"Poikilodermatous mycosis fungoides"Bloom, Bradley; Marchbein, Shari; Fischer, Max; KAMINO, HIDEKO; Patel, Rishi; Latkowski, Jo-Ann
Poikilodermatous mycosis fungoides (MF) is a variant of MF, formerly referred to as poikiloderma vasculare atrophicans. The lesions are classically characterized by large plaques of hypopigmentation and hyperpigmentation with atrophy and telangiectases. The plaques may be asymptomatic or mildly pruritic and typically involve the major flexural areas and trunk. Poikilodermatous MF has an early stage (IA-IIA) at diagnosis and a male predominance. Poikilodermatous MF shows an atypical T-cell infiltrate in the papillary dermis with evidence of epidermotropism, epidermal atrophy, dilated blood vessels in the dermis, melanophages, and melanin incontinence. Recent studies suggest a predominance of a CD8+, CD4- immunophenotype. Treatment modalities are similar to classic MF with phototherapy being the most common first-line therapy. Poikilodermatous MF has an excellent prognosis..
"Verrucous and macular sarcoidosis"Defelice, Taylor; Fischer, Max; KAMINO, HIDEKO; Cohen, David; Latkowski, Jo-Ann
We present a case of cutaneous sarcoidosis in a 77-year-old woman without systemic disease, who presented with two subtypes of lesions: erythematous and hyperpigmented macules on the arms and anterior upper legs and erythematous, hyperkeratotic nodules, some crateriform, on the lower legs and feet. Whereas cutaneous sarcoidosis is present in up to one-third of cases and may present with a wide variety of lesions, verrucous sarcoidosis is uncommon. Diagnosis is by clinical and histopathologic exclusion of other entities, which include other causes of granulomatous inflammation such as infections and foreign-body reactions. Management options include topical, intralesional, or systemic glucocorticoids, antimalarials, tetracycline derivatives, and methotrexate..
"Acrokeratosis verruciformis"Defelice, Taylor; Robinson, Maria; Patel, Rishi; KAMINO, HIDEKO
We present a case of acrokeratosis verruciformis of Hopf in a 58-year-old woman with multiple, skin-colored, flat-topped, hyperkeratotic papules on the dorsal aspects of the hands and feet. Her lesions appeared at age 13 and her son developed similar lesions at about the same age. Acrokeratosis verruciformis of Hopf is a rare genodermatosis, which is distinct from keratosis follicularis; sporadic cases have also been described. Management options include cryotherapy, keratolytics, superficial ablation with CO2 or Nd:YAG lasers, topical retinoids, and acitretin..
"Eruptive basal-cell carcinomas in the setting of human immunodeficiency virus infection"Gordon Spratt, Elizabeth A; Fischer, Max; KAMINO, HIDEKO
Patients with human immunodeficiency virus are known to have an increased risk for development of cutaneous malignant conditions. We present a 55-year-old man with a history of an isolated squamous-cell carcinoma five years prior, who presented with 27 cutaneous lesions, which had developed over the course of the preceding year. This occurred in the context of a new diagnosis of human immunodeficiency virus (HIV) infection and acquired immune deficiency syndrome (AIDS). The initiation of anti-retroviral therapy and subsequent restoration of CD4 counts was associated with an increase in the number of lesions. Histopathologic examination of representative tumors showed basal-cell carcinomas, which lead to the diagnosis of eruptive basal-cell carcinomas in the setting of HIV-related immunosuppression. The incidence and epidemiology of malignant conditions that are related and non-related to AIDS are reviewed as well as discussion of the current literature regarding presentation, treatment, and prevention of non-melanoma skin cancers in patients with HIV infection..
