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Open Access Publications from the University of California

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, and by the Northern California Veterans Administration.

Volume 21, Issue 7, 2015

Original

Psoriasis disease severity affects patient satisfaction with treatment

Background: Patients with psoriasis are often dissatisfied with available treatments, but contributing factors are not well defined.

Objective: Examine relationships between psoriasis severity, patient characteristics, and treatment satisfaction.

Methods: Patients with psoriasis were classified into mild and moderate-to-severe groups based on self-reported data. Demographics, comorbidities, symptoms, and multiple treatment satisfaction outcomes were compared between groups. Predictors of patient satisfaction with treatment were examined using linear regression models.

Results: The analyses included 773 patients (407 mild; 366 moderate-to-severe). The percentage of patients reporting satisfaction with treatment was low overall, ranging from 8.6% to 61.7% for the mild and 13.9% to 49.5% for the moderate-to-severe group. Satisfaction among biologics users was also low (≤53%; 50% of satisfaction rates <40%). Regression results consistently showed greater dissatisfaction with current treatment among moderately to severely affected patients.

Conclusion: Many psoriasis patients were dissatisfied with their treatment; moderate-to-severe patients expressed significantly less satisfaction than mild patients.

Review

Beta blocker treatment for infantile hemangiomas

Infantile hemangiomas (IH) are common childhood vascular tumors.  Treatment of IH has undergone rapid change in recent years.  Since 2008, oral propranolol has been used to treat complicated IH and has proven superior to previously used therapies.  More recently, the efficacy of other systemic beta blockers, specifically atenolol and nadolol, has been reported.  In addition, topical timolol solution has been effective for treatment of smaller, more superficial IH.  The purpose of this article is to review the current literature of beta-blocker therapy for IH.

Commentary

Successful treatment of psoriasis with ustekinumab in patients with multiple sclerosis

Psoriasis is a chronic inflammatory disease, evolving from a complex interplay of genetic and environmental factors. In the recent years, we have seen much progress in understanding the immunopathogenesis of psoriasis, paving the way for new therapies with biologics. Currently, the most commonly used biologics in psoriasis are TNF inhibitors etanercept, infliximab and adalimumab, and the IL-12/23 inhibitor ustekinumab. As TNF inhibitors are contraindicated in patients with multiple sclerosis, ustekinumab remained the only biologic available for these patient before the recent approval of Secukinumab, an IL-17A inhibitor. Herein we report two patients with multiple sclerosis and comorbid psoriasis successfully treated with ustekinumab without progression of their multiple sclerosis. Our cases demonstrate that ustekinumab is a reasonably safe choice in this patient population. We also briefly reviewed new therapies currently under investigation, which will undoubtedly further expand our armamentarium for the treatment of psoriasis in patients with neuromuscular diseases.

Case Report

Acquired cutis laxa associated with cutaneous mastocytosis

Cutis laxa is characterized by dramatic wrinkling of skin that is lacking in elasticity due to inherent defects in dermal elastic fibers. Cutis laxa can be caused by genetic and metabolic disorders. It can also be acquired, possibly resulting from inflammatory processes with destruction of elastic fibers. This report describes a 26-year old woman who developed acquired cutis laxa and cutaneous mastocytosis leading to premature aging. She represents a unique co-occurrence of these two separate disease entities. To our knowledge, there has been only one published case report of acquired cutis laxa occurring in association with urticaria pigmentosa in a 4-year old girl. Our case would be a second case that exhibits the coexistence of these two disorders in an adult female.

Alopecia as the Presenting Symptom of Syphilis

Alopecia can be one of the many symptoms of secondary syphilis and the clinical presentations include essential syphilitic alopecia or symptomatic syphilitic alopecia. In this report, we present a case of a patient with essential syphilitic alopecia whose sole presenting symptom of syphilis was alopecia. Despite an initial negative rapid plasma reagin (RPR) test, he was ultimately found to have syphilis on scalp biopsy. His alopecia improved following treatment with benzathine penicillin. This presentation serves as a reminder to clinicians to be cognizant of alopecia as a presenting sign of syphilis. A review of the specificity and sensitivity of the typical tests used for the diagnosis is presented.

