Charles H. Dicken

The clinical and histopathologic spectrums of urticarial vasculitis: Study of forty cases

Urticarial skin lesions may occur in patients as a manifestation of necrotizing vasculitis. We describe a series of forty patients with idiopathic chronic urticaria and histologic features of necrotizing vasculitis. On the basis of clinical evaluation, we have classified urticarial vasculitis into two major groups: (1) hypocomplementemic (sixteen patients, ten of whom had evidence of renal disease) and (2) normocomplementemic (twelve patients with systemic disease and twelve with only cutaneous involvement). Most patients with hypocomplementemia presented with arthritis, and some had abdominal pain or airway compromise. Although patients with normocomplementemia and systemic disease had a less severe clinical course, four exhibited renal disease that was characterized by microhematuria and proteinuria. Direct immunofluorescence microscopy of the skin aids in assessing renal involvement in some cases of hypocomplementemic urticarial vasculitis, particularly when IgG and IgM are deposited at the basement membrane. There seems to be a spectrum of disease in urticarial vasculitis, ranging from benign cutaneous lesions to systemic disease.

Retinoids: A review

The retinoids are synthetic derivatives of vitamin A. Isotretinoin (13-cis-retinoic acid) is now being widely used in the United States for severe acne and etretinate is available in Europe and other countries for psoriasis. These drugs are also effective for a number of other skin diseases. This is an attempt to review basic knowledge of retinoids with which the practicing dermatologist should be familiar, to review the current status of studies, and to speculate on the present and future roles of these drugs in dermatology.

A clinical and histopathologic study of granulomatous rosacea.

A retrospective clinical and histopathologic study of 53 patients with granulomatous rosacea was undertaken. The patients had a broad clinical spectrum of lesions that ranged from primarily erythema to papulonodular lesions. Extrafacial lesions occurred in 15% of patients. Histologic examination showed mixed lymphohistiocytic inflammation (primarily lymphocytic inflammation in 40% of patients and primarily histiocytic with a few giant cells in 34%), epithelioid granulomas in 11% of patients, and epithelioid granulomas with caseation necrosis in 11%. Most patients had a good response to oral antibiotic therapy. Granulomatous rosacea is not a distinct disease but can be regarded and treated as a subtype of rosacea.

Treatment of classic pityriasis rubra pilaris

BACKGROUND Treatment of classic pityriasis rubra pilaris, which almost always progresses to a generalized erythroderma with marked, often disabling keratoderma of the palms and soles, remains problematic. OBJECTIVE Our purpose was to evaluate the results of treatment in a recent period during which the retinoid group of drugs has been available. METHODS The clinical course of 75 patients with classic pityriasis rubra pilaris seen from 1982 to 1992 was reviewed. RESULTS Of 15 patients treated with isotretinoin, 10 had complete and 2 had partial clearing. Of six treated with etretinate, four had clearing. All eight patients treated with methotrexate had a favorable response. Other forms of treatment, including Goeckerman regimen, corticosteroids, vitamin A, and cyclosporine, were ineffective. CONCLUSION Early diagnosis and early treatment with retinoids appear to offer the best chance for clearing of pityriasis rubra pilaris. If retinoids fail or cannot be used, methotrexate should be considered.

Epidermolysis bullosa acquisita: A heterogeneous disease

The question of whether epidermolysis bullosa acquisita (EBA) is a distinct entity prompted a review of the Mayo Clinic experience. Twelve patients with this diagnosis were seen from 1955 through 1979, and their records were reviewed. Particular attention was directed to immunofluorescence microscopy (IF), which had been performed in seven patients. Direct IF was positive in seven patients, and indirect IF was positive in four patients. Although many cases of EBA may remain distinct, we believe that the morphologic appearance known as EBA may represent a final common pathway of disease expression in a heterogeneous group of patients with a variety of more specific diseases and that trauma serves as a localizing factor. Our findings indicate that cicatricial pemphigoid probably is the most common specific diagnosis but that all cases of EBA are not represented by this diagnosis. Furthermore, we conclude that when IF is performed, exclusion of other bullous diseases, which is a key criterion for the diagnosis of EBA, is more difficult than previously recognized.

Evaluation of isotretinoin treatment of hidradenitis suppurativa

Eight patients with a long-standing hidradenitis suppurativa were treated with isotretinoin, 0.71 to 1.2 mg/kg/day, as a single agent for 4 months and have had follow-up of at least 2 months. The clinical status was judged as cleared in one patient, almost cleared in three patients, improved in one patient, not changed in two patients, and worse in one patient.

Isotretinoin treatment of Darier's disease

A total of 104 patients with Dariers disease were treated with 13- cis -retinoic acid (isotretinoin) at nineteen medical centers. Clinical evaluation by both physicians and patients indicated that the drug is very effective in alleviating Dariers disease but does not produce prolonged remissions.

The therapeutic response of urticarial vasculitis to indomethacin

Ten patients with urticarial vasculitis, characterized clinically by persistent painful urticarial lesions, angioedema, recurrent arthralgia, abdominal pain, and low-grade fever, were selected for study. All patients had histologic evidence of leukocytoclastic vasculitis in the urticarial lesions. Results of direct immunofluorescence microscopy of urticarial lesions were positive in all nine of the patients tested. Treatment with indomethacin in dosages from 25 mg three times daily to 50 mg four times daily resulted in complete clearing of all disease manifestations in six of ten patients within 17 days and partial improvement in three. In eight of the ten patients, disease activity recurred within 48 hours after discontinuation of the use of indomethacin. Gastrointestinal irritation was the only side effect noted. Indomethacin is proposed as an effective mode of therapy in a disorder unresponsive to treatment with conventional medications for urticaria, including high-dose corticosteroids.

Isotretinoin treatment of pityriasis rubra pilaris

Five patients with pityriasis rubra pilaris were treated with isotretinoin from September 1982 through 1985. Isotretinoin at an average dose of 1.16 mg/kg/day for 16 to 24 weeks caused complete or almost complete clearing in four of the five patients.

Bowel-associated dermatosis-arthritis syndrome: Bowel bypass syndrome without bowel bypass

The bowel bypass syndrome is a well-recognized complication in patients who have had jejunoileal bypass for morbid obesity. It consists of an influenza-like illness with increased temperature, chills, polyarthralgia, myalgia, and inflammatory papules and pustules that are 2 to 4 mm in diameter and that usually appear on the extremities and the upper part of the trunk. A patient is reported who had an identical bowel-associated dermatosis-arthritis syndrome but who had not undergone a jejunoileal bypass operation. Seven previously reported similar cases are reviewed.