"Single versus multiple primary melanomas: Old questions and new answers"Hwa C; Price LS; Belitskaya-Levy I; Ma MW; Shapiro RL; Berman RS; KAMINO H; Darvishian F; Osman I; Stein JA
BACKGROUND: In patients with multiple primary melanomas (MPM), mean tumor thickness tends to decrease from the first melanoma to the second melanoma, and prognosis may be improved compared with the prognosis for patients who have a single primary melanoma (SPM). In this study, the authors compared the clinicopathologic features of patients with MPM and SPM to better characterize the differences between these 2 groups and to determine whether or not there is an inherent difference in tumor aggression. METHODS: In total, 788 patients with melanoma who were enrolled prospectively in the Interdisciplinary Melanoma Cooperative Group database from 2002 to 2008 were studied. Patients with SPM and with MPM were compared with regard to clinical and primary melanoma characteristics. RESULTS: Of 788 patients with melanoma, 61 patients (7.7%) had 2 or more primary melanomas. The incidence of developing a second primary melanoma 1 year and 5 years after initial melanoma diagnosis was 4.1% and 8.7%, respectively, and most of the risk accumulated within the first year. The incidence of MPM was greater in patients aged >/=60 years than in those aged </=60 years. The absence or presence of mitosis and other tumor characteristics did not differ significantly between patients with SPM and patients with MPM (P = .61). CONCLUSIONS: No difference was observed in the presence or absence of mitoses, a marker of tumor proliferation, in SPM and MPM. Because it has been demonstrated that the presence of mitosis is a powerful prognostic marker, the current findings suggested that the tumors behave similarly in patients with SPM and patients with MPM. The authors concluded that differences in tumor thickness and prognosis between SPM and MPM more likely are caused by factors other than tumor biology, such as increased surveillance. Cancer 2012;. (c) 2012 American Cancer Society.
"Localized pemphigus with vegetative features"Lehrhoff, Stephanie; Miller, Kristen; Fischer, Max; KAMINO, HIDEKO; Meehan, Shane
We present a 58-year-old Jamaican man with an eight-month history of an isolated, vegetative, eroded, and crusted plaque on the posterior aspect of the scalp. One month prior to referral to the Charles C. Harris Skin and Cancer Pavilion he developed a few, scattered erosions on the extremities. A biopsy specimen showed suprabasal acantholysis along with deposition of C3 and IgG on a direct immunofluorescence test. Localized pemphigus is a rare variant of pemphigus vulgaris. Localized pemphigus presents with a localized plaque that remains localized for an extended period of time without generalized bullae formation. Our patient represents an unusual case of localized pemphigus of the scalp with vegetative features..
"Hypergammaglobulinemic purpura of Waldenstrom"Lewin, Jesse M; Hunt, Raegan; Fischer, Max; KAMINO, HIDEKO; Franks, Andrew G Jr
We report a case of a 33-year-old-woman with a one-year history of bilateral lower extremity vasculitis and laboratory evidence of hypergammaglobulinemia with otherwise unremarkable routine laboratory and rheumatologic studies. Her clinical picture, together with histopathologic evidence of leukocytoclastic vasculitis, favor a diagnosis of hypergammaglobulinemic purpura of Waldenstrom..
"Purpura annularis telangiectoides"Miller, Kristen; Fischer, Max; KAMINO, HIDEKO; Meehan, Shane; Cohen, David
Purpura annularis telangiectoides of Majocchi (PATM) is a clinical variant of pigmented purpuric dermatoses. It is characterized by asymptomatic or mildly pruritic, erythematous, annular patches that vary in size. The patches exhibit central clearing and peripheral cayenne pepper petechiae. It classically occurs on the lower legs but may be more generalized. Histopathologic features include a lymphocytic capillaritis with subsequent capillary leakage and extravasated erythrocytes, which is typical of all pigmented purpuric dermatoses. The pathogenesis of PATM is unknown, but it may uncommonly be associated with underlying diseases such as hematologic disorders and rarely cutaneous T-cell lymphoma. There is no standard effective treatment..
"Perifollicular Langerhans cell histiocytosis"Mir, Adnan; Fischer, Max; KAMINO, HIDEKO; Soter, Nicholas A
A 25-year-old man presented with a 13-year history of an erythematous, papular eruption of his face and trunk, which was treated in the past as acne and psoriasis with isotretinoin and methotrexate, respectively. Histopathologic examination demonstrated an infiltrate of Langerhans cells, which was consistent with Langerhans cell histiocytosis. The epidemiology, clinical presentation, and treatment options of this disease are reviewed..
