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Review of thymic pathology in 30 cats and 36 dogs.

Data are presented from 30 cats and 36 dogs in which thymic disease was recognised clinically or on postmortem examination. The diagnoses included thymic lymphoma (19 cats, 12 dogs), thymoma (five cats, 18 dogs), thymic branchial cyst formation or cystic change (one cat, four dogs), thymic hyperplasia (two cats), congenital hypoplasia (one cat, one dog), thymic haemorrhage (one cat, one dog) and thymic amyloidosis (one cat). Thymic lymphoma occurred in younger dogs and cats, and was recorded equally among domestic shorthaired and purebred (especially Siamese) cats. Eight cats with thymic lymphoma were tested for feline leukaemia virus and four were positive. Thymoma occurred more frequently in older cats and dogs, and in Labradors and German shepherd dogs. Thymic tumours were associated with paraneoplastic hypercalcaemia (six dogs), megaoesophagus (two dogs) or interface dermatitis with basement membrane immune complex deposition (one cat). Non-neoplastic thymic diseases were associated with myasthenia gravis (one cat), pemphigus foliaceus (one cat) and superficial necrolytic dermatitis (one cat).

Amyloidosis↗

Bacterial pneumonia associated with corticosteroid therapy in three horses.

Three horses developed severe pulmonary infections while being treated with systemic corticosteroids for other diseases. Two of them had an immune-mediated skin disease, compatible with a diagnosis of pemphigus foliaceus, and one had severe chronic obstructive pulmonary disease. Case 1 developed diffuse pneumonia from which Streptococcus zooepidemicus and Bacteroides melaninogenicus were isolated, and it responded to antibiotic therapy. Case 2 developed septicaemia, pulmonary thrombosis and pneumonia associated with Escherichia coli, and died during a peracute illness with signs of disseminated intravascular coagulation. Case 3 developed focal pneumonia from which S zooepidemicus was isolated. This horse was destroyed at the owner's request and no treatment was attempted.

Adrenal Cortex Hormones↗

Efficacy of the treatment of dogs with leishmaniosis with a combination of metronidazole and spiramycin.

Twenty-seven dogs infected naturally with Leishmania infantum were used in a randomised controlled trial to compare the clinical and parasitological efficacy of an oral treatment with a combination of metronidazole and spiramycin (13 dogs) with the efficacy of conventional treatment with meglumine antimonate and allopurinol (14 dogs) as controls. In the test group one dog had to be withdrawn from the treatment because it developed pemphigus foliaceus; 10 of the dogs were clinically responsive but none was cured parasitologically. In the control group four dogs were withdrawn from the treatment because of side effects; eight of the dogs were clinically responsive but none was cured parasitologically. The control group showed signs of improvement after an average of 30 days, whereas the test group did not show signs of improvement until after an average of 45 days.

Allopurinol↗

Treatment of staphylococcal scalded skin syndrome.

Humans are a natural reservoir for Staphylococcal aureus. Colonization begins soon after birth and predisposes to infection. S. aureus is one of the most common causes of skin infection, giving rise to folliculitis, furunculosis, carbuncles, ecthyma, impetigo, cellulitis and abscesses. In addition, S. aureus may cause a number of toxin-mediated life-threatening diseases, including staphylococcal scalded skin syndrome (SSSS). Epidermolytic toxins released by certain S. aureus strains cause SSSS by cleaving the epidermal cell adhesion molecule, desmogelin-1, resulting in superficial skin erosion. Recent experiments have revealed similarities in the pathophysiology of SSSS and pemphigus foliaceus, an autoimmune disorder that is characterized by antibodies targeting the same epidermal attachment protein. SSSS typically affects neonates and infants but may also occur in predisposed adults. It is painful and distressing for the patient and parents, although most cases respond to antibiotic treatment. Mortality is low in infants but can be as high as 67% in adults, and is dependent on the extent of skin involvement and the comorbid state. Thus, the management of adults who develop SSSS remains a major therapeutic challenge. The antibody response against the toxins neutralizes their effect and prevents recurrence or limits the effects to the area of infection, which is known as bullous impetigo.

Animals↗

[Dermatosis among the Xavánte from the Pimentel Barbosa Indian Reservation, Mato Grosso, (Brazil)].

The authors present the results of a dermatological survey conducted among the Xavánte Indians from the Pimentel Barbosa Reservation, state of Mato Grosso, Brazil Scabies, pediculosis, and pioderma were the most frequent diseases, clearly related to poor hygiene. The finding of perleche indicates the presence of nutritional deficiency. Endemic pemphigus foliaceus (fogo selvagem) was the most serious dermatological affection found in this population.

