Cerebriform Nuclei Research Articles

‘Could it be mycosis fungoides?’: an approach to diagnosing patch stage mycosis fungoides

Mycosis fungoides (MF) is the most common primary cutaneous T cell lymphoma, which is characterised in its early stages by epidermotropism of small to medium-sized T lymphocytes with cerebriform nuclei. Originally described by Alibert in 1806, MF is classically a disease of adults, although children and adolescents can be affected, and it typically has a protracted, indolent course. Routine dermatopathology practice involves many biopsies submitted with a clinical query regarding the possibility of MF. These are not always straightforward, as the histological features can be variable and are not always readily distinguishable from several other clinical differential diagnoses. Whilst modern molecular testing modalities can assist, even these do not always enable a definitive diagnosis of MF in its early stages. We have reviewed the histopathological features of early MF and currently recognised subtypes and the role of immunohistochemistry and emerging molecular techniques in the diagnosis of this condition. We also outline our approach to a biopsy where the question of ‘Could it be MF?’ has been proposed.

Cutaneous T-cell lymphoma with nasal necrosis.

Mycosis fungoides is a rare T-cell cutaneous lymphoma that poses a unique diagnostic challenge given its heterogeneous presentation. The refractory case presented here highlights the need for advances in early diagnostic technologies. A 73-year-old diabetic man presented with erythema and necrosis of the nose. He initially had a 7 mm squamous cell carcinoma on the nose which was excised with Mohs surgery. The wound rapidly progressed to full thickness necrosis of the left nasal ala without pain or fever. During subsequent reconstruction with a nasolabial flap and cartilage graft, biopsies of the nasal margin showed severe inflammation without malignancy. The reconstructed area failed to heal and serial cultures grew multiple species of bacteria which were treated with several courses of antibiotics. Erythema of the nose and face continued to progress over 7 months and new lesions appeared, including skin necrosis at the site of previous left ala flap, right dorsum of nose and right cheek (Fig. 1). Computed tomography (CT) scan showed no involvement of sinuses or facial bones. Repeat biopsy showed acute and chronic inflammation with no malignancy and no organisms. A few weeks later a new erythematous plaque developed on the back (Fig. 2) and biopsy showed lymphocytic infiltrate with a monoclonal T-cell population (Figs. 3, ​,4).4). Repeat assay of previous nasal biopsy showed the same T-cell receptor gene rearrangement leading to the diagnosis of cutaneous T-cell lymphoma, consistent with mycosis fungoides (MF). The patient was staged with positron emission tomography-CT scan showing no visceral disease despite developing new lesions in his groin in addition to new lesions on his back and face. He was treated with a combination of systemic chemotherapy, radiation and phototherapy. At 16 month follow up he has persistent skin lesions, with progression of tumor burden. Fig. 1 Facial involvement of mycosis fungoides with significant nasal necrosis at site of previous nasolabial flap. Fig. 2 Erythematous plaque distant to original lesion with biopsy results demonstrating cutaneous T cell lymphoma. Fig. 3 Diffuse lymphocytic infiltrate through the dermis with inconspicuous epidermotropism (H&E, ×6.3). Fig. 4 Immunohistochemical study demonstrates a predominance of lymphocytic cells labeling with CD4 with some extension into the epidermis (×12.6). This case highlights the diagnostic difficulty seen in many cases of cutaneous T-cell lymphoma described in the literature. The diagnosis of cutaneous T-cell lymphomas, the majority of which are classified as Mycosis fungoides or Sezary syndrome, requires integration of both clinical and histopathological information [1]. The diagnosis of early MF is often difficult given its heterogeneous clinical and pathologic presentations [2,3]. Histopathologically, early MF may resemble chronic inflammatory dermatoses with reactive T cells and other immune cells [2,4]. Adjuvant techniques such as immunophenotyping and T cell receptor gene rearrangement studies can help make the diagnosis in some difficult cases [3]. The classic histopathology of MF is characterized by lymphocytes with cerebriform nuclei and a haloed appearance that display epidermotropism or populate the dermoepidermal junction [2,4]. Histological diagnosis of early disease typically requires several follow up skin biopsies [3]. Given the difficulty of diagnosis, clinic-pathologic correlation is crucial for early MF diagnosis [3], though the use of novel immunohistochemical and molecular biology techniques has been discussed as a method of helping with diagnosis [4]. Classic presentation of MF includes patches and plaques on non-sun exposed areas that may slowly evolve [2]. The patient's atypical clinical presentation likely contributed to the difficulty with diagnosis. Treatment goals are typically determined by extent of disease, prognostic factors, quality of life, and patient comorbidities [5]. Staging is determined by skin, lymph node, viscera and blood involvement; in early disease, prognosis is generally favorable [5]. Therapy for early disease often includes topical corticosteroids, topical nitrogen mustard and phototherapy, total skin electron beam therapy and/or low-dose local radiation [5]. Systemic therapy is used in advanced cases or cases refractory to topical therapy. Interferons and retinoids are commonly used first-line, with histone deacetylase inhibitors, alemtuzumab also possible therapeutic agents. Chemotherapy is generally reserved for treatment refractory or rapidly progressive disease [5]. In this patient, chemotherapy was appropriate given his treatment refractory disease, with appropriate alterations in treatment after toxicities developed.

