Folliculotropic Mycosis Fungoides as a Posttransplant Lymphoproliferative Disorder.

Posttransplant lymphoproliferative disorder (PTLD) is a known complication of solid organ transplantation. The majority are B cell in origin and related to Epstein-Barr virus infection. T-cell PTLD is much less common; most are Epstein-Barr virus negative and have a worse prognosis. Primary cutaneous T-cell lymphoma (CTCL) as a presentation of PTLD is rare. CTCL has a less favorable prognosis in transplant patients compared with that in immune-competent patients. Herein, we report a case of a 13-year-old boy who developed folliculotropic mycosis fungoides, a rare subtype of CTCL, subsequent to renal transplantation. To our knowledge, this is the first report of this type of PTLD in a pediatric patient.

The skin and HIV: no superficial matter.

The vast majority of HIV-infected patients experience some type of skin disorder; these may broadly be categorized as infectious, neoplastic, or inflammatory. Additionally, primary pruritus afflicts a considerable percentage of HIV-infected individuals, and an attempt should be made to identify potential underlying triggers. Chronic itch, whether related to an underlying cutaneous, systemic, or psychiatric illness, can have a profound effect on quality of life. Therapy for inflammatory skin disorders may involve initiation of antiretroviral therapy in those who have not yet started such treatment, oral antihistamines, topical corticosteroids, topical antipruritic agents, and skin moisturizers. Because topical corticosteroids are often a necessary component of the therapeutic armamentarium for skin diseases, practitioners are encouraged to become familiar with the appropriate indications, strengths, and formulations of available preparations. In some instances, psychiatric medications or phototherapy may be necessary for the treatment of HIV-associated skin disorders, particularly for patients experiencing refractory itch. Although psoriasis is not more frequent among HIV-infected patients than in the general population, it can be more severe and debilitating for those who are HIV infected. Our understanding of psoriasis in the setting of HIV infection has evolved and new therapies for psoriasis have recently become available. This article summarizes a presentation by Sareeta R. S. Parker, MD, at the IAS-USA continuing education program held in Atlanta, Georgia, in April 2014.

Is non-alcoholic steatohepatitis a predisposing factor to porphyria cutanea tarda?

Porphyria cutanea tarda (PCT) is a disease caused by a deficiency of the fifth enzyme of the heme biosynthetic pathway in the liver that manifests in the skin as blistering and fragility of predominantly sun-exposed skin. It occurs in individuals with environmental and/or genetic risk factors such as estrogen use, hepatitis C infection and hemochromatosis gene mutations. This report highlights a case of PCT which manifested in an individual with non-alcoholic fatty liver disease (non-alcoholic steatohepatitis; NASH). We propose that NASH may have been a contributing factor for the development of PCT in our patient.

The head, neck, and systemic manifestations of levamisole-adulterated cocaine use.

Systemic complications of levamisole-adulterated cocaine (LAC) use have recently been described. The objective of this review is to increase awareness of these manifestations among oral and maxillofacial surgeons. LAC exposure through inhalation, nasal insufflation, or injection can induce cutaneous vasculopathy and hematologic abnormalities such as neutropenia or agranulocytosis. Unlike other vasculopathies involving the skin, LAC-induced vascular injury frequently manifests with purpuric and necrotic lesions that involve the face and ears. Oral manifestations have also been reported but are not yet well characterized. The aforementioned hematologic manifestations are not uncommon, and patients exposed to LAC are potentially at higher risk for infectious complications. When manifestations of LAC affect the head, neck, and oral cavity, oral and maxillofacial surgeons may be the first providers to encounter the patient. Early recognition of the clinical signs and laboratory abnormalities will better allow for distinguishing LAC-related effects from various clinical mimics, will facilitate appropriate patient management, and may further contribute to the understanding of the biological effects of LAC.

Acitretin for the treatment of cutaneous T-cell lymphoma.

