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Open Access Publications from the University of California

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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, and by the Northern California Veterans Administration.

Volume 20, Issue 8, 2014

Review

Social impact of the burden of psoriasis: effects on patients and practice

The raised, scaly, and erythematous plaques associated with psoriasis can be cosmetically disfiguring, which may provoke disgust, fear, and aversion in others. Consequently, the social stigma of psoriasis can be devastating for patients, evoking feelings of shame and anxiety about how they are perceived. In recent years, appreciation of psoriasis as a disease that can cause social distress and impairment has increased. This review discusses the manifold social burdens of psoriasis; different and emerging therapies that may mitigate these burdens by improving outcomes associated with the underlying disease; and psoriasis management in the context of healthcare reform changes focused on assessing the quality and value of patient care. The social impact of psoriasis is substantial (eg, affecting interpersonal relationships, sexual function, intimacy, occupational success). Undertreatment of psoriasis continues, despite evidence that biologic agents may lessen the physical and social burdens and provide greater patient satisfaction than conventional therapy. Changes in healthcare place an even greater emphasis on measurable outcomes, including patient satisfaction. Increased understanding of the social burden of psoriasis may lead to provision of more comprehensive, holistic care that is in concordance with the evolving restructured reimbursement system.

Risk of tuberculosis with the use of anti-TNF medications in psoriasis: incidence, screening and management

Tumor necrosis factor (TNF) plays an important role in containing mycobacterial infections. With the rapidly increasing role of TNF inhibitors in dermatology, tuberculosis (TB) is becoming an important and worrisome concern to dermatologists. This paper aims to provide a comprehensive review on the incidence of TB in patients treated with anti-TNF, the variety of TB screening methods, and management of these cases. Various national recommendations have been highlighted. The monoclonal antibodies, infliximab and adalimumab, appear to be more associated with the risk of TB reactivation than the soluble receptor etanercept. Tuberculosis associated with TNF inhibitors, in contrast to classical TB, is more likely to be disseminated, atypical, extra pulmonary, and life threatening. Vigilance for typical and atypical presentations of active TB is mandatory until the end of therapy. Although tuberculin standard test (TST) has been the gold standard for screening of latent TB infection (LTBI) for close to a century, it has several inadequacies and may be unreliable in patients with widespread psoriasis. Interferon gamma release assays (IGRAs) with better diagnostic specificity and sensitivity are a promising adjunct to diagnose LTBI at present. Although appropriate screening and treatment of LTBI will lower the risk of reactivation to a great extent, no chemoprophylactic regimen is fully protective.

Case Report

A verrucous presentation of mycosis fungoides

Mycosis Fungoides (MF) is the most common cutaneous T-cell lymphoma and can have a variety of clinical and histological manifestations, including erythrodermic, pustular, bullous, hypo/hyperpigmented, and verrucous forms. We describe a case of a 59-year-old woman who presented with verrucous hyperkeratotic plaques on her distal fingertips, dorsal feet, and areolae that were subsequently biopsied and shown to be mycosis fungoides. This case highlights one of the many atypical manifestations of MF and underscores the need to have a high clinical suspicion for the disease.

A case of generalized eruptive histiocytosis in a 23-year-old man

Generalized eruptive histiocytosis (GEH) is an exceedingly rare type of Factor XIIIA-positive histiocytopathy, characterized by symmetric distribution of red-brown papules on the trunk, proximal extremities, and face. It is diagnosed by the integration of the clinical features, light microscopic findings, and phenotypic profile. Herein, we report an unusual presentation of GEH in a 23-year-old man, who had a striking papular and erythrodermic component to his lesions.

