflammatory response. Accordingly we suggest classification of PAPA syndrome as an autoinflammatory disease. This CD2BP1-mediated biochemical pathway(s) may function in common inflammatory disorders with apparent etiological overlap, such as rheumatoid arthritis and inflammatory bowel disease.

Acne Vulgaris↗
PubMed2001

Pyoderma vegetans.

BACKGROUND: Pyoderma vegetans is a rare condition that is clinically characterized by large verrucous plaques with elevated borders and multiple pustules. The etiology of this disorder remains unknown. OBJECTIVES: We describe a 24-year-old woman with rapidly evolving pyoderma vegetans. Our patient had the unique additional findings of a highly elevated serum IgE level and a history of hidradenitis suppurativa. CONCLUSIONS: Pyoderma vegetans is diagnosed on clinical and histological criteria. Differentiation must be made from disorders such as pyoderma gangrenosum, Sweet's syndrome, and deep fungal infections. We illustrate a case of pyoderma vegetans and review the literature on this rare disorder. Clinical and histological criteria for diagnosis are presented, as well as differentiation from some mimicking disorders.

Adult↗
PubMed1989

Superficial granulomatous pyoderma: clinicopathologic spectrum.

Superficial granulomatous pyoderma is a form of pyoderma gangrenosum characterized by superficial ulceration and a chronic course. Histopathologic examination shows a granulomatous response. We report two new cases of superficial granulomatous pyoderma in detail and briefly review the clinical data of five previous cases included in a recent series. These lesions typically begin as single furunculoid papules that most commonly occur on the trunk and that may arise at sites of surgical treatment or other pathergic stimuli. The lesions progress to superficial ulcers with a relatively clean base and vegetative borders. Tetracycline, minocycline, sulfapyridine, dapsone, and intralesionally administered corticosteroids have been effective anti-inflammatory agents, producing healing and allowing avoidance of the use of systemic corticosteroids in the management of most patients with superficial granulomatous pyoderma.

Adult↗
PubMed1976

Postoperative progressive gangrene: a reminder.

A classical example of progressive postoperative gangrene is described and the relevant literature has been reviewed. After consideration of the evolution and the clinical appearances of the condition, of the consistency of the associated bacterial flora and of the response to antibiotic therapy alone, we suggest that progressive postoperative gangrene is a distinct entity and separate from pyoderma gangrenosum.

Adult↗
PubMed1978

A diffuse pustular eruption associated with ulcerative colitis.

Two patients each with a two-year history of chronic ulcerative colitis developed, over a 24- to 48-hour period, painful pustules involving the skin and oral mucosa. The pustular eruption was associated with a severe exacerbation of the colitis. Culture of the lesions and of the blood failed to reveal an infectious cause for the eruption. Histologic study of a pustule in case 2 revealed suppurative folliculitis. The lesions subsided rapidly in case 1 when intravenous hydrocortisone and intravenous and intramuscular antibiotics were administered, and further resolution occurred after colectomy. A similar rapid resoultion of the pustules occurred in case 2 after initiation of treatment with intramuscular cortisone acetate and antibiotics and with colectomy. This pustular eruption may represent a variant of pyoderma gangrenosum.

Adult↗
PubMed1988

Pustular ulcerative dermatosis of the scalp.

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.

Adolescent↗
PubMed1998

[Pyostomatitis-pyodermatitis vegetans uncovering a case of Crohn disease].

BACKGROUND: Pyostomatitis-pyodermatitis vegetans is an uncommon condition associated with chronic inflammatory bowel disease in 75% of the cases, usually hemorrhagic rectocolitis. CASE REPORT: A 48-year-old man was referred for recent development of pustulous lesions of the lips and buccal mucosa and weight loss. He complained of abdominal pain and intermittent diarrhea which had persisted for more than one year. During the last three months, a pseudotumoral plaque with a pustulous rim had developed over the two distal phalanxes of the right middle finger in association with ungueal lysis and nodular, vegetating and crusted lesions on the lateral aspect of the left arm. Small pustules covered the entire buccal mucosa excepting the tongue and the glans forming a typical snail trace aspect. Bacterial and mycological samples were negative. The histology reports for skin and mucosa were similar: epithelial hyperplasia, intra- and subepithelial granulocyte micro-abscesses and polymorphous infiltration of the superficial derma with numerous neutrophils and eosinophils. There was a discrete interkeratinocytic fluorescence at direct immunofluorescence but indirect immunofluorescence was negative. Anti-desmogleine 1 immunolabeling showed typical normal skin uptake and immunotransfer was negative. Digestive tract endoscopy and histopathology examination of the bowel specimens confirmed the diagnosis of Crohn's disease. Clinical manifestations improved dramatically with prednisone. DISCUSSION: This case of pyostomatitis-pyodermatitis vegetans involved several aspects rarely reported in the literature: a) the cutaneomucosal signs were inaugural; b) the association with Crohn's disease; c) the presence of lesions to the genital mucosa; d) the unusual localization of the inaugural skin manifestations. This clinical entity has now been clearly distinguished from pemphigus vegetans. There was however a long debate on the similar clinical, histological and even immunological expressions. We suggest that pyostomatitis-pyodermatitis vegetans belongs to the spectrum of neutrophilic dermatoses and other authors even propose it is a clinical form of pyoderma gangrenosum.