BACKGROUND: Bexarotene is the only Food and Drug Administration-approved retinoid for the treatment of cutaneous T-cell lymphoma (CTCL) and is associated with a relatively high frequency of adverse effects. Acitretin has anecdotally been reported to be effective for CTCL. OBJECTIVE: We sought to determine the effectiveness and tolerability of acitretin as primary or adjuvant therapy for CTCL. METHODS: We conducted a retrospective chart review of patients with CTCL treated with acitretin at a single tertiary care center. RESULTS: A total of 32 patients with CTCL were included: 29 had mycosis fungoides, 2 had Sézary syndrome, and 1 had CTCL not otherwise specified. Median patient age was 55 years; 56% were male; 47% were white, 47% black, and 6% other. In all, 3% of patients were stage IA, 69% stage IB/IIA, 16% stage IIB, 6% stage III, and 6% stage IV. Six patients received acitretin alone; 26 received acitretin in addition to another CTCL therapy. The overall response rate was 59%. In all, 25% of patients had stable disease and 16% had progressive disease. Median duration of response was 28 months. Adverse effects were generally mild with 5 patients discontinuing therapy because of these. LIMITATIONS: In this small retrospective chart review, many patients were on other CTCL therapies while on acitretin; therefore precise assessment of response to acitretin alone was difficult. CONCLUSIONS: Acitretin is well tolerated and potentially effective for early-stage CTCL. Response to acitretin, either as adjuvant therapy monotherapy, is comparable with the response to oral agents currently approved for this disease.

Hodgkin lymphoma presenting as generalized pruritus in an adolescent.

Pruritus is a common manifestation of Hodgkin lymphoma (HL), and given its high frequency, inclusion of itching as a B symptom of HL has been proposed. We present a 16-year-old adolescent boy with treatment-refractory eczema of 2 years' duration. Physical examination revealed a thin adolescent boy with widespread excoriations, but no eczematous or primary cutaneous lesions were identifiable. Lymph node examination revealed palpably enlarged nodes in the cervical and supraclavicular regions. Laboratory studies revealed leukocytosis and an elevated lactate dehydrogenase level. Diffuse lymphadenopathy was detected on a chest radiograph, and excisional lymph node biopsy revealed HL (nodular sclerosing subtype). The patient was classified as HL stage IIIB (Ann Arbor staging classification) after further evaluation. Chemotherapy was initiated followed by radiation therapy. The patient's pruritus markedly improved within 2 cycles of chemotherapy; however, his HL relapsed and additional salvage combination chemotherapy followed by high-dose chemotherapy and autologous stem cell transplant were required. This case underscores the need for a complete history as well as a careful skin and systemic evaluation in patients presenting with long-term pruritus, including children and adolescents.

Autoimmune blistering diseases in the elderly.

Autoimmune blistering diseases are a significant cause of morbidity and mortality in the elderly population. Given the advancing age of the population, the incidence of these disorders, particularly bullous pemphigoid, is expected to rise. This contribution reviews autoimmune immunobullous disorders of particular relevance in the elderly population. These include bullous pemphigoid, cicatricial pemphigoid, epidermolysis bullosa acquisita, pemphigus, paraneoplastic pemphigus, and linear immunoglobulin A bullous dermatosis. Because therapy and management of individual immunobullous dermatoses differ, establishing the diagnosis is often critically important. An overall approach to bullous diseases in the elderly, as well as key clinical features, appropriate diagnostic tests, microscopic findings, immunofluorescence microscopy patterns, and molecular targets for select disorders are reviewed. Elucidation of antigenic targets at the molecular level has allowed for development of serum enzyme-linked immunofluorescence assays, which have enhanced diagnostic accuracy for several autoimmune blistering disorders. Given the relative rarity of these diseases, large randomized trials evaluating efficacy of various treatments are few, and therapy for most immunobullous disorders in the elderly has not been standardized. Despite this, appropriate therapeutic considerations for each condition are presented and the evidence for them is reviewed.