Elevated levels of antibodies against phosphatidylserine/prothrombin complex and/or cardiolipin associated with infection and recurrent purpura in a child: a forme fruste of antiphospholipid syndrome?

Antiphospholipid syndrome is an autoimmune disorder characterized by the occurrence of venous and arterial thrombosis, as well as morbidity in pregnancy, in the presence of anti-phospholipid antibodies. The diagnosis of antiphospholipid syndrome is usually established based on clinical and laboratory findings by strictly following the 2006 Sapporo classification. However, the diagnosis remains challenging owing to the ongoing debates on the serological criteria. We report a case we describe as forme fruste antiphospholipid syndrome in which these criteria were not fulfilled. Purpura appeared repeatedly in a female infant starting from the age of 6 months and following episodes of upper respiratory infections and vaccinations. The levels of anti-cardiolipin IgG antibodies and anti-phosphatidylserine/prothrombin complex antibodies were elevated in accordance with these events. Histopathological evaluation revealed multiple small vessel thrombi in the dermis and adipose tissue. After 2 weeks of treatment with aspirin and heparin, the cutaneous symptoms subsided. Infection has long been associated with antiphospholipid syndrome, and anti-phosphatidylserine/prothrombin antibodies are considered a new marker for the diagnosis of antiphospholipid syndrome. Forme fruste antiphospholipid syndrome should be considered even if the antiphospholipid syndrome diagnostic criteria are not completely fulfilled, especially in the presence of elevated levels of anti-phosphatidylserine/prothrombin antibodies and known preceding infections.

Case Presentation

A case of hair re-pigmentation from a scalp melanoma

Hair re-pigmentation in adults is a rare phenomenon. We describe a 58-year-old woman who developed hair re-pigmentation on her vertex scalp as a marker of underlying melanoma. Histopathology revealed a nodular melanoma that was surrounding but not invading follicular epithelium. To our knowledge, there have only been 4 other previously published cases describing hair re-pigmentation in the setting of scalp melanoma. Focal hair re-pigmentation in adults should prompt a thorough evaluation for an underlying melanoma.

Linear acral pseudolymphomatous angiokeratoma of children with associated nail dystrophy

Acral pseudolymphomatous angiokeratoma of children (APACHE) is a rare entity that typically occurs on the extremities of young females. Although linear arrangement of cutaneous lesions has been rarely reported, accompanying nail dystrophy has not been linked with this condition to our knowledge. We describe a case of linearly-oriented infiltrative papules and nodules on the index finger of a young female with associated onychodystrophy. Histology demonstrated a heavy lymphocytic infiltrate with plasma cells and proliferation of blood vessels consistent with APACHE. Our case is unique given the linear array of cutaneous lesions and associated nail dystrophy.

“Cyrano nose” associated with hepatic hemangiomas successfully treated with propranolol

Hemangioma of the nasal tip is commonly described as “Cyrano nose” and his treatment is extremely difficult because of its location and possible severe aesthetic complications like definitive nasal deformation. We describe a patient who presented at two months of age with a “Cyrano nose” associated with multiple hepatic and cutaneous hemangiomas, which completely resolved after therapy with propranolol. Treatment was well tolerated and aesthetic result was excellent.

Blue rubber bleb nevus syndrome: a rare multisystem affliction

About 200 cases of blue rubber bleb nevus syndrome (BRBNS) have been reported in the literature. The disorder affects both sexes equally and the occurrence is mostly sporadic except for a few reports of cases with autosomal dominant inheritance pattern. Herein we report an 11-year-old girl with progressive BRBNS and onset at 5 years of age.

A case of acanthosis nigricans coexisting with mycosis fungoides

Acanthosis nigricans maligna (ANM) is a paraneoplastic phenomenon most commonly associated with visceral adenocarcinoma, in particular, gastric adenocarcinoma. Clinically, AMN is characterized by rapidly progressive symmetrical skin thickening and hyperpigmentation of the intertriginous areas with peripheral acrochorda. The diagnosis is made by a detailed medical work-up for occult malignancies with particular emphasis on endocrinological diseases. We report a 67-year-old man that presented clinically with acanthosis nigricans, in which a subsequent diagnosis of mycosis fungoides was made.

