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Dermatology Online Journal

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About

Dermatology Online Journal is an open-access, refereed publication intended to meet reference and education needs of the international dermatology community since 1995. Dermatology Online Journal is supported by the Department of Dermatology UC Davis.

Volume 20, Issue 7, 2014

Original

Non-random geographic distribution of patients with cutaneous T-cell lymphoma in the Greater Pittsburgh Area

Background: Environmental hazards may play a role in the etiology of cutaneous T-cell lymphoma (CTCL). Some studies have found an increased incidence of CTCL among workers in chemical science, transportation, and manufacturing industries, but other studies have not. This discrepancy may be attributable to population migration, complicating accurate assessment of lifetime exposures. The Pittsburgh population has very low migration rates and most CTCL patients seen at the University of Pittsburgh Medical Center (UPMC) Cutaneous Lymphoma Center are life-long local residents. The Greater Pittsburgh Area used to be an industrial hub. There are residential communities positioned within close proximity to inactive industrial sites that continue to contain pollutants.

Objective: To determine whether CTCL patients’ residences cluster within specific Pittsburgh regions, in particular, those with high levels of environmental pollutants.

Methods: Our study included patients diagnosed with CTCL at the UPMC Cutaneous Lymphoma Center between 2000 and 2012. We mapped the longitudinal and latitudinal coordinates of patients’ residences at diagnosis, superfund sites, toxic release inventory sites, particular matter levels, and dermatologists’ offices using ArcMap 10.1. We then performed a SaTScan analysis using zip codes to assess for geographic clustering of patients’ residences in the Pittsburgh metropolitan statistical area. We assessed for a correlation between case distribution and both environmental hazards sites and dermatologist density in the area.

Results: We identified 274 patients with CTCL in the Greater Pittsburgh area. We identified a statistically significant geographic cluster (p<.001) in zip code 15213, which is the most densely populated neighborhood in Pittsburgh and the site of the region’s only CTCL clinic.  We observed no relationship between the locations of superfund sites, toxic release inventory sites, or particular matter levels and CTCL case distribution.

Conclusion: Our findings do not support an association between exposure to environmental toxins and CTCL. CTCL cases clustered in areas with the highest population density, which also happen to include a regional CTCL center. To evaluate a possibility of urban pollutants playing a role in etiology of CTCL, dermatologist density and access to care need to be addressed as potential confounders in the future studies.

Commentary

Dermatology on Instagram

The novel photo-sharing social networking platform, Instagram, has an impressive following of 75 million daily users, with a predominantly younger and female demographic.  This study investigated the presence of dermatology-related content on Instagram.  The most popular professional dermatological organizations, dermatology journals, and dermatology related patient advocate groups on Facebook and Twitter, determined from a prior study, were searched for established profiles on Instagram.  In addition, dermatology-related terms (i.e. dermatology, dermatologist, alopecia, eczema, melanoma, psoriasis, and skin cancer) and dermatology-related hashtags (i.e. #dermatology, #dermatologist, #melanoma, #acne, #psoriasis, and #alopecia) were searched.  None of the top ten dermatological journals or professional dermatological organizations were found on Instagram.  Although only one of the top ten patient advocate groups related to dermatology conditions, Melanoma Research Foundation, had an Instagram presence, there were many private offices, cosmetic products, and some patient advocacy groups.  This novel social networking platform could grant dermatology journals and other professional organizations a unique opportunity to reach younger demographic populations, particularly women, with the potential for true educational and life-changing impact.

Review

Topical treatments of skin pain: a general review with a focus on hidradenitis suppurativa with topical agents