Ulcerative granulomatous mycosis fungoides.

A 42-year-old white male military recruit presented with a 2-year history of painful ulcerations on the skin of his flanks and thighs. Prior skin biopsies were nondiagnostic but raised the suspicion of an infectious etiology due to the presence ofa granulomatous infiltrate. His medical history was significant for herpes zoster and eczema, and, on review of systems, he had a 1-year history of progressive fatigue and night sweats. Examination revealed approximately one dozen 1- to 5-cm indurated, dusky violaceous plaques on his trunk and lower extremities. Several of the plaques, including one on his right flank, had overlying deep ulcerations (Figure 1A and 1B). Bilateral inguinal lymphadenopathy was present.

Follicular mucinosis: clinical, histologic, and molecular remission with minocycline.

Follicular mucinosis is an uncommon inflammatory disorder characterized histologically by mucin accumulation in the follicular epithelium. The condition is generally divided into primary and secondary forms, the latter being frequently associated with mycosis fungoides. Lesional skin T-cell clonality has been documented in some patients with follicular mucinosis, even those with no histologic evidence of cutaneous lymphoma. In this report, we describe a patient with clonal idiopathic primary follicular mucinosis who had complete clinical, histologic, and molecular remission with minocycline therapy.

Leishmania tropica-induced cutaneous and presumptive concomitant viscerotropic leishmaniasis with prolonged incubation.

BACKGROUND: Leishmaniasis includes a spectrum of diseases caused by protozoan parasites belonging to the genus Leishmania. The disease is traditionally classified into visceral, cutaneous, or mucocutaneous leishmaniasis, depending on clinical characteristics as well as the species involved. Leishmania tropica is one of the causative agents of cutaneous leishmaniasis, with a typical incubation period of weeks to months. Observation We describe a 17-year-old Afghani girl who had lived in the United States for 4 years and who presented with a 6-month history of pretibial ulcerations, 9.1-kg weight loss, abdominal pain, splenomegaly, and extreme fatigue. Histopathologic examination and culture with isoenzyme electrophoresis speciation of her skin lesions confirmed the presence of L tropica. In addition, results of serum laboratory and serological studies were highly suggestive of concomitant visceral involvement. The patient was treated with a 28-day course of intravenous pentavalent antimonial compound sodium stibogluconate with complete resolution of her systemic signs and symptoms and improvement of her pretibial ulcerations. CONCLUSIONS: This is an exceptional case in that our patient presented with disease after an incubation period of years rather than the more typical weeks to months. In addition, this patient had confirmed cutaneous involvement, as well as strong evidence of viscerotropic disease caused by L tropica, a species that characteristically displays dermotropism, not viscerotropism.

Mortality of bullous pemphigoid: an evaluation of 223 patients and comparison with the mortality in the general population in the United States.

BACKGROUND: There are large discrepancies in reported mortality for bullous pemphigoid (BP). OBJECTIVE: We sought to determine the mortality of a large cohort of patients with BP and compare this with age-matched control subjects. METHODS: Data were collected on 223 patients with a new diagnosis of BP between 1998 and 2003 through our cutaneous immunofluorescence laboratory databases. The mortality of patients with BP was compared with that of age-matched control subjects in the general US population. RESULTS: The 1-, 2-, and 5-year mortality was 0.23 (95% confidence interval=0.18, 0.29), 0.37 (95% confidence interval=0.31, 0.44), and 0.50 (95% confidence interval=0.42, 0.57), respectively. However, relative to age-matched control subjects, no difference in expected mortality was detected. LIMITATIONS: This was a retrospective cohort analysis. CONCLUSIONS: Mortality of patients with BP is more likely related to advanced age and associated medical conditions than to disease-specific factors.

A report of Epstein-Barr virus-positive primary cutaneous natural killer-/T-cell lymphoma.

