A case of pachydermodactyly

( Haryeong Ryu ),( Seulki Lee ),( Jeongsoo Kim ),( Chulhyun Yun ),( Jooyoung Roh ),( Jongrok Lee ),( Jinok Baek ) 대한피부과학회 2016 대한피부과학회 학술발표대회집 Vol.68 No.1

Pachydermodactyly (PDD) is a rare, distinct form of digital fibromatosis characterized by acquired, asymptomatic swellings on the back and side of the proximal phalanges or proximal interphalangeal joint areas of the fingers. In case of patients with repetitive hand movements, appreciable improvement has been observed after cessation of the traumatic activity. Intralesional triamcinolone injections and localized surgical resection also have resulted in reduction of soft tissue swelling. A 18-year-old male gave a three year history of progressive swelling of fingers affecting predominantly the proximal interphalangeal joints of the index, middle, 4th, 5th fingers of both hands. The swelling was along the lateral aspects of the joints. There was no history of trauma or pain, and he had a full range of movement. Clinical examination was otherwise unremarkable. He also denied repetitive hand-rubbing movements. There was no family history of the disorder. X-ray of the hands showed soft tissue swelling only, no bony abnormality. Histopathologic examination showed epidermal hyperplasia with acanthosis and hyperkeratosis. Collagen fibers in the reticular dermis are thickened and haphazardly arranged, and increased deposits of connective tissue mucin. Misdiagnosis of the periarticular abnormalities as polyarticular juvenile idiopathic arthritis may lead to needless investigations and inappropriate treatment. Herein, we report a rare case of PDD

[P453] Granulomatous pigmented purpuric dermatosis: : A precursor of mycosis fungoides?

( Haryeong Ryu ),( Seha Park ),( Seulki Lee ),( Jungsu Kim ),( Heejoo Kim ),( Jinok Baek ),( Hyangjoon Park ),( Jooyoung Roh ) 대한피부과학회 2017 대한피부과학회 학술발표대회집 Vol.69 No.1

Pigmented purpuric dermatoses (PPD) are vascular disorders of varying morphology and unknown etiology. There are 5 major forms of PPD and the unusual presentations include a granulomatous form. PPD may be biologically related to mycosis fungoides (MF). A 15-year-old girl presented with purpuric and pigmented macules on left dorsum of foot for two years. The first biopsy from the lesional skin showed a band-like lymphoplasmocytic infiltrate in the superficial and deep dermis with RBC extravasation and pigmentary incontinence in papillary dermis. Lymphocytic atypia and epidermotropism were observed. She was diagnosed as MF based on the first biopsy from other clinic. Second biopsy was performed on 1 month later. It revealed a dense lymphoplasmocytic infiltrate occupying papillary and reticular dermis and epidermotropism was not observed. Immunohistochemically lymphocytes were CD3+, CD4+, CD8+, CD20+, and negative for CD30 expression. T-cell receptor gene rearrangement analysis performed on the second biopsy demonstrated polyclonality. Laboratory findings, peripheral blood smear, and PET CT were all within normal limits. For early phase of MF, histopathologic, immunohistochemical, and molecular work-up are not sufficient for definite diagnosis. Taken together with a progressive clinical course and peculiar pathologic findings, it should be monitored for the development of early phase MF superimposed on granulomatous variant of PPD.

A case of pediatric hidradenitis suppurativa in girl

( Haryeong Ryu ),( Seulki Lee ),( Jeongsoo Kim ),( Chulhyun Yun ),( Jooyoung Roh ),( Jinok Baek ),( Jongrok Lee ) 대한피부과학회 2016 대한피부과학회 학술발표대회집 Vol.68 No.1

