Lauren C. Hughey

Trichodysplasia of immunosuppression treated with oral valganciclovir

Trichodysplasia of immunosuppression (TOI) is a newly described rare clinicopathologic entity usually found in transplant patients on chronic immunosuppressive medications or with hematologic malignancies. TOI consists of a cutaneous eruption of spiny papules predominantly affecting the face and arms. The distinct histopathologic picture includes abnormally maturing anagen follicles with hyperkeratotic infundibula and excessive inner root sheath differentiation. Given that intranuclear viral particles have been noted on electron microscopy of skin biopsy in several patients, a viral etiology is suspected. These viral particles are similar in shape and size to a polyoma virus, however, the virus has not been identified to date. There are reports of improvement of TOI with topical cidofovir or tazarotene 0.5% gel, but to our knowledge there has not been a report of near-complete resolution with oral valganciclovir. We report a case of TOI in a heart transplant patient on a chronic immunosuppressive regimen of sirolimus, mycophenolic acid and prednisone that demonstrated almost complete response to treatment with systemic valganciclovir.

Fulminant myocarditis as a late sequela of DRESS: Two cases

Greg P. Bourgeois, MD1, Jennifer A. Cafardi, MD1, Vlada Groysman, MD1, Salpy V. Pamboukian, MD MSPH3, James K. Kirklin, MD4, Aleodor A. Andea, MD2, and Lauren C. Hughey, MD1 1Department of Dermatology, University of Alabama at Birmingham, EFH 414, 1530 3rd Ave S., Birmingham, AL 35294, USA 2Department of Pathology, University of Alabama at Birmingham, PD6A 149, 619 19th St S., Birmingham, AL35294 USA 3Department of Cardiology, University of Alabama at Birmingham, THT 321, 1530 3rd Ave S., Birmingham, AL 35294, USA 4Department of Cardiothoracic Surgery, University of Alabama at Birmingham, THT 760, 1530 3rd Ave S., Birmingham, AL 35294, USA

Recognizing large-cell transformation of mycosis fungoides

BACKGROUND Although patients with mycosis fungoides (MF) typically experience an indolent disease course, a minority undergo a process of large-cell transformation (LCT), which often heralds more aggressive disease and shortened survival. Regrettably, most dermatologists are unfamiliar with LCT, and even fewer understand how to recognize it clinically. Because a diagnosis of LCT typically triggers more aggressive therapy and/or referral to cutaneous T-cell lymphoma (CTCL) centers, it is paramount for clinicians to be able to recognize suspect lesions visually. OBJECTIVE LCT is diagnosed histologically; however, diagnostic biopsy is performed only if transformed lesions are suspected clinically. Because the literature provides little information on what clinical features should lead to suspicion of LCT, we sought to identify and categorize the presentations of LCT to aid in its recognition. METHODS We identified 14 patients with biopsy-proven LCT confirmed by a board-certified dermatopathologist experienced with this diagnosis. The clinical presentations of LCT, timing of its evolution, and treatment regimens were evaluated by chart and photograph review. RESULTS We devised 3 categories that clinically represent LCT: (1) LCT occurring as a new, solitary nodule within a classic MF patch or plaque, (2) LCT occurring as abrupt onset of multiple scattered papules and/or nodules without spontaneous resolution, and (3) LCT occurring within new or enlarging tumors. LIMITATIONS A larger number of reviewed cases might reveal additional clinical presentations of LCT. CONCLUSIONS Dermatologists may use our categories of clinical indicators to recognize and diagnose LCT earlier, allowing implementation of more aggressive treatment regimens when appropriate.

Ultrastructural and molecular confirmation of the trichodysplasia spinulosa-associated polyomavirus in biopsies of patients with trichodysplasia spinulosa.

Trichodysplasia spinulosa (TS) is a rare and only recently characterized cutaneous disease occurring in immunocompromised patients. The disease is characterized by spiny follicular papules on clinical examination and by the presence of viral inclusions at ultrastructural examination. In the last year, this virus has been identified as a new member of the polyomavirus family and designated as TS‐associated polyomavirus (TSPyV). We report two organ transplant patients with this disease in which we were able to identify the TSPyV at ultrastructural and molecular level from formalin‐fixed paraffin‐embedded biopsies of lesional skin. Similar to prior described cases, the patients presented with follicular papules which were concentrated on the central face and associated with alopecia. Histopathology of both cases showed dilated follicular infundibula plugged with cornified eosinophilic cells containing large trichohyaline granules. Transmission electron microscopy on paraffin‐embedded tissue in case 1 showed 28‐nm intracellular viral particles morphologically consistent with polyoma virus. For both cases the presence of TSPyV was confirmed by polymerase chain reaction with virus‐specific primers followed by identification by direct sequencing. These two cases show the presence of the newly described TSPyV in TS further establishing its association with this distinctive disease.

Approach to the hospitalized patient with targetoid lesions

Approaching the hospitalized patient with skin disease can be daunting. This article focuses on a practical approach to the patient with targetoid lesions. The discussion focuses on differentiating erythema multiforme from Stevens–Johnson syndrome and toxic epidermal necrolysis. In addition, the article offers a concise review of the broader differential diagnosis of targetoid lesions including ecthyma gangrenosum, fixed drug eruption, erythema multiforme‐like drug reaction, vasculitis, acute hemorrhagic edema of infancy, erythema chronicum migrans, connective tissue diseases, and blistering diseases.