Folliculotropic mycosis fungoides: clinicopathological analysis of 17 patients.

Folliculotropic mycosis fungoides (FMF) represents a variant of MF characterized by hair follicle invasion of mature, CD4-positive small lymphoid cells with cerebriform nuclei. The disease displays resistance to standard treatment modalities and has an unfavourable course. Clinical analysis of 17 patients with FMF collected between 2005 and 2012, investigation of tumour cells and involved hair follicle. Re-evaluation of clinical data, wide panel immunohistochemistry investigation on paraffin-embedded biopsy material, T-cell receptor gene rearrangement analysis of the samples. Male and older age group predominance, frequent head-neck involvement, acneiform lesions, keratotic plugs, cysts, nodules, follicular papules, alopecia and classic mycosis fungoides-like plaques represented the main clinical characteristics. Treatment response showed a wide range from transient complete response to therapy resistance and death due to the disease. The pathological alterations: folliculotropism, mild epidermotropism, follicular plugging, mucinous degeneration of hair follicle, basaloid hyperplasia, syringotropism were similar to those observed previously. The first case of a CD8-positive folliculotropic mycosis fungoides - with unusual clinical presentation - is reported here. Nestin overexpression of mesenchymal cells of the isthmic and suprabulbar regions of hair follicle and the reappearance of dermal nestin-expressing cells were observed in association with immature dendritic cell hyperplasia. Altered CK19 expression was detected suggesting a potential role of follicular keratinocytes in the disease process. It was found that a proportion of neoplastic T cells constantly express programmed death-1 receptor in our patients contrary to classic mycosis fungoides. The spectrum of the clinical manifestation and the course of folliculotropic mycosis fungoides are broad and differ from classic mycosis fungoides. Folliculotropic neoplastic T-cell proliferation is associated with activation of inflammatory reactive T- and B-lymphoid cells, mesenchymal cells and changes in the hair follicle.

Cytotoxic Variant of Mycosis Fungoides with CD8+ CD56+ Phenotype: A Case Report and Review of Literature.