Hidradenitis suppurativa is a chronic, recurrent inflammatory disorder of the hair follicles that affects apocrine gland bearing sites, such as the axillae and inguinal and perianal areas. Hidradenitis suppurativa is uncommon in children, with onset generally occurring well after puberty, typically between 20 and 24 years of age. We present an 8-year-old girl whose lesions commenced at the age of 6 years as painful nodules in the buttock. There was no family history of hidradenitis suppurativa or severe acne. Examination revealed few erythematous papules, nodules, and atrophic scars in the buttock. Axillary lesion was absent. She had not attained menarche and the breasts and pubic hair were Tanner stage 1. No evidence of androgen excess was noted on physical examination. A skin biopsy showed superficial dermal perivascular lymphocytic infiltration and presence of acute inflammation of apocrine glands. After the exclusion of bacterial and mycotic infections, a diagnosis of hidradenitis suppurativa was made based on the characteristic clinical presentation and the skin biopsy result. The patient had previously been treated with oral and topical antibiotics for intermittent periods with no improvement. She was treated oral roxithromycin (2.5 mg/kg/day) which marked improvement was noted after 4 weeks, and 2 months later she was in complete remission. Herein, we report a rare case of hidradenitis suppurativa in pediatric.

A case of Woringer-Kolopp disease on the hand

( Haryeong Ryu ),( Jeongsoo Kim ),( Chulhyun Yun ),( Jihoon Kim ),( Hyunchul Kim ),( Jongrok Lee ),( Jooyoung Roh ),( Jinok Baek ) 대한피부과학회 2015 대한피부과학회 학술발표대회집 Vol.67 No.1

Woringer-Kolopp disease is a rare variant of mycosis fungoides and it is also known as localized pagetoid reticulosis. It is slowly presented as a solitary, localized hyperkeratotic patch or a plaque on the extremities follows a benign course. Effective treatments for Woringer-Kolopp disease include skin-directed therapies such as topical nitrogen mustard, high-potency topical steroids, and phototherapy that may include PUVA, narrow-band UVB, or both. For small and localized lesions, surgical excision may be pursued. A 39-year-old man had presented with a 3-month history of an asymptomatic plaque on his hand. Physical examination showed a solitary round, erythematous, scaly plaque, 10 mm in size, with a slightly raised edge, on the dorsum of his left hand. A skin biopsy showed numerous atypical lymphocytes had infiltrated into the upper dermis and expanded into the epidermis with a pagetoid pattern. These pagetoid atypical cells were strongly positive for CD3, CD8, T-cell intracellular antigen-1, and they were negative for CD20, CD30 and CD56. Some reactive lymphoid cells in the dermis were positive for CD4. Further general examination revealed no evidence of systemic involvement. He was treated with surgical excision, and there has been no recurrence during the last 9 months of follow-up period. Herein, we report a rare case of Woringer-Kolopp disease.

Severe cutaneous adverse effects in patients after Influenza vaccination

( Haryeong Ryu ),( Jeongsoo Kim ),( Chulhyun Yun ),( Jihoon Kim ),( Jinok Baek ),( Jooyoung Roh ),( Jongrok Lee ) 대한피부과학회 2015 대한피부과학회 학술발표대회집 Vol.67 No.1

Influenza vaccines are the primary method for controlling influenza and its complication. In Korea, annual influenza immunization for all children 6 months through 59 months of age and children who are at increased risk of severe complications from influenza viral infection is included in the national immunization program. Severe side effects of influenza vaccination are rare. Mild adverse reactions of influenza vaccination are relatively frequent, such as erythema, pains, fevers, myalgia, headaches and so on. We report rare cases of severe cutaneous adverse effect in two patients after influenza vaccination. The first case is Stevens-Johnson syndrome in a 3-year-old boy which developed 4 days after influenza vaccination. Physical examination showed erythematous scaly patches on his whole body, bilateral conjunctiva injection and superficial erosion with crust on lips. He had been egg allergy. His serum was determined to be high levels of white egg specific allergen. The patient was treated with systemic steroid, topical steroid and antihistamine for 2 weeks, the lesions were improved. The second case is generalized exanthematous eruption in a 4-year-old boy which developed 6 days after influenza vaccination. Confluent erythematous maculopapules were located on his whole body. The patient was treated with systemic steroid and antihistamine for 1 week, the lesions were improved.

