Vincent Liu

Mycophenolate in dermatology

Originally used to treat psoriasis nearly three decades ago, mycophenolic acid, reformulated as mycophenolate mofetil (MMF), has been rediscovered by the world of dermatology. As a relatively well‐tolerated immunosuppressive used in organ transplant recipients, MMF has recently been reported to show promise for several dermatologic conditions, including psoriasis, pemphigus vulgaris, pyoderma gangrenosum, bullous lichen planus, and even connective tissue diseases such as lupus erythematosus and dermatomyositis. Although not intended to be exhaustive, this review discusses MMF with regard to its basic pharmacology, its side effects, and its reported efficacy in a variety of dermatologic indications. Relevant literature was retrieved by a Medline search combining the terms ‘mycophenolate’ or ‘mycophenolic acid’ and ‘skin’ or ‘skin disease’ or a number of specific conditions (‘psoriasis’, ‘dermatitis’, ‘eczema’, ‘pemphigoid’, ‘pemphigus’, ‘vasculitis’, ‘pyoderma gangrenosum’, ‘Crohns disease’, ‘graft‐versus‐host disease’, ‘lichen planus’). As MMF has only been recently re‐introduced for dermatologic application, the nature of much of the literature is admittedly that of case reports or case series. Nevertheless, the results are sufficiently promising to warrant further larger, control studies.

Reappraisal of the provisional entity primary cutaneous CD4+ small/medium pleomorphic T-cell lymphoma: A series of 10 adult and pediatric patients and review of the literature

BACKGROUND Primary cutaneous CD4(+) small/medium-sized pleomorphic T-cell lymphoma (PCSM-TCL) was defined as a provisional entity in the 2005 World Health Organization-European Organization for Research and Treatment of Cancer classification of cutaneous lymphomas. A limited number of reports describe a generally indolent but heterogeneous condition. OBJECTIVE We reviewed the concept of PCSM-TCL when applied to our experience and to the published literature. METHODS We conducted a retrospective chart review and a PubMed search to identify all reported cases of PCSM-TCL from 2005 to 2010. RESULTS Ten patients, including 4 of the youngest described, were identified in our institution, and their clinical and pathologic features were analyzed. All had a benign clinical course. Ten reports of patients with PCSM-TCL were reviewed and commonalities were found within an otherwise variable spectrum of clinical presentation, pathology, and biologic behavior. LIMITATIONS This study was retrospective, follow-up was short term in some cases, and data were limited in a number of published reports. CONCLUSIONS Our experience of 10 cases that met the diagnostic criteria of the provisional entity PCSM-TCL highlights its occurrence in children and further supports characterization of this condition as a clonal T-cell lymphoproliferative disorder with indolent behavior.

Case records of the Massachusetts General Hospital. Weekly clinicopathological exercises. Case 35-2004. A 68-year-old man with end-stage renal disease and thickening of the skin.

From the Department of Dermatology, Lahey Clinic, Burlington, Mass. (S.L.M.); the Division of Rheumatology, Department of Medicine (J.K.), the Department of Dermatology (B.T.M.), and the Division of Dermatopathology, Department of Pathology (V.L.), Massachusetts General Hospital; and the Departments of Dermatology (S.L.M., B.T.M.), Medicine (J.K.), and Pathology (V.L.), Harvard Medical School.

Vancomycin-induced linear IgA disease manifesting as bullous erythema multiforme.

Background:  Vancomycin‐induced linear immunoglobulin A (IgA) disease, an autoimmune, blistering disease in response to vancomycin administration, is characterized by a subepidermal, vesiculobullous eruption and linear IgA deposition along the basement membrane zone on direct immunofluorescence.

Indolent CD8+ lymphoid proliferation of the ear: report of two cases and review of the literature.

Indolent CD8+ lymphoid proliferation of the ear is a recently described cutaneous lymphoid proliferation that clinically presents with slow growing lesion(s) on the ear(s). In cases reported to date, there has been indolent clinical behavior and no evidence of systemic involvement. Characteristic histopathologic features include a non‐epidermotropic diffuse dermal infiltrate of CD8+ T‐lymphocytes with a lymphoblast‐like appearance. T‐cell clonality has also been observed. Herein, we present two patients who show clinical, histopathologic and immunophenotypic features similar to the original index cases described by Petrella et al. In addition, we review the literature regarding this unusual lymphoid proliferation and provide evidence that this entity represents a phenotypic variant of primary cutaneous CD4+ small/medium‐sized pleomorphic T‐cell lymphoma, a concept proposed by Beltraminelli et al. in 2009. Differentiation between this lymphoproliferative process and other more aggressive CD8+ lymphomas is essential in avoiding excessive treatment of a condition that combines worrisome histopathology with indolent clinical behavior.