Mycosis fungoides (MF) is the most common cutaneous Tcell lymphoma. Approximately 50% of all primary cutaneous lymphomas are MF[1]. These lymphomas are comprised of epidermotropic collections of small- to medium-sized T lymphocytes with cerebriform nuclei. The neoplastic cell shows a mature CD3+, CD4+, CD45RO+, CD8– memory T-cell phenotype. However, rarely, MF with a CD4–, CD8+ cytotoxic T-cell phenotype has been reported[2-5]. The World Health Organization (WHO)-European Organization for Research and Treatment of Cancer (EORTC) classification for cutaneous lymphomas describes three types of cutaneous lymphomas that express CD56: 1) subcutaneous panniculitis-like T-cell lymphoma, 2) extranodal natural killer (NK)/T-cell lymphoma, nasal type, and 3) CD4+/CD56+ hematodermic neoplasm (blastic NKcell lymphoma)[1]. However, the report does not mention, MF with CD56 expression. Earlier in 2003, a report by the EORTC cutaneous lymphoma task force workshop described cytotoxic/natural killer cell cutaneous lymphomas and divided them into eight categories[6]. One of these categories was the CD56+, cytotoxic variant of MF. This report presents a rare CD8+, CD56+ variant of MF having the cytotoxic phenotype.

Clinical Features and Histological Findings of 17 Patients with Chronic Actinic Dermatitis

ObjectivesThis study was designed to evaluate the clinical, histological and immunohistochemical findings and treatment of chronic actinic dermatitis in Korean patients. MethodsSeventeen Korean patients with chronic actinic dermatitis were enrolled for this study. The clinical and histological findings and the results of phototest were reviewed with medical records, clinical photographs and pathologic slides. We also reviewed the effectiveness of the treatments in all patients with chronic actinic dermatitis. ResultsIn all patients with chronic actinic dermatitis, pruritus was severe, and the patients present in the early stages with erythemas on the face, neck and the back of the hands. As the eruption progresses, it became lichenified and scaly plaques and papules developed. The face, upper extremity and neck were most commonly affected. The most common abnormal results of the phototests were decreased MED-UVB alone. In 8 patients with actinic reticuloid, histopathologic findings showed irregular acanthosis, parakeratosis, spongiosis, atypical hyperchromatic cells with cerebriform nuclei, epidermotropism, Pautrier-like microabscess, deep perivascular lymphocytic infiltrates, vertically-streaked collagen in the papillary dermis, stellate and multinucleated fibroblasts. Treatment includes topical tacrolimus and corticosteroid, oral corticosteroid, azathioprine and cyclosporine. ConclusionsOur study showed classic clinical and histological findings. The most common abnormal results of the phototests were decreased MED-UVB alone. Topical steroid, tacrolimus and systemic cyclosporine, azathioprine are effective in treating chronic actinic dermatitis.

Mycosis fungoides in children and adolescents

Mycosis fungoides (MF) is a low-grade lymphoproliferative disorder that is histologically characterized by an epidermotropic proliferation of small or medium-sized atypical T lymphocytes with cerebriform nuclei and is primarily a disease of the elderly. It is rare in children and adolescents; in large series of MF published in the literature, only a small percentage of patients were observed to have onset of MF below adolescence. It was believed that a younger age of onset of MF may herald a poorer prognosis. Recently, with improved knowledge of the basic nature of the disease and refined diagnostic facilities, a few series of MF among children and adolescence have appeared and have drawn more awareness to the behavior of MF in this age group. This review summarizes the clinicoepidemiological features, treatment and prognosis of MF among children and adolescents based on the current literature.

Sezary syndrome: diagnosis and management

We describe a case of a delayed diagnosis of Sezary syndrome in a patient with longstanding generalised pruritus, erythroderma, alopecia and nail dystrophy. We highlight some of the difficulties in diagnosis despite the fact that all patients have a characteristic circulating malignant T-cell lymphocyte in peripheral blood. These cells with cerebriform nuclei should be sought in peripheral blood films and further evaluated using flow cytometry in all patients with an erythrodermatous presentation. We summarise current treatment options although, as with all rare diseases, there are few randomised controlled clinical trials to inform decisions on optimal therapy for each individual patient.

