Charles Camisa

Vesiculobullous systemic lupus erythematosus

Vesicles and bullae complicating systemic lupus erythematosus (SLE) are relatively uncommon. Two young women with SLE presented with vesiculobullous eruptions on sun-exposed areas that resembled dermatitis herpetiformis (DH) histologically. There were active visceral manifestations of SLE in both patients, including mesangioproliferative glomerulonephritis. Granular deposits of IgG and/or IgM, along with IgA, were demonstrated along the basement membrane of skin by direct immunofluorescence microscopy. Review of fifteen additional cases of vesiculobullous SLE reported in the literature suggests that this cutaneous manifestation of SLE is associated with a high incidence of IgA deposits in skin and glomerulonephritis. The following criteria for the diagnosis of a distinct subset of vesiculobullous skin lesions occurring in patients with SLE are proposed: (1) a diagnosis of SLE based upon American Rheumatism Association (ARA) criteria; (2) vesicles and bullae arising upon but not limited to sun-exposed skin; (3) histopathology compatible with DH; (4) negative indirect immunofluorescence for circulating basement membrane zone (BMZ) antibodies; (5) direct immunofluorescence reveals IgG and/or IgM and often IgA at the BMZ.

Vesiculobullous systemic lupus erythematosus: A report of four cases

A vesiculobullous eruption is now recognized as a specific but rare cutaneous complication of systemic lupus erythematosus. Four additional cases are reported in whom the five previously proposed criteria were met. Increased activity of systemic lupus erythematosus affecting other organ systems was documented in three of four cases. All four patients demonstrated a positive lupus band, and three of four showed granular deposits of IgA along the basement membrane zone (BMZ). Evidence of glomerulonephritis was obtained in three of four cases, which resulted in death in one. The higher than expected incidence of IgA deposits in skin and renal disease in patients with vesiculobullous eruption of systemic lupus erythematosus is again confirmed. The eruption cleared in all four cases with either dapsone, 50 mg daily, or high doses of corticosteroids and immunosuppressive agents.

Treatment of oral erosive lichen planus with systemic isotretinoin

Six patients with symptomatic oral erosive lichen planus were treated with systemic isotretinoin (10 to 60 mg daily) for 8 weeks. Five (83%) showed subjective and objective improvement at completion of therapy, but the improvement was slight. Relapse occurred in four patients within 2 months after the drug was stopped; one was lost to follow-up. Because of the minimal improvement and adverse side effects, no patient wished to be re-treated with isotretinoin.

The coexistence of systemic lupus erythematosus and psoriasis

Systemic and discoid lupus erythematosus (LE) have both been reported in association with psoriasis. Four additional patients who had systemic LE are reported. All of them had a high titer of antinuclear antibodies (ANAs) and were SS-A(Ro)-negative. Only one patient exhibited photosensitivity. SS-A(Ro) antibodies do not appear to be specific markers for the coexistence of LE and psoriasis, and screening for them in all psoriatics prior to ultraviolet B phototherapy is not recommended. Pitfalls in the management of patients with systemic LE and psoriasis are discussed.

Paraneoplastic pemphigus : a distinct autoimmune vesiculobullous disorder associated with neoplasia

A vesiculobullous disease termed paraneoplastic pemphigus with distinct autoantibodies was newly described in 1990. All reported cases have occurred in patients with a history of neoplasia, including lymphoma, chronic lymphocytic leukemia, poorly differentiated sarcoma, and benign thymoma. As in pemphigus vulgaris, intraepithelial clefts with acantholysis are noted histopathologically, and intercellular binding of immunoreactants is seen with direct immunofluorescence studies of mucous membrane and skin biopsies. However, immunoreactants may also be found along the basement membrane zone in paraneoplastic pemphigus. Indirect immunofluorescence using rat bladder epithelium as substrate shows an intercellular pattern that appears to be highly specific for paraneoplastic pemphigus. We report a patient with non-Hodgkins lymphoma of 8 years duration who developed severe erosive stomatitis and lichenoid dermatitis after receiving chemotherapy for a relapse of lymphoma. Her case illustrates the typical features of the disorder described as paraneoplastic pemphigus. Neoplasia-associated pemphigus may be a more precise term for this disorder because the course of the blistering eruption does not always parallel the course of the underlying cancer. The clinical features, histopathologic findings, and immunofluorescence findings of this unique syndrome are reviewed.

