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PubMed1980

Globular deposits of immunoglobulins and complement in the papillary dermis. Clinical significance.

Globular deposits of immunoglobulins in the papillary dermis have been reported to occur in certain dermatoses, particularly in lichen planus. The clinical correlation of these deposits in 52 skin biopsy specimens reviewed by light and fluorescent microscopy was studied. These cases included five of lichen planus, 24 of lupus erythematosus or related diseases, four of dermatitis herpetiformis, three of drug eruption; two each of bullous pemphigoid, erythema nodosum, porphyria cutanea tarda; one each of vitiligo, pyoderma gangrenosum, neurodermatitis, erythema multiforme, granuloma annulare, vasculitis, epidermolysis bullosa simplex, Rothmund-Thompson syndrome, and four of unspecified dermatoses. Using an arbitrary scale of 1-4 based on the frequency of deposits, 3+ and 4+ deposits were identified in all five cases of lichen planus, as well as in six cases of lupus erythematosus, one of drug eruption, one of bullous pemphigoid, one of erythema nodosum, the vitiligo, vasculitis, and Rothmund-Thompson syndrome cases, and two cases of unspecified dermatoses; other cases showed only 1+ and 2+ deposits. In all five cases of lichen planus, the deposits contained IgM and C3, and in addition, IgA was present in four, IgG and fibrinogen in three. Among non-lichen planus cases, C3 was detected in 11 of 49, and fibrinogen in only four of 49. These findings indicate that globular deposits of Ig in the dermis, though suggestive, are not pathognomonic of lichen planus.

Biopsy↗
PubMed2003

Degos' disease: a distinctive pattern of disease, chiefly of lupus erythematosus, and not a specific disease per se.

Degos' disease, known confusingly as malignant strophic papularis, is an uncommon condition of unknown cause characterized by distinctive infarctive lesions in the skin, gastrointestinal tract, and central nervous system; the lesions at the two latter sites often result in death. We deem Degos' disease to be analogous to lupus erythematosus in the sense that each is fundamentally a systemic pathologic process involving several organs, among them the skin, but, moreover, we regard Degos' disease, in most instances, to be an actual manifestation of lupus erythematosus. Histopathologically, the findings in sections of tissue of skin lesions of Degos' disease are indistinguishable from those of one expression of cutaneous lupus erythematosus; immunopathologically, some patients with morphologic findings stereotypical of Degos' disease display signs characteristic of lupus erythematosus. For these reasons, we consider Degos' disease to be a distinctive pattern of disease, rather than a specific disease per se, just as are erythema multiforme, erythema nodosum, leukocytoclastic vasculitis, Sweet's syndrome, and pyoderma gangrenosum, to name but five of scores of them. The singular pattern that is designated Degos' disease usually is an expression of lupus erythematosus, but, episodically, of conditions like dermatomyositis and rheumatoid arthritis.

Adolescent↗
PubMed1997

Crohn's-like complications in patients with ulcerative colitis after total proctocolectomy and ileal pouch-anal anastomosis.

Total proctocolectomy with ileal pouch-anal anastomosis (IPAA) has become an established surgical procedure for ulcerative colitis. Occasional patients who have undergone IPAA develop persistent or recurrent episodes of pouchitis (chronic pouchitis), from which a subset also develop gastrointestinal and systemic complications that are identical to those seen in Crohn's disease. These complications include enteric stenoses or fistulas in the pouch or pouch inlet segment, perianal fistulas or abscesses, pouch fistulas, arthritis, iridocyclitis, and pyoderma gangrenosum. The development of Crohn's-like gastrointestinal complications in a patient with chronic pouchitis frequently engenders concern that the pathologist misinterpreted the proctocolectomy specimen as ulcerative colitis instead of Crohn's disease. We describe eight patients who developed chronic pouchitis and Crohn's-like complications after IPAA and total proctocolectomy. In each case, concern was voiced about misinterpretation of the proctocolectomy specimen as ulcerative colitis instead of Crohn's disease after the development of the Crohn's-like complications. Preoperatively, all eight patients had characteristic clinical, radiographic, and pathologic features of ulcerative colitis. Review of the pathology specimens indicated that all eight had ulcerative colitis. Crohn's-like complications are most likely related to chronic pouchitis, which probably is a form of recrudescent ulcerative colitis within the novel environment of the pouch. A diagnosis of Crohn's disease after IPAA surgery should only be made when reexamination of the original proctocolectomy specimen shows typical pathologic features of Crohn's disease, Crohn's disease arises in parts of the gastrointestinal tract distant from the pouch, pouch biopsies contain active enteritis with granulomas, or excised pouches show the characteristic features of Crohn's disease, including granulomas. There were no histologic differences in the total colectomy specimens between the eight ulcerative colitis study patients and 16 control ulcerative colitis patients who had a favorable clinical outcome after IPAA surgery groups. Crohn's-like complications and chronic pouchitis does not necessarily imply an incorrect original interpretation of ulcerative colitis by the pathologist.