Síndrome de Graham-Little-Piccardi-Lassueur: variante disseminada de líquen plano folicular

Graham-Little-Piccardi-Lassueur syndrome is a rare lichenoid dermatosis. It is characterized by the triad of scarring alopecia of the scalp, alopecia of the axilla and or groin, and keratotic follicular papules of the body. The present paper reports on two cases affecting young women. Histopathological findings suggest the disorder represents a generalized form of lichen planus follicularis.

Histiocytoid Sweet syndrome treated with azathioprine: a case report

Histiocytoid Sweet syndrome (HSS) is a rare histologic variation of Sweet syndrome (SS) predominantly exhibiting mononuclear histiocytoid cells instead of neutrophils. We report a 22-year-old woman with HSS, who, after minimal improvement with colchicine and dapsone, had significant improvement of her cutaneous eruption and systemic symptoms following empiric treatment with azathioprine. Since azathioprine has historically been known to cause SS, this case highlights a previously unreported treatment response for the histiocytoid variant.

A fibrous papule with abundant CD34-immunoreactive ganglion-like multinucleated giant cells: a case report and review of the literature

Fibrous papules present clinically as benign, asymptomatic, dome-shaped, flesh colored papules on the face. Histologically, fibrous papules are characterized by fibrous stroma with fibroblasts and dilated blood vessels. Multiple variants of fibrous papules have been reported. Although scattered multinucleated cells in fibrous papules have been well described, we report a fibrous papule with abundant multinucleated ganglion-like giant cells that were immunoreactive with CD34. Recognition of such fibrous papule variants is important to avoid misdiagnosis as potentially more worrisome and/or aggressive melanocytic, soft tissue, or neural lesions that may require more aggressive treatment. Indeed, fibrous papules do not commonly appear on the differential diagnosis for lesions with multinucleated giant cells or ganglion-like cells and consideration should be given to their inclusion in the appropriate clinical setting.

Photo Vignette

Jejunal adenocarcinoma with cutaneous metastasis

Small bowel adenocarcinoma (SBA) is a rare primary gastrointestinal malignancy. We present a 60-year old man who developed a cutaneous metastasis of jejunal adenocarcinoma to his neck. This case highlights the clinicopathologic and immunophenotypic features of this uncommon cutaneous metastasis.

Unilateral hyperkeratotic plaques along blaschko lines

Epidermolytic ichthyosis (or epidermolytic hyperkeratosis) classically presents with erythroderma and increased fragility (blistering) at birth or soon thereafter. In later life, erythroderma and blistering improve gradually and the clinical picture is dominated by hyperkeratotic plaques in flexures and around joints. Linear epidermolytic hyperkeratosis is a unique, uncommon clinical variant and the absence of erythroderma and blistering are its hallmark. Linear lesions may be localized or generalized andunilateral or bilateral. Herein we report a 6-year-old girl with unilateral epidermolytic ichthyosis.

Letter

Pitiríase rotunda: uma apresentação incomum

Pityriasis rotunda disorder of keratinization clinically described as persistent, large, sharply defined circular patches of ichthyosiform scaling with no inflammatory changes. Patients with pityriasis rotunda may be classified into one of two groups, which are based on ethnicity, number of lesions, family history, and association with systemic diseases. A 45-year-old woman presented with asymptomatic, sharply-demarcated, round, scaly, hyperpigmented lesions on her leg that had been present for several years. Our patient did not have systemic disease or malignancy and so does not fit into groups already described.

Early diagnosis of subungual squamous cell carcinoma of the hallux

Primary subungual squamous cell carcinoma (SCC) of the toe is rare in the literature and frequently late or misdiagnosed. Lack of awareness among physicians of the specific clinical findings, the indolent course of the disease, and the high prevalence of benign conditions of the nail unit may account for delays in diagnosis. In addition to difficulties in identifying the true incidence of this tumor, a standardized therapeutic approach does not currently exist. We present a case of subungual SCC of the first toe without bone invasion. The tumor was diagnosed early, treated with conservative surgical excision, and showed no recurrence or metastasis after 2 years of follow-up.