Fig 1. Multiple small hyperpigmented papules, vesicles,and violaceous plaques on palms and dorsal aspect ofhands.

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populations is around 2%.5Given the pivotal involve-ment of FcgRIIB in regulating the balance betweentolerance and autoimmunity, the rarity of our pa-tient’s genotype, and her severe clinical course, wespeculate that the FcgRIIB-Ile187Thr polymorphismmay act as an immunogenetic modifier in BP. Theseverity (and chronicity) of this case could be par-tially explained by the presence of -187Thr receptoron B cells, leading to the increased, sustained, andunrepressed production of anti-BP180 antibodies.

Antonio Guilabert, MD,a Francisco Lozano, MD,b

Pilar Iranzo, MD,a Bel�en Su�arez-Casas�us,b

Isabel Martinez-De Pablo,a Marc Juli�a, MD,a

and Jose Manuel Mascar�o, Jr, MDa

Departments of Dermatologya and Immunology,b

Institut de Investigacions Biom�edicas August Pi ISunyer (IDIBAPS), Hospital Clinic of Barcelona,Barcelona, Spain

Supported in part by a grant from the Instituto deSalud Carlos III, Ministerio de Sanidad yConsumo.

Conflict of interest: None declared.

Correspondence to: Antonio Guilabert, MD, De-partment of Dermatology, Hospital Clinic, C/Villarroel 170, 08036 Barcelona, Spain

E-mail: tonovidal@gmail.com

REFERENCES

1. Chen JY, Wang CM, Ma CC, Luo SF, Edberg JC, Kimberly RP, et al.

Association of a transmembrane polymorphism of Fcgamma

receptor IIb (FCGR2B) with systemic lupus erythematosus in

Taiwanese patients. Arthritis Rheum 2006;54:3908-17.

2. Tarasenko T, Dean JA, Bolland S. Fc gamma RIIB as a modulator of

autoimmunedisease susceptibility. Autoimmunity2007;40:409-17.

3. Li X, Wu J, Carter RH, Edberg JC, Su K, Cooper GS, et al. A novel

polymorphism in the Fcgamma receptor IIB (CD32B) trans-

membrane region alters receptor signaling. Arthritis Rheum

2003;48:3242-52.

4. Floto RA, Clatworthy MR, Heilbronn KR, Rosner DR, MacAry PA,

Rankin A, et al. Loss of function of a lupus-associated

FcgammaRIIb polymorphism through exclusion from lipid rafts.

Nat Med 2005;11:1056-8.

5. Magnusson V, Zunec R, Odeberg J, Sturfelt G, Truedsson L,

Gunnarsson I, et al. Polymorphisms of the Fc gamma receptor

type IIB gene are not associated with systemic lupus erythema-

tosus in theSwedishpopulation.Arthritis Rheum2004;50:1348-50.

doi:10.1016/j.jaad.2009.02.017

Adult T-cell lymphoma/leukemia presenting aspagetoid reticulosis of the palms and soles

To the Editor: A 40-year-old Jamaican man presentedwith a 6-month history of a pruritic eruption on hishands and feet. On examination, he had multiple,

violaceous plaques on the palms and soles (Fig 1).Biopsy revealed a lymphocytic infiltrate at thedermoepidermal junction with basal layer epider-motropism and pagetoid mononuclear cell infiltra-tion (Fig 2). The patient reported that he had beenhuman T-cell lymphotropic virus type 1 (HTLV-1)positive since 2001.He was started on a regimen ofPUVA therapy with a presumptive diagnosis of pag-etoid reticulosis (Woringer-Kolopp) or mycosis fun-goides palmaris et plantaris, a variant of cutaneousT-cell lymphoma.

The patient failed to improve and rapidly devel-oped new papules and nodules on his head, neck,chest, and forearms. Flow cytometry revealed lym-phoid cells of varying size with irregular, convolutednuclei (‘‘cerebriform’’ cells). Fifty percent of periph-eral blood lymphocytes expressed CD2, CD4, andCD25, without CD7 or CD8. Liver function test valueswere elevated, and peripheral leukocytes numbered185,000/mm3, with 20% atypical lymphocytes. Hewas hospitalized for hypercalcemia and found tohave inguinal lymphadenopathy; cyclophospha-mide, Adriamycin, vincristine and prednisone(CHOP) chemotherapy was begun. He did not im-prove on this regimen or on zidovudine with inter-feron. He then failed to respond to weeklymethotrexate, cyclophosphamide, prednisone, andpulse intravenous etoposide. Bone marrow allografttransplantation led to initial improvement, but thepatient developed pneumonia and died.