P091 : Clinical efficacy of alitretinoin in patients with palmoplantar pustular psoriasis

( Chulhyun Yun ),( Haryeong Ryu ),( Jihoon Kim ),( Joonseok Choi ),( Jinok Baek ),( Jongrok Lee ),( Jooyoung Roh ) 대한피부과학회 2014 대한피부과학회 학술발표대회집 Vol.66 No.2

Background: Palmoplantar pustular psoriasis(PPP) is a chronic inflammatory skin disease which is often resistant to treatment. Oral alitretinoin is a vitamin A derivative that has emerged as a novel treatment for recalcitrant chronic hand eczema. The effect of alitretinoin for recalcitrant PPP is still poorly investigated. Objectives: The aim of this study is to evaluate the therapeutic effect of alitretinoin in recalcitrant PPP. Methods: Six patients with moderate to severe PPP unresponsive to conventional treatment were treated with oral alitretinoin 30mg once daily for 12 weeks. Efficacy was assessed at week 12 by modified Palmoplantar Pusutular Psoriasis And Severity Index (m-PPPASI), Physician’s Global Assessment (PGA), Patient’s Global Assessment (PaGA). Results: At baseline, the mean m-PPPASI was 26.03 (range: 5.0-43.8). The mean m-PPPASI score at week 12 was 3.88 (range: 0-10.7). At week 12, 83.3% (n=5) of patients achieved an m-PPPASI 75. PGA response of ‘clear (n=2, 33.3%)’ or ‘almost clear(n=1, 16.6%)’ was reported in 50.0% of patients. PaGA results were consistent with PGA in 3 patients rating their disease as ‘clear(n=2, 33.3%)’ or ‘almost clear(n=1, 16.7%)’ at the end of treatment. Conclusion: Alitretinoin might be suggested as a new treatment modality for the patients who have failed to respond to conventional therapy. Further randomized controlled study with large number of patients and comparison with conventional treatment is pending.

Squamous cell carcinoma in situ successfully treated with ingenol mebutate 0.015%

( Jeongsoo Kim ),( Seulki Lee ),( Haryeong Ryu ),( Chulhyun Yoon ),( Jinok Baek ),( Jongrok Lee ),( Jooyoung Roh ) 대한피부과학회 2016 대한피부과학회 학술발표대회집 Vol.68 No.1

Cutaneous squamous cell carcinoma(cSCC) is the second most common skin cancer in the US. Many cSCCs are small low-risk cancers and are usually effectively managed using minor surgical procedures. However, alternative treatment options are sometimes required, for example, when a patient refuses a surgical procedure, surgical treatment is contraindicated due to possible morbidity or cosmetic deformity and patient condition is compromised by age or the presence of comorbidities. Topical therapy is usually preferred by these patients. Ingenol mebutate has a dual mechanism of action involving the rapid induction of cellular death and cellular cytotoxicity triggered by neutrophils and antibodies. And it has limited local side effect and is applied easily. A 92-year-old Asian woman presented at our dermatology clinic with an erythematous, rough, oozing, crusted lesion of the scalp. In 2011, she has been diagnosed with actinic keratosis of the scalp but dropped out. At this presentation the lesion had became more severe and was biopsied under the suspicion of SCC. We decided to treat the lesion with ingenol mebutate gel (0.015%) daily, and administered two cycles of 3 consecutive days per month for two months. One month after the second cycle the lesion had almost resolved and this was confirmed by biopsy. We report a rare case of cSCC in situ that was treated by ingenol mebutate that is considered an alternative to surgery.

Marginal zone B-cell lymphoma associated with MALT lymphoma of parotid gland

( Jeongsoo Kim ),( Seulki Lee ),( Haryeong Ryu ),( Chulhyun Yoon ),( Jinok Baek ),( Jongrok Lee ),( Jooyoung Roh ) 대한피부과학회 2016 대한피부과학회 학술발표대회집 Vol.68 No.1

Cutaneous Marginal zone B-cell lymphoma (CMZL) is known as extranodal mucosa associated lymphoid tissue (MALT) lymphoma arising at skin accounting for 11% of various extranodal organs. CMZL has indolent behavior and excellent prognosis but recurrence is common. Typically, it manifests as single or multiple, clustered erythematous or violaceous papules, plaques, or nodules. The lesions occur most commonly on the trunk and upper extremities. Histologically, CMZL is composed of polymorphous infiltrate that includes centrocyte-like, monocytoid, and lymphoplasmacytoid lymphocytes and plasma cells. CMZL can be managed with either conservative skin-directed strategies, such as surgical excision and topical steroid, or radiotherapy. A 78-year-old male patient presented with round elevated soft plaque on his back for 2 years. He was diagnosed as MZL of the parotid gland, and underwent surgical excision, radiotherapy and chemotherapy (CHOP) 9 years ago. Histopathologic of the skin showed nodular infiltrate of lymphocytes forming dense, irregulary shaped aggregates in the reticular dermis to subcutaneous tissue. Immunohistochemistry findings revealed positive for CD20, Bcl-2, CD5 and negative for Cyclin D1. There was no evidence of recurrence of previous lymphoma and no sign of metastasis with PET-CT. He was treated with radiotherapy. We report a rare case of CMZL that was formed from patient who had been diagnosed as MZL of the parotid gland.