English Abstract↗

Influence of long-term treatment with tetracycline and niacinamide on antibody production in dogs with discoid lupus erythematosus.

OBJECTIVE: To evaluate the effect of long-term treatment with tetracycline and niacinamide on antibody production in dogs by measuring postvaccinal serum concentrations of antibodies against canine parvovirus and canine distemper virus. ANIMALS: 10 dogs receiving long-term treatment with tetracycline and niacinamide (treatment group) and 10 healthy dogs (control group). PROCEDURE: The treatment group included 9 dogs with discoid lupus erythematosus and 1 dog with pemphigus foliaceus on long-term treatment (> 12 months) with tetracycline and niacinamide. The control group included 10 healthy dogs with no clinical signs of disease and no administered medications for the past 3 months. Blood samples were obtained from all dogs by jugular venipuncture. Serum antibody titers against canine parvovirus and canine distemper virus antigens were measured, using hemaglutination inhibition and serum neutralization, respectively, and compared between groups. RESULTS: A significant difference in antibody titers between treatment- and control-group dogs was not found. All dogs had protective antibody titers against canine distemper virus, and 8 of 10 dogs from each group had protective titers against canine parvovirus infection. CONCLUSION AND CLINICAL RELEVANCE: These results provide evidence that long-term treatment with tetracycline and niacinamide does not interfere with routine vaccinations and thus does not seem to influence antibody production in dogs.

Animals↗

[Successful treatment of autoimmune bullous diseases with a combination therapy of ciclosporin and corticosteroid].

Three patients with autoimmune bullous diseases, pemphigus vulgaris, pemphigus foliaceus and bullous pemphigoid, were treated with a combination therapy of ciclosporin and corticosteroid. These patients responded to systemic low-dose prednisolone (or dexamethasone) and low-dose ciclosporin therapy; the result was prolonged complete remission. The addition of low-dose ciclosporin may produce enhanced clinical effects of steroid therapy without increasing any significant side effects.

Adult↗

Toxicity of longterm low dose D-penicillamine therapy in rheumatoid arthritis. Cooperative Systematic Studies of Rheumatic Disease Group.

After a controlled, double blind multicenter trial of D-penicillamine in the treatment of rheumatoid arthritis, 148 patients were followed for an additional year in an open label trial. Complications of longterm D-penicillamine included rash, gastrointestinal manifestations, proteinuria, bone marrow depression, myasthenia gravis, myositis, acute febrile reaction and pemphigus foliaceus. With the exception of one patient with myasthenia gravis, all adverse reactions resolved after withdrawal of D-penicillamine. While the complications of D-penicillamine therapy are usually dose related, these reactions can occur at any time and at any dose. Continued and frequent longterm monitoring of D-penicillamine therapy is required.

Adolescent↗

[Inflammatory skin diseases and hypereosinophilia].

Among the dermatoses associated with tissue eosinophilia are pemphigus vegetans, bullous pemphigoid, granuloma facial and Wells' syndrome. Eosinophilic spongiosis can occur in the early stage of pemphigus. Pemphigus herpetiformis, a recently introduced term, does not represent a well defined entity but develops into either pemphigus vulgaris or pemphigus foliaceus. Granuloma facial is basically vasculitis. Eosinophilic cellulitis or Wells' syndrome shows numerous eosinophils which degranulate and through confluence of their granules form the so-called flame figures.

Cellulitis↗

Acute pancreatitis in two dogs given azathioprine and prednisone.

Acute pancreatitis was diagnosed in 2 dogs given azathioprine and prednisone. Prednisone and azathioprine had been given as immunosuppressive therapy for pemphigus foliaceus in dog 1 and for polymyositis in dog 2. Azathioprine was discontinued in both dogs. In dog 1, prednisone was reinstituted on day 6 of hospitalization. Prednisone was continued throughout the period of hospitalization in dog 2. Both dogs recovered without complication. Glucocorticoid therapy has been associated with the development of pancreatitis. In human beings, a common side effect of azathioprine is the development of drug-induced pancreatitis. Definitive identification of azathioprine as the cause of pancreatitis in these dogs was not possible; the owners refused to permit retreatment with the drug. Therefore, the synergistic action between these 2 drugs could not be ruled out as the cause of pancreatitis.

Acute Disease↗

Bullous transient acantholytic dermatosis.