Indirect immunofluorescence microscopy of lichen planus

Indirect immunofluorescence microscopy using serum and lesional skin revealed a lichen planus specific antigen (LPSA) in 80% of patients with lichen planus. This antigen is found only in the stratum granulosum and stratum spinosum. It was demonstrated in skin lesions in Caucasians and Negroes in South Africa and the U. S. A. but was not found in the skin of normal people or patients with other dermatoses. Indirect immunofluorescence should prove useful in distinguishing atypical forms of lichen planus from other dermatoses.

Methoxsalen is a potent inhibitor of the metabolism of caffeine in humans

The acute effect of a single oral dose of methoxsalen on the pharmacokinetics of caffeine was investigated in five nonsmoking volunteers with psoriasis. Caffeine, 200 mg orally, was administered to each subject at baseline before treatment with methoxsalen. One week later each subject was given a single oral dose of 1.2 mg/kg methoxsalen 1 hour before administration of another oral dose of 200 mg caffeine. The clearance of caffeine declined markedly from 110 ± 17 ml/min (mean ± SE) in the control study to 34 ± 5 ml/min after methoxsalen. During the period of maximum inhibition the mean elimination half‐life of caffeine increased from 5.6 hours at baseline to 57 hours after administration of methoxsalen. The peak concentration of caffeine and the time to reach the peak concentration of caffeine were not affected by pretreatment with methoxsalen. Thus, methoxsalen, administered acutely, is a potent inhibitor of caffeine metabolism in humans with psoriasis. Results of this investigation suggest that the elimination of concurrently administered drugs may be inhibited in patients receiving methoxsalen. In comparison with other drugs, methoxsalen is the most potent inhibitor of drug metabolism in humans. Other work has shown that inhibition of drug metabolism by methoxsalen is associated with both extensive covalent binding of metabolite(s) of methoxsalen to liver microsomal protein in vitro and in vivo and inactivation of cytochrome P‐450.

Naevoid basal-cell carcinoma syndrome with unilateral neoplasms and pits

A patient with the naevoid basal‐cell carcinoma syndrome (NBCCS) is reported in whom non‐aggressive neoplasms developed predominantly on the right side of the body. It is proposed that a post‐zygotic somatic mutation is most likely to be responsible for the unilateral manifestation of the syndrome in this patient.

Autosomal Dominant Keratoderma, Ichthyosiform Dermatosis and Elevated Serum Beta-Glucuronidase

Five patients in a large pedigree with autosomal dominant palmoplantar keratoderma and/or an ichthyosiform dermatosis were studied. Biopsies of the keratoderma, a remote hyperkeratotic plaque on an arm and the ichthyosis all revealed the same histopathologic changes including hyperkeratosis with round retained nuclei. All 5 patients had elevated serum beta-glucuronidase activity. The significance of beta-glucuronidase in this and other skin diseases is discussed.

Lichen planus pemphigoides: Report of a case with oral lesions

Lichen planus pemphigoides is a rare condition characterized by the coexistence of lichen planus and bullous pemphigoid. Oral lesions have been reported but have not been studied immunopathologically. We describe a 59-year-old white man with cutaneous and oral lesions of lichen planus pemphigoides. Biopsies were done on these lesions, and the specimens were examined by routine light microscopy and immunofluorescent techniques. Fine keratotic striae on the anterior buccal mucosa were clinically consistent with oral lichen planus. Perilesional tissue associated with ulceration of the posterior buccal mucosa showed histologic and immunopathologic changes consistent with bullous pemphigoid.