Fine needle aspiration cytology of unilesional mycosis fungoides d′emblee

Primary cutaneous T-cell lymphoma is a rare lymphoproliferative disorder accounting for 2% of all lymphomas. Mycosis fungoides (MF) is a rare, albeit commonest form of primary cutaneous T cell lymphoma. MF d’emblee is an uncommon variant which is easily mistaken clinically for epithelial malignancy. Diagnosis at cytology is challenging due to low degree of suspicion, rare occurrence and diverse morphology. We report a case of 51-year-old male presenting with a solitary nodulo-ulcerative lesion over right thigh. Smear showed atypical lymphocytes with hyper-convoluted cerebriform nuclei along with few mature lymphocytes consistent with MF. To our knowledge, this is the first report of unilesional MF d’emblee diagnosed at fine needle aspiration cytology (FNAC). Our case emphasizes that FNAC is an accurate method for early diagnosis and clinical staging of patients with MF.

Tubulolobular carcinoma of the breast with grooved and cerebriform nuclei: Failure to identify this specific subtype in a case during routine fine needle aspiration cytology and histopathological diagnosis

Tubulolobular carcinoma (TLC) is a rare tumor of the breast in which histologic features of both tubular and lobular carcinoma are combined. We report a case of TLC, in which the specific subtype was missed at routine cytologic and histopathological examination. A 69-year-old woman presented with a right breast lump. Imaging studies indicated a malignant lesion in right upper quadrant. Routine fine needle aspiration (FNA) cytology diagnosis was a duct cell carcinoma (small cell type). In a setting of cystic thyroid lesions, presence of excessive nuclear grooves, and rare intranuclear cytoplasmic inclusion, metastatic papillary thyroid carcinoma was also considered. However, both these possibilities were not supported by immunocytochemical findings (estrogen receptor+, thyroglobulin-, and chromogranin-). The histopathology diagnosis was invasive duct cell carcinoma. Review of FNA smears and paraffin sections led to the diagnosis of TLC, which was supported by positive immunohistochemical stainings for markers like e-cadherin and β-catein.

Naproxen-induced pseudolymphoma syndrome: a case report

Drug-induced pseudolymphoma syndrome is a rare form of adverse cutaneous drug reaction. Its clinical and histological presentation may mimic mycosis fungoides, which sometimes leads to misdiagnosis and unnecessary treatments. We describe the case of a 65-year-old man with a generalized pruritic and confluent maculopapular eruption. His history of skin lesions was concordant with medication and resolved after discontinuation. However, microscopic examination showed characteristic features of mycosis fungoides, including Pautrier-like microabscesses and cerebriform nuclei of atypical lymphocytes. The immunohistochemical study showed CD4/CD8 infiltrate ratio to be 1 in the epidermis. The histopathological result of a second skin biopsy was compatible with drug eruption. This experience demonstrates that combining clinical history, histological and immunohistochemical findings is crucial for a definite diagnosis of PLS.

Primary cutaneous T-cell lymphoma: Two rare presentations

Cutaneous T-Cell Lymphoma (CTCL) is a subset of non-Hodgkin's T-cell lymphomas which presents with malignant lymphocytic infiltration of the skin. The disease spectrum is characterized by clonal proliferation of helper T-cells of the CD4 phenotype in the skin. Mycosis Fungoides (MF), the commonest variant of CTCL, is characterized clinically by an indolent clinical course with subsequent evolution of patches, plaques andtumours and histologically by the infiltration of the epidermis by medium-sized to large atypical T-cells with cerebriform nuclei [1]. Although not very rare worldwide, it is commonest among Africans and relatively uncommon among Asians. Hypopigmented MF is a rare variant of patch stage MF in the dark skinned. Folliculotropic MF is an uncommon variant characterized by folliculotropic T-cell infiltrates with or without mucinous degeneration of the hair follicles.