Adolescent↗
PubMed1998

Drugs associated with vasculitis.

Newer agents of particular interest to rheumatologists are increasingly associated with vasculitis. There is now good evidence that treatment with hematopoietic growth factors, including granulocyte colony-stimulating factor and granulocyte-macrophage colony-stimulating factor, can cause or mimic vasculitides such as Sweet's syndrome and pyoderma gangrenosum. Similarly, there is strong evidence that clinical use of interferons is associated with a variety of autoimmune phenomenon rarely including vasculitis. The increased availability of testing for antineutrophil cytoplasmic antibody (ANCA) has widened the clinical spectrum of systemic vasculitides. There are now many reports that treatment with either hydralazine or propylthiouracil is associated with ANCA-positive vasculitis. A small number of case reports also implicate penicillamine and minocycline as agents capable of inducing an ANCA-associated vasculitis. Clinicians should be aware of the potential of certain drugs to cause vasculitis and especially cautious in using these agents in patients with known vasculitis.

Antibodies, Antineutrophil Cytoplasmic↗
PubMed1992

Simultaneous appearance of a unique common epitope in fetal colon, skin, and biliary epithelial cells. A possible link for extracolonic manifestations in ulcerative colitis.

The tissue distribution of a previously identified M(r) 40 K epithelial autoantigen in ulcerative colitis was examined in situ in the fetal tissue by immunocytochemical method, using an immunoglobulin M (IgM) monoclonal antibody (7E12H12). Fetal-autopsy tissue from 9 to 19 weeks of development (11-21 gestational weeks) gestations, including specimens of colon (20 specimens), skin (19), small intestine (12), gallbladder (15), liver (20), spleen (20), kidney (3), urinary bladder (3), and umbilical cord/placenta (25) were examined. 7E12H12 reactive epitope appeared first in a few colonic epithelial cells at 10 weeks of gestation. By 11 weeks, 7E12H12 reactivity was clearly evident in most of the colonic epithelial cells. Goblet-cell reactivity was evident from 15 weeks of gestation. Enterocytes lining the small intestine from all the specimens were negative. In the skin, earliest immunoreactivity was evident in the basal layer of epidermis at 10 weeks of development, and by 11 weeks, the staining was intense and localized exclusively to squamous epithelial cells of the epidermis. The immunoreactivity in the fetal gallbladder mucosa was also evident earliest at 11 weeks of development. The staining was distinct in the mucosal epithelial cells, and localized along the periphery of the cells and in the apical areas. None of the other tissue specimens reacted with 7E12H12. These results demonstrate that the 7E12H12 reactive epitope is novel, is shared by colon, skin, and biliary epithelium, and appears more or less simultaneously in the three organs at 10 to 11 weeks of development of the fetus. A possible link of this crossreactive protein or proteins in the immunopathogenesis of ulcerative colitis, sclerosing cholangitis, and pyoderma gangrenosum is discussed.

Antibodies, Monoclonal↗
PubMed2006

Extraintestinal manifestations of inflammatory bowel diseases.

Inflammatory bowel diseases are associated with extraintestinal manifestations involving almost every organ system in the body. They occur in approximately 20% to 40% of patients with inflammatory bowel diseases. Immune-related and genetic mechanisms play an important role in the pathogenesis of these complications. Peripheral arthritis, erythema nodosum, and episcleritis respond to the treatment of the underlying intestinal inflammation, whereas axial arthropathy, pyoderma gangrenosum, and uveitis do not. Immunomodulator therapy, particularly with biologic agents has been shown to be effective in treating some of the extraintestinal manifestations. Early recognition and treatment are crucial in preventing major morbidity.

