No abstract

Thirty patients who fulfilled the criteria for complete and incomplete types of Behçet's syndrome were studied to determine the histopathologic changes of erythema nodosum-like lesions. Lymphocytic vasculitis was observed in 12 (40%) of the cases, but it was only focal in areas of severe lymphocytic inflammation and only mild in degree. No diffuse vasculitis was seen. It is our opinion that the observed lymphocytic vasculitis is only secondary to moderate or severe lymphocytic inflammation. Twelve cases (40%) revealed septal panniculitis, 10 cases (33%) demonstrated lobular panniculitis with moderate to marked inflammation and fat cell necrosis, and 8 cases (27%) showed mild and nonspecific inflammation in the panniculus. Therefore, there is a spectrum of histopathologic changes of erythema nodosum-like lesions in Behçet's syndrome, similar to that of erythema nodosum secondary to other systemic disorders.

Twelve cases of pachyonychia congenita were reviewed. The mode of inheritance was autosomal dominant. The clinical features of these patients included thickened nails, hyperkeratosis of the palms and soles, thinning of hair or alopecia, painful bullae or ulcerations of the palms and soles, leukokeratosis oris, verrucous lesions of the extremities, hyperhidrosis, premature eruption of teeth, paronychial infections, epidermal cysts with milia, and corneal dyskeratosis at times associated with cataracts. Biopsy from the plantar lesions usually revealed marked hyperkeratosis, acanthosis, moderate hypergranulosis, and minimal dermal inflammatory infiltration. Treatment with keratolytic agents and lubricants is indicated to areas of palmar and plantar hyperkeratosis but usually produces only transient benefit. Squamous cell carcinoma developed in one of the patients over the site of chronic plantar ulcerations. Areas of chronic bullous formation or ulceration should be observed for possible skin malignancy.

Light microscopic studies of 55 patients with complete and incomplete types of Behçet's syndrome were reviewed. We reported here 39 skin biopsies from 30 patients with cutaneous lesions of oral, and genital ulcers, folliculitis, erythema multiforme, and thrombophlebitis. Leukocytoclastic vasculitis was seen in 5 of 12 (42%) cases of oral ulcers and 2 of 7 (29%) of genital ones. Lymphocytic vasculitis was demonstrated in 3 of 12 (25%) cases of oral ulcers and 4 of 7 (57%) of genital ulcers. However, the vasculitis was observed only locally in the areas with moderate or severe inflammation. From the biopsy specimens of our cases with fully developed mucocutaneous lesions of Behçet's syndrome, it appears that the forms of vasculitis that are often emphasized in the literature are a phenomenon secondary to the intense inflammation seen in such cutaneous lesions.

A 40-year-old man who had received long term immunosuppressive treatment for 14 years following kidney transplantation developed multiple skin lesions on both antecubital fossae, scalp, and both lower extremities. Histopathologic findings from three skin regions revealed characteristic features of epidermolytic hyperkeratosis, verruca vulgaris, and disseminated superficial porokeratosis, respectively. Although immunocompromised individuals may demonstrate verruca vulgaris or porokeratosis, disseminated epidermolytic acanthoma (DEA) has not been reported to be associated with immunosuppressed status. We suggest that immunosuppression may play a role in the pathogenesis of DEA, as shown in our case.