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Pagetoid reticulosis in lymphomatoid papulosis type D and mycosis fungoides, mimicking aggressive epidermotropic CD8+ (“Berti”) lymphoma
Minimally differentiated acute myeloid leukemia (FAB AML-M0) with overlap features of blastic plasmacytoid dendritic cell neoplasm presenting as leukemia cutis in a young woman
(Poster reference number 5710)
Kerry A. Lavigne, MD, Geisinger Medical Center, Department of Dermatology, Danville, PA, United States; Dirk Elston, MD, Geisinger Medical Center, Departments of Dermatology and Dermatopathology, Danville, PA, United States; Jagadeesh Ramdas, MD, Geisinger Medical Center, Department of Pediatric Hematology/Oncology, Danville, PA, United States; R. Patrick Dorion, MD, Geisinger Medical Laboratories, Departments of Hematology, Anatomic Pathology, Hematopathology, Danville, PA, United States; Victor John Marks, MD, Geisinger Medical Center, Department of Dermatology, Danville, PA, United States Minimally differentiated acute myeloid leukemia (FAB AML-M0) is a rare variant of myeloid leukemia accounting for \5% of AML diagnoses. A distinct subgroup of myeloid neoplasm called blastic plasmacytoid dendritic cell neoplasm (BPDCN) has been recently described, characterized by clonal expansion of immature plasmacytoid dendritic cells. We report an unusual overlap of immunohistochemical features between BPDCN and AML-MO in a 17-year-old female who presented with extensive leukemia cutis and a normal CBC. Bone marrow biopsy showed extensive leukemia infiltration and a diagnosis of minimally differentiated acute myeloid leukemia with T-lymphoid aberrancy (FAB AML-M0) was made given the expression of a CD33+, CD38+, HLA-DR+ myeloid phenotype with moderate CD13+, weak CD117+, and aberrant strong CD4+ and CD7+ antigen positivity. Concurrent skin biopsy demonstrated a nodular infiltrate of large pleomorphic blue cells filling the dermis with associated mitotic figures. The infiltrate extended to the subcutaneous fat and stained CD4+, CD123+, BCL-2+, and weakly BCL-6+ and CD43+. Notably both cutaneous and bone marrow samples were CD56e. The patient responded very well to standard AML based chemotherapy leading to the disappearance of all the skin nodules and is in complete morphological remission after 3 courses of chemotherapy. In 2008, the fourth edition of the WHO classification delineated blastic plasmacytoid dendritic cell neoplasm (BPDCN), replacing prior designations such as agranular CD4+ natural killer cell leukemia, blastic natural killer leukemia/lymphoma, and agranular CD4+/CD56+ hematodermic neoplasms. The proposed relationship between myelogenous leukemias and BPDCN has been attributed to the plastic nature of the primitive precursor cells. AML-M0 features low myeloperoxidase (MPO) expression along with myeloid surface antigen such as CD13+, CD33+, or CD15+, as well as an early progenitor immunophenotype with CD34+, terminal deoxynucleotidyltransferase (TdT+), HLA-DR+, or CD117+. BPDCN is generally MPOe, CD4+, CD56+, CD123+, and CD43+. Challenges arise in cases that fail to express one of these markers, such as CD56 in our case, as well as from the overlap of markers between AML and BPDCN. Given the rarity of these two entities, especially in the pediatric population, this case demonstrates key immunophenotypic markers which when analyzed with a larger series may help clarify commonalities in histogenesis.
Porokeratoma: A different entity or a variant of verrucous (hyperkeratotic) porokeratosis?