Case Presentation

Subcutaneous sarcoidosis without systemic involvement

Subcutaneous sarcoidosis is a rare variant of cutaneous sarcoidosis, which typically presents as single or multiple, indurated, ill-defined plaques, typically on the upper extremities. Granulomas consisting of macrophages with multinucleated giant cells and sparse lymphocytic inflammation are confined to the subcutaneous tissue, rather than to their usual location within the dermis in typical lesions of cutaneous sarcoidosis. An association between subcutaneous sarcoidosis and systemic involvement has been reported, although response to treatment and prognosis remain good. We report a case of a middle-aged woman with subcutaneous sarcoidosis, with negative work-up for systemic involvement of sarcoidosis. Interestingly, family history was significant for a son who died from complications of pulmonary sarcoidosis. The patient was successfully treated with a tapering course of oral prednisone in combination with hydroxychloroquine.

Rapid response of tattoo-associated cutaneous sarcoidosis to minocycline: case report and review of the literature

Importance: Cutaneous sarcoidosis can present in pre-existing tattoos. Previous reports suggest modest improvement with systemic or topical corticosteroids or other immunomodulating medications. Tetracyclines have anti-inflammatory properties and have been shown to be efficacious in non-tattoo associated cutaneous sarcoidosis. The pharmacology of minocycline suggests that its higher concentration in the skin may improve its efficacy in the treatment of cutaneous granulomas.Case Report: We present a case of a 35-year-old man with a history of pulmonary sarcoidosis who developed raised plaques within tattoos present for over 10 years. Skin biopsy findings revealed non-caseating granulomas consistent with cutaneous sarcoidosis. The patient was started on minocycline 100mg twice daily and had resolution of pruritus in four days and improvement of sarcoidal plaques within one week.Conclusions: To our knowledge, this is the first report of cutaneous sarcoidosis in tattoos treated with minocycline. Our patient’s rapid response to minocycline suggests that minocycline may be a quickly effective medication for cutaneous sarcoidosis and should be considered as a therapeutic option given its favorable side-effect profile.

Basal cell carcinomas in a young woman with Steinert’s disease.

Steinert’s disease or Myotonic dystrophy type I (DM1) is an autosomal dominant disease characterized by myotonia, muscular dystrophy, cataracts, hypogonadism, frontal balding, and electrocardiographic alterations.Several tumors have been associated with DM1 such as pilomatricoma, thymomas and insulinomas. Herein, we describe the unusual onset of multiple basal cell carcinomas in a young woman with DM1.

Axillary basal cell carcinoma in patients with Goltz-Gorlin syndrome: report of basal cell carcinoma in both axilla of a woman with basal cell nevus syndrome and literature review

Background: Basal cell carcinoma of the axilla, an area that is not usually exposed to the sun, is rare. Individuals with basal cell nevus syndrome, a disorder associated with a mutation in the patch 1 (PTCH1) gene, develop numerous basal cell carcinomas.Purpose: To describe a woman with basal cell nevus syndrome who developed a pigmented basal cell carcinoma in each of her axilla and to review the features of axillary basal cell carcinoma patients with Goltz-Gorlin syndrome.Methods: Pubmed was used to search the following terms: axillary basal cell carcinoma and basal cell nevus syndrome. The papers and their citations were evaluated.Results: Basal cell nevus syndrome patients with basal cell carcinoma of the axilla were observed in two women; this represents 2.5% (2 of 79) of the patients with axillary basal cell carcinoma. Both women had pigmented tumors that were histologically nonaggressive. The cancers did not recur after curettage or excision.Conclusions: Basal cell carcinoma of the axilla has only been described in 79 individuals; two of the patients were women with pigmented tumors who had basal cell nevus syndrome. Similar to other patients with axillary basal cell carcinoma, the tumors were histologically nonaggressive and did not recur following treatment. Whether PTCH1 gene mutation predisposes basal cell nevus patients to develop axillary basal cell carcinomas remains to be determined.

Basal cell carcinoma of the perineum

Basal cell carcinoma (BCC) is the most common nonmelanoma skin cancer. Most BCCs are found on areas of UV-damaged skin, The study of BCCs of sun-protected regions, however, suggests a more complex pathogenesis. We present a case of BCC of the perineum in a man with no previous history of skin cancer. This is the first report of BCC in this region and one of a small body of cases arising on or near the genital and perianal regions.

