Successful Treatment of Generalized Essential Telangiectasia With 6-Mercaptopurine.

Generalized essential telangiectasia (GET) is a notoriously difficult to treat disorder with no current satisfactory treatments. This case and discussion report the use of 6-mercaptopurine (6-MP) as a successful treatment for GET. Moreover, we show that GET may represent a state of increased angiogenesis, a paradigm shift from the current understanding that these telangiectasias represent dilatations of only pre-existing vessels. This new view of GET may drive others to look at novel agents for treatment.

J Drugs Dermatol. 2017;16(3):280-282.

Adiposis dolorosa.

We report a 46-year-old woman with a nine-year history of obesity; chronic diffuse pain in the adipose tissue of her medial upper arms, lower trunk, and thighs; multiple biopsy-proven lipomas and angiolipomas; and a lipomatous pseudomass of the breast. Her systemic symptoms included generalized weakness, fatigue, memory impairment, and arthralgias. Although some of the lesions were tender, most were only appreciated with palpation. Her clinical history and histopathologic data suggested adiposis dolorosa (Dercum's disease). Owing to the chronic pain, an interdisciplinary approach with the use of analgesics and doxycycline has been initiated.

Familial benign chronic pemphigus (Hailey-Hailey disease).

We present an atypical case of familial benign chronic pemphigus (Hailey-Hailey disease) that manifested with relapsing, flaccid vesicles and erosions, which were limited to the upper chest, anterior aspect of the neck, and anterior aspects of the upper arms without intertriginous involvement. Although individual eruptions in this patient demonstrated asymmetry, relapses did not obey a segmental distribution. To the best of our knowledge, no other patient has been described with symmetric lesions that were localized solely to the anterior upper body without a prior history of lesions at commonly affected disease sites, which include skin folds, the back, and the posterior and lateral aspects of the neck. This unusual presentation of Hailey-Hailey disease highlights the variable nature of the disease.

Porokeratosis palmaris et plantaris disseminata or a disseminated late-onset variant of porokeratotic eccrine ostial and dermal ductal nevus (PEODDN) with follicular involvement.

A 48-year-old woman presented with a four-year history of pruritic, hyperkeratotic, spiny papules that began on her chest and spread to her extremities, groin, palms, face, and scalp where it caused non-scarring alopecia. Histopathologic features included cornoid lamella, which is the hallmark of porokeratosis. However, the patient's constellation of findings does not meet diagnostic criteria for any of the five clinical variants of porokeratosis. Her presentation is most compatible with either porokeratosis palmaris et plantaris disseminata (PPPD), which is a rare variant of punctate porokeratosis that can involve any area of the body or late-onset porokeratotic eccrine ostial and dermal ductal nevus (PEODDN), which is a rare, benign hamartoma of the eccrine sweat glands with porokeratotic histopathologic features that has been reported to occasionally have systemic involvement. Treatment of either condition is difficult although there have been reported successes with cryotherapy, surgical excision, and CO2 laser. Since porokeratosis is a disorder of keratinization and our patient has prominent follicular involvement, isotretinoin may be another reasonable therapeutic option.

Acquired smooth-muscle hamartoma.

A 52-year-old woman presented with an occasionally pruritic, hyperpigmented and hypertrichotic, indurated plaque on the left upper arm that initially developed during childhood. Histopathologic examination showed changes that were consistent with a smooth-muscle hamartoma. Cutaneous smooth-muscle hamartomas are uncommon benign neoplasms. Most lesions are congenital, but there have been a few reports of acquired lesions. These lesions have been described as part of a spectrum of neoplasms that include Becker nevi since they share many clinical and histopathologic features.

Generalized discoid lupus erythematosus.

A 79-year-old woman presented with a four-year history of generalized, erythematous, indurated plaques on the malar areas, back, and extremities. The lesions had been recalcitrant in the past to topical glucocorticoid therapy. A skin biopsy specimen was diagnostic of discoid lupus erythematosus (DLE). A minority of patients with DLE progress to develop systemic lupus erythematosus although generalized DLE is more frequently associated with systemic involvement than is limited disease. Standard therapy of cutaneous lupus includes broad spectrum sunscreens, topical and intralesional glucocorticoids, and antimalarial agents.

Generalized essential telangiectasia.