Eosinophils play a role in the pathogenesis of psoriasis by stimulating inflammatory cytokine and chemokine production and neutrophil chemotaxis

( Jeongsoo Kim ),( Seha Park ),( Seulki Lee ),( Haryeong Ryu ),( Jinok Baek ),( Jooyoung Roh ),( Hyangjoon Park ),( Heejoo Kim ) 대한피부과학회 2017 대한피부과학회 학술발표대회집 Vol.69 No.2

Background: Eosinophilic cytokine profile including IL-23/Th17 axis and their immunomodulatory interaction with other immune cells imply their possible involvement in psoriasis pathogenesis. Objectives: This study was to examine the potential role of eosinophils in imiquimod (IMQ)-induced psoriasiform dermatitis using ΔdblGATA mice, an eosinophil lineage-ablated mouse line. Methods: After topical IMQ application, inflammatory cytokine gene expression and Inflammatory cell populations were analyzed by qRT-PCR and flow cytometry, respectively. Results: Psoriatic features like epidermal thickening, erythema, and scale were less prominent in ΔdblGATA mice after IMQ treatment. Inflammatory cytokines important in psoriasis, like IL-1β, IL-6, IL-22, IL-23, and IL-17, and Th1-/neutrophil-related chemokines CCL2 and CXCL12 were also downregulated, and neutrophils were constantly decreased in the skin, LN, and spleen of ΔdblGATA mice compared to WT mice after IMQ treatment. Although IMQ application increased eosinophils in the skin of WT mice, the absolute cell count was much lower than neutrophils, a major player in psoriasis. Conclusion: The small size of eosinophil population and constant reduction of neutrophil in ΔdblGATA mice than WT mice after IMQ treatment suggest that eosinophils would influence psoriasis indirectly via their inflammatory profile and affecting other mediators in psoriasis, such as neutrophils.

High fluence 1064-nm Q-Switched Nd:YAG laser : safe and effective treatment of cafe-au-lait macules in Asian patients

( Jeongsoo Kim ),( Seha Park ),( Seulki Lee ),( Haryeong Ryu ),( Heejoo Kim ),( Jooyoung Roh ),( Hyangjoon Park ),( Jinok Baek ) 대한피부과학회 2017 대한피부과학회 학술발표대회집 Vol.69 No.2

Background: Cafe-au-lait macules (CALMs) are benign cutaneous hyperpigmentary disorders. Usually, laser therapies for cosmetic concerns result in more severe side effects in the people of Asian descent than that of Caucasians. Unfortunately, there is no gold standard for the laser treatment of CALMs in skin of people of Asian descent. Objectives: To investigate the efficacy and safety of a high fluence 1064-nm Q-switched neodymium-doped yttrium aluminum garnet (Nd:YAG) laser treatment of CALMs in Asian patients. Methods: The medical records of thirty-five Korean patients (age range: 1 to 40 years old, mean age : 18.5 years) diagnosed with isolated CALMs were reviewed retrospectively. The patients were treated with a 1064-nm Q-switched Nd:YAG laser. The parameters were a spot size of 7 mm, a fluence of 2.2-2.4 J/cm2 with a slow single sliding-stacking pass, and a pulse rate of 10Hz with a one-week interval for 20-50 sessions. Results: At the week of the final treatment, all treated CALMs showed considerable pigmentation removal without any permanent side effects, such as scaring, mottled hypopigmentation, and post-inflammatory hyperpigmentation (PIH). All treated CALMs showed more than 50% clinical improvement. No recurrence was observed in any of the patients after 12 months of follow up. Conclusion: A high fluence 1064-nm Q-switched Nd:YAG laser treatment of CALMs in Asian patients is a safe and effective method without side effects and recurrence.