"Dissecting cellulitis of the scalp"Mundi, Jyoti P; Marmon, Shoshana; Fischer, Max; KAMINO, HIDEKO; Patel, Rishi; Shapiro, Jerry
Dissecting cellulitis of the scalp is a chronic, relapsing, inflammatory disease of the scalp that results in scarring alopecia. We present a case of a 32-year-old man with recalcitrant disease who is now responding to treatment with isotretinoin. The pathogenesis, clinical presentation, disease associations, and histopathological findings are reviewed. Treatment can be challenging. The literature on medical and surgical therapeutic options is reviewed..
"Eruptive vellus hair cysts"Patel, Utpal; Terushkin, Vitaly; Fischer, Max; KAMINO, HIDEKO; Patel, Rishi
Eruptive vellus hair cyst (EVHC), described initially in 1977, is a benign dermatologic condition that is characterized by the sudden appearance of monomorphic, follicular, asymptomatic, small papules in children and young adults. The diagnosis is based on the histopathologic findings of stratified-squamous epithelium with a granular layer that surrounds a cystic space filled with laminated keratin and a variable number of vellus hair cysts. EVHC can be associated with steatocystoma multiplex. A current hypothesis suggests that EVHC originates from a cystic change at the insertion of the pilosebaceous duct. EVHC is primarily treated for cosmesis with retinoids, surgery, and lasers..
"Necrobiotic xanthogranuloma"Rose, Amy; Robinson, Maria; KAMINO, HIDEKO; Latkowski, Jo-Ann
Necrobiotic xanthogranuloma (NXG) is a rare, chronic, progressive, non-Langerhans histiocytosis that is strongly associated with hematologic malignant conditions. Only about 100 cases have been reported in the literature since it was first described in 1980. It is important for dermatologists to recognize NXG and initiate a prompt hematologic evaluation. IgG kappa is the most frequently discovered monoclonal gammopathy (65%), followed by IgG lambda (35%), and, much less commonly, IgA. Although no modality has been shown to be consistently effective, therapeutic options include glucocorticoids (topical, intralesional, and/or systemic), alkylating agents (chlorambucil and cyclophosphamide). interferon alpha, antimetabolites, antibiotics, thalidomide, and plasmaphersis..
"Metastatic hepatocellular carcinoma in a liver transplant patient presenting as cheek nodules"Mandell, Jenny A; Tlougan, Brook E; Patel, Rishi R; Warycha, Melanie A; KAMINO, HIDEKO; Haglof, Karen J
"Morphea with discoid lupus erythematosus" [Case Report]Mir, Adnan; Tlougan, Brook; O'Reilly, Kathryn; Tzu, Julia; Meehan, Shane; KAMINO, HIDEKO; Franks, Andrew G Jr
The presence of lupus erythematosus with morphea in the same patient has rarely been reported. In this case, we describe a woman with the overlap of discoid lupus erythematosus with superficial morphea, diagnoses that are supported by histopathologic features and laboratory studies..
"Clinical Relevance of Detection of Lymphovascular Invasion in Primary Melanoma Using Endothelial Markers D2-40 and CD34"Rose, Amy E; Christos, Paul J; Lackaye, Dan; Shapiro, Richard L; Berman, Russell; Mazumdar, Madhu; KAMINO, HIDEKO; Osman, Iman; Darvishian, Farbod
GRANTS:UL1-RR024996/RR/NCRR NIH HHS/United States
Immunohistochemistry (IHC) using endothelial markers may facilitate the detection of lymphovascular invasion (LVI) in primary melanoma; however, the clinical implications of enhanced detection are unknown. We evaluated whether the use of lymphatic endothelial marker D2-40 and panvascular marker CD34 increases LVI positivity relative to routine histology alone and then evaluated the prognostic relevance of LVI detected using these markers in terms of disease-free (DFS) and overall survival (OS). A total of 246 primary melanomas were assessed for LVI using D2-40, CD34, and routine histology. Associations between LVI positivity and clinicopathologic variables, DFS, and OS were compared using univariate and multivariate analyses. The use of endothelial markers increased the rate of LVI positivity (18% using D2-40 and/or CD34 vs. 3% by routine histology, P<0.0001). On univariate analysis, IHC-detected LVI was significantly associated with more adverse clinicopathologic variables (thickness, ulceration, mitoses, and nodular subtype) compared with LVI detected by routine histology (thickness and ulceration only). In a multivariate model controlling for stage, LVI detected using IHC markers remained a significant marker of both reduced DFS [hazard ratio (HR), 2.01; 95% confidence interval (CI): 1.27-3.18; P=0.003] and OS (HR, 2.08; 95% CI: 1.25-3.46; P=0.005). Results show that D2-40 and CD34 increase the detection of LVI in primary melanoma and that cases missed by routine histology have prognostic relevance.