We have been three cases of a bullous variant of transient acantholytic dermatosis. Skin biopsy specimens showed histopathologic changes identical to pemphigus foliaceus, but immunofluorescent studies showed no tissue-fixed or circulating antibodies to intercellular antigens. The bullous eruption lasted a few weeks to several months and healed without scarring or recurrence. The lesions healed with topical and/or systemic corticosteroid therapy.

Acantholysis↗

[Sporadic superficial pemphigus in the child: 2 cases].

INTRODUCTION: Excepting the endemic foliaceus form, childhood pemphigus is uncommon. We report two cases of pemphigus foliaceus in children with typical clinical manifestations. CASE REPORTS: Case n(o) 1. A 5-year-old girl was seen for a vesiculobullous crusted dermatosis involving the trunk and the face which had developed over the last 5 months, predominantly in periorificial and fold localizations. Histology showed intragranulous acatholysis. Direct skin immunofluorescence was positive for anti-intercellular substance IgG and C3. Indirect immunofluorescence was positive for anti-intercellular substance antibodies at 1/500. The diagnosis of superficial pemphigus was retained and the child was given dapsone associated with systemic prednisone (1.5 then 2.5 mg/kg/d). Dapsone was stopped on day 15 due to poor hematological tolerance. Outcome was favorable allowing withdrawal of prednisone at 18 months. Case n(o) 2. A 6-year-old had developed since the age of 18 months a generalized and polycyclic pruriginous erythemato-squamous dermatosis with oozing discharge which started and predominated on the face (periorificial zones). Trace element (copper, selenium, zinc) and vitamin (A, E and B1) assays were within the normal range. Glucagon was normal. Histological examinations of several biopsies were non-contributive. Diagnosis of pemphigus foliaceus was finally obtained after repeated direct immunofluorescence tests which revealed anti-intercellular substance IgG. Indirect immunofluorescence was negative. The child was given prednisone (2 mg/kg/d). DISCUSSION: In children, pemphigus foliaceus has an exceptional frequency and diagnosis is often made quite late (mean 8 months). The diagnosis should always be entertained in children who develop chronic extensive erythemato-squamous and crusted dermatosis, even if formation is absent. Direct skin immunofluorescence confirms the diagnosis and should be repeated if negative in cases with highly suggestive clinical presentations. It would be reasonable to attempt "minor" treatments as the first line approach. Systemic corticosteroids are however the treatment of choice despite the risk of classical side effects. Childhood pemphigus foliaceus is not an attenuated clinical form of adult pemphigus. Mortality is not negligible and is close to that in adults.

Adult↗

IgG subclasses in pemphigus in Indian and UK populations.

The autoimmune blistering disease pemphigus is more common in the Indian subcontinent than in the UK. This study of 19 patients from Oxford, UK and 39 patients from New Delhi, India demonstrates that the incidence of the disease subtypes is different in the two countries. In the UK the commonest subtypes are pemphigus vulgaris and foliaceus with equal prevalence (both eight of 19), but in India pemphigus vulgaris is the most frequent (31 of 39), while pemphigus foliaceus is uncommon (three of 39) and with equal prevalence to the other subtypes. These populations also differ with a younger age at onset in the Indian patients (36.9 India; 52.7 UK) though the sex distribution is the same. Study of the immunopathology shows that the antibodies produced by patients in the two countries do not differ significantly, and are predominantly of the IgG4 subclass. The antibody produced does not vary with the subtype of pemphigus or the age or sex of the patient. Although there are considerable differences between the two groups of patients this difference is not reflected by the subclass of auto-antibody response.

Adult↗

[Tunisian pemphigus. Apropos of 70 cases. (Experience of the dermatology department of La Rabta Hospital 1974-1992)].

INTRODUCTION: Pemphigus frequently seen in Tunisia has specific characteristics distinguishing it from the European or American forms. Between 1974 and 1992, we observed 70 cases in the dermatology unit of the La Rabta University Hospital in Tunis. Most patients were young (mean age 39 yr), predominantly women (80%). Deep pemphigus (pemphigus vulgaris and vegetans) occurred in 56% and superficial pemphigus (seborrheic and foliaceus) in 44%. High-dose corticosteroids were given as initial treatment (1.4 mg/kg/day). Infectious complications were frequent. Mortality was 14%. Pemphigus in Europe and North America usually occurs in adults in the 50-60 year age range. Superficial forms are rare (2-10%). In Tunisia, pemphigus is similar to Brazilian pemphigus with high frequency and predominant superficial forms (pemphigus foliaceus).

Adult↗

Microscopic Nikolsky's sign.

