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Sulfapyridine and sulfones decrease glycosaminoglycans viscosity in dermatitis herpetiformis, ulcerative colitis, and pyoderma gangrenosum.

Shortly after the introduction of sulfa drugs, sulfapyridine was found to have unique therapeutic properties, unrelated to antibacterial activity. Later, sulfones were found to share the same properties. The disorders initially improved were dermatitis herpetiformis, pyoderma gangrenosum, subcorneal pustular dermatosis, acrodermatitis continua, impetigo herpetiformis and ulcerative colitis. They were also sometimes helpful in many other disorders. They are effective in select disorders characterized by edema followed by granulocytic inflammation or edema followed by vesicle or bullae formation. The sulfones work in low doses in leprosy and their mode of action is not fully understood. Several pieces of experimental information are available. It is proposed that these drugs are entering or influencing the protein moiety of glycosaminoglycans and decreasing tissue viscosity. This decreased tissue viscosity prevents edema and dilution of tissue fluid and decreases acute inflammation and vesicle and bullae formation.

Clofazimine

Malignant pyoderma.

Malignant pyoderma is a destructive, ulcerating skin disease that occurs chiefly in young adults. Only eight cases of this rare disease have been reported. The head and neck have been involved in all cases, and a predilection for the periauricular region has been noted in several of the cases. Although the disease is progressive and chronic, responses to high-dose systemic corticosteroids have been noted, but usually a flare is associated with a reduction in dose. An additional case is described in which therapy with isotretinoin and sulfapyridine led to complete remission.

Adolescent

Chlorambucil--an effective corticosteroid-sparing therapy for pyoderma gangrenosum.

A 21-year-old man, with a past history of severe aphthosis and arthritis between age 5 and 15 years, presented at age 18 years with a large leg ulceration, which developed after minor trauma. At that time he was otherwise healthy and taking no medications. Between 1983 and 1986 the patient failed to respond to intensive topical care to the ulcer, two attempted skin grafts, oral prednisone (up to 200 mg/day), sulfasalazine, dapsone (200 mg/day), multiple trials of intralesional triamcinolone acetonide, hyperbaric oxygen, azathioprine, methotrexate, clofazimine, minocycline, and three courses of pulse methylprednisolone therapy. Therapy with chlorambucil (4 mg/day) resulted in progressive improvement and complete healing with eventual cessation of all other therapy. The use of chlorambucil in pyoderma gangrenosum may be an effective adjunctive steroid-sparing therapy.

Administration, Oral

[Pseudo-septicemic pyoderma gangrenosum and breast cancer. Apropos of a case caused by an intramuscular injection].

We report a case of pyoderma gangrenosum (PG) mimicking a severe infectious skin disease in a woman with metastatic breast cancer. PG started at the site of an intramuscular injection administered a few days previously, and it subsequently extended. The skin disease was cured by high-dose corticosteroid therapy and clofazimine, but it marked a turn for the worst in the course of the breast cancer which became rapidly fatal.

Breast Neoplasms

German shepherd dog pyoderma: a genetic disorder.

In this study the pedigrees of 42 German Shepherd dogs with German Shepherd dog Pyoderma (GSP) were analysed. Parents, littermates and offspring of the affected dogs were traced and their owners were questioned on characteristic skin lesions. Evidence suggesting an autosomal recessive trait was found. Breeders are advised to exclude affected animals and their relatives from further breeding.

Animals

Investigations on the role of flea antigen in the pathogenesis of German shepherd dog pyoderma (GSP).

Skin reaction patterns to the intradermal injection of a whole-body flea extract were examined in five physically healthy dogs and in 24 dogs with German Shepherd dog Pyoderma (GSP) at 15 and 30 minutes and at 1, 2, 4, 8, 24, 48 and 72 hours after the injection. In 10 out of 24 GSP dogs a positive skin reaction was observed macroscopically after 15 minutes. Delayed reactions at 24 or 48 hours were not observed. In the control group neither immediate nor delayed reactions were observed. The histopathologic skin changes were basically the same in both groups: an initial polymorphonuclear reaction followed by a mononuclear cell reaction. In the GSP dogs, however, these changes occurred earlier and were more prolonged than in the normal dogs. No flea-antigen-specific IgGd antibodies could be demonstrated by enzyme-linked immunosorbent assay. It is concluded that delayed type hypersensitivity to flea antigen does not play a role in the pathogenesis of GSP. Immediate type hypersensitivity may contribute to the disease in some cases.

Animals

Investigations on the role of staphylococci in the pathogenesis of German shepherd dog pyoderma (GSP).