(Poster reference number 4735)
Ana Batalla, MD, Dermatology Service, Complejo Hospitalario de Pontevedra (SPAIN), Pontevedra, Spain; Beatriz Aranegui, MD, Dermatology Service, Complejo Hospitalario de Pontevedra (SPAIN), Pontevedra, Spain; Carlos De la Torre, MD, PhD, Dermatology Service, Complejo Hospitalario de Pontevedra (SPAIN), Pontevedra, Spain; Elena Ros on, MD, Dermatology Service, Complejo Hospitalario de Pontevedra (SPAIN), Pontevedra, Spain Background: The term porokeratoma (defining an acanthoma with features of porokeratosis) was coined by Santa Cruz in 2007 as a new entity with a distinct pattern of cornoid lamellation from typical porokeratosis. We present a case of porokeratoma and review its characteristics and controversies. Case report: A 78-year-old man with paraplegia secondary to poliomyelitis and ankylosing spondylarthritis presented with a 4-year history of slightly painful and slowly growing lesions on his left buttock. The patient had been unsuccessfully treated with cryotherapy 2 years before. Clostridiopeptidase and protease ointment had been occasionally applied. On physical examination, there were six well defined verrucous lesions, with a central depression filled with keratinous material. The main differential diagnoses were perforating diseases, giant comedones, or viral warts. Surgical removal of the biggest lesion was performed. Histologic study showed papillomatosis and multiple epidermal depressions filled with cornoid lamellae. Below the lamellae, the granular layer was absent, and there were few dyskeratotic cells. Areas between cornoid lamellae showed orthokeratotic hyperkeratosis. The diagnosis of porokeratoma was made. Discussion: Porokeratoma is clinically and pathologically a distinct form of porokeratosis. It usually appears as scaling plaques, papules, or nodules with central hyperkeratosis and occasional verrucous appearance. Microscopically, it is composed of multiple cornoid lamellae throughout the entire lesion, instead being present only at the borders, with no central epidermal atrophy, and with well demarcation from the uninvolved skin. It is also remarkable that previous reported cases of porokeratoma showed solitary lesions, except in one case from Kanitakis et al, who reported a patient who suffered from ankylosing spondylarthritis, with multiple lesions of porokeratoma, being very similar to our case. Nevertheless, some authors disagree with the term porokeratoma, and consider that it only refers to a verrucous variant of porokeratosis, suggesting the unification of the different terms which have been used in the literature to design this entity. Because of the similarities with porokeratosis and the potential but not still reported malignant transformation of porokeratoma, excision and close follow-up is indicated in these patients. Commercial support: None identified.
Commercial support: None identified.
Pagetoid reticulosis in lymphomatoid papulosis type D and mycosis fungoides, mimicking aggressive epidermotropic CD8+ (‘‘Berti’’) lymphoma
(Poster reference number 5311)
Mina Ally, MBBS, St. John’s Institute of Dermatology, London, United Kingdom; Alistair Robson, MBChB, St. John’s Institute of Dermatology, London, United Kingdom Pagetoid reticulosis is a histologic pattern in which there is extensive epidermotropism by lymphoid cells, often without Pautrier microabscess formation. It is a feature associated with WoringereKolopp disease, KetroneGoodman disease, aggressive epidermotropic CD8+ (Berti) lymphoma, and the recently described lymphomatoid papulosis (LyP) type D. We present three cases of clinical LyP, which in 4 biopsies had a histologic pattern of pagetoid reticulosis mimicking Berti’s lymphoma, and a single case of mycosis fungoides (MF) with a Pagetoid reticulosis histology. In each there was florid epidermotropism without Pautrier microabscesses. In 2 cases, the immunophenotype was CD4+ T cell, but the third case of LyP appeared to have markedly epidermotropic CD4+ and CD8+ cells. Case 1 was a 71-year-old man with nodules on his right breast and inner thigh; the highly pleomorphic neoplastic cells had a CD45RA+ CD2+ CD5+ CD30+ phenotype. Case 2, a 64-year-old male, with a nodule on his left arm, had atypical cells that had focal CD45RO+, and were CD30+ CD2+. Case 3 was an 84-year-old man with penile papules CD2+ CD5+ CD45RO+ and CD30+. Case 4 was a 63-year-old man with MF, including folliculotropic disease. There was a very high Ki-67 index in all biopsies. The tumor cells were more pleomorphic than those seen in Berti lymphoma, and differed from the typical Berti immunophenotype: CD8+ CD45RA+ CD2e CD5e. The LyP patients were treated as appropriate, without disease progression. Similarly, the patient with MF was treated with conventional topical and systemic therapy. Pagetoid reticulosis is a very distinctive histologic pattern most commonly associated with aggressive epidermotropic CD8+ (Berti) lymphoma and only rarely seen in other forms of cutaneous lymphoma and the benign solitary form of pagetoid reticulosis, WoringereKolopp. Pathologists should be aware of this histologic pattern in LyP and, rarely in MF, and not to misdiagnose Berti lymphoma, which has a median survival of 12 months and requires urgent and intensive treatment.
