Junctional adhesion molecule-A is abnormally expressed in diffuse cutaneous systemic sclerosis skin and mediates myeloid cell adhesion.

OBJECTIVE: To investigate the role of junctional adhesion molecule-A (JAM-A) in the pathogenesis of systemic sclerosis (SSc). METHODS: Biopsy specimens from proximal and distal arm skin and serum were obtained from patients with SSc and normal volunteers. To determine the expression of JAM-A on SSc dermal fibroblasts and in SSc skin, cell surface ELISAs and immunohistology were performed. An ELISA was designed to determine the amount of soluble JAM-A (sJAM-A) in serum. Myeloid U937 cell-SSc dermal fibroblast and skin adhesion assays were performed to determine the role of JAM-A in myeloid cell adhesion. RESULTS: The stratum granulosum and dermal endothelial cells (ECs) from distal arm SSc skin exhibited significantly decreased expression of JAM-A in comparison with normal volunteers. However, sJAM-A was increased in the serum of patients with SSc compared with normal volunteers. Conversely, JAM-A was increased on the surface of SSc compared with normal dermal fibroblasts. JAM-A accounted for a significant portion of U937 binding to SSc dermal fibroblasts. In addition, JAM-A contributed to U937 adhesion to both distal and proximal SSc skin. CONCLUSIONS: JAM-A expression is dysregulated in SSc skin. Decreased expression of JAM-A on SSc ECs may result in a reduced response to proangiogenic basic fibroblast growth factor. Increased JAM-A expression on SSc fibroblasts may serve to retain myeloid cells, which in turn secrete angiogenic factors.

Cephalexin rash in infectious mononucleosis.

The ampicillin rash occurring in cases of infectious mononucleosis is well documented. Similar phenomena have also been observed with other antibiotics. The case of a patient with infectious mononucleosis treated with cephalexin who later showed a rash is presented and the previous literature is reviewed. The rash seen in this patient, who was treated with cephalexin, may be similar to the rash seen with ampicillin treatment of patients with infectious mononucleosis. The mechanism by which these eruptions occur is not completely understood, although there are many interesting theories. Perhaps rashes during infectious mononucleosis in association with other antibiotics are actually more common, but just not recognized. A heightened awareness of this possibility may therefore lead to recognition of more cases and further understanding of this entity.

Costochondritis presenting as a peculiar skin lesion.

Costochondritis is an important complication of median sternotomy that can present months to years after surgery. Delay in diagnosis may result in more disability and increased medical costs. The varied clinical manifestations of costochondritis are reviewed and a case is presented.

Erythema with features of seborrheic dermatitis and lupus erythematosus associated with systemic 5-fluorouracil.

Multiple cutaneous reactions have been reported in association with topical and systemic 5-fluorouracil therapy. Three patients are reported who noted facial erythema with features of both seborrheic dermatitis and lupus erythematosus following the administration of systemic 5-fluorouracil.

Malignant transformation of oral lichen planus: case report and review of the literature.

Lichen planus is a mucocutaneous disorder well known to both dermatologists and dentists. Traditional belief holds that oral lichen planus predisposes to the development of squamous cell carcinoma of the oral cavity. We present a case that illustrates such a malignant transformation in a patient who smoked and had actinically damaged skin.

Procainamide-induced urticarial vasculitis.

Dermatologists are often faced with the difficulty of evaluating drug reactions in patients receiving multiple medications. Unfortunately, few drugs produce distinctive lesions; many types of medications can produce identical eruptions. One common drug-induced eruption is urticaria. We report a specific eruption due to procainamide: urticarial vasculitis.

Lichenoid drug eruption secondary to propranolol.

Propranolol, a widely prescribed beta-adrenergic receptor blocker, has occasionally been associated with adverse cutaneous reactions. We present a case of ulcerative lichenoid drug eruption of the penis secondary to propranolol therapy. This unusual clinical presentation has not been described previously in the literature.

Autoimmune hemolytic anemia in a patient with mycosis fungoides.

We report a case of autoimmune hemolytic anemia in a patient with mycosis fungoides. We propose that autoimmune hemolytic anemia may be induced by cutaneous lymphoproliferative diseases. Thus, hemolysis should be considered as a mechanism of anemia in patients with mycosis fungoides.

Cutaneous lesions of histoplasmosis with transepidermal elimination in a patient with acquired immunodeficiency syndrome.

Histoplasma capsulatum is a dimorphic pathogenic fungus that produces a variety of self-limiting disease syndromes in healthy persons, but commonly disseminates in immunocompromised hosts. Although histoplasmosis has been recently described in patients with acquired immunodeficiency syndrome, it is an uncommon finding, occurring in fewer than 0.5 percent of patients with acquired immunodeficiency syndrome. We are reporting a second case of disseminated histoplasmosis presenting as keratotic papules with transepidermal elimination of Histoplasma capsulatum. Our case demonstrates the importance of including histoplasmosis in the differential diagnosis of keratotic papules occurring in patients with acquired immunodeficiency syndrome.

Skin signs of systemic disease. When the problem is more than skin-deep.

The cutaneous manifestations of systemic diseases are diverse. In some cases, they are the first signs of an underlying disorder, such as Cowden's disease, dermatomyositis, and Lyme disease. Sister Mary Joseph's nodule (metastatic involvement of the umbilicus) is an ominous sign of internal malignant disease. Drug-induced skin necrosis may result from therapy with coumarin (Coumadin, Panwarfin, Sofarin) or heparin.