Mycosis Fungoides in the Pediatric Population: Report from an International Childhood Registry of Cutaneous Lymphoma

There are limited data on the clinical presentation and progression of pediatric cutaneous lymphoma. This study focuses on the clinical characteristics of pediatric patients with mycosis fungoides (MF). This descriptive study presents clinical characteristics of 22 pediatric patients with MF, enrolled in the international Childhood Registry for Cutaneous Lymphomas (CRCL). The mean ages at onset and at diagnosis were 7.5 (SD 3.8 years) years and 9.9 (SD 3.4) years, respectively. The most common MF presentation was patch stage (68%), followed by hypopigmentation (59%) and plaque stage disease (50%). Epidermotropism and lymphocytic atypia were the most common pathologic features, found in 89% and 85%, respectively. Cerebriform nuclei were noted in 42%, and Pautrier microabscesses were seen in 16% of cases. A cytotoxic pattern was more commonly seen (67% vs 33%), and clonality was detected in 21% (3 of 14) of patients. All patients presented with early-stage disease and received skin-directed therapy (topical steroids, 73%; light therapy, 54%; or combination therapy, 35%). Pediatric patients with MF present in the first decade of life, with early-stage disease and unusual forms such as hypopigmented variant. Further patient enrollment will provide information regarding natural history, treatment response, and overall prognosis of pediatric cutaneous T-cell lymphoma (CTCL).

Mycosis fungoides presenting as a pustular eruption

A 78-year-old woman presented with a generalized and figurate erythema with marginal lamellar scaling and pustulation. In addition, scaly, erythematous, and atrophic plaques with occasional pustules were found on the trunk, arms and legs (Fig. 1). Several skin biopsy specimens showed two distinct phenomena: a) a sub- and intracorneal pustule and b) a bandlike lymphocytic infiltrate of atypical lymphocytes with cerebriform nuclei. The atypical lymphocytes showed extensive epidermotropism and intraepidermal abscess formation (Fig. 2).

Investigation on clinicopathologic features of lymphomatoid papulosis

To investigate the clinicopathologic features, immunophenotype and prognosis of lymphomatoid papulosis (LyP). Clinicopathologic analysis, immunohistochemical staining (LSAB and EliVision method) and in situ hybridization for EBER were undertaken in this study. Thirteen cases of LyP were studied, derived from six male and seven female patients with a median age of 26.4 years. The most common presentation was multiple symptomless papules or nodules, involving predominately the extremities and trunks. Histologically, the tumor primarily involved the dermis and subcutaneous layer. Six tumors were type A, one was type B and six were type C. The main infiltration patterns were wedge-shaped, band-like, sheet-like or nodular. There was epidermotropism in eight cases. Immunohistochemical staining showed that the large tumor cells in all 12 types A and C cases expressed CD30. All 13 cases expressed two to three T-cell associated antigens (CD3, CD5 or CD45RO) and one to three cytotoxic granule associated antigens (TIA-1, GrB or Perforin). All cases expressed CD4, four expressed CD8, and one expressed CD15. Only one case expressed CD20; and all cases were negative for ALK-1. The tumor cells showing epidermotropism had CD3(+), CD4(+) and CD8(-) phenotype in most cases. Only one case was EBER1/2 positive. Follow up information was available in 12 patients; all were alive at the end of the follow up period. LyP has distinctive clinicopathologic features and immunophenotype with favorable prognosis. In types A and C, the atypical cells showing epidermotropism were similar to those in MF, these cells possess cerebriform and hyperchromatic nuclei. The epidermotropic tumor cells and the CD30(+) large cells may be derived from different clones. EB virus may not be correlated with LyP.

CD8+ Lymphomatoid Papulosis in a Patient with Simulataneous Type A, Type B, and Type C Lesions

Lymphomatoid papulosis (LyP) is a cutaneous CD30+ lymphoproliferative disorder characterized by recurrent papulonecrotic or papulonodular lesions with histologic features of a malignant lymphoma and an excellent prognosis. Three morphologic types (A, B, and C) of LyP exist and typically display an immunophenotype of CD4+, CD8−, and CD30+. We present a case of a 63‐year‐old woman with a one‐year history of relapsing papulonecrotic skin eruptions. Biopsy of a lesion on the forearm showed a superficial and deep dermal wedge‐shaped infiltrate with large lymphoid cells (Type A LyP). A lesion on the abdomen showed a band‐like superficial dermal infiltrate composed of small lymphoid cells with extensive epidermotropism and cerebriform nuclei (Type B LyP). Lastly, a lesion on the buttock histologically showed a dense, diffuse dermal infiltrate composed of a mixture of small and large lymphoid cells (Type C LyP). In all three lesions, the atypical lymphocytes were immunoreactive to CD8 and CD30 and nonreactive to CD4. CD8 positive cases of LyP are rare in the literature and tend to occur in pediatric patients. Moreover, while approximately 10% of patients may present with lesions of both the type A and type B types, cases showing all three types of LyP are exceedingly rare.

