Hyun-Ho Cho

Oral Hairy Leukoplakia Which Occurred as a Presenting Sign of Acute Myeloid Leukemia in a Child

Oral hairy leukoplakia (OHL) is caused by the reactivation of a previous Epstein-Barr virus (EBV) infection in the epithelium of the tongue. Most lesions are characterized by corrugated whitish patches on the lateral border of the tongue. It is frequently associated with AIDS, but cases in patients with other immunosuppressed states have also been reported. In leukemia patients, OHL is rarely encountered, and appears only after chemotherapy. We report a case of OHL which occurred as a presenting sign of acute myeloid leukemia (AML) in a previously healthy 15-year-old child. A 15-year-old boy presented with a whitish patch on the left lateral border of the tongue. The biopsy specimen revealed papillomatosis, hyperkeratosis, acanthosis and ballooning degeneration in the stratum spinosum. The patient was EBV seropositive, and PCR analysis of EBV DNA in the lesional tissue was positive. After the diagnosis of OHL in dermatologic department, the patient was referred to pediatrics due to the abnormal peripheral blood smear, and was diagnosed with AML.

Two cases of pyodermatitis-pyostomatitis vegetans

Pyodermatitis‐pyostomatitis vegetans (PPV) is a rare chronic pustular and vegetating mucocutaneous dermatosis. The oral lesions present as multiple, friable and yellowish pustules, which form “snail tracts” and rupture easily. The cutaneous lesions begin as crusted erythematous papulopustules that coalesce to form large vegetating plaques, usually in the axillae, genital area and scalp. Cutaneous lesions usually develop at the same time as the oral lesions or thereafter. PPV is usually considered as a specific marker for inflammatory bowel disease since the concurrence of PPV and inflammatory bowel disease has been reported in approximately 70% of cases. We report two patients who showed typical clinical and histopathological features of PPV, but were not accompanied by inflammatory bowel disease.

Narrowband UVB treatment of progressive macular hypomelanosis

BACKGROUND Little information is available on effective treatments for progressive macular hypomelanosis (PMH). To our knowledge, only one case of narrowband ultraviolet B (NB-UVB) therapy as an efficient treatment for patients with PMH has been reported in the recent literature. OBJECTIVE We aimed to investigate the clinical features of PMH in Koreans and to determine the therapeutic efficacy of NB-UVB therapy in the management of PMH. METHODS We performed an uncontrolled prospective study designed to evaluate the usefulness of NB-UVB therapy in PMH. A total of 23 patients with PMH were enrolled in the study. Of these, 17 patients underwent treatment with NB-UVB therapy once or twice weekly and were eligible for analysis. The remaining 6 patients were lost to follow-up before completion of the treatment. Repigmentation was evaluated by two dermatologists using photographic documentation. RESULTS In our trial, NB-UVB therapy was used successfully in 9 of 16 patients (56.2%), who showed more than 90% repigmentation. We found that 13 of 16 patients (81.3%) experienced at least 50% repigmentation. The repigmented sites showed an excellent color match. No signs of recurrence have been detected in 11 of these 16 patients (68.7%) up to the present time (13.2 ± 8.2 months of follow-up). LIMITATIONS Our study includes a small number of subjects examined, and it was an uncontrolled and non-double-blind study. CONCLUSIONS The findings of this study suggest that NB-UVB therapy is an effective and safe method for use in the treatment of PMH.