Hidradenitis Supprurativa (HS) is a painful chronic follicular disease.  Few papers have addressed pain control for this debilitating condition.  Possible topical agents include tricyclic antidepressants, opioids, anticonvulsants, NSAIDs, NMDA receptor antagonists, local anesthetics and other agents. The first line agents for the topical treatment of the cutaneous pain of HS are diclonefac gel 1% and liposomal xylocaine 4% and 5% cream or 5% ointment. The chief advantage of topical xylocaine is that is quick acting i.e. immediate however with a limited duration of effect 1-2 hours.  The use of topical ketamine, which blocks n-methyl-D-aspartate receptors in a non-competitive fashion, might be a useful tool for the treatment of HS pain.  Topical doxepin, which available in a 5% commercially preparation (Zonalon®) , makes patients drowsy and is not useful for controlling the pain of HS . Doxepin is available in a 3% or 3.3% concentration (which causes less drowsiness) from compounding pharmacies and can be used in compounded analgesic preparations with positive effect.  Topical doxepin is preferred over use of topical amitriptyline because topical doxepin is more effective.  Nevertheless, topical amitriptyline increase of the tactile and mechanical nociceptive thresholds and can be used for topical pain control in compound mixture of analgesics .  Gabapentin and pregablin can also be used compounded with other agents in topical analgesic preparations with positive topical anesthetic effect.  Capsaicin is not useful for topical treatment of the pain of HS.  Sometimes compounded of anesthetics medications such as ketamine 10%, bupivacaine 1%, diclofenac 3%, doxepin 3% or 3.3%, and gabapentin 6% can extend the duration of effect so that medication only needs to be used 2 or 3 times a day.  Still in my experience the easiest to get and most patient requested agent is topical diclonefac 1% gel.

Case Report

CD30 expression in cutaneous B-cell and post-transplant peripheral T-cell lymphoma: report of 2 cases

CD30 expression is the hallmark of the cutaneous CD30+ lymphoproliferative disorders, lymphomatoid papulosis and anaplastic large cell lymphoma. We report CD30 expression in cutaneous follicle center cell lymphoma and in cutaneous post-transplant peripheral T-cell lymphoma. Histopathologists should be aware of CD30 expression in cutaneous lymphomas outside the realm of so-called CD30+ lymphoproliferative disorders to avoid diagnostic errors and improper medical treatment.

Cutaneous erosions: a herald for impending pancytopenia in methotrexate toxicity

Psoriatic plaque erosion is a rare toxic side effect of low-dose methotrexate  (LDMTX) that has been reported during the treatment of psoriasis and described as a herald for impending pancytopenia. Fatalities from this have rarely been reported. Even rarer is methotrexate (MTX)-induced erosions of clinically normal skin in patients without a history of psoriasis. We report 3 rare presentations of MTX-induced cutaneous erosions, 2 fatalities occurring with MTX-induced psoriatic plaque erosions, and the sixth reported case of MTX-induced erosions with no prior history of psoriasis. Each were elderly patients on proton pump inhibitors with a history of chronic non-steroidal anti-inflammatory drug (NSAID) use. They all presented with acute onset of erosions after a recent change in their MTX dose. Pancytopenia followed in each case. Physicians’ awareness of the sequelae in MTX-induced cutaneous erosions is imperative so MTX can be discontinued and treatment instituted to prevent fatal bone marrow suppression.

Case Presentation

Recurrent basal cell carcinoma with intracranial invasion: a case report and literature review

Basal cell carcinoma (BCC) is the most common malignancy in humans. We present a man with a recurrent BCC of the scalp that presented as an intracranial tumor 18 years after original excision.

A rare pigmentary disorder in two non-identical siblings: Griscelli Syndrome –type 3

Griscelli Syndrome (GS) is a rare autosomal recessive disorder characterized by pigmentary dilution of the hair and skin (partial albinism). Three different types (1-3) caused by mutation in three different genes have been described. Patients with GS type 1 have primary central nervous system dysfunction; type 2 patients commonly develop hemophagocytic lymphohistiocytosis and type 3 patients present with partial albinism only. Two siblings discussed here had silvery hair, eyebrows and eyelashes since birth with no features suggestive of immunodeficiency or neurological impairment, making it an even rarer presentation of Griscelli Syndrome, type 3. Diagnosis was confirmed on light microscopy (LM) of hair shafts. Both GS1 and GS2 have been described earlier. However, extensive search of the literature failed to reveal a similar presentation from Indian origin. This is the first ever report of GS-3 in non-identical siblings from India.

Pilomatrix carcinoma: a rare cause of facial tumor

Pilomatrix carcinoma is a rare malignant tumor that originates from hair matrix cells. It is not usually considered in a differential diagnosis owing to its low incidence. We present a case of this uncommon entity and review the literature.

Sclerosing lymphangitis of penis- literature review and report of 2 cases

Sclerosing lymphangitis of the penis is a condition related to vigorous sexual activity, manifesting as an asymptomatic firm cord –like swelling around the coronal sulcus of the penis. Since, it is self-limiting, only reassurance along with abstinence from sexual activity are required. In addition to reporting two new cases, we review and discuss the medical literature for this condition.