Localized bullous pemphigoid occurring in a surgical wound.

Localized forms of bullous pemphigoid have been reported to occur in sites of trauma. The case of a 79-year-old female who developed blisters at the site of an abdominal surgical wound is reported. The diagnosis of bullous pemphigoid was made by histology and immunofluorescence studies. The reported cases of localized bullous pemphigoid associated with surgical wounds are reviewed.

Lymphocytic vasculitis: is it a specific clinicopathologic entity?

Seventy-one biopsy specimens taken at the Mayo Clinic from June 1977 through May 1981 demonstrated lymphocytic vasculitis. All specimens met the criteria for lymphocytic vasculitis, defined as (1) a predominantly lymphocytic infiltrate that involves and surrounds blood vessel walls, (2) fibrinoid necrosis of blood vessel walls, and (3) endothelial cell hyperplasia. Other histologic findings such as thrombosis, extravasation of erythrocytes, ulceration, epidermal infarction, and evidence of nuclear dust were seen only occasionally. Hypocomplementemia and other serologic abnormalities were very rare, even when lymphocytic vasculitis was extensive. The clinical diagnoses varied, with drug reaction (12 patients) and chronic urticaria (10 patients) being most frequent. In 32 cases, no specific diagnosis could be made at the time of dermatologic dismissal. In the remaining 39 cases, the diagnoses were varied and no definite clinical categories can be applied to them. We also observed typical lymphocytic vasculitis in some cases of other clinical entities, such as nodular scabies, erythema multiforme, and urticarial vasculitis, and so forth. We conclude that lymphocytic vasculitis is probably not a specific clinicopathologic entity but is more likely a reactive process, secondary to severe lymphocytic inflammation in the skin.

Pyoderma faciale: a clinical study of twenty-nine patients.

Pyoderma faciale is a distinctive entity. Twenty-nine patients with this process were seen in the Mayo Clinic from 1969 to 1980. Twenty-seven patients had follow-up that ranged from 1 month to 11 years, and twenty-two had follow-up of 3 years or more. Clinical features that characterize the patients were (1) female predominance, (2) onset later than teenage acne vulgaris, generally at 19 to 40 years of age, (3) rapid onset and progression, (4) facial involvement with sparing of the back and chest, (5) cysts, swelling, and purulent drainage with a lack of comedones, and (6) paucity of systemic complaints. Patients were treated with multiple forms of therapy simultaneously, often including Vleminckx packs, oral antibiotics, incision and drainage, ultraviolet B, and intralesional steroids. Of twenty-five patients available for follow-up at 1 year, twenty-three had achieved remission, though fifteen patients required ongoing treatment to maintain optimal control. Twenty-three patients had scarring as a sequela. Patients with pyoderma faciale represent a subset of patients with acne in whom the outlook is favorable with appropriate therapy.

Cutaneous cryptococcosis simulating pyoderma gangrenosum.

A 33-year-old white man with a history of chronic ulcerative colitis presented with multiple cutaneous ulcers and an indurated cellulitic area on his right thigh. Clinically, the ulcers were considered to represent pyoderma gangrenosum. However, tissue biopsy revealed copious yeast forms that were identified as Cryptococcus neoformans. The indurated area on the right thigh later ulcerated, and tissue culture of this area also revealed Cryptococcus. This case illustrates that when pyoderma gangrenosum is diagnosed, the possibility of deep fungal infection should be considered and excluded by appropriate studies.

An association between C1 esterase inhibitor deficiency and lupus erythematosus: report of two cases and review of the literature.

Two patients who had lupus erythematosus and C1 esterase inhibitor deficiency are described. The data on eleven previously reported cases are reviewed and summarized with those from our own cases. Early complement component depletion secondary to C1 esterase inhibitor deficiency may predispose to the development of lupuslike syndromes by impairing the ability of the organism to handle foreign antigen. Further study of this unique group of patients may provide insight into the pathogenesis of immune complex disease.

Griseofulvin therapy of lichen planus.

Thirty-one patients who had lichen planus treated with griseofulvin were randomly selected for review from a group of patients with lichen planus seen at the Mayo Clinic between January 1976 and June 1980; two patients were excluded because of lack of adequate follow-up. Of the 11 patients with only oral lesions, 6 showed a marked improvement or complete remission. Of the 18 patients with lichen planus involving one or more sites with or without oral lesions, 15 had cutaneous lesions. Three of the 15 had improvement of their cutaneous lesions; however, 1 of the 3 continued to develop new lesions, although old ones were improving. In patients with recalcitrant, symptomatic oral lichen planus, a trial of griseofulvin would seem justified. Success in patients with cutaneous lichen planus is less likely; however, griseofulvin may afford relief in selected patients. This study indicates that further prospective studies are needed to clarify the efficacy of griseofulvin in lichen planus.

Pigmented Bowen's disease arising from pigmented seborrheic keratoses.

We report three cases of pigmented Bowen's disease that clinically and histologically had features of seborrheic keratoses. We speculate about the mechanism of pigmentation in these lesions and suggest that they arise from pigmented seborrheic keratoses.