A Case of Peripheral T-cell Lymphoma, Unspecified Presenting as Multiple Ulcerative Tumors

( Dong-uk Cheon ),( Ju-wang Jang ),( Sung-soo Han ),( Hyun-min Seo ),( Joung-soo Kim ) 대한피부과학회 2019 大韓皮膚科學會誌 Vol.57 No.3

Peripheral T cell lymphoma, unspecified (PTCL-U) comprises a heterogenous group of mature T-cell lymphomas that do not match with any defined T-cell entities in the current classification system. A 68-year-old man presented with extensive erythematous to brownish ulcerative tumors with yellowish discharge on the neck, trunk, and both upper extremities that had persisted for the past 7 months. Histological findings showed medium- to large-sized pleomorphic lymphocytes with cellular atypia infiltrating the deep dermis and subcutis. Immunohistochemical analysis of specimens from this patient revealed positive staining for CD2, CD45, and granzyme B and mildly positive staining for CD3, CD4, CD30, and CD79a. Based on these clinico-pathological findings, the patient was finally diagnosed with PTCL-U. We report herein a rare case of PTCL-U presenting as multiple ulcerative tumors. (Korean J Dermatol 2019;57(3):152∼155)

[FCT 12] A case of lichen amyloidosis treated with oral alitretinoin

( Dong Uk Cheon ),( Won Seon Koh ),( Oh Su Jin ),( Jeong Eun Kim ),( Joo Yeon Ko ),( Young Suck Ro ) 대한피부과학회 2017 대한피부과학회 학술발표대회집 Vol.69 No.1

Lichen amyloidosis (LA) is a pruritic eruption of multiple hyperkeratotic and hyperpigmented papules frequently distributed over shins and extensor aspect of arms. Although several therapeutic strategies for LA were reported, standardized treatment has not been established. Up to now, there has been only one case report, in which LA was successfully treated with alitretinoin (9-cisretinoic acid) originally approved for the treatment of recalcitrant chronic hand eczema. A 49-year-old woman presented with localized brownish papules on the left forearm and right elbow for 3 months. Although she has taken oral cyclosporine (100-300 mg/day) and applied topical steroid for the treatment of atopic dermatitis (AD) for 7 years, the brownish papules on forearm and elbow have progressed and became more prominent. A biopsy specimen obtained from a brownish papule of left forearm showed epidermal hyperplasia and homogeneous pinkish materials in the dermal papilla. Congo red stain revealed deposits of amyloid within the dermal papillae. Based on these findings, she was diagnosed with LA and we decided to treat her lesions with oral alitretinoin. 6-month course of oral alitretinoin 30mg/day produced a marked improvement in the thickness and color of the hyperkeratotic papules without aggravation of the patient`s AD. Herein, we suggest that oral alitretinoin can be a valuable therapeutic option for treatment- resistant LA.

[P531] Nevoid hyperkeratosis of the nipple and areola successfully treated with Er :YAG laser

( Dong Uk Cheon ),( Won Seon Koh ),( Su Jin Oh ),( Jeong Eun Kim ),( Joo Yeon Ko ),( Young Suck Ro ) 대한피부과학회 2017 대한피부과학회 학술발표대회집 Vol.69 No.1

Nevoid hyperkeratosis of the nipple and areola (NHNA) is a benign condition of unknown origin characterized by persistent verrucous thickening and hyperpigmentation of the nipple and areola. It mostly affects women in the second or third decade of life, mainly during pregnancy. There is no uniformly effective treatment and several therapeutic options such as topical retinoic acid, topical calcipotriene ointment, cryotherapy, and ablative laser have been tried with variable results. A 22-year-old woman presented with asymptomatic blackish verrucous plaques on both nipples and areola which have developed for 5 years. Verrucous papules first appeared in left nipple and gradually involved areola, which eventually coalesced into extensive plaque lesion. There was neither combined dermatologic nor systemic diseases in our patient. Biopsy specimen from left areola revealed hyperkeratosis, papillomatosis, irregular filiform acanthosis, rete ridge anastomosis, and basal hyperpigmentation. From these clinicopathologic findings, she was diagnosed with NHNA and we started to treat her with Er: YAG laser. Skin lesions improved dramatically with only 1 treatment session and there was no gross remnant lesion. Herein, we report a case of NHNA successfully treated with Er: YAG laser, which has rarely been reported in dermatologic literatures.

