Faculty Bibliography

A 61-year-old woman with a history of chilblains and systemic lupus erythematosus (SLE) for 15 years presented with annular, erythematous, scaly papules and plaques on her face, neck, chest, abdomen, back, arms, and legs. A biopsy specimen showed a destructive interface dermatitis with extensive epithelial cell necrosis, which was consistent with lupus erythematosus with combined subacute cutaneous lupus and erythema multiforme-like features. These findings are most compatible with a diagnosis of Rowell syndrome. Rowell syndrome and its relation to lupus erythematosus and erythema multiforme are discussed

We describe a case of non-scarring, generalized, cutaneous and mucosal subepidermal bullous dermatosis that is characterized histopathologically by a neutrophilic infiltrate and strong linear staining with both IgA and IgG along the basement-membrane zone. Autoantibodies to collagen VII of both the IgA and IgG4 subtypes were detected by indirect immunofluorescence test, which led led to a diagnosis of epidermolysis bullosa aquisita (EBA). EBA is a subepidermal bullous disorder that is mediated by autoantibodies, which are directed against type VII collagen. The distinct clinical presentations of EBA are reviewed and discussed in the context of the unique autoantibody profile of this case

A 37-year-old woman presented with a one-year history of asymptomatic, red-brown patches and plaques on the abdomen and extremities, in the context of Raynaud phenomenon and anti-centromere antibodies. Two biopsy specimens confirmed the diagnosis of inflammatory morphea. Even in the absence of initial symptoms to support systemic disease, patients presenting with morphea in the setting of Raynaud phenomenon or anti-centromere antibodies deserve close surveillance for the possibility of CREST syndrome and systemic sclerosis

A 64-year-old woman presented with a one-and-one-half year history of an enlarging, red-brown, firm plaque on the left thigh, with numerous, scattered, indurated, hyperpigmented patches on the lower extremities. Histopathologic examination of the largest plaque confirmed the diagnosis of erythema elevatum diutinum, which is a rare form of leukocytoclastic vasculitis that is associated with many disease entities, which include human immunodeficiency virus infection, malignant conditions, hematologic abnormalities, chronic infection, and autoimmune and connective-tissue disorders. The treatment of choice is dapsone; however, several other treatment modalities have been reported to be of benefit

Raynaud's phenomenon is a common clinical disorder for which patients frequently seek the expertise and care of dermatologists. It is manifested by recurrent vasospasm of the fingers and toes, often associated with exposure to cold temperature or emotional stress. The phenomenon is named after Maurice Raynaud, who, as a medical student, defined the first case in 1862 as episodic, symmetric, acral vasospasm characterized by pallor, cyanosis, suffusion, and a sense of fullness or tautness, which may be painful. Despite more than 140 years of research, the pathophysiology of Raynaud's phenomenon continues to elude investigators. Accordingly, although many pharmacologic treatments have been reported, there is still no cure or gold standard therapy. Further, response to treatment varies and is difficult to predict. Recently, there has been renewed interest in finding the pathogenetic mechanisms of Raynaud's phenomenon, an effort that has led to more potential targeted therapeutics. The purpose of this review is to discuss recent breakthroughs in the pathogenesis and treatment of Raynaud's phenomenon

A 75-year-old woman with end-stage renal disease on hemodialysis presented with a 2-month history of progressive skin thickening of the lower extremities. A punch biopsy specimen showed plump fibroblasts entrapping collagen bundles and positive staining for CD34 and procollagen. These changes were consistent with a diagnosis of nephrogenic fibrosing dermopathy (NFD). Nephrogenic fibrosing dermopathy is a rare, sclerosing disorder in patients with renal failure, which may be mistaken for scleromyxedema. The etiology of NFD is unclear but may be associated with systemic involvement, antecedent surgical procedures, gadolinium, or an underlying hypercoagulable state. The treatment is limited, and only a few reported cases have shown remission after stopping dialysis in transient renal failure

A 32-year-old woman and her 35-year-old sister presented with plaques of scleroderma en coup de sabre. The younger sister's disease was more severe and preceded the older sister's by 10 years. This is the second reported case of familial en coup de sabre, and the first case of horizontal transmission. Treatment of the younger sister with antimalarials and narrow-band ultraviolet B (NB-UVB) phototherapy slowed disease progression and reversed hair loss. The observation that NB-UVB was effective in this case of linear scleroderma suggests that it may be indicated as a therapy for cutaneous scleroderma

A 40-year-old man presented with hardening of the skin of his hands and upper back, which had slowly worsened with time. His medical history included insulin-dependent diabetes mellitus since childhood. Histopathologic features of a biopsy specimen from the skin of his back showed a thick reticular dermis with collagen bundles in a haphazard array, which were separated by increased deposits of connective-tissue mucin. Scleredema and diabetic sclerodactyly are both well recognized skin findings that may occur in patients with diabetes mellitus. It is important to differentiate this condition from scleroderma. Treatment is difficult, and therefore many modalities have been used. This patient has improved with aminobenzoate, colchicine, and DMSO gel

The 1982 ACR classification criteria have become de facto diagnostic criteria for systemic lupus erythematosus (SLE), but a review of the criteria is necessary to include recent diagnostic tests. The criteria were not developed with the help of dermatologists, and assign too much weight to the skin as one expression of a multiorgan disease. Consequently, patients with skin diseases are classified as SLE based mostly on skin symptoms. We discuss specific problems with each dermatologic criterion, but changes must await a new study. We suggest the following guidelines for such a study, aimed at revision of the criteria. 1) The SLE patient group should be recruited in part by dermatologists. 2) The study should evaluate an appropriate international ethnic/racial mix, including late onset SLE as well as pediatric patients. 3) All patients should have current laboratory and clinical evaluations, as suggested in the paper, to assure the criteria can be up-to-date. This includes anti-SS-A and anti-SS-B antibodies and skin biopsies for suspected cutaneous lupus erythematosus except for nonscarring alopecia and oral ulcers. 4) The study should be based on a series of transparent power calculations. 5) The control groups should represent relevant differential diagnoses in numbers large enough to assess diagnostic problems that might be specific to these differential diagnoses. In order to demonstrate specificity of the criteria with a 95% confidence interval between 90 and 100%, each control group of the above should have at least 73 patients