Harold O. Perry

Pyoderma gangrenosum: Classification and management

Pyoderma gangrenosum (PG) has four distinctive clinical and histologic variants. Some have morphologic and histologic overlapping features with other reactive neutrophilic skin conditions. PG often occurs in association with a systemic disease, and the specific clinical features of the skin lesion may provide a clue to the associated disease. Management of PG depends on its type and severity and usually requires aggressive local and systemic treatment.

Pyoderma gangrenosum: clinicopathologic correlation and proposed diagnostic criteria

Pyoderma gangrenosum is a rare but significant cause of ulcerations. It is a diagnosis of exclusion. Herein, we suggest diagnostic criteria and some historical perspectives on the diagnosis of pyoderma gangrenosum.

Treatment of cicatricial (benign mucous membrane) pemphigoid with dapsone

Treatment of cicatricial pemphigoid is a problem because patients afflicted are elderly, the disease is chronic, systemic agents required for control are potentially toxic (particularly to older patients), and the disease is often in an advanced stage when the diagnosis is established and requires aggressive therapy for control. We selected dapsone as an alternative anti-inflammatory agent because it has provided control in a subset of patients with bullous pemphigoid, five of six who had oral lesions. Twenty-four patients with cicatricial pemphigoid were treated with dapsone. Twenty (83.3%) had partial or complete control with mild to no inflammatory activity. Control was obtained within 4 weeks in eleven of the twenty patients. The use of dapsone was discontinued in two patients because it failed to control their disease and in four patients (two with control) because of drug-related side effects. Cicatricial pemphigoid may be added to the list of dapsone-responsive dermatoses.

Histopathologic and immunopathologic study of pyoderma gangrenosum

Sixty‐three patients with pyoderma gangrenosum were seen and studied at the Mayo Clinic from 1971 to 1980. Biopsies from the erythcmatous border or necrotic edge of the pyoderma gangrenosum lesions usually demonstrated a characteristic pathogenic morphologic evolution. The early lesions revealed mild to moderate perivascular lymphocytic infiltrate associated with endothelial swelling. The fully developed lesions demonstrated necrosis in addition to a dense lymphocytic infiltration surrounding as well as involving the blood vessels. Extravasation of erythro‐cytes and thrombosis sometimes were seen. Ulceration, infarction, and abscess formation were found in the later stages of evolution. Direct immunofluorescence results were positive in the blood vessels of 36 of 65 (55%) specimens. IgM, C3, and fibrin were found in the papillary and retieular dermal vessels. IgG and IgA were only occasionally present. Pyoderma gangrenosum appears to be a reactive process that is manifested as a vasculilis. Biopsy material from the advancing active erylhc‐matous border has early characteristic derniatopathologic findings of lymphocytic vasculitis. Cutaneous vascular immune deposits suggest an immune pathogenesis of either an immune complex disease or lymphocytotoxic reaction.

Scleredema: A review of thirty-three cases

A review of thirty-three cases of scleredema, with particular reference to clinical and histologic findings, revealed that the disease was often of insidious onset and the course was usually prolonged. A preceding respiratory tract infection was uncommon and usually did not indicate a short course. The patients were divided into two groups according to the presence or absence of diabetes. Diabetes, when present, was typically the late-onset, insulin-dependent type and difficult to control. In many of these patients, the onset of scleredema was so subtle that it went unnoticed by the patient until pointed out by an examining physician.

Incidence of skin cancers in patients with atopic dermatitis treated with coal tar: A 25-year follow-up study

Coal tar ointments have been used for many years in the treatment of chronic eczematoid dermatitis. The possible carcinogenic effect of coal tar ointment and ultraviolet light (Goeckerman regimen), considered individually or in combination, has been of concern to physicians. A 25-year follow-up study was completed on 426 patients with atopic dermatitis and neurodermatitis who were hospitalized and treated with coal tar ointments and ultraviolet light at the Mayo Clinic from 1950 through 1954. These results were compared with those from a similar study that examined patients with psoriasis who also were treated with coal tar ointments and ultraviolet light. The results of this study suggest that the incidence of skin cancer is not significantly increased above the expected incidence for selected populations of the United States. This study offers reassurance about the relatively small risk of skin cancer developing in patients with atopic dermatitis and neurodermatitis treated with coal tar ointments and ultraviolet light (Goeckerman regimen).

Olmsted syndrome—congenital palmoplantar and periorificial keratoderma

We are reporting the third case of the Olmsted syndrome, an entity that has received little attention in the medical literature. The syndrome consists of congenital diffuse, sharply marginated keratoderma of the palms and soles, onychodystrophy , constrictions of digits, and periorificial keratoses. Our patient, in addition, had congenital universal alopecia, absence of a premolar tooth, leukokeratosis of oral tissue, and hyperlaxity of the joints. The differential diagnosis includes hidrotic ectodermal dysplasia of the Clouston type, pachyonychia congenita, acrodermatitis enteropathica, keratoma hereditarium mutilans of Vohwinkel , mal de Meleda , and other forms of palmoplantar keratodermas . This patient had originally been reported as having acrodermatitis enteropathica, a condition with which the Olmsted syndrome can easily be confused.

Malignant Pyodermas Revisited

The concept of malignant pyoderma (MP) has created controversy since its origin. The distinction of this disease from pyoderma gangrenosum was based on clinical criteria and response to treatment. Herein we discuss our current ideas on this entity and its possible relationship to Wegeners granulomatosis (WG). Follow-up data from the three original cases of MP are reported, as well as additional clinical and laboratory data from cases subsequently thought to represent MP. Many of these cases have similar clinical features such as facial and periauricular ulceration and occasionally signs or symptoms of WG, including positive titers of antineutrophil cytoplasmic antibodies (with a diffuse cytoplasmic staining pattern) (cANCA). MP represents a distinctive clinical disorder and may be a dermal manifestation of WG. Some cases of MP may represent pyoderma gangrenosum or other undefined systemic illnesses. Such cases of WG can be distinguished on the basis of clinical, histopathologic, and laboratory evidence including cANCA titers. MP should no longer be used as a final clinical diagnosis.

Plaquelike cutaneous mucinosis: Its relationship to reticular erythematous mucinosis

Nine patients with plaquelike cutaneous mucinosis, including the three described originally in 1960, are presented. The clinical manifestations of asymptomatic persistent, erythematous, infiltrated papules, either isolated or coalescing into plaques, in the midline of the back or chest are suggestive of the diagnosis. The dermal histopathologic findings of perivascular and periappendageal round cell infiltrates and mucin (acid mucopolysaccharides) on special stains confirm the diagnosis. This syndrome is more common among women, may be aggravated by exposure to ultraviolet light, and may subside spontaneously after long periods. We believe the condition of these patients is identical to that described as reticular erythematous mucinosis syndrome.

Adenoma sebaceum of Pringle: a clinicopathologic review, with a discussion of related pathologic entities.

“Adenoma sebaceum of Pringle” (ASP) is a misnomer. The tumor is not an adenoma and is not derived from sebaceous glands. The lesion is characterized by dermal fibrosis and associated vascular proliferation and dilatation. Changes in contiguous sebaceous glands and other adnexal structures are merely secondary. Thus, “angiofibroma” would be a more appropriate name. The histologic changes in ASP (and in related pathologic lesions) suggest that it is a hamartoma rather than a true neoplasm. However, the embryologic tissue of its origin is not definitively known.