Complete Cytogenetic Response in a Case of T-PLL with a Complex Karyotype after Treatment with Alemtuzumab.

T-prolymphocytic leukemia (T-PLL) is an aggressive T-cell leukemia characterized by the proliferation of prolymphocytes with a mature postthymic T-cell phenotype and commonly involves the blood, bone marrow, lymph nodes, liver, spleen, and skin. Leukemic cells appear as small to medium-sized prolymphocytes or small variant cells, occasionally with cerebriform nuclei. T prolymphocytes are CD2+, CD3+, and CD7+ and may express CD4+CD8−, CD4+CD8+, or CD4−CD8+ markers. The most frequent chromosomal abnormalities are t(14;14)(q11;q32), inv(14)(q11q32), t(X;14)(q28;q11), i(8)(q10), and t(8;8)(p12;q11). Although patients treated with conventional chemotherapy generally have a poor prognosis, alemtuzumab (Campath®) has been associated with good clinical responses in patients with T-PLL. Routine cytogenetic analysis to determine the response to alemtuzumab has not been performed in patients with T-PLL. This study reports the complete cytogenetic remission achieved following alemtuzumab treatment in a single patient with T-PLL. The 41-year old male patient presented with weight loss, night sweats, diffuse lymphadenopathy and hepatosplenomegaly, in the absence of any skin rash. Peripheral blood counts were: platelets 128 x 109/L and leukocytes 93.6 x 109/L, and hemoglobin values were 12.5 g/dL. The lymphocytes were mostly medium-sized with round to irregular nuclei; some cerebriform cells were also observed, with intermediate chromatin, single evident nucleoli, and a basophilic cytoplasm. Human T-cell lymphotrophic virus-1 serology was negative. Immunophenotypic studies showed CD2+, CD3+, CD5+, CD7+, CD4−, CD8+, CD1−, terminal deoxynucleotidyl transferase-negative (TdT-), and T-cell receptor a/b+. Classic cytogenetics and spectral karyotyping (SKY) were performed and an abnormal karyotype was observed in 18 metaphases analyzed: 46, t(X;14)(q28;q11), t(Y;14)(q12;q11), r[i(8)(q10)]. These abnormalities have not been previously reported in cases of T-PLL. The patient then underwent chemotherapy with CHOP, and subsequent therapy with fludarabine. The patient was then treated with alemtuzumab, 30 mg twice a week, for a total of 7 doses. The patient achieved complete clinical, hematological, and cytogenetic remission, characterized by lack of symptoms, reduction of lymph nodes, liver, and spleen size to normal volumes, and the disappearance of prolymphocytes from peripheral blood. Cytogenetic studies performed 7 months after treatment by classic cytogenetics and SKY revealed a karyotype of 46,XY[20]. This study is the first to demonstrate a complete cytogenetic remission following treatment with alemtuzumab in a patient with T-PLL that was refractory to standard chemotherapy. This is especially impressive in a patient with a complex karyotype and with different cytogenetic alterations not previously described.