A 56-year-old woman presented with multiple, erythematous cutaneous vessels that blanched with diascopy and were symmetrically distributed over both lower and upper extremities. The lesions had been present for approximately 6 years and were asymptomatic. Generalized essential telangiectasia (GET) is an idiopathic syndrome of widespread, asymptomatic telangiectases of unknown cause. In our patient, an extensive laboratory evaluation showed low levels of circulating vitamin C as well as the presence of urinary matrix metalloproteinases. The relevance of these abnormal findings is unclear. No uniformly effective treatment exists for GET. Our patient was started on oral doxycycline and was referred for laser therapy.

Pharmacokinetics of topical calcineurin inhibitors in adult atopic dermatitis: a randomized, investigator-blind comparison.

OBJECTIVE: We sought to compare pharmacokinetics of pimecrolimus cream 1% and tacrolimus ointment 0.1% in adults with extensive, moderate to severe atopic dermatitis. Secondary end points included efficacy and safety. METHODS: Patients received twice-daily treatment for 13 days. Blood concentrations of pimecrolimus and tacrolimus were measured at days 1, 5, and 13. Treatment success was defined as an Investigators' Global Assessment score of 0 (clear) or 1 (almost clear). RESULTS: Tacrolimus was detectable in 36% of blood samples and pimecrolimus was detectable in 12%. In patients with measurable blood drug concentrations, systemic exposure to tacrolimus (mean area under the curve(0-10h) < 9.7 ng.h/mL; n = 7) was higher than to pimecrolimus (mean area under the curve(0-10h) < 2.5 ng.h/mL; n = 2). Whole-body treatment success (day 13) was achieved in 1 of 18 (5.6%) and 2 of 19 (10.5%) patients treated with pimecrolimus and tacrolimus, respectively, and face/neck treatment success in 5 of 18 (27.8%) and 5 of 19 (26.3%) patients, respectively. Patients included in the study were adult patients with severe atopic dermatitis. The results and conclusions drawn from this study population may not be applicable for the majority of patients with atopic dermatitis who have mild to moderate disease. CONCLUSION: Pimecrolimus appears to be associated with lower systemic drug exposure than tacrolimus.

Etanercept does not effectively treat moderate to severe alopecia areata: an open-label study.

In this prospective, open-label pilot study, we evaluated the safety and efficacy of etanercept, a TNF-alpha inhibitor, in the treatment of moderate to severe alopecia areata, alopecia totalis, or alopecia universalis. Seventeen otherwise healthy adults with moderate to severe alopecia areata were enrolled. The primary outcome measure was the extent of hair regrowth during and after the end of treatment as evaluated by the Severity of Alopecia Tool (the SALT score). After between 8 and 24 weeks of continuous treatment with etanercept 50 mg given subcutaneously twice weekly, significant regrowth of hair was not shown in any of the subjects treated. Based on these results, etanercept appears to be ineffective in treating subjects with treatment-refractory, moderate to severe alopecia areata, alopecia totalis, or alopecia universalis.

Necrobiotic xanthogranuloma.

A 67-year-old man presented with a 13-year history of slowly enlarging yellow-red plaques on the face and lower extremity. A biopsy specimen was consistent with necrobiotic xanthogranuloma. Necrobiotic xanthogranuloma is a slowly progressive histiocytic disease that is associated with paraproteinemia in most cases; however, its pathogenesis remains unclear. Although there is no first-line therapy, anecdotal reports have shown variable benefit with chemotherapeutic agents.

Psoriasis: to cut or not to cut, what say you?

BACKGROUND: A common problem facing patients suffering from psoriasis is the need for surgery that requires incision through active psoriatic skin. Many patients have been denied surgery because of the fear of an increased risk of infection, decreased wound healing ability, and worsening of the psoriatic lesions. OBJECTIVE: To assess the practices and beliefs of dermatologists and surgeons (orthopedic and plastic surgeons) faced with the decision of whether to operate through active psoriatic skin. METHODS: Dermatologists, orthopedic surgeons, and plastic surgeons selected from various professional membership lists from five representative cities were sent a survey to ascertain their opinions on operating on active psoriatic skin. Psoriatic patients were also given forms asking about the severity of their psoriasis and whether they had ever been denied surgery. RESULTS: Dermatologists are more likely to condone surgery in active psoriatic skin and to believe that there is not a risk of increased infection or decreased wound healing than are orthopedic surgeons and plastic surgeons. These findings are statistically significant (P<0.05). CONCLUSION: With proper preoperative measures and dermatologic treatment, surgery can be performed on active psoriatic skin in most cases with minimal reservations, although a controlled, prospective study is necessary to arrive at a definitive conclusion.