Solitary plaque on the scalp as a primary manifestation of Hodgkin lymphoma: a case report and review of the literature

Cutaneous Hodgkin lymphoma is infrequent and typically occurs after extensive involvement of the lymph nodes. The condition decreased significantly in incidence in the past two decades, likely owing to the new treatment protocols composed of chemotherapy, radiotherapy and stem cell transplantation. Nevertheless, recognition of this uncommon but significant disease manifestation is important from a prognostic and therapeutic perspective. We are sharing a recent case of Hodgkin lymphoma where the primary presentation appeared as a solitary plaque on the left side of the occipital scalp, clinically suspected to represent a ruptured follicular cyst. The patient underwent excisional biopsy. Histological assessment revealed Hodgkin lymphoma affecting the skin. Radiological studies showed no regional lymphadenopathy. However, two enlarged lymph nodes were identified in the mediastinum and were positron emission tomography avid. The patient underwent systemic treatment without further histopathological examination of these two lymph nodes. Not being clear if these enlarged two lymph nodes were related to his cutaneous disease or not, we cannot be sure if the patient was afflicted either by primary cutaneous Hodgkin lymphoma or by secondary cutaneous involvement because of hematogenous spread. In either case, primary or secondary cutaneous Hodgkin disease is an extreme rarity. The literature is critically reviewed.

Association of Dermatology Consultation With Accuracy of Cutaneous Disorder Diagnoses in Hospitalized Patients: A Multicenter Analysis

topical lidocaine cream, 4%, is typically used to produce anesthesia 60 minutes after application.4,6 Our results suggest that the use of microneedle pretreatment can reduce the incubation time to 30 minutes—perhaps even to 10 minutes in more pain-sensitive individuals—to achieve sufficient anesthesia for needle insertion. Our study has several limitations. First, the pain stimulus was needle based and it is not known how laseror scalpelbased pain would have been altered. Second, our study was limited to men; future studies should include women. Finally, this study evaluated pain on the volar forearms, but pain sensitivity can vary by anatomical site. Because the participants served as their own controls and the anatomical site at each pain assessment had a symmetrically placed control, the role of the site was minimized. The study should be repeated using other anatomical sites. Randomized clinical trials should be conducted with laseror scalpel-based stimuli to simulate other dermatologic procedures. In particular, studies in a pediatric population can help clarify the role of microneedle pretreatment in topical anesthesia before invasive procedures. Regardless, the findings of this studyarepromising,showingthatmicroneedlesofferaminimally painful method of enhancing the effects of topical anesthesia.

Rickettsia parkeri: eschar diagnosis.

lymphomas after TNF-alpha blockade has been reported. TNF-alpha blockade likely inhibits both innate and acquired immunity and may compromise immune surveillance for malignancy. The relation between our patient’s dermatitis and her Berti lymphoma is unclear. Her dermatitis may have represented a smoldering CTCL that transformed into Berti lymphoma, or the latter may have developed anew in the setting of immunosuppression. This case suggests that corticosteroid-refractory, histologically nonspecific dermatitis should be evaluated carefully and managed cautiously. Not all psoriasiform dermatitis is psoriasis or is safely treated as such. A high suspicion for possible CTCL and a low threshold for repeat biopsies to establish a diagnosis should be maintained when considering immunomodulatory therapy.

Practical Management of CD30⁺ Lymphoproliferative Disorders.

Primary cutaneous CD30⁺ lymphoproliferative disorders (LPDs) account for approximately 25% of cutaneous lymphomas. Although these LPDs are clinically heterogeneous, they can be indistinguishable histologically. Lymphomatoid papulosis rarely requires systemic treatment; however, multifocal primary cutaneous anaplastic large cell cutaneous lymphoma and large cell transformation of mycosis fungoides are typically treated systemically. As CD30⁺ LPDs are rare, there is little published evidence to support a specific treatment algorithm. Most studies are case reports, small case series, or retrospective reviews. This article discusses various treatment choices for each of the CD30⁺ disorders and offers practical pearls to aid in choosing an appropriate regimen.

Concomitant Mycosis Fungoides and Vitiligo: How Mycosis Fungoides May Contribute to Melanocyte Destruction

Background: Few reports have described vitiligo developing in patients with cutaneous T-cell lymphoma (CTCL). Objective: We sought to identify possible factors that might predispose patients with CTCL to vitiligo. Methods: Patient demographics, CTCL disease characteristics and treatments were analyzed in 25 patients with CTCL who developed vitiligo. Cox proportional hazards modeling was used to identify associations of risk factors with the development of vitiligo. Results: Younger age, later CTCL disease stage (stages IIB-IV) and presence of a CD8+CD4- mycosis fungoides phenotype were associated with the development of vitiligo. After adjusting for disease stage, increased risk of vitiligo was associated with methotrexate and CD4 antibody therapies (although the total number of patients with these was small), while decreased risk was associated with nitrogen mustard and PUVA therapies. Conclusions: No single feature was common to all of our patients, suggesting that multiple factors may contribute to the development of vitiligo in a patient-specific fashion.