HTLV-1 was the first retrovirus associated directlywith malignancy.1 Twenty million to 30 million peo-ple are infected worldwide, especially in endemicareas: southwest Japan, Central and South America,southeastern United States, Central and West Africa,and the Caribbean. Ninety percent of serologic-positive patients remain asymptomatic,2 with a cu-mulative 3% to 5% lifetime risk of developing adultT-cell leukemia/lymphoma (ATLL).3 Latency be-tween infection and disease is 10 to 30 years.4,5

Fig 2. Skin biopsy specimen from left palm revealscollections of mononuclear cells throughout the epidermisand the cornified layer. There is a patchy band-likelymphocytic infiltration at the dermoepidermal junctionwith focal basal layer epidermotropism. (Hematoxylin-eosin stain; original magnification: 34.)

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HTLV-1 infects several cell types, but primarilyCD41 T-helper lymphocytes.4 Transmission occursprimarily during sexual activity, with intravenousdrug use and blood transfusion and via breast milk.Infection is a prerequisite for ATLL, a malignancywith a spectrum of cutaneous manifestations andvaried clinical course.1 Most patients with ATLLpresent with the leukemic form of the disease.Clinical manifestations include skin lesions, lym-phadenopathy, and hepatosplenomegaly. Skin le-sions are often a presenting sign, ranging fromlocalized papules and vesicles to keloidal nodulesand erythroderma. The unique feature of our patientwas involvement of his palms and soles.

Slowly evolving ATLL often resembles mycosisfungoides, having similar clinical, histopathologic,and immunophenotypic findings, which compli-cates early detection. Diagnosis is aided by exami-nation of peripheral blood cells, with leukemic cellsappearing as characteristic multilobate cells called‘‘flower cells’’.

Immunophenotypic findings of CD21/CD31/CD41/CD7-/CD8-/CD251 also suggest ATLL. Strongexpression of the interleukin 2 receptor a chain(CD25) can help distinguish ATLL from S�ezary syn-drome. Demonstration of clonality of HTLV-1 proviralDNA is essential for diagnosis.

As our patient demonstrated, treatment of ATLLremains disappointing, although clinical trials ofnew therapies such as MCNU and carboplatinappear promising. Our patient also demonstratesthat clinicians should maintain an index of suspicionsufficient to identify such patients when theyappear.

Bethany Grubb, MPAS, PA-C, Deborah B. Henderson,MD, and Amit G. Pandya, MD

Department of Dermatology, University of TexasSouthwestern Medical Center, Dallas

Funding sources: None.

Conflicts of interest: None declared.

Correspondence to: Bethany L. Grubb, PA-C,Department of Dermatology, 5323 Harry HinesBlvd, Dallas, TX 75390-9069.

E-mail: bethany.grubb@utsouthwestern.edu

REFERENCES

1. Uchiyama T. Human T cell leukemia virus type 1 (HTLV-1) and

human diseases. Annu Rev Immunol 1997;15:5-37.

2. Nicot C. Current views in HTLV-1-associated adult T cell

leukemia/lymphoma. Am J Hematol 2005;78:232-9.

3. Muller AM, Ihorst G, Mertelsmann R, Engelhardt M. Epidemiol-

ogy of non-Hodgkin’s lymphoma (NHL): trends, geographic

distribution, and etiology. Ann Hematol 2005;84:1-12.

4. Incan M, Antoniotti O, Gasmi M, Fonck Y, Chassagne J,

Desgrandges C, et al. HTLV-1-associated lymphoma presenting

as mycosis fungoides in an HTLV-1 non-endemic area: a viro-

molecular study. Br J Dermatol 1995;132:983-8.

5. Yamada Y, Tomonaga M. The current status of therapy for adult

T-cell leukaemia-lymphoma in Japan. Leuk Lymphoma 2003;44:

611-8.

doi:10.1016/j.jaad.2010.02.007

Rituximab used as a first-line single agent inthe treatment of pemphigus vulgaris

To the Editor: Pemphigus vulgaris (PV) is a life-threatening autoimmune blistering disease affectingthe skin and mucosa. Patients with severe diseaserequire high-dose, long-term corticosteroids or otherimmunosuppressant drugs and are often slow inachieving a remission. The adverse effects of long-term treatment with such agents are well documentedand associated with a mortality of 5%. There isincreasing evidence for the use of the monoclonalanti-CD20 antibody, rituximab, in the treatment ofsevere and refractory PV.1-4 In these reports rituximabhas been used as an adjuvant agent or after othertreatment modalities failed. We believe this is the firstreport of a patient treated with rituximab as a first-linesingle agent to induce a rapid sustained remission.

A 52-year-old man presented with a 2-monthhistory of painful erosions on the glans penis andfrank blisters and erosions over the torso and face.There was marked ulceration over the buccal mu-cosae and hard and soft palate (Fig 1). Given theextent of oral disease he had a hoarse voice anddifficulty eating, as a result he had lost 10 kg inweight. PV was confirmed on skin biopsy specimenfrom a lesion on the chest and on direct and indirectimmunofluorescence. While awaiting confirmationof the diagnosis oral prednisolone was started at