Giant melanoma of the abdomen: case report and revision of the published cases

Malignant melanoma presenting as a giant cutaneous mass is rarely observed in clinical practice. A few patients with giant melanoma have been reported, Herein, we document our experience with a patient with giant cutaneous melanoma of the abdomen and review the features of previously reported individuals.

Multifocal cutaneous alternariosis in a 70-year-old kenyan renal transplant patient

Alternaria species are a group of dematiaceous fungi that are ubiquitous in nature and are becoming an increasingly important cause of disease in immunocompromised patients.  We present a case of a 70 year old renal transplant recipient with multiple areas of cutaneous Alternaria infections likely introduced during local trauma.  Treatment has required a combination of systemic therapy and surgical excision.  This case illustrates the importance of recognizing fungal infections with cutaneous manifestations, such as alternariosis, in immunosuppressed patients.

Striated muscle hamartoma presenting as a chin cyst in a newborn

Striated muscle hamartoma is a rare, benign mesenchymal neoplasm that typically arises in the midline of a newborn patient. We report a clinically and histopathologically classic case of striated muscle hamartoma presenting as a chin cyst in a newborn female.

Successful treatment of facial papules with electrodessication in a patient with birt-hogg-dubé syndrome

We report a case of a 51-year-old Hispanic female who presented with a several year history of multiple flesh colored papules of cosmetic concern on the nose and medial cheeks. Biopsies revealed fibrofolliculoma and trichodiscoma. The patient was referred for genetic testing and was found to be positive for the FLCN gene defect, confirming a diagnosis of Birt-Hogg-Dubé syndrome. Further work-up with screening renal ultrasound and CT scan of the thorax and abdomen was unrevealing. For treatment of these skin lesions, dermasanding was attempted initially with only minimal benefit. She subsequently had multiple lesions treated with electrodessication at a low setting and was very pleased with the results. Curettage was not performed and importantly, there has yet to be a recurrence of lesions treated with only hyfrecation.

Photo Vignette

Bloodroot associated eschar

We present a case of a 60-year-old woman who, after using a blemish cream purchased at a health store, presented to our clinic with a large eschar on the right neck.  The active ingredient in this cream was found to be Sanguinaria canadensis, a known escharotic.  This substance is popularly marketed as a natural remedy for a host of skin diseases such as blemishes, moles, warts, skin tags, basal cell carcinomas, squamous cell carcinomas, actinic keratoses, and even melanoma.  The patient was treated with topical corticosteroids but is still left with a scar from the healed eschar.  Dermatologists must be aware of the increasing popularity of “natural” medicinal skin cancer therapies, such as bloodroot-containing “cancer salves” and “herbal cures,” and emphasize to their patients that conventional excision, micrographic surgery, electrodessication, and/or curettage are highly successful treatments for most skin cancers with little morbidity.

Rapidly growing pigmented tumor on a scalp nevus sebaceous of a pediatric patient: Observation or excision

A 14-year-old girl presented with a new, rapidly growing, pigmented tumor on a previously existing yellowish, verrucous plaque on the scalp. The patient received complete surgical excision. Routine histology ruled out basal cell carcinoma (BCC) and the histological diagnosis was pigmented trichoblastoma arising in nevus sebaceous (NS). It is important to define management for new lesions developing in pediatric patients with existing nevus sebaceus.

Sclerotic atrophic plaques associated with a tattoo

Lichen sclerosus is a chronic inflammatory disease, usually of the anogenital area, that causes intractable itching and soreness. Less commonly, it may have extragenital involvement in 15 to 20% of cases. Lichen sclerosus has been reported at sites of injury as a Koebner phenomenon. We report a case of lichen sclerosus at the site of a tattoo with simultaneous genital involvement.

Chronic myelomonocytic leukemia can present with diffuse planar xanthoma

There is a documented association between diffuse planar xanthoma and chronic myelomonocytic leukemia. Previous accounts have hypothesized that patients with multiple lesions or extensive cutaneous disease are more likely to have an underlying abnormality of the reticuloendothelial system. However, we document a 62-year-old woman with a large pruritic yellow-orange plaque on the chest and lower anterior neck consistent with planar xanthoma that was discovered to have chronic myelomonocytic leukemia. Solitary large plaques of planar xanthoma should be considered in the same fashion as diffuse planar xanthoma and warrant a prompt hematologic evaluation.

Purplish tender nodule on the arm

We present a patient with a painful glomus tumor of the right upper arm.