Skin reaction patterns to intradermal injections of a Staphylococcus intermedius antigen were examined in physically healthy dogs and in dogs with German Shepherd dog Pyoderma (GSP) at 15 and 30 minutes and at 1, 2, 4, 6, 8, 24, 48 and 72 hours after the injection. In both groups the skin histopathology revealed an aspecific inflammatory response of an early polymorphonuclear reaction, followed by a mononuclear cell reaction at 24 and 48 hours. It is concluded that hypersensitivity to staphylococcal antigens does not play a role in the pathogenesis of GSP.

Animals

Squamous syringometaplasia in lobular panniculitis and pyoderma gangrenosum.

Squamous metaplasia of eccrine sweat glands has been most frequently described in chronic cutaneous ulcerations with associated epidermal hyperplasia. We found examples of the process in skin biopsy specimens from five patients: three had associated lobular panniculitis and two had lesions of pyoderma gangrenosum. The metaplasia was located in the mid-to-deep reticular dermis in all five patients and extended into the superficial subcutis in one. Immunohistochemical stains for CEA and S-100 protein were used to accentuate the relationship of the metaplastic islands with eccrine ducts. It is postulated that necrosis of a portion of the eccrine duct is the stimulus for this process.

Adenoma

Case report: severe pyoderma associated with familial Mediterranean fever--favorable response to colchicine in three patients.

Familial Mediterranean fever (FMF) is an autosomal recessive disorder that occurs most frequently among Sephardic Jews and Armenians. It is characterized by recurrent episodes of fever, peritonitis, pleuritis, and arthritis. Skin lesions are seen in some patients. Diagnosis of FMF usually is made on clinical grounds only, typically when recurrent attacks of abdominal pain, fever, and arthritis are observed in a patient with an appropriate ethnic background and family history. To date, there are no specific diagnostic laboratory tests for FMF. Three patients with severe recurrent Pyoderma are covered in this report. In all three cases, the cutaneous lesions were associated with clinical manifestations of FMF and responded to colchicine therapy favorably. The importance of such an association and its therapeutic consequences are emphasized.

Adolescent

Cutaneous vasculitis, hypersensitivity vasculitis, erythema nodosum, and pyoderma gangrenosum.

Cutaneous vasculitis encompasses a highly heterogeneous group of disorders of diverse etiology, pathogenesis, and clinical features. Recent reexamination of clinical pathologic features have yielded no insights assisting our understanding of current clinical classification schemes and strengthening our clinical approach. Erythema nodosum is a common cutaneous disorder for which new etiologies continue to be described. Pyoderma gangrenosum is an uncommon ulcerative cutaneous condition that remains a therapeutic problem.

Erythema Nodosum

Pyoderma gangrenosum: recognition and management.

Pyoderma gangrenosum, a progressive necrotizing and ulcerative disease of the skin, presents serious problems in diagnosis and management to physicians not familar with the entity. The clinical basis of the diagnosis is outlined, and the possible etiological mechanisms are reviewed. Treatment is primarily nonsurgical, relying on oral administration of corticosteroids and conservative local care of the ulcers.

Diagnosis, Differential

Pseudoepitheliomatous hyperplasia and pyoderma gangrenosum after a brown recluse spider bite.

Brown recluse spider bites may result in extensive soft tissue injury, causing months of disability. We have described a patient who had a series of extensive surgical debridements after envenomation. Despite skin grafting, persistent cutaneous lesions and extensive satellitosis progressed to involve the entire lower extremity. A recent biopsy showed pseudoepitheliomatous hyperplasia and pyoderma gangrenosum complicating the original injury. Although the role of early surgical excision and newer forms of medical treatment including dapsone and antivenom are still in evolution, recent reports suggest that the majority of patients will respond to medical therapy and may not require any surgical intervention.

Animals

A case of pyoderma vegetans and the follicular occlusion triad.

A case of pyoderma vegetans (PYV) and hidradenitis suppurativa (HS) is described. Our patient had a previous history of perifolliculitis capitis abscedens et suffodiens and acne conglobata. Direct immunofluorescence findings ruled out pemphigus vegetans and suggested a potential pathogenic mechanism.

Acne Vulgaris

Pyoderma gangrenosum treated with cyclosporin A.

A 49-year-old woman with a 14-year history of pyoderma gangrenosum unresponsive to a variety of systemic and topical treatments was given oral cyclosporin A (CyA), a potent immunosuppressive agent widely used in organ transplantation for the prophylaxis of acute graft-versus-host disease. Improvement and subsequent healing of the lesions was evident within 3 weeks of starting treatment and has continued for the duration of treatment (12 months).

Adult

Development of folliculitis and pyoderma gangrenosum in association with abdominal pain in a patient following treatment with isotretinoin.

A 19-year-old man with severe acne developed extensive scalp folliculitis and later superficial pyoderma gangrenosum following treatment with isotretinoin. A cyclical neutropenia was noted and bone marrow findings suggested myelodysplasia. We believe that isotretinoin was implicated in the development of overt symptoms in this patient whose haematological condition was previously asymptomatic.

Abdominal Pain