Hydroxyurea-induced amyopathic dermatomyositis presenting with heliotrope erythema

Hydroxyurea (HU) is a chemotherapeutic agent used for the treatment of myeloproliferative disorders such as chronic myeloid leukemia, polycythemia vera, and essential thrombocytosis. We describe a 69-year-old man who had essential thrombocytosis and developed amyopathic dermatomyositis after long-term HU therapy. He presented with Gottron papules and heliotrope erythema. The former has been described in all cases of HU-induced dermatomyositis; the latter has been seen in a few cases of that disorder.

Granuloma faciale treated with topical dapsone: a case report

Granuloma faciale (GF) is an unusual, treatment-resistant skin disorder that commonly affects the face. Several medical and surgical interventions are available that offer varying degrees of benefit. Both the condition and the treatment modalities can lead to significant disfigurement. The use of oral dapsone in the treatment of GF has been described in the literature, but there are no reports, to our knowledge, of the use of topical dapsone 5% gel (Aczone; Allergan Inc, Irvine, CA). We present a case of a patient with GF on the nasal tip successfully treated with topical dapsone.

Multiple labial melanotic macules occurring after topical application of calcineurin inhibitors

Topical calcineurin inhibitors are widely used to treat inflammatory dermatoses for their steroid-sparing advantage. Herein, we report a patient with chronic lip dermatitis who developed multiple labial melanotic macules after application of tacrolimus 0.1% ointment and pimecrolimus 1% cream. Prior and current reports raise concerns for potential development of pigmented lesions associated with topical calcineurin inhibitor use. These reports highlight the need for careful risk-benefit assessment when prescribing topical calcineurin inhibitors for inflammatory dermatoses, especially when used on sun-exposed sites.

Photo Vignette

Hydroa vacciniforme: a rare photodermatosis

Hydroa vacciniforme (HV) is a rare photodermatosis characterized by a recurrent vesiculopapular eruption with varioliform scarring. The pathogenesis remains unknown. Herein we present a case to emphasize the importance of recognizing this condition and its clinical mimickers, which include other photodermatoses and lymphoma.

Zosteriform collagen nevus in a young boy

Zosterifom connective tissue nevus is a rare kind of connective tissue nevi composed of collagen, elastin, or glycosaminoglycan, which was first reported by Steiner 1944. Herein, we report a young boy with a collagen nevus that presented in a zosteriform distribution.

Self regressing skin-colored papules with acneiform scarring over the face

Lupus miliaris disseminata faciei is a chronic inflammatory dermatosis of unknown etiology, which mostly involves the face in young adults. Earlier attempts had been made to relate it to tuberculosis, rosacea, and sarcoidosis without any strong evidence. For the past few years it has been considered as a distinct entity by various authors and was given a new nomenclature, “facial idiopathic granulomas with regressive evolution” (FIGURE). We also support this new nomenclature and present a young man with facial idiopathic granulomas and scarring consistent with this diagnosis.

Hennekam syndrome: a rare cause of primary lymphedema

Hennekam syndrome (HS) is an autosomal recessive disorder characterized by the association of lymphedema, intestinal lymphangiectasia, moderate mental retardation, and facial dysmorphism. We describe a 14-year-old girl affected with Hennekam syndrome.

Multifocal epithelial hyperplasia: report of 3 cases

Multifocal epithelial hyperplasia (MEH) is a rare disorder characterized by multiple painless discrete and soft flattened papules on the oral mucosa. It is caused by human papilloma virus 13 and 32. The frequency of this disease varies widely from one geographic region to another. Generally it is very rare in Asia. Herein we report 3 Iranian cases with oral lesions, which showed clinical and histopathological characteristics of MEH disease. Two of them were siblings and HPV13 was detected in one of the patients.