P5915Treatment of eczematoid and vitiliginous graft versus host disease withextracorporeal photopheresis and alemtuzumab

Choon Chiat Oh, MBBS, Singapore General Hospital Dermatology Unit,Singapore; Haur Yueh Lee, MBBS, Singapore General Hospital DermatologyUnit, Singapore

This is a case report of a 36-year-old Indian womanwho underwent peripheral bloodstem cell transplant for anaplastic large cell lymphoma. Three weeks posttransplant,she developed generalized erythema which progressed to erythroderma withdiffuse erythema and scaling affecting [90% of body surface area. There werealso discrete areas of depigmentation on her limbs and trunks. This was associatedwith eosinophilia. She had no previous personal nor family history of atopy. Herdonor (sister) similarly had no previous history of skin diseases. Serial skin biopiesshowed features of spongiosis and scattered apoptotic keratinocytes in the epider-mis and perifollicular region. A diagnosis of eczematoid and vitiliginous GVHD wasmade. Her condition was progressive despite the use of immunosuppressants. suchas prednisolone, mycophenolic acid, tacrolimus. and potent topical corticosteroidtherapy. A decision was made to initiate alemtuzumab as well as extracorporealphotopheresis. This resulted in resolution of her condition and repigmentation ofthe previous depigmented areas.

AB142

cial support: None identified.

Commer

P7073Treatment of vemurafenib-related cutaneous side effects with acitretin

Susan Chon, MD, University of Texas MD Anderson Cancer Center, Houston, TX,United States; Brittany Sambrano, University of Texas Medical School at Houston,Houston, TX, United States; Elizabeth Geddes, MD, University of Texas atHouston Dermatology, Houston, TX, United States

Vemurafenib is a BRAF kinase inhibitor that improves the survival of patients withmetastatic melanoma, who have the V600E BRAF mutation. The development ofcutaneous neoplasms, including squamous cell carcinomas (SCCs), keratoacan-thomas (KAs), and hyperkeratotic papules is one of the most common adverseeffects of this therapy. These lesions usually develop on sun-damaged skin and oftenwithin the first 8 to 12weeks of therapy, suggesting a potentiating effect of the BRAFinhibitor on preexisting mutations. Dermatologists are often challenged by themanagement of these neoplasms. In patients with numerous precancerous andcancerous lesions, repeated cryotherapy, biopsies, and surgeries can cause signif-icant discomfort, disfigurement, increased risk of infection, and a tremendousburden to the patient. Systemic retinoids, such as isotretinoin and acitretin, havebeen used for chemoprophylaxis in individuals at high risk of developing manynonmelanoma skin cancers, such as immunosuppressed solid organ transplantrecipients. These agents may reduce and delay the growth of skin cancers byexerting their effects during the promotion and progression stages of carcinogen-esis. We report a series of two patients (patient A and patient B) with stage IVmetastatic melanoma who presented to our dermatology clinic for evaluation of aflorid eruption of hyperkeratotic neoplasms (verrucae, actinic keratoses, and SCCs)within 1 month of initiating vemurafenib. These numbered over 160 in A and over60 in B. Despite cryotherapy with liquid nitrogen to verrucae and actinic keratoses,plus Mohs micrographic surgery for SCCs, multiple new neoplasms (over 100 and20, respectively) were found at the next follow-up visit 4 weeks later. Within 2months from initial presentation to our clinic, a course of oral acitretin wasprescribed to each patient. After 1 month of acitretin, substantially fewer neoplasmswere observed (6 in A and 0 in B). The patients continued acitretin for 2 months,serum lipids and liver function tests were monitored and remained acceptable.Although not definitive, these cases suggest that acitretin may have a role inchemoprevention of a subset of patients with vemurafenib-associated neoplasmsand slowing the progression of more aggressive SCCs and KAs. Future studies toevaluate this acitretin may substantially improve the morbidity associated withvemurafenib.

cial support: None identified.

