Folliculotropic Sézary syndrome: Report of a case

P8067Folliculotropic S�ezary syndrome: Report of a case

Brian Hinds, MD, MS, University of Louisville, Louisville, KY, United States; CindyOwen, MD, University of Louisville, Louisville, KY, United States; Janine Malone,MD, University of Louisville, Louisville, KY, United States

Background: S�ezary syndrome (SS) is an aggressive form of cutaneous T-celllymphoma (CTCL) characterized by erythroderma, generalized lymphadenopathy,and circulating neoplastic T-lymphocytes with cerebriform nuclei (S�ezary cells) inthe skin, lymph nodes, and the peripheral blood. Folliculotropic mycosis fungoides(FMF) coexisting with classic SS is a rare phenomenon, previously suggested as anew variant of CTCL, termed folliculotropic SS (FSS).

Case report: A 76-year-old black man presented for evaluation of severe pruritusrecalcitrant to medium potency topical steroids and oral gabapentin. Review ofsystems was positive for night sweats and weight loss. His medical history wassignificant for renal insufficiency and prostate cancer statusepost-brachytherapy.Physical examination revealed widespread exfoliative erythroderma with hyperpig-mented patches and admixed edematous follicular papules. In addition, the patientexhibited leonine facies, madarosis, frontoparietal alopecia, generalized lymphade-nopathy, and keratotic papules on the palms. Skin biopsies obtained from the Lmedial arm, R deltoid, and R upper chest revealed a superficial and deep perivascularand interstitial infiltrate of atypical lymphoid cells. The atypical lymphocytes stainedpositively with CD3 and CD4. CD30 immunostaining was negative and large cellscomprised \25% of the infiltrate. Follicular and perifollicular infiltration byneoplastic T-cells was brisk in some foci and associated with follicular mucindeposition. Lymph node and bone marrow biopsies revealed similar cytologicalfindings. Flow cytometry enumerated an aberrant T cell population with CD4+CD7e

and CD4+CD26e immunophenotype. A diagnosis of FSS (stage IVA1) was estab-lished. Extracorporeal photophoresis (ECP), oral bexarotene, and subcutaneousinterferon alpha produced modest improvement in peripheral T-cell CD4 counts,decreasing by 50% at 6months, but the patient continues to have significant clinicaldisease and guarded prognosis.

Conclusion: FSS is a rare and unique variant of CTCL with only 15 cases described inthe current English literature. It is important to recognize FSS as a new variant ofCTCL, and in doing so, characterize the clinical impact of folliculotropism versusclassic SS. Overall, a diagnosis of FSS should be strongly considered if anerythrodermic patch in a patient with lymphadenopathy and leukemic involvementexhibits salient histologic features of FMF.

AB116

cial support: None identified.

P8060Granulomatous folliculitis and anterior uveitis: A case report

Marcella Markthaler, Hospital universitario de Canarias, La Cuesta, La Laguna,Spain; Ana De Andr�es Del Rosario, Hospital Universitario de Canarias, La Cuesta,La Laguna, Spain; Estela Garc�ıa-Peris, Hospital Universitario de Canarias, LaCuesta, La Laguna, Spain; Francisco Guimer�a Mart�ın-Neda, Hospital Universitariode Canarias, La Cuesta, La Laguna, Spain; Mar�ıa del Mar Pestana-Eliche, HospitalUniversitario de Canarias, La Cuesta, La Laguna, Spain; Marta Garc�ıa-Bust�ınduy,Hospital Universitario de Canarias, La Cuesta, La Laguna, Spain; Rosa Rodr�ıguez-Rodr�ıguez, Hospital Universitario de Canarias, La Cuesta, La Laguna, Spain

Introduction: Granulomatous folliculitis has been described in the context ofmultiple diseases. It is characterized by a granulomatous reaction suited next to thepilosebaceous follicles. It is mostly observed as a reactive pattern of a local skininfection produced by dermatophytes, bacterias or parasites like Demodex. Wepresent a case of rare noninfective granulomatous folliculitis.

Case report: A 59-year-old manwith history of chronic benign idiopathic neutropenia,presents in the context of an anterior uveitis, normochromic, normocytic anemia,bilateral pulmonary calcified hilar adenopathy and splenomegaly, symptomaticpapules on the chest and face. In physical examination multiple papules, some ofthem seated perifollicularly, could be observed. They had an erythematous, brilliantaspect, some centrally umbilicated similar to lesions of molluscum contagiosum, of asize between 2 to 4mm, in a symmetric distribution.Histologic examination revealed agranulomatous infiltration composed of plasmatic cells, epitheloid cells and multinu-cleate giant cells surrounding follicles in the dermis, respecting the apparently normalepidermis. Parameters of infectious or autoimmune diseases were negative. Serumangiotensin converting enzyme (ACE) level was elevated. Complete remission ofuveitis, anemia and skin lesions without scaring and normalization of ACE value couldbeobserved after systemic corticoid treatment.With all this clinical and analytical data,sarcoidosis appeared to be the most probable diagnosis of the patient.

Discussion: Sarcoidosis is a multisystemic disease involving any organ of the body,especially affecting the lungs, lymph nodes, skin, liver, spleen, and eyes. Also thebone marrow can be affected producing anemia. Sarcoidosis must be approached asa diagnosis of exclusion. In the differential diagnosis of noncaseating granulomatoushistology, we have to consider some forms of tuberculosis, tuberculoid leprosy,nodular granulomatous perifolliculitis, primary perforating granulomatous follicu-litis, foreign body reactions, syphilis, S�ezary syndrome, etc.