Atypical intravascular CD30+ T‐cell proliferation following trauma in a healthy 17‐year‐old male: first reported case of a potential diagnostic pitfall and literature review

Herein, we present the first report of a reactive atypical intravascular CD30+ T‐cell proliferation. Our patient developed the condition after trauma, and he has followed a benign clinical course. This observation represents a potential diagnostic pitfall for intravascular lymphoma and adds to the list of reactive conditions that may be associated with an atypical CD30+ T‐cell infiltrate.

Case 35-2004: A 68-Year-Old Man with End-Stage Renal Disease and Thickening of the Skin

From the Department of Dermatology, Lahey Clinic, Burlington, Mass. (S.L.M.); the Division of Rheumatology, Department of Medicine (J.K.), the Department of Dermatology (B.T.M.), and the Division of Dermatopathology, Department of Pathology (V.L.), Massachusetts General Hospital; and the Departments of Dermatology (S.L.M., B.T.M.), Medicine (J.K.), and Pathology (V.L.), Harvard Medical School.

Exploring the Physiological Link between Psoriasis and Mood Disorders

Psoriasis is a chronic, immune-mediated skin condition with a high rate of psychiatric comorbidity, which often goes unrecognized. Beyond the negative consequences of mood disorders like depression and anxiety on patient quality of life, evidence suggests that these conditions can worsen the severity of psoriatic disease. The mechanisms behind this relationship are not entirely understood, but inflammation seems to be a key feature linking psoriasis with mood disorders, and physiologic modulators of this inflammation, including the hypothalamic-pituitary-adrenal axis and sympathetic nervous system, demonstrate changes with psychopathology that may be contributory. Cyclical disruptions in the secretion of the sleep hormone, melatonin, are also observed in both depression and psoriasis, and with well-recognized anti-inflammatory and antioxidant activity, this aberration may represent a shared contributor to both conditions as well as common comorbidities like diabetes and cardiovascular disease. While understanding the complexities of the biological mechanisms at play will be key in optimizing the management of patients with comorbid psoriasis and depression/anxiety, one thing is certain: recognition of psychiatric comorbidity is an imperative first step in effectively treating these patients as a whole. Evidence that improvement in mood decreases psoriasis severity underscores how psychological awareness can be critical to clinicians in their practice.

Papular mycosis fungoides: report of two patients, literature review, and conceptual re-appraisal

Recent reports of 10 patients have proposed a papular variant of mycosis fungoides (MF), characterized by the appearance of papules in the absence of patches and the presence of histopathologic features of classic patch/plaque stage MF. Given the overlapping clinical and pathologic features between this proposed entity and lymphomatoid papulosis (LyP)‐type B, however, the optimal classification for such cases remains somewhat unclear. Herein, two patients are described who presented to the dermatology clinic with persistent erythematous papular eruptions on the trunk, upper and lower extremities and histopathology compatible with MF. Of note, these patients represent the oldest reported cases of papular MF, and one patient had documented peripheral blood involvement by atypical CD4+ cells. The clinicopathologic characteristics of this purported entity suggest that it may occupy the intersection between MF and CD30+ lymphoproliferative disorders. These two cases expand the clinicopathologic spectrum of papular MF to include older individuals, and further emphasize the importance of recognition of this morphology as a possible MF manifestation. Furthermore, consideration of our cases in conjunction with the previously documented 10 other patients in the literature, offers potential insight into the relationship between MF and CD30+ lymphoproliferative disorders.

Lymphoplasmacytic plaque in children: a report of two new cases with review of the literature.

Lymphoplasmacytic plaque in children has been proposed as a rare, emerging clinicopathologic entity characterized by solitary, extratruncal, asymptomatic papules and plaques that are typically found in healthy young Caucasian females. Biopsy of these lesions reveals a dermal lymphoplasmacytic infiltrate with or without epithelioid granulomas. Two unique patients with lymphoplasmacytic plaque in children are presented in this report, including a 26‐month‐old female with a lesion on her finger, who represents both the youngest described patient and the first documented with a finger lesion, as well as a 17‐year‐old young woman with a left thigh lesion, who represents the patient with the longest clinically and histopathologically observed lesion to date. These two additional patients corroborate the experience of lymphoplasmacytic plaque in children in the six previously reported cases and further expand the clinicopathologic spectrum of the disease. Recognition of lymphoplasmacytic plaque in children is important to facilitate distinction from potential differential considerations, including lymphoproliferative disorders and infectious conditions, particularly as the experience to date appears to suggest that lymphoplasmacytic plaque in children represent a reactive (pseudolymphomatous) condition.