A comparative study of the efficacy of H2 receptor antagonist, prokinetics, and mucosal protective agents in improving cyclosporine-induced gastrointestinal complications

( Dong Uk Cheon ),( Ju Wang Jang ),( Won Seon Koh ),( Su Jin Oh ),( Jeong Eun Kim ),( Joo Yeon Ko ),( Young Suck Ro ) 대한피부과학회 2017 대한피부과학회 학술발표대회집 Vol.69 No.2

Background: Cyclosporine (CS) has been used successfully for various skin diseases. However, sometimes it is difficult to manage patients due to its adverse events, especially gastrointestinal (GI) discomforts in their clinical practices. Objectives: The aim of this study was to compare the effectiveness of three GI agents for improving CS-induced GI complications. Methods: We performed a prospective study of 60 patients with CS-induced GI complications. The participants were randomly assigned to receive H2 receptor antagonist (H2RA), prokinetics, or mucosal protective agents (MPA), each of 20 patients. Changes in severity of GI symptoms were evaluated by a validated GI Symptom Rating Scale (GSRS) and Dyspepsia Symptom Severity Index (DSSI) at baseline and at 2 and 4 weeks after treatment. Results: All the medications significantly improved GSRS and DSSI after 2- and 4-week of treatment compared with baseline except that 2-week treatment with prokinetics didn’t improve GSRS. There was no significant difference in effectiveness among the medications but GSRS of the 2-week treatment with MPA was better than that of prokinetics (p=0.046). Conclusion: We confirmed CS-induced GI complications were effectively treated with practical GI agents including H2RA, prokinetics, and MPA. However, considering that prokinetics needs more time to have symptomatic relief, H2RA and MPA would be preferred as practical option to maintain patients’ compliance.

[P530] Treatment of disseminated superficial actinic porokeratosis with oral alitretinoin

( Dong Uk Cheon ),( Sung Soo Han ),( Oh Su Jin ),( Jeong Eun Kim ),( Joo Yeon Ko ),( Young Suck Ro ) 대한피부과학회 2017 대한피부과학회 학술발표대회집 Vol.69 No.1

Disseminated superficial actinic porokeratosis (DSAP) is a chronic keratinization disorder characterized by numerous papules distributed over sun-exposed area. Several therapeutic options showed only limited success without any standard treatment. Up to now, there has been no report about a case of DSAP treated with alitretinoin (9-cis-retinoic acid) approved for the treatment of recalcitrant chronic hand eczema. Patient 1 was a 26-year-old woman presented with 2-year history of brownish macules on both extremities. Histopathologic findings showed keratin-filled invagination of the epidermis and marginal parakeratosis, leading to a diagnosis of DSAP. Treatment with oral alitretinoin (10mg/day) was started and her skin lesions showed marked improvement after 7 months. Patient 2 was a 65-year-old man suffering from pruritic generalized brownish macules for 20 years. Numerous therapies including topical corticosteroid and cryotherapy had been tried without clinical improvement. Histopathologic findings showed a column of parakeratosis overlying an invagination of the epidermis. He was diagnosed with DSAP and oral alitretinoin (30mg/day) was administrated. A 4-month treatment with alitretinoin led to notable clinical improvement. Herein, we suggest that alitretinoin represents a promising treatment option for DSAP.

[P192] Follicular T-cell lymphoma initially presented with hypereosinophilic syndrome

( Dong Uk Cheon ),( Sung Soo Han ),( Oh Su Jin ),( Jeong Eun Kim ),( Joo Yeon Ko ),( Young Suck Ro ) 대한피부과학회 2017 대한피부과학회 학술발표대회집 Vol.69 No.1

Hypereosinophilic syndrome (HES) is a spectrum of entities defined by persistent blood eosinophilia and at least two evidence of tissue eosinophilia without any secondary causes of eosinophilia. HES often presents with various cutaneous manifestation such as pruritic erythematous macu1opapules. Follicular T-cell lymphoma is a unique clinicopathologic entity derivation from follicular helper T cells and categorized from peripheral T-cell lymphoma. A 62-year-old woman presented with itchy erythematous maculopapules on whole body combined with chronic cough and multiple lymphadenopathy which have lasted for 15 months. She was treated with systemic and topical corticosteroid under impression of contact dermatitis and asthma, but there was no improvement. Prominent eosinophilia and high IgE level were observed and biopsy from right forearm revealed eosinophilic infiltration in the upper dermis. Prominent eosinophilic infiltrations were also found at biopsy from stomach and sigmoid colon. Excisional biopsy from inguinal lymph node showed diffuse effacement of nodal architecture composed of pleomorphic atypical lymphocytes, stained positive for CD2, CD3, CD4, CD5 CD21, 23, Bcl-2 and Bcl-6. From these findings, she was diagnosed with HES associated with follicular T-cell lymphoma. Herein, we report a rare case of cutaneous manifestation originated from HES associated with follicular T-cell lymphoma.

Lymphangioma circumscriptum of the vulva

( Dong Uk Cheon ),( Ju Wang Jang ),( Sung Soo Han ),( Hyun-min Seo ),( Hee Joon Yu ),( Joung Soo Kim ) 대한피부과학회 2018 대한피부과학회 학술발표대회집 Vol.70 No.1

Lymphangioma circumscriptum is a benign lymphatic malformation characterized by dilatation of lymphatic channels in deep dermal and subcutaneous layers. It can occur as either a congenital abnormality or acquired damage to previously normal lymphatic channels such as lymphadenectomy and radiotherapy. Generally, it is located in chest, thigh, buttock, axilla and tongue but rarely in the vulva. A 31-year-old woman presented with verrucous plaques around the vulva which has lasted for 10 years. The lesion had gradually increased in size without any subjective symptoms. Punch biopsy was performed under the impression of condyloma acuminatum. Histological examination revealed cystically dilated dermal lymphatics lined by single layer of endothelial cells. Immunohistochemical stains were positive for CD31, CD34 and D2-40. Polymerase chain reaction for human papillomavirus DNA was negative. Based on these findings, the patient was diagnosed with lymphangioma circumscriptum. It was totally excised and the patient has shown no evidence of recurrence for 8 months. Herein, we report a rare case of primary lymphangioma circumscriptum of the vulva.