Commer

J AM ACAD DERMATOL

P6884Two cases of cutaneous involvement in chronic myelomonocytic leuke-mia: A rare type of leukemia cutis

Elena Sotomayor, MD, Hospital Universitario de La Princesa, Madrid, Spain;Amaro Garc�ıa-D�ıez, MD, Hospital Universiatrio de la Princesa, Madrid, Spain;Javier Fraga, MD, Hospital Universitario de la Princesa, Madrid, Spain; MarLlamas-Velasco, MD, Hospital Universiatrio de la Princesa, Madrid, Spain;Maximiliano Arag€u�es, MD, Hospital Universiatrio de la Princesa, Madrid, Spain;Patricia Garc�ıa-Mart�ın, MD, Hospital Universiatrio de la Princesa, Madrid, Spain

Background: Chronic myelomonocytic leukemia (CMML) is a myelodysplasticsyndrome/myeloproliferative syndrome (MDS/MPN), characterized by a peripheralmonocytosis ([1 3 109/L) associated with dysmyelopoiesis. Specific infiltration ofCMML is uncommon (approximately 30 case reports), we amend 2 clinicallypeculiar cases. Case 1: An 84-year-old man with stable CMML complained of a 1-yearhistory of infiltrated, pruritic, erythematous and purplish papules, widely spread onthe trunk and extremities. Skin biopsy showed a dermal and perivascular infiltrateconstituted by mature lymphocytes mixed with monocytoid atypical cells, whichstained positive for CD43, myeloperoxidase, CD68 and CD123, whereas negative forCD1a and langerin. With these findings we diagnosed specific infiltration of CMML.The hematologic disease continued stable. A month later we started him onprednisone and hydrea with an important improvement of the skin, but unfortu-nately, a month later, he died of a respiratory infection. Case 2: A 78-year-old mandeveloped pruritic erythematous papules and macules that first appeared on hisarms and then spread on his trunk and legs. After a few biopsies that were notconclusive, we finally observed an amorphous proliferation of immature mono-cytoid cells which stained positive for CD43, lysozyme, myeloperoxidase, and CD 68that indicated skin infiltration of CMML. He presented maintained peripheral blood(PB) monocytosis for 4 years with thrombocytopenia episodes and 6 months beforeskin involvement a slight dysmyelopoiesis in the bone marrow aspiration. After theonset of cutaneous lesions, he developed leukocytosis and a new bone marrowbiopsy indicated CMML with blast increase. Patient was treated with hydroxyureabut he died after different infectious complications a few weeks later.

Discussion: Specific infiltration of CMML often presents as an erythematousmaculopapular rash or numerous scattered small nodules usually with a purplecoloration as in our first patient but sometimes they can mimick other dermatoses.Diagnosis is often quite difficult because histopathological findings are not usuallyconclusive and it requires high degree of suspicion an summarize clinical,hystopathologic and analytic findings. It is basic an early diagnosis as cutaneouslesions often precludes a blastic transformation, such as in our second case.

cial support: None identified.

Commer

P6367Wegener granulomatosis of the penis

Julie Iwasaki, MD, Henry Ford Hospital, Detroit, MI, United States; Tor Shwayder,MD, Henry Ford Hospital, Detroit, MI, United States

Wegener granulomatosis is an uncommon multisystem autoimmune disease ofunknown etiology. It usually involves the renal, pulmonary, and respiratory systems.Its hallmark features include necrotizing granulomatous inflammation and vasculitisof small and medium sized blood vessels. We describe a case of a 62-year-old manwho presented with nonpainful necrosis of his penis over the past 4 months. He hadno other systemic symptoms, no other skin lesions, and had no problems withurination. Histopathology of the penis showed necrotizing granulomatous inflam-mation and vasculitis. Laboratory test results showed a C-ANCA level of 320. Thepatient then developed purpuric nodules on his lower legs, which were biopsiedand showed similar vasculitis with granulomatosis. We present this case as necrosisof the penis is a rare initial presentation of Wegener granulomatosis.

cial support: None identified.

Commer

APRIL 2013