Primary cutaneous CD4+ small/medium sized pleomorphic T-cell lymphoma: A study of 3 cases
Commercial support: None identified.
Commercial support: None identified.
APRIL 2012
(Poster reference number 5335)
Mina Ally, MBBS, St. John’s Institute of Dermatology, London, United Kingdom; Alistair Robson, MBChB, St. John’s Institute of Dermatology, London, United Kingdom Primary cutaneous CD4+ small/medium sized pleomorphic T-cell lymphoma is listed as a provisional entity in the WHO-EORTC classification of cutaneous lymphomas. We describe three further cases, each presenting as a solitary lesion. A 52-year-old woman presented with a rapidly growing nodule behind her right ear. Biopsy revealed a dense nodular nonepidermotropic lymphoid infiltrate. This infiltrate contained medium sized atypical hyperchromatic cells and occasional blasts. Immunocytochemistry confirmed a predominantly CD3+CD4+CD21/ phenotype with admixed CD8+ cells and numerous CD20+ cells. There was a clonal gene rearrangement of the T-cell receptor. Staging investigations were normal, and follow-up at 11 months showed no evidence of disease. A 44-year-old woman presented with a nodule in the left pre-auricular area which had been slowly enlarging over 2 to 3 months. Skin biopsy showed almost identical histologic features. Immunocytochemistry confirmed a predominantly CD3+CD4+ phenotype, with a heavy B-cell population. The lesion was excised and there has been no recurrence in 1 year of follow-up. A 25-year-old woman presented with a lesion on the left side of her chest. Skin biopsy showed a dense infiltrate with scattered atypical cells. Immunocytochemistry confirmed a CD3+CD4+ phenotype with many admixed B cells. T cell receptor analysis confirmed a clonal V-gamma rearrangement. The patient is being treated with superficial radiotherapy to the excision site. These cases represent further examples of this rare entity; despite the cytologic pleomorphism, the prognosis is very good, and locally directed radiotherapy is the treatment of choice.
J AM ACAD DERMATOL
AB81
(Poster reference number 5710)
Kerry A. Lavigne, MD, Geisinger Medical Center, Department of Dermatology, Danville, PA, United States; Dirk Elston, MD, Geisinger Medical Center, Departments of Dermatology and Dermatopathology, Danville, PA, United States; Jagadeesh Ramdas, MD, Geisinger Medical Center, Department of Pediatric Hematology/Oncology, Danville, PA, United States; R. Patrick Dorion, MD, Geisinger Medical Laboratories, Departments of Hematology, Anatomic Pathology, Hematopathology, Danville, PA, United States; Victor John Marks, MD, Geisinger Medical Center, Department of Dermatology, Danville, PA, United States Minimally differentiated acute myeloid leukemia (FAB AML-M0) is a rare variant of myeloid leukemia accounting for \5% of AML diagnoses. A distinct subgroup of myeloid neoplasm called blastic plasmacytoid dendritic cell neoplasm (BPDCN) has been recently described, characterized by clonal expansion of immature plasmacytoid dendritic cells. We report an unusual overlap of immunohistochemical features between BPDCN and AML-MO in a 17-year-old female who presented with extensive leukemia cutis and a normal CBC. Bone marrow biopsy showed extensive leukemia infiltration and a diagnosis of minimally differentiated acute myeloid leukemia with T-lymphoid aberrancy (FAB AML-M0) was made given the expression of a CD33+, CD38+, HLA-DR+ myeloid phenotype with moderate CD13+, weak CD117+, and aberrant strong CD4+ and CD7+ antigen positivity. Concurrent skin biopsy demonstrated a nodular infiltrate of large pleomorphic blue cells filling the dermis with associated mitotic figures. The infiltrate extended to the subcutaneous fat and stained CD4+, CD123+, BCL-2+, and weakly BCL-6+ and CD43+. Notably both cutaneous and bone marrow samples were CD56e. The patient responded very well to standard AML based chemotherapy leading to the disappearance of all the skin nodules and is in complete morphological remission after 3 courses of chemotherapy. In 2008, the fourth edition of the WHO classification delineated blastic plasmacytoid dendritic cell neoplasm (BPDCN), replacing prior designations such as agranular CD4+ natural killer cell leukemia, blastic natural killer leukemia/lymphoma, and agranular CD4+/CD56+ hematodermic neoplasms. The proposed relationship between myelogenous leukemias and BPDCN has been attributed to the plastic nature of the primitive precursor cells. AML-M0 features low myeloperoxidase (MPO) expression along with myeloid surface antigen such as CD13+, CD33+, or CD15+, as well as an early progenitor immunophenotype with CD34+, terminal deoxynucleotidyltransferase (TdT+), HLA-DR+, or CD117+. BPDCN is generally MPOe, CD4+, CD56+, CD123+, and CD43+. Challenges arise in cases that fail to express one of these markers, such as CD56 in our case, as well as from the overlap of markers between AML and BPDCN. Given the rarity of these two entities, especially in the pediatric population, this case demonstrates key immunophenotypic markers which when analyzed with a larger series may help clarify commonalities in histogenesis.