"Hypertrophic discoid lupus erythematosus"Farley-Loftus, Rachel; Elmariah, Sarina B; Ralston, Jonathan; KAMINO, HIDEKO; Franks, Andrew G Jr
Hypertrophic discoid lupus erythematosus is a distinct form of chronic cutaneous (discoid) lupus, which is characterized by hyperkeratotic plaques that typically are observed over the face, arms, and upper trunk. We present the case of a 43-year-old man with verrucous plaques that were distributed symmetrically over the face, who initially was treated with oral antibiotics and topical glucocorticoids for acne vulgaris. A biopsy specimen confirmed the diagnosis of hypertrophic discoid lupus erythematosus. The clinical and histopathologic features of this clinical variant are reviewed.
"Morphea, diabetes mellitus type I, and celiac disease: case report and review of the literature"Firoz, Elnaz F; KAMINO, HIDEKO; Lehman, Thomas J A; Orlow, Seth J
An 11-year-old girl with a history of diabetes mellitus type I and celiac disease presented with multiple, depressed patches of purple-brown skin on the right lower extremity and central back, with histopathologic features of early morphea. Though morphea may coexist with other autoimmune diseases, its presentation with both diabetes mellitus type I and celiac disease has not yet been described.
"Familial benign chronic pemphigus (Hailey-Hailey disease)"Hunt, Raegan; O'Reilly, Kathryn; Ralston, Jonathan; KAMINO, HIDEKO; Shupack, Jerome L
We present an atypical case of familial benign chronic pemphigus (Hailey-Hailey disease) that manifested with relapsing, flaccid vesicles and erosions, which were limited to the upper chest, anterior aspect of the neck, and anterior aspects of the upper arms without intertriginous involvement. Although individual eruptions in this patient demonstrated asymmetry, relapses did not obey a segmental distribution. To the best of our knowledge, no other patient has been described with symmetric lesions that were localized solely to the anterior upper body without a prior history of lesions at commonly affected disease sites, which include skin folds, the back, and the posterior and lateral aspects of the neck. This unusual presentation of Hailey-Hailey disease highlights the variable nature of the disease.
"Elastic fiber pattern in regressing melanoma: a histochemical and immunohistochemical study"KAMINO, HIDEKO; Tam, Sam; Roses, Daniel; Toussaint, Sonia
BACKGROUND: Although histopathologic identification of regression of melanoma is usually straightforward, sometimes it can be difficult to distinguish it from scarring fibrosis. Therefore, this study investigates the elastic fiber pattern in melanomas associated with either regression or scars. METHODS: We compared 33 invasive melanomas with the fibrosing stage of regression to 10 cases of invasive melanomas with scarring fibrosis. None of the regression cases had a prior surgical procedure. Elastic fiber patterns were evaluated with Verhoeff's elastic van Gieson stain (EVG) and elastin immunostain. RESULTS: Elastin immunostain was superior to EVG in revealing the elastic fiber patterns. Both regression and scars had decreased to absent elastic fibers in the areas of fibrosis. However, areas of regression had a well-defined compressed layer of thin elastic fibers pushed down from the papillary dermis to the base of the fibrosis. In contrast, the base of scars lacked this compressed elastic layer and had instead an abrupt transition to the thick elastic fibers of the spared reticular dermis. CONCLUSIONS: We have identified distinct changes of the elastic tissue network, which more accurately define the presence of regression in melanoma and distinguish it from scarring fibrosis.