Kevin Pehr

Coexistence of erythromelalgia and Raynaud's phenomenon

Erythromelalgia is characterized by spontaneous recurrent episodes of redness, heat, and pain of the extremities that can be triggered or worsened by heat. Raynauds phenomenon occurs in response to cold exposure and presents as pallor of the fingers or toes, often followed by cyanosis and rubor. Although the 2 conditions may appear to be opposites in symptomatology and clinical presentation, there are very rare reports of their coexistence. A case of coexistent erythromelalgia and Raynauds phenomenon is presented. The pathophysiology is reviewed to elucidate a common mechanism underlying some cases of the 2 seemingly opposite conditions. A review of the literature indicates that causative and pathophysiologic similarities between the 2 conditions may exist in some cases. Rare reports of coexistence of the 2 disease processes further strengthen such research findings.

Linear Lichen Planopilaris of the Trunk: First Report of a Case

Background: Lichen planopilaris (LPP) is believed to be a follicular variant of lichen planus that affects pilosebaceous units, mainly of the scalp. An extremely rare variant of LPP is a linear form, which follows the lines of Blaschko. Of the five previously documented cases of linear LPP, all were limited to the face. Objective: We report the case of a 34-year-old male who presented with a nonpruritic eruption on the trunk consisting of erythematous, keratotic, folliculocentric papules following Blaschkos lines. Results: Biopsy revealed lichenoid and interface dermatitis involving the basilar epidermis and hair follicles, as well as apoptotic keratinocytes, consistent with LPP. Conclusion: This represents the first documented case of LPP, following the Blaschkos lines, in a nonfacial distribution.

Juvenile xanthogranuloma in a 77-year-old man.

A 77-year-old white man presented with a 2-month history of nontender, nonpruritic, smooth, yellow-red papules. He stated that the first lesion appeared on his anterior left thigh, followed by several others on his lower extremities, abdomen, and back, as well as one on the right forearm; none developed on his face, neck, chest, irides, or mucous membranes. He further stated that three of the early lesions (all smaller than 3 mm) had regressed spontaneously in 2 to 3 weeks, whereas the others had grown quickly to their full size of 4 to 7 mm (Fig. 1). At the time we first saw him, he had a total of 12 papules. On physical examination, the mucocutaneous findings were as noted above, and one lesion was removed for histopathology. Family history was negative for xanthomatous lesions, hyperlipidemia, diabetes mellitus, cancer, and neurofibromatosis. Laboratory studies included complete blood count, chemistry panel, urinalysis, and lipid profile, all of which were normal. At his second visit, six papules were treated empirically: the one on the right anterior thigh was injected with 0.25 mL of triamcinolone acetate 40 mg/mL (10 mg total dose), five other lower extremity lesions were treated with a 10-second application of liquid nitrogen, using a cottontipped applicator. Three weeks later, the papule that had been injected showed no resolution, whereas those that had been treated by cryotherapy showed good-to-moderate regression; however, the patient had developed six new lesions, for a total of 18. Six papules were marked for test purposes and treated with liquid nitrogen for varying lengths of time. At follow-up 1 month later, the results were excellent (Fig. 2 and Table 1). All remaining lesions received further cryotherapy. The patient was quite satisfied with the treatment, and indicated that he would return if any new lesions occurred. Upon follow-up some months later, all existing lesions had flattened completely, and no new ones had appeared. Hematoxylin and eosin staining showed a diffuse dermal infiltrate, consisting mainly of histiocytes, many of them having foamy cytoplasm. Admixed with these were a large

DERMATOLOGY IN A WAR ZONE: A PERSIAN GULF EXPERIENCE

The clinical experience of two US Army dermatologists during the recent Gulf War (Operation Desert Shield/Storm) are presented, with comparison with dermatologic experience in previous wars and in civilian practice.

Comprehensive analysis of cutaneous T-cell lymphoma (CTCL) incidence and mortality in Canada reveals changing trends and geographic clustering for this malignancy.

Previous reports of geographic clustering of cutaneous T‐cell lymphoma (CTCL) in Texas, Pittsburgh, and Sweden as well as the occurrence of CTCL in married couples and family members raise a possibility of the existence of an external and potentially preventable trigger(s) for this rare skin cancer.

No show : Incidence of nonattendance at a dermatology practice in a single universal payer model

Background: Nonattendance at scheduled appointments is a major problem. Previous studies have shown rates between 17 and 31%. Most US studies found the type of payer to be the greatest determinant of attendance rates. Objectives: This study examines the no-show rate in a private dermatology practice under a single universal payer model, including the effects of old versus new patient, gender, day of the week, month, and weather. Results: The overall rate of nonattendance was lower than in all previous studies (7.79%), with the only statistically significant variable being established versus new patients. Limitations: Certain demographic data investigated in previous studies (eg, age, socioeconomic status) were not assessable. Data are from a single office. Conclusion: The no-show rate in a single universal payer, private practice model is low, especially for established patients.