Nikolsky's sign is a useful clinical sign in patients with active pemphigus. It does not however, indicate the level of split in the skin, and is seldom present in partially-treated patients. The purpose of this study was two fold: (i) to document the microscopic or subclinical counterpart of clinical Nikolsky's sign; and (ii) to improve the diagnostic yield of routine histopathology in pemphigus patients in whom the lesions are infected/old and are therefore not useful for histopathology, and when intact blisters are not present; this is particularly useful for institutions in which immunofluorescence facilities are not available. Pemphigus patients were allocated to one of two groups. Group A patients (n = 23) were subjected to manual tangential pressure over the perilesional skin before a biopsy specimen was taken from that site; group B patients (n = 14) were subjected to a biopsy without the tangential pressure technique. Group C consisted of 37 healthy volunteers who were subjected to the tangential pressure technique before a biopsy. Histopathological changes of pemphigus vulgaris or foliaceus were present in 73.9% patients in group A, 28.6% in group B and none in the control group C. Tangential pressure as described below can produce microscopic changes in the epidermis which are diagnostic of pemphigus. These changes are produced at the suprabasal level in pemphigus vulgaris and intraepidermally in pemphigus foliaceus. This technique is of value in those parts of the world where immunofluorescence facilities are not readily available.

Biopsy↗

Detection of IgA autoantibodies to desmogleins by an enzyme-linked immunosorbent assay: the presence of new minor subtypes of IgA pemphigus.

OBJECTIVE: To examine the frequency of antidesmoglein 1 (Dsg1) and antidesmoglein 3 (Dsg3) IgA autoantibodies in IgA pemphigus. DESIGN: We developed an enzyme-linked immunosorbent assay against recombinant Dsg1 and Dsg3 to detect IgA autoantibodies. PATIENTS: Twenty-two patients with IgA pemphigus were studied. Among them, 10 patients had subcorneal pustular dermatosis type, 9 patients had intraepidermal neutrophilic IgA dermatosis type, and 3 patients had pemphigus foliaceus-like clinical features. RESULTS: Of the 22 cases of IgA pemphigus, 3 cases were positive for anti-Dsg1 IgA antibodies and only 1 case was positive for anti-Dsg3 IgA antibodies. In those 4 cases, there were no IgA autoantibodies against other components of the keratinocyte cell surfaces because preincubation with the respective recombinant desmogleins removed the immunoreactivity on immunofluorescence. All 10 patients with subcorneal pustular dermatosis type IgA pemphigus were positive against desmocollin 1 expressed on COS-7 cells. No target antigen was detected in the other 8 cases. CONCLUSIONS: Desmogleins were recognized by IgA antibodies of a few patients with IgA pemphigus. Considering that subcorneal pustular dermatosis type IgA pemphigus recognizes desmocollin 1, autoimmune targets of IgA pemphigus are more heterogeneous than previously considered.

Autoantigens↗

Pemphigus in El Salvador. An eight-year study (1970-1977).

Of 23 patients with pemphigus seen between 1970 and 1977, 18 had foliaceus and five had vulgaris (ratio of 3.73:1). The characteristics of pemphigus foliaceus (age of onset, disease among sisters, presence of lesions resistant to corticotherapy) suggest Brazilian pemphigus foliaceus (fogo selvagem).

Adolescent↗

Intercellular IgA dermatosis.

We report three cases of intercellular IgA dermatosis (IAD) and review the literature. IAD is a spectrum of vesiculobullous or vesiculopustular diseases mediated by intercellular IgA deposition. The clinical picture may vary from a vesiculopustular eruption with centrifugal evolution mainly involving the trunk and extremities, to the typical picture of classic pemphigus variants (foliaceus, vegetans). Histologically, infiltrating polymorphonuclear cells (mainly neutrophils) are observed in the epidermis with formation of pustules and bullae at various levels. However cases with typical histological features of pemphigus (variants) are described. Direct immunofluorescence on perilesonal skin typically displays intercellular IgA deposition at different levels or throughout the epidermis and indirect immunofluorescence often detects low levels of circulating antibodies. The disease has been repeatedly reported in association with monoclonal IgA gammopathy. Most cases respond to dapsone. In some cases IgA is directed against known pemphigus antigens whereas their targets in other cases are newly discovered antigens (105 kD, 115 kD, 120 kD). We observe a heterogeneity within the clinical, histological and immunological characteristics of the disease. Many reported cases feature various combinations of these characteristics. We therefore consider IAD as a disease spectrum with IgA pemphigus (clinical and histological pemphigus) at one end and intercellular IgA vesiculopustular dermatosis at the other end.

Aged↗