Autoimmunity↗
PubMed1976

The extra-intestinal complications of Crohn's disease and ulcerative colitis: a study of 700 patients.

The records of a series of 700 patients with inflammatory bowel disease, 498 with Crohn's disease and 202 with ulcerative colitis, have been analyzed to determine the relative incidence and characteristic features of their extra-intestinal manifestations. The group with Crohn's disease included 62 with colitis, 223 with ileocolitis, and 213 with regional enteritis. A consideration of the clinical patterns and an understanding of their pathophysiology suggested a subdivision into two main groups: one "colitis related" and one related to the pathophysiology of the small nonspecific third group. Group A, colitis related, comprises joint, skin, mouth, and eye disease. The complications might be immunologically determined, were closely associated with active inflammation, and often responded to medical or surgical treatment of the underlying bowel disease. They occurred in 36% of the entire series of patients: joints were involved in 23%, skin in 15%, and mouth and eye each in 4%. Pyoderma gangrenosum was observed most often in ulcerative colitis and erythema nodosum most often in granulomatous colitis. The incidence of Group A complications was higher in disease involving the colon (42%) than in disease restricted exclusively to the small bowel (23%). There were interrelationships among the various members of Group A, with multiple manifestations occurring in a third of affected patients. Group B, related to small bowel pathophysiology, includes malabsorption, gallstones, kidney stones, and non-calculous hydronephrosis and hydroureter. Disorders in this group were generally related to the severity of the disease in the small bowel and tended to persist even in the absence of active inflammation. In contrast to Group A, this group occurred most frequently in small bowel disease, and least in colonic disease. Malabsorption was virtually confined to the patients with small bowel disease (10% incidence), while gallstones and renal stones were also both more frequent in Crohn's disease (11% and 9% respectively), the latter usually in association with small bowel resection or ileostomy. Group C, found in a small percentage of patients, consists of nonspecific complications, including osteoporosis (3%), liver disease (5%), peptic ulcer (10%), and amyloidosis (1%).

Amyloidosis↗
PubMed1997

Sweet's syndrome: an unusual cutaneous feature of Crohn's disease or ulcerative colitis. The South West Gastroenterology Group.

Sweet's syndrome is characterized by tender, red inflammatory nodules or papules, usually affecting the upper limbs, face or neck. It is part of the group of acute neutrophilic dermatoses that includes pyoderma gangrenosum, but can be distinguished by its appearance, distribution and histological features. Four patients with Sweet's syndrome and Crohn's disease are reported. A total of 30 cases from the literature suggest that Sweet's syndrome is an unusual extraintestinal manifestation of either Crohn's disease or ulcerative colitis. There is a strong predilection for women (87%), patients with colonic disease (100%) and those with other extraintestinal features (77%). The rash is associated with active disease in 67-80%, but may precede the onset of intestinal symptoms in 21% and has been reported 3 months after proctocolectomy for ulcerative colitis.

Adult↗
PubMed2002

Clinical significance of granuloma in Crohn's disease.

Crohn's disease (CD) is diagnosed from information obtained clinically, pathologically, and radiologically. One important pathologic finding is a granuloma, which is helpful when a positive diagnosis of CD will affect treatment. Whether the presence of a granuloma has any clinical implication is not clear. We conducted a retrospective study to determine whether a granuloma found on a biopsy sample is associated with disease severity, fistulizing or perianal disease, frequent relapses, and extraintestinal manifestations. Eighty-two patients were identified who had a biopsy or bowel resection for CD between 1990 and 1994 at a tertiary referral center; 21 (25.6%) had a granuloma. This group was compared with a group of 61 patients without a granuloma. Forty-five percent were male (n = 37), mean age at diagnosis was 42.6 years (median, 39.5 years), mean disease duration at presentation was 8.8 years (median, 4.8 years), and mean follow-up duration was 2 years (range, 1 day to 10.2 years). No significant differences were demonstrated between the two groups by the Fisher exact test with regard to fistulizing or perianal disease, oral aphthous ulcers, disease severity, axial or peripheral arthralgia, episcleritis, anterior uveitis, erythema nodosum, or pyoderma gangrenosum.

Adult↗
PubMed1995

Management of unusual causes of ulcers of lower extremities.