Conclusion: This casemay represent a rare cutaneous variant of sarcoidosis associatedwith systemic sarcoidosis.Thereareonly a fewcases reported in literatureofcutaneoussarcoidal granulomas related to the pilosebaceous follicles like in our case.

cial support: None identified.

J AM ACAD DERMATOL

P8278Initial stage of elephantiasis nostras verrucosa in a 30 years lasting giantlymphedema

Estela Garc�ıa Peris, MD, Hospital Universitario de Canarias, Santa Cruz deTenerife, Spain; Francisco Guimer�a Mart�ın-Neda, MD, PhD, Hospital Universitariode Canarias, Santa Cruz de Tenerife, Spain; Irene Latour �Alvarez, MD, HospitalUniversitario de Canarias, Santa Cruz de Tenerife, Spain; Marcella Markthaler,MD, Hospital Universitario de Canarias, Santa Cruz de Tenerife, Spain; Mar�ıaPestana Eliche, MD, Hospital Universitario de Canarias, Santa Cruz de Tenerife,Spain; Marta Garc�ıa Bust�ınduy, MD, PhD, Hospital Universitario de Canarias,Santa Cruz de Tenerife, Spain; Rosa Rodr�ıguez Rodr�ıguez, MD, HospitalUniversitario de Canarias, Santa Cruz de Tenerife, Spain

Introduction: Elephantiasis nostras verrucosa is a nonfilarial chronic lymphedemacomplication that causes progressive cutaneous hypertrophy and severe disfigura-tion of the body part affected.

Case report: An86-year-oldwhitewomancame toour officebecause of 30years lastingleft leg chronic lymphedema that progressively developed after lymphadenectomyperformedduring the surgical treatmentof a cervical cancer. Shehadbeen treatedwithdifferent conservative measures by different doctors. Cutaneous lesions started toappear on her lymphedematous leg 10 years ago and they had slowly and progressivebeen worsening until the time of consultation. On examination, edematous papulo-nodules andvegetatingpapuloplaques, giving a verrucous appearance,were observed.Skin biopsy was performed showing hyperkeratosis and hyperplasia in the epidermisand, severe edema with a lymphohistiocytic and plasmatic inflammatory infiltrate inthe dermis. According to the medical history, physical examination, and histologicfindings, a diagnosis of elephantiasis nostras verrucosa was made. Medical treatmentwith topical retinoid was started in order to improve cutaneous surface.

Discussion: Elephantiasis nostras verrucosa (ENV) is an uncommon entity caused bychronic lymphedema. ENV can be either congenital (primary lymphedema) orproduced by a nonfilarial infection or other secondary forms (secondary lymphe-dema). Clinically, it is characterized by the enlargement of an anatomic regiontogether with skin changes such as edema and hyperkeratotic papulonodulesshowing a verrucose or cobblestone-like appearance. ENV may be unilateral orbilateral and it is commonly observed in the lower extremities. Diagnosis is based onpatient history, physical examination and typical cutaneous lesions. Skin biopsy andimaging techniques may be useful to identify secondary causes of lymphedema.Early stages of the disease show a pseudoepitheliomatous hyperplasia with hyper-keratosis of the epidermis and a dermal papillae and sweat glands lost, whileadvanced stages show a fragmentation and separation of elastic fiberswith extensivefibrous tissue hyperplasia in the dermis. ENV is almost always progressive, andusually management of advanced stages results unsatisfactory, hence the impor-tance of a diagnosis as early as possible. Initial treatment includes conservativemeasures and medical management, being surgery the last option. Nevertheless,there is still no standard treatment for this disabling cutaneous illness.

cial support: None identified.

P8556Interstitial granulomatous dermatitis as the presenting manifestation ofmyeloma

Sabra Abner, MD, University of Louisville-Division of Dermatology, Louisville, KY,United States; Janine Malone, MD, University of Louisville-Division ofDermatology, Louisville, KY, United States; Jeffrey Callen, MD, University ofLouisville-Division of Dermatology, Louisville, KY, United States

Interstitial granulomatous dermatitis was first described as a distinct disease entityby Ackerman in 1993. It has been reported to be associated with variousmedications and autoimmune and lymphoproliferative diseases. We present apatient who presented with interstitial granulomatous dermatitis that was foundto have stage I myeloma after systemic evaluation. A 55-year-old white manpresented with a 1-year history of a minimally symptomatic eruption on his upperback, arms, forearms, and hands. There were no changes in his medications for[1year. He had a medical history of a retroperitoneal schwannoma that was excisedwith negative margins 1 month before presentation, a history of diabetes mellitus,hypertension, and arthritis. His medications were metformin, telmisartan, vitaminsB1, B6, and naproxen. On physical examination, he had erythematous to violaceouspatches and plaques, some with trailing scale; 1 on his right forearm had an annularborder and was infiltrated. Punch biopsies revealed a patchy interstitial histiocyticinfiltrate with elastophagocytosis. Laboratory evaluation including a complete bloodcell count, comprehensive metabolic panel, and urinalysis had minimal abnormal-ities. An immunofixation electrophoresis revealed an elevation of his IgA level andanM-spike with IgA kappa that was too small to be quantified. Autoimmune serologywas negative. This patient was referred to hematology for evaluation where a bonemarrow biopsy was performed with 20% to 25% IgA kappa restricted plasma cells.There were no lytic lesions on bone survey. He was started on a bisphosphonate,with no further treatment required, and his rash faded shortly after presentation.This is the first case, to our knowledge, where interstitial granulomatous dermatitishas been the initial presentation of myeloma. This case highlights the importance ofa thorough systemic work-up in patients who present with interstitial granuloma-tous dermatitis.

cial support: None identified.

MAY 2014