Porokeratoma: A different entity or a variant of verrucous (hyperkeratotic) porokeratosis?
(Poster reference number 4735)
Ana Batalla, MD, Dermatology Service, Complejo Hospitalario de Pontevedra (SPAIN), Pontevedra, Spain; Beatriz Aranegui, MD, Dermatology Service, Complejo Hospitalario de Pontevedra (SPAIN), Pontevedra, Spain; Carlos De la Torre, MD, PhD, Dermatology Service, Complejo Hospitalario de Pontevedra (SPAIN), Pontevedra, Spain; Elena Ros on, MD, Dermatology Service, Complejo Hospitalario de Pontevedra (SPAIN), Pontevedra, Spain Background: The term porokeratoma (defining an acanthoma with features of porokeratosis) was coined by Santa Cruz in 2007 as a new entity with a distinct pattern of cornoid lamellation from typical porokeratosis. We present a case of porokeratoma and review its characteristics and controversies. Case report: A 78-year-old man with paraplegia secondary to poliomyelitis and ankylosing spondylarthritis presented with a 4-year history of slightly painful and slowly growing lesions on his left buttock. The patient had been unsuccessfully treated with cryotherapy 2 years before. Clostridiopeptidase and protease ointment had been occasionally applied. On physical examination, there were six well defined verrucous lesions, with a central depression filled with keratinous material. The main differential diagnoses were perforating diseases, giant comedones, or viral warts. Surgical removal of the biggest lesion was performed. Histologic study showed papillomatosis and multiple epidermal depressions filled with cornoid lamellae. Below the lamellae, the granular layer was absent, and there were few dyskeratotic cells. Areas between cornoid lamellae showed orthokeratotic hyperkeratosis. The diagnosis of porokeratoma was made. Discussion: Porokeratoma is clinically and pathologically a distinct form of porokeratosis. It usually appears as scaling plaques, papules, or nodules with central hyperkeratosis and occasional verrucous appearance. Microscopically, it is composed of multiple cornoid lamellae throughout the entire lesion, instead being present only at the borders, with no central epidermal atrophy, and with well demarcation from the uninvolved skin. It is also remarkable that previous reported cases of porokeratoma showed solitary lesions, except in one case from Kanitakis et al, who reported a patient who suffered from ankylosing spondylarthritis, with multiple lesions of porokeratoma, being very similar to our case. Nevertheless, some authors disagree with the term porokeratoma, and consider that it only refers to a verrucous variant of porokeratosis, suggesting the unification of the different terms which have been used in the literature to design this entity. Because of the similarities with porokeratosis and the potential but not still reported malignant transformation of porokeratoma, excision and close follow-up is indicated in these patients. Commercial support: None identified.
Commercial support: None identified.