Subcutaneous fat necrosis of newborn associated with maternal gestational diabetes mellitus

( Dong Uk Cheon ),( Ju Wang Jang ),( Sung Soo Han ),( Hyun-min Seo ),( Hee Joon Yu ),( Joung Soo Kim ) 대한피부과학회 2018 대한피부과학회 학술발표대회집 Vol.70 No.1

Subcutaneous fat necrosis of the newborn is a rare form of panniculitis characterized by firm subcutaneous nodules and plaques on the fat pads-rich portion of the body. It usually occurs in full-term infants with associated perinatal problems such as obstetric trauma, hypoxia, meconium aspiration and maternal gestational diabetes mellitus. Although it generally has benign course showing spontaneous regression within a few months, close monitoring is needed until the complete resolution of the lesion as complications could lead to hypocalcemia which is fetal if untreated. A 6-day-old neonate with a history of maternal gestational diabetes mellitus presented with a 3x3 cm sized erythematous to violaceus indurated plaque on back which has lasted for 2 days. He had a mild fever and leukocytosis. Histological examination revealed lobular panniculitis with a infiltration of lymphohistiocytes and radially arranged needle shaped cholesterol clefts in adipocytes. Based on these findings, he was diagnosed with subcutaneous fat necrosis of newborn. We excluded the possibility of sclerema neonatorum because there was no vasculitis. Herein, we report a case of subcutaneous fat necrosis of newborn, which has rarely been reported in the dermatologic literatures in Korea.

Pustular psoriasis induced by infliximab in a patient with Crohn's disease

( Dong Uk Cheon ),( Ju Wang Jang ),( Sung Soo Han ),( Hyun-min Seo ),( Hee Joon Yu ),( Joung Soo Kim ) 대한피부과학회 2018 대한피부과학회 학술발표대회집 Vol.70 No.1

Infliximab is a chimeric monoclonal antibody inducing potent anti-inflammatory responses by neutralizing biological activity of tumor necrosis factor (TNF)-α. It is widely used to treat various immune system-associated diseases such as Crohn's disease, rheumatoid arthritis, ankylosing spondylitis, psoriasis and psoriatic arthritis. However, infliximab can cause cutaneous adverse effects including erythema, urticaria, pruritus and lupus-like eruption. Rarely, it can provoke paradoxical aggravation or induction of psoriasis. A 28-year-old man with a history of Crohn's disease presented with erythematous scaly plaques with pustules on whole body which has lasted for a month. Punch biopsy was performed and histopathologic findings were consistent with pustular psoriasis. He had been treated with infliximab (10 mg/kg, intravenously) every 8 weeks for the past six years. We stopped using infliximab considering the possibility of paradoxical skin eruptions resulted from infliximab and most of the lesions showed complete regression. Though the exact pathophysiology of this paradoxical skin reaction is not clear, hypothesis has been suggested that a production of interferon-α is induced by dermal plasmacytoid dendritic cells and upregulated certain chemokine receptors provoke the migration and activation of T lymphocytes into the skin. Herein, we report an interesting case of pustular psoriasis paradoxically induced by infliximab.

Drug-induced subepidermal bullous disease with an atypical clinical manifestation

( Dong Seok Shin ),( Ju Wang Jang ),( Dong Uk Cheon ),( Won Seon Koh ),( Jeong Eun Kim ),( Joo Yeon Ko ),( Young Suck Ro ) 대한피부과학회 2019 대한피부과학회 학술발표대회집 Vol.71 No.1

It has been widely accepted that subepidermal bullous diseases could be induced by diverse drugs. Bullous pemphigoid and linear IgA bullous dermatosis are common examples of them. It is important to consider possible association with medication when patients present with bullous skin lesions after receiving multiple regimens. A 47-year-old man presented with generalized tense bullae for 2 weeks. He had been treated with systemic corticosteroid, methotrexate, hydroxychloroquine, NSAIDs, and Tenofovir for 1 year under the diagnosis of rheumatoid arthritis and chronic hepatitis B. Cefixime and proton pump inhibitors had also been administered for 2 weeks to manage infective colitis. Histopathologic examination revealed subepidermal blister but the results of both direct and indirect immunofluorescence studies were all negative. He was treated with systemic corticosteroid and dapsone with cessation of all the other medications. After skin lesions improved, they recurred 2 times since then with taking NSAID and dipeptidyl peptidase-4 inhibitor to treat diabetes mellitus, respectively. Based on these findings, he was diagnosed as drug-induced bullous disease. Herein, we report a drug-induced subepidermal bullous disease with negative immunofluorescence results.