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Biomedical subjects

Bruce E Strober

Publications and source records attributed to Bruce E Strober.

6 recordsLinked to original sources

Erythrokeratodermia variabilis.

A 47-year-old woman presented with both transient, figurate, erythematous patches and fixed, hyperkeratotic plaques on the upper extremities. Her cutaneous disease began in childhood and affects numerous siblings and her daughter. Her clinical presentation, history, and histopathologic analysis were consistent with erythrokeratodermia variabilis, which is a genodermatosis linked to mutations in the gene encoding for the gap-junction protein, connexin 31.

Connexins↗

Pachyonychia congenita, type II.

A 5-year-old girl presented with extensor hyperkeratotic papules and subungual hyperkeratosis with nail-plate discoloration affecting all twenty nails. The mother reported that her daughter had natal teeth. By report, the father has a similar history and constellation of clinical findings. The patient's clinical presentation and history was consistent with pachyonychia congenita, which is a genodermatosis linked to mutations in the genes encoding keratins 6, 16, and 17.

Child, Preschool↗

Generalized morphea.

A 69-year-old man presented with symmetric erythematous, hyperpigmented, and ivory-colored plaques on the trunk and extremities. His clinical history, laboratory analysis, and histopathologic examination were consistent with generalized morphea, a subtype of localized scleroderma.

Aged↗

The Köbner phenomenon and psoriatic arthritis.

Psoriatic arthritis (PsA) affects a large percentage of patients with psoriasis. Similar to the cutaneous disease of psoriasis, PsA displays an isomorphic response (ie, the propensity to develop at traumatized sites). In some patients, traumatized joints that subsequently develop PsA are the initial manifestation of psoriasis, preceding the skin disease by months to years. Dermatologists should screen patients with psoriasis for accompanying PsA and consider recently traumatized joints that remain arthritic to be a component of this disease.

Arthritis, Psoriatic↗

Etanercept for the treatment of psoriasis: combination therapy with other modalities.

Etanercept is a self-administered medication that has FDA approval for the treatment of rheumatoid arthritis, juvenile rheumatoid arthritis, psoriatic arthritis, and ankylosing spondylitis. Etanercept is a human fusion protein of the tumor necrosis factor receptor (TNFR) and the Fc region of IgG1 that binds to and presumably inhibits the pro-inflammatory and pro-proliferative activity of the tumor necrosis factor (TNF). A recent multisite, randomized, double-blind, placebo-controlled study conclusively demonstrates that etanercept as monotherapy effectively treats patients with moderate-to-severe plaque psoriasis. This effect is dose-responsive, with the etanercept 50 mg twice-weekly dose significantly more effective than the 25 mg twice-weekly dose in reducing the Psoriasis Area and Severity Index (PASI) score over both 12 and 24 weeks of continuous therapy. Nevertheless, clinical trials do not instruct the dermatologist on how to practically integrate etanercept into a patient's pre-existing treatment regimen. Many psoriasis patients are already on other systemic therapies or have a medical history that necessitates a tailored approach to their therapy. Further, in some patients, etanercept at 25 mg twice weekly is ineffective in maximally clearing a patient of psoriasis. Below are cases that demonstrate how etanercept can be combined with other medications in order to both maximize clinical efficacy and minimize potential risk.

Acitretin↗