Unclassified mature T cell leukemia with cerebriform nuclei

Unclassified mature T cell leukemia with cerebriform nuclei

Cns Involvement From Mycosis Fungoides With CD 30 Transformation

Mycosis fungoides with central nervous system (CNS) involvement is a rare event. Most cases evolve from progressive tumor stage skin disease or CD30 large cell transformation. Cases with CD 30 transformation can show CNS involvement without advanced skin disease. We report a 63 year‐old woman with 6 year history of mycosis fungoides with patch and plaque stage disease who developed a single 2.5 cm tumor on the left chest without lymphadenopathy. Histology revealed a dense infiltrate of small atypical lymphocytes with cerebriform nuclei. Immunohistochemistry showed CD 4+, CD 43+ and CD 30‐ staining. Within 2 weeks she developed ataxia and right‐sided weakness. Magnetic resonance imaging (MRI) revealed left frontal and brainstem masses. Stereotactic biopsy showed a dense infiltrate of atypical lymphocytes with large convoluted nuclei. The immunohistochemistry showed a population of CD 4+, CD 43+, Alk‐1‐ cells with CD 30+ transformation. Gene rearrangement studies for the gamma T cell receptor on the skin patch and tumor as well as brain tumor failed to reveal a monoclonal population in any of the specimens. No other areas of involvement were found on complete staging. This case is an example of tumor stage MF with CD 30 transformation and CNS involvement.

Cutaneous T‐cell Lymphoma of 12 Years Duration Initially Presenting in a Two Year Old

A 14‐year‐old Caucasian girl presented with an eczematous patch on the left proximal inner arm of 12 years duration. She had previously been treated with emollients and topical steroids without complete resolution. The patient denied constitutional symptoms but did note eczematous patches on her buttocks of one‐year duration and new eczematous patches on her inner left thigh and right inner upper arm. Upon exam, all lesions were slightly scaly without induration. The primary lesion was a poikilodermatous 4 × 10 cm rough plaque. No lymphadenopathy was noted. A total of five lesional biopsies were obtained. All of the biopsies demonstrated a superficial infiltrate of atypical lymphocytes with cerebriform nuclei and prominent epidermotropism including the formation of Pautrier’s microabcesses. The diagnosis of mycosis fungoides was rendered. This case is significant for its onset at two years of age, the benignity of its course over a 12 year period and the long duration before definitive diagnosis was obtained. Mycosis fungoides needs to be considered in the differential diagnosis of recalcitrant eczematous disease even in young children.

Cases from the Osler Medical Service at Johns Hopkins University

PRESENTING FEATURES: An 85-year-old black woman presented to the Osler Medical Service complaining of a pruritic, erythematous scaly rash that was on her right thigh and abdomen and that had been worsening over the prior 3 months. She also complained of increasing fatigue, decreased exercise tolerance, and a 5-lbs weight loss. There was no orthopnea, paroxysmal nocturnal dyspnea, fevers, chills, or night sweats. She denied recent travel and exposures to or contact with ill people. Her past medical history was unremarkable. There was no history of eczema, atopy, or dermatologic conditions. Her only medication was a baby aspirin taken daily. On physical examination, she was afebrile, her blood pressure was 110/72 mm Hg, and her pulse was 82 beats per minute with a room air oxygen saturation 98%. She was mildly obese but in no apparent distress. She had 1-cm anterior cervical lymphadenopathy bilaterally and a 1-cm left axillary lymph node. Cardiovascular and chest examination was unremarkable. Her abdomen was soft and nontender, with a faint, erythematous rash that was mildly scaly but nontender in her abdominal skin fold. Her right thigh showed a large erythematous area, approximately 15 x 20 cm, which was scaly with multiple areas of discoloration (Figure 1). There was no palpable mass. She had slight edema in her right leg. Her left thigh was normal. Neurologic examination was nonfocal. Laboratory studies were notable for a white blood cell count of 17,000 cells/microL, with a differential of 46% lymphocytes. The absolute lymphocyte count was 8000 cells/microL. Hematocrit was 28%. Platelet count was normal. A comprehensive metabolic panel was normal. A peripheral blood smear (Figure 2) showed numerous atypical lymphocytes with cerebriform nuclei. Peripheral blood flow cytometry showed the presence of a clonal population of T cells that expressed CD4 and CD5 but showed a loss of CD7. What is the diagnosis?