Treatment of any wound begins with the correct diagnosis. The vast majority of chronic wounds can be classified as pressure ulcers, venous ulcers, insensate ulcers, or arterial insufficiency ulcers. This article focuses on wounds of unusual origin that must be considered in evaluation. All of these wounds are difficult to treat, are more likely to occur in specialized clinical settings, may provide clues to underlying systemic illnesses, and can be life-threatening. Unusual wounds discussed include atrophe blanche, calciphylaxis, leukocytoclastic vasculitis, necrobosis lipoidica, necrotizing fasciitis, and pyoderma gangrenosum.

Humans↗
PubMed2005

Caspase recruitment domain 15 mutations and rheumatic diseases.

PURPOSE OF REVIEW: The purpose of this article is to review the foundational work and current developments on a group of rheumatic disorders associated with mutations in the caspase recruitment domain 15/nucleotide oligomerization domain 2 gene. RECENT FINDINGS: To date, there are at least 10 arthritic conditions for which specific genetic mutations have been demonstrated. They include familial Mediterranean fever; tumor necrosis factor receptor associated periodic syndrome; hyper immunoglobulin D syndrome; neonatal onset multisystemic inflammatory disease; pyogenic arthritis pyoderma gangrenosum and acne; Muckle-Wells syndrome; familial cold autoinflammatory syndrome; immunodysregulation, polyendocrinopathy, enteropathy, X-linked syndrome; Crohn's disease; and familial and sporadic sarcoid granulomatous arthritis. This review focuses on recent progress in the last two diseases and the caspase recruitment domain 15 genetic defects with which they are associated. Up to 50% of patients with familial granulomatous arthritis (Blau's syndrome), 90% of those with sporadic granulomatous arthritis (early-onset sarcoidosis), and 40% of individuals with Crohn's disease have documented mutations in the caspase recruitment domain 15 gene. SUMMARY: Although histologically, Crohn's disease and familial and sporadic sarcoid granulomatous arthritis are distinct from rheumatoid arthritis because of the defining presence (albeit in not all cases) of non-caseating granulomata in the synovial and intestinal tissues, respectively, they still represent a promising model of both chronic synovitis and uveitis. In addition, once the actual mechanism is discovered by which defects of the caspase recruitment domain 15 gene product lead to chronic arthritis, it may uncover unsuspected biologic targets for therapeutics.

Caspases↗
PubMed1981

IgA-associated inhibition of polymorphonuclear leukocyte chemotaxis in neutrophilic dermatoses.

The chemotactic activity of normal human polymorphonuclear leukocytes (PMNs) confronted with heat inactivated sera from patients with psoriasis as well as various chronic proliferative diseases was determined using modified Boyden chambers. By the addition of phorbol myristate acetate (PMA) at a concentration of 1 ng/ml the chemoattractant activities of the sera were greatly potentiated. However, the chemotactic migration of normal PMNs was strongly inhibited by sera from patients with long standing and wide spread psoriasis, pyoderma gangrenosum, severe acne conglobata, Sweet syndrome, and some patients with chronic arthritis following rheumatoid fever. In acute guttate psoriasis and atopic dermatitis increased migratory activities were seen. The inhibition of chemotaxis correlated with increased serum IgA levels as determined by radial immuno diffusion. Column chromatography (Sephacryl S-300) revealed serum fractions of strong inhibitory potency at a molecular weight near 200,000 Dalton. These inhibitory fractions were seen in patients with long standing neutrophil related diseases and could not be detected in normal control sera. It appears that inhibition of PMN chemotaxis is a secondary phenomenon and may play an autoregulatory role in PMN related inflammation.

Adolescent↗
PubMed2006

Langerhans cell histiocytosis associated with myelodysplastic syndrome in adults.

BACKGROUND: Myelodysplastic syndrome (MDS) is a group of bone marrow disorders associated with dyplasia of myeloid elements that may have cutaneous manifestations including infections, vasculitis, Sweet's syndrome, pyoderma gangrenosum, erythema elevatum diutinum, and leukemia cutis. These cutaneous manifestations are attributed to the underlying bone marrow defect. Langerhans cell histiocytosis (LCH) is primarily a pediatric disease, and rarely LCH has been described in association with pediatric MDS. We are aware of only a single case report of LCH associated with MDS in an adult. METHODS: We report two new cases of LCH in elderly patients with underlying MDS. The specimens were examined by routine microscopy as well as immunohistochemical stains for S100 protein and CD1a. RESULTS: Both patients were elderly men with established diagnoses of MDS. One presented with a solitary pruritic papule while the other had a 2-year history of erythematous papules involving the trunk and extremities. Histologic examination revealed intraepidermal and dermal collections of mononuclear cells with reniform nuclei. The cells were strongly positive for S100 and CD1a, confirming their identity as Langerhans cells. CONCLUSION: Cutaneous LCH may be associated with underlying MDS in adults and should be considered in the differential diagnosis of cutaneous eruptions in patients with MDS.