Colonoscopy↗
PubMed1979

Blastomycosis-like pyoderma.

Seven patients with blastomycosis-like pyoderma had skin lesions of four months' to six years' duration. The criteria for the diagnosis of blastomycosis-like pyoderma include the clinical presentation of large verrucous plaques with multiple pustules and elevated border, pseudoepitheliomatous hyperplasia with abscess histologically, and the growth of at least one pathogenic bacteria from the culture of a tissue-biopsy specimen. The differential diagnosis includes deep fungus infection (especially North American blastomycosis), bromoderma, pyoderma gangrenosum, mycobacterial infections, giant keratoacanthoma, and squamous cell carcinoma. Generally, the patients had one or more conditions that could have affected their systemic or local immunologic competence to infection. We believe that the clinical and histologic features in these cases of blastomycosis-like pyoderma were produced by an unusual, exaggerated, vegetating-tissue reaction to a primary or secondary bacterial infection.

Adult↗
PubMed1990

Inappropriate diagnosis of necrotizing arachnidism. Watch out Miss Muffet--but don't get paranoid.

A 28-year-old man presented with painful progressive necrosis of the skin of his left leg which was associated with general toxicity and a high fever. A working diagnosis of necrotizing arachnidism was made, but no improvement followed two weeks of therapy with analgesics, antibiotics and antihistamines. His symptoms responded rapidly to prednisolone therapy, and a diagnosis of pyoderma gangrenosum was confirmed by biopsy. This subsequently proved to be associated with early acute myelomonocytic leukaemia.

Adult↗
PubMed1991

Cutaneous manifestations of neutrophilic disease. A study of seven cases.

Seven patients with a complex form of neutrophilic dermatosis are reported. Clinically, they had variable associations of four types of lesions: blisters/pustules, plaques, nodules and ulcerations. Histologically, a neutrophilic infiltrate was observed at variable levels in the epidermis, dermis and subcutis. Systemic manifestations were present in all cases (general symptoms, joint, renal, ocular and lung involvements). Three patients had an associated disease (myelodysplasia, metastatic carcinoma, IgG gammopathy). Steroids were the most efficient treatment. These observations, as well as a review of the literature, support the opinion that the neutrophilic dermatosis represents a continuous spectrum encompassing four well-defined entities: subcorneal pustular dermatosis, Sweet's syndrome, erythema elevatum diutinum and pyoderma gangrenosum. We propose that the different patterns of the neutrophilic dermatosis are the most obvious manifestations of a potentially multisystemic neutrophilic disease and allow its recognition.

Adult↗
PubMed1981

[Pyoderma gangraenosum].

Pyoderma gangrenosum is a relatively rare, destructive, inflammatory disease of unknown cause which may present as a purely cutaneous disorder or may be associated with an underlying internal disease (ulcerative colitis, Crohn's disease, rheumatoid arthritis, multiple myeloma, lymphoma and others). Four selected patients are described which reflect the clinical spectrum of this condition; these cases and a review of the literature serve as a background for analysis of pyoderma gangraenosum as an entity and a discussion of its pathogenesis.

Aged↗
PubMed1986

Cutaneous changes associated with inflammatory bowel disease.

Inflammatory bowel disease often begins during adolescence, but may affect younger children. Associated skin lesions are not uncommon. Occasionally, cutaneous alterations may precede the onset of signs or symptoms of the gastrointestinal disorder. Erythema nodosum and pyoderma gangrenosum are the most common associated specific skin changes, but numerous others may be found. Although the skin condition often responds to treatment of the bowel, it may require additional therapy.

Child↗