The EuroSCAR study: Cannot agree with the conclusions

To the Editor: We would like to add another perspective to Forestier’s article on sunscreen development published in the Journal’s May 2008 supplement. He identified the need for further development of ultraviolet light (UV) filters that provide balanced protection against both UVA and UVB solar radiation. We believe that these efforts should also consider the potential environmental effects of sunscreens. Runoff of pharmaceutical products, especially antibiotics and hormonal agents used in both human and veterinary medicine, have had significant unanticipated environmental effects. Recent investigations suggest that run-off of personal care products (PCPs), including sunscreens, may have similar consequences. The US Environmental Protection Agency regards UV filters as environmental contaminants because they are measurable in many aquatic ecosystems. Environmental risk assessments show that current levels of filtering agents can have deleterious effects at microscopic and macroscopic levels. Sunscreen agents that are dispersed in bodies of water decrease the penetration of UV light, which in turn affects aquatic organisms that depend on light for metabolic and reproductive functions. A recent study showed certain cosmetic sunscreen agents (3-benzylidene camphor and 4-methylbenzylidene camphor) disrupt the androgen and estrogen balance in laboratory rats and their progeny. The experiment’s exposure levels of 4-methylbenzylidene were consistent with amounts detected in freshwater fish in Swiss lakes. In addition, an in situ study of oceanic coral showed that sunscreen agents can deplete symbiotic zooxanthellae, the microscopic algae that provide the photosynthesis essential for coral’s growth. Without these symbionts, the coral becomes bleached, which further disrupts marine ecosystems. To date, relatively little attention has been directed at the potential long-term direct and indirect effects that PCPs have on the health of the environment and human populations. As the controversies regarding the health benefits and risks associated with sun exposure continue, we recommend including the inadvertent effects of PCPs on the environment and on human populations in these discussions.

Granuloma annulare associated with hypercalcemia secondary to hyperparathyroidism.

2 Bastuji-Garin S, Rzany B, Stern RS, et al. Clinical classification of cases of toxic epidermal necrolysis, Stevens–Johnson syndrome and erythema multiforme. Arch Dermatol 1993; 129: 92–96. 3 Metry DW, Jung P, Levy ML. Use of intravenous immunoglobulin in children with Stevens–Johnson syndrome and toxic epidermal necrolysis: seven cases and review of literature. Pediatrics 2003; 112: 1430–1436. 4 Princ C, Kerdel FA, Padilla SR, et al. Treatment of toxic epidermal necrolysis with high-dose intravenous immunoglobulin. Arch Dermatol 2003; 139: 26–32.

Pustular flagellate dermatitis after consumption of shiitake mushrooms

Lentinula edodes, or shiitake mushroom (SM), is typically grown in Eastern Asia and used in traditional Asian medicine and cuisine. Recently, SM became popular in Western culture and is now the second most commonly consumed mushroom in the world.1, 2 Although rare, adverse reactions to SM have been previously reported. In mushroom farm workers, contact dermatitis, contact urticaria, rhinitis, and hypersensitivity pneumonitis have been described. In the general population, oral intake of raw SM has been associated with a typical flagellate eruption, known as shiitake dermatitis (SD).3 Shiitake dermatitis, also termed shiitake toxicoderma or flagellate mushroom dermatitis, classically manifests 12 hours to 5 days after the ingestion of SM.1, 4 Since its first description in 1977, SD has been described in approximately 100 patients, predominantly Japanese.3, 5 Recently, a few cases were reported in Europe and in the United States.1 The mechanism underlying SD has not been fully elucidated. Although the eruption is usually considered nonallergic,6 5 cases of SD resulted in positive delayed skin prick testing, suggesting that delayed-type systemic hypersensitivity may be important in its pathogenesis.2, 3, 4, 7, 8, 9 We report the first case of SD in Canada and, to our knowledge, the first case of pustular SD. In our patient, positive delayed skin prick testing to SM was documented.

Tazarotene 0.1% Cream as Monotherapy for Early-Stage Cutaneous T-Cell Lymphoma.

Background: Numerous treatments are available for cutaneous T-cell lymphoma (CTCL), including systemic retinoids. Very few data are available on topical retinoids. Objectives: The aim of this study was to evaluate the safety and efficiency of tazarotene as monotherapy for early-stage CTCL. Methods: An open-label, prospective study of tazarotene as monotherapy for stages IA to IIA CTCL was conducted. Index lesions on 10 patients were followed for 6 months on treatment, plus at least 6 months off treatment. Results: Six patients (60%) showed complete response (CR). Erythema, scaling, thickness, and lesion area decreased progressively throughout treatment. The mean time to CR was 3.8 months; CR was durable for at least 6 months in 83%. Of the 4 patients (40%) without CR, 2 (20%) had stable disease and 2 (20%) stopped the medication because of local side effects; none showed progression. Conclusions: This is the first Canadian trial providing evidence that topical tazarotene has excellent potential as a monotherapy agent for stages I to IIA CTCL.