Aged↗
PubMed2006

Cyclophosphamide pulse therapy followed by azathioprine or methotrexate induces long-term remission in patients with steroid-refractory Crohn's disease.

BACKGROUND: In patients with steroid-refractory Crohn's disease, the therapeutic goal is to achieve both rapid remission and maintenance of clinical response. AIM: To evaluate the long-term benefit in patients treated with cyclophosphamide pulse therapy and azathioprine or methotrexate, a combination shown to be effective in a recent pilot study. METHODS: Sixteen patients with acute steroid-refractory Crohn's disease participated in a prospective open-labelled uncontrolled pilot study between December 1998 and June 2003. All had a median number of 4 monthly pulses of intravenous cyclophosphamide (750 mg) and were followed until relapse of the disease. RESULTS: Thirteen of 16 patients (81%) achieved remission within 8 weeks after two pulses of cyclophosphamide in combination with azathioprine or methotrexate, with a Crohn's Disease Activity Index decrease from 294 to 111 (median). Remission sustained for 19 months (median, range: 1-45). Moreover, eight patients with pyoderma gangrenosum and erythema nodosum who responded to cyclophosphamide have maintained their remission for up to 30 months. CONCLUSIONS: In steroid refractory patients with Crohn's disease, cyclophosphamide is highly effective to induce remission. This uncontrolled study indicates that cyclophosphamide-induced remission is long-lasting under standard immunosuppressive therapy.

Adult↗
PubMed1990

Skin manifestations in congenital deficiency of leucocyte-adherence glycoproteins (CDLG).

In congenital deficiency of leucocyte-adherence glycoproteins (CDLG) there is an immunodeficiency with impaired leucocyte function and cutaneous and extracutaneous infections occur. In more than 30% of cases the condition has a fatal course. We report the skin manifestations of three siblings with CDLG in which areas of skin necrosis occurred that resembled pyoderma gangrenosum.

Adult↗
PubMed2005

Thalidomide: dermatological indications, mechanisms of action and side-effects.

Thalidomide was first introduced in the 1950s as a sedative but was quickly removed from the market after it was linked to cases of severe birth defects. However, it has since made a remarkable comeback for the U.S. Food and Drug Administration-approved use in the treatment of erythema nodosum leprosum. Further, it has shown its effectiveness in unresponsive dermatological conditions such as actinic prurigo, adult Langerhans cell histiocytosis, aphthous stomatitis, Behçet's syndrome, graft-versus-host disease, cutaneous sarcoidosis, erythema multiforme, Jessner-Kanof lymphocytic infiltration of the skin, Kaposi sarcoma, lichen planus, lupus erythematosus, melanoma, prurigo nodularis, pyoderma gangrenosum and uraemic pruritus. This article reviews the history, pharmacology, mechanism of action, clinical uses and adverse effects of thalidomide.

Adult↗
PubMed2006

Update on the use of ciclosporin in immune-mediated dermatoses.

Immune-mediated dermatoses, such as psoriasis and atopic dermatitis, affect a significant proportion of the population. Although most cases are not life threatening, these diseases can have a profound effect on the sufferer's quality of life and that of their family. Systemic therapy, such as ciclosporin, is often indicated for severe or recalcitrant disease. The efficacy of ciclosporin in the treatment of psoriasis and atopic dermatitis has been established and clinical data also demonstrate its efficacy in treating less common but equally challenging conditions such as pyoderma gangrenosum, lichen planus, autoimmune bullous disease, recalcitrant chronic idiopathic urticaria and chronic dermatitis of the hands and feet. The risk of potential adverse events associated with ciclosporin is greatly reduced if current treatment and monitoring guidelines are followed.

Arthritis, Psoriatic↗