Pagetoid reticulosis in lymphomatoid papulosis type D and mycosis fungoides, mimicking aggressive epidermotropic CD8+ (‘‘Berti’’) lymphoma
(Poster reference number 5311)
Mina Ally, MBBS, St. John’s Institute of Dermatology, London, United Kingdom; Alistair Robson, MBChB, St. John’s Institute of Dermatology, London, United Kingdom Pagetoid reticulosis is a histologic pattern in which there is extensive epidermotropism by lymphoid cells, often without Pautrier microabscess formation. It is a feature associated with WoringereKolopp disease, KetroneGoodman disease, aggressive epidermotropic CD8+ (Berti) lymphoma, and the recently described lymphomatoid papulosis (LyP) type D. We present three cases of clinical LyP, which in 4 biopsies had a histologic pattern of pagetoid reticulosis mimicking Berti’s lymphoma, and a single case of mycosis fungoides (MF) with a Pagetoid reticulosis histology. In each there was florid epidermotropism without Pautrier microabscesses. In 2 cases, the immunophenotype was CD4+ T cell, but the third case of LyP appeared to have markedly epidermotropic CD4+ and CD8+ cells. Case 1 was a 71-year-old man with nodules on his right breast and inner thigh; the highly pleomorphic neoplastic cells had a CD45RA+ CD2+ CD5+ CD30+ phenotype. Case 2, a 64-year-old male, with a nodule on his left arm, had atypical cells that had focal CD45RO+, and were CD30+ CD2+. Case 3 was an 84-year-old man with penile papules CD2+ CD5+ CD45RO+ and CD30+. Case 4 was a 63-year-old man with MF, including folliculotropic disease. There was a very high Ki-67 index in all biopsies. The tumor cells were more pleomorphic than those seen in Berti lymphoma, and differed from the typical Berti immunophenotype: CD8+ CD45RA+ CD2e CD5e. The LyP patients were treated as appropriate, without disease progression. Similarly, the patient with MF was treated with conventional topical and systemic therapy. Pagetoid reticulosis is a very distinctive histologic pattern most commonly associated with aggressive epidermotropic CD8+ (Berti) lymphoma and only rarely seen in other forms of cutaneous lymphoma and the benign solitary form of pagetoid reticulosis, WoringereKolopp. Pathologists should be aware of this histologic pattern in LyP and, rarely in MF, and not to misdiagnose Berti lymphoma, which has a median survival of 12 months and requires urgent and intensive treatment.
Primary cutaneous CD4+ small/medium sized pleomorphic T-cell lymphoma: A study of 3 cases
Commercial support: None identified.
Commercial support: None identified.
APRIL 2012
(Poster reference number 5335)
Mina Ally, MBBS, St. John’s Institute of Dermatology, London, United Kingdom; Alistair Robson, MBChB, St. John’s Institute of Dermatology, London, United Kingdom Primary cutaneous CD4+ small/medium sized pleomorphic T-cell lymphoma is listed as a provisional entity in the WHO-EORTC classification of cutaneous lymphomas. We describe three further cases, each presenting as a solitary lesion. A 52-year-old woman presented with a rapidly growing nodule behind her right ear. Biopsy revealed a dense nodular nonepidermotropic lymphoid infiltrate. This infiltrate contained medium sized atypical hyperchromatic cells and occasional blasts. Immunocytochemistry confirmed a predominantly CD3+CD4+CD21/ phenotype with admixed CD8+ cells and numerous CD20+ cells. There was a clonal gene rearrangement of the T-cell receptor. Staging investigations were normal, and follow-up at 11 months showed no evidence of disease. A 44-year-old woman presented with a nodule in the left pre-auricular area which had been slowly enlarging over 2 to 3 months. Skin biopsy showed almost identical histologic features. Immunocytochemistry confirmed a predominantly CD3+CD4+ phenotype, with a heavy B-cell population. The lesion was excised and there has been no recurrence in 1 year of follow-up. A 25-year-old woman presented with a lesion on the left side of her chest. Skin biopsy showed a dense infiltrate with scattered atypical cells. Immunocytochemistry confirmed a CD3+CD4+ phenotype with many admixed B cells. T cell receptor analysis confirmed a clonal V-gamma rearrangement. The patient is being treated with superficial radiotherapy to the excision site. These cases represent further examples of this rare entity; despite the cytologic pleomorphism, the prognosis is very good, and locally directed radiotherapy is the treatment of choice.
J AM ACAD DERMATOL
AB81