We describe a patient who presented with Epstein-Barr virus-positive tumor-stage primary cutaneous lymphoma. Our patient had previously been treated with oral methotrexate for long-standing rheumatoid arthritis. Tissue analysis revealed large tumor cells that were surface CD2- and CD3-positive; T-cell-restricted intracellular antigen-positive; CD56-, CD20-, and CD30-negative; and stained positively for Epstein-Barr virus. Our case is noteworthy for several reasons. Although the presence of rheumatoid arthritis and therapy with methotrexate are putative risk factors for the development of immune suppression-related and Epstein-Barr virus-related lymphomas, the vast majority of lymphomas in this setting are of B-cell origin, and rarely are these primary cutaneous in nature. In addition, our patient's tumor displayed an unusual phenotype, with immunophenotypic features suggestive of an atypical natural killer-/T-cell lymphoma. Methotrexate was withdrawn, and our patient was successfully treated with local radiotherapy. She has remained in complete remission 28 months since diagnosis.

Intravenous cidofovir-induced resolution of disfiguring cutaneous human papillomavirus infection.

Human papillomavirus infection is one of the most common and most distressing cutaneous diseases in patients with HIV infection. It is also a common, and often therapeutically challenging, infection in individuals who are immunologically competent. A wide range of therapeutic options exists for treating cutaneous human papillomavirus infections, but none is uniformly effective. In this report we describe a man with HIV-1 infection and disfiguring facial verruca vulgaris who demonstrated complete clinical response to intravenous cidofovir. Our report provides further support for the use of intravenous cidofovir as therapy for treatment-resistant and/or widespread cutaneous human papillomavirus infection.

Eosinophilic folliculitis in HIV-infected women: case series and review.

BACKGROUND AND OBJECTIVE: Dermatologic conditions are often presenting signs of HIV infection and may be the sole cause of morbidity in patients who have otherwise stable HIV disease. Eosinophilic folliculitis is a pruritic, follicular eruption that typically manifests late in the course of HIV infection. Most published reports of eosinophilic folliculitis have been in HIV-infected men. In those reports, a characteristic truncal distribution was present, with involvement of the head, neck, and upper extremities commonly seen as well. The objective of this study was to better characterize the presentation of eosinophilic folliculitis in women. METHODS: We conducted a retrospective chart review of six HIV-seropositive women with eosinophilic folliculitis previously seen in our dermatology clinics. We also reviewed the literature for cases of eosinophilic folliculitis in women and for clinical and therapeutic aspects of the condition, particularly in women. RESULTS: In our case series, we found that eosinophilic folliculitis in women may predominantly affect the face and mimic acne excoriée. A review of the literature of HIV-associated eosinophilic folliculitis in women supports these findings. Regarding treatment, many therapies are available, but none is uniformly effective. CONCLUSION: Given the dramatic rise in the incidence of HIV infection in women, who now represent nearly 50% of adults living worldwide with HIV/AIDS, a heightened awareness of HIV-related dermatoses in women is essential. HIV-associated eosinophilic folliculitis should be considered in the differential diagnosis of chronic, pruritic, papular facial eruptions in females.

Treatment of cutaneous T-cell lymphoma/mycosis fungoides.

The cause of mycosis fungoides is unknown and, with the possible exception of very early stage disease, no cure is available. Fortunately, patients with MF have a number of therapeutic options and partial and complete remissions are achievable. Because it is not curable, the burden for patients with this disease involves the need for lifelong therapy and monitoring, and meticulous skin care. Despite its indolent nature in most individuals, the disease has a tremendous psychological impact, not only because of the visible nature of the skin lesions, but also due to the rarity of the disease and its chronicity. Knowledge of this disease, therapeutic options, and expectations of therapy will enhance care of patients afflicted with mycosis fungoides. Ongoing research provides hope that in the future, therapy to induce long-lasting remission, or even cure, will become available. Since the submission of this manuscript, vorinostat (Zolinza), an orally administered histone inhibitor, has been FDA approved for treating skin manifestations in patients with CTCL.