Recurrent basal cell carcinoma following ablative laser procedures

BACKGROUND In Korea, many patients diagnosed with basal cell carcinoma (BCC) have a history of laser ablations of undiagnosed lesions. OBJECTIVE To evaluate the clinical/pathological and surgical features of BCC developing from undiagnosed lesions following laser ablations (not full-face cosmetic ablations) and to compare them with primary BCCs. METHODS This study enrolled 359 patients with 373 biopsy-proven BCC lesions. All of the patients were treated by Mohs micrographic surgery (MMS) at the Department of Dermatology, Pusan National University Hospital from 1998 to 2008. BCC was classified by previous treatment history of lesion ablative laser: post-laser BCC vs primary BCC. We conducted a retrospective study through clinical photographs, pathology slides, and MMS sheets. RESULTS Among 373 BCCs, 58 lesions (15.5%) were post-laser BCCs. The post-laser BCC group was younger (59.9 vs 65.4 years, P = .001), but had a longer disease interval until pathologic diagnosis (7.18 vs 3.33 years, P < .0001) than the primary BCC group. The post-laser BCC group had a greater frequency of the micronodular pattern (22.4% vs 10.8%, P = .01), required more stages of excision (2.69 ± 1.63 vs 2.15 ± 1.05, P < .001), and had fewer cases with one Mohs stage excision (10.3% vs 27%, P = .006) than the primary BCC group. LIMITATIONS We could not identify the type of laser used in all 58 cases; instead, we supposed that most of the patients were likely treated with the carbon dioxide laser. CONCLUSIONS The results demonstrated that the post-laser BCC group had a longer disease interval to diagnosis, a more aggressive histologic pattern, and required more stages of excision in MMS than the primary BCC group.

Efficacy and Safety of Calcipotriol/Betamethasone Dipropionate Ointment for the Treatment of Trachyonychia: An Open-Label Study.

Background Despite efforts to treat trachyonychia, there is no promising treatment modality. Objective This study evaluated the efficacy and safety of calcipotriol plus betamethasone dipropionate ointment on trachyonychia. Methods A total of 39 patients with 432 nails affected by trachyonychia were enrolled. All patients applied calcipotriol/betamethasone ointment once daily without occlusion for 6 months. Outcome measures were assessed by physicians global assessment (degree of roughness: 0, clear; 1, mild; 2, moderate; 3, marked; 4, severe) at all time points. Results After 6 months of therapy, 98.6% (426/432) of nails showed significant clinical improvement; 4.2% were completely free from nail lesions. The mean physician global assessment score decreased significantly from 3.5 to 1.7 points (p< 0.05). No serious side effects were reported, except mild pruritus and erythema in 2 patients. Conclusion This is the first study to prospectively evaluate the efficacy and safety of calcipotriol/betamethasone ointment for the treatment of trachyonychia. The results indicate topical calcipotriol/betamethasone is an effective and safe treatment for symptom improvement of trachyonychia.

Two cases of generalized pustular psoriasis: successful treatment with infliximab.

Dear Editor: In case 1, a 41-year-old woman presented with a 3-year history of recurrent pustules on erythematous skin. The patient had been treated with methotrexate, sulfasalazine and cyclosporine for psoriatic arthritis and generalized pustular psoriasis (GPP) for 2 years. However, these treatments failed to yield satisfactory improvement. Physical examination revealed the presence of extensive erythematous patches with pustules over the entire body (Fig. 1A) and inflammatory arthritis was noted in the distal interphalangeal, sacroiliac and knee joints. We decided to use infliximab (3 mg/kg) to treat the GPP and psoriatic arthritis. The patients pustular lesions cleared quickly within 48 hours after the first injection. After the second injection of infliximab (3 mg/kg), marked improvement in arthralgia was observed (Fig. 1B). She has since been receiving infliximab 3 mg/kg every 8 weeks as maintenance therapy. No recurrence of pustules or arthritis symptoms has been detected during 12 months of follow-up. Fig. 1 Pustular psoriasis in case 1 (A). Marked improvement was observed after the second injection of infliximab (B). In case 2, a 39-year-old woman with a 20-year history of plaque type psoriasis was admitted to our department for lower limb cellulitis. Rapid resolution of cellulitis was achieved after systemic antibiotic treatment (cefazolin 6 g/day). Three days after antibiotic treatment, she experienced abrupt onset of pustules on erythematous skin affecting more than 90% of body (Fig. 2A, B). She was initially treated with infliximab (3 mg/kg) combined with acitretin (30 mg/day) for pustular flares. Within 48 hours of the first infusion, the pustules were resolved, but diffuse erythema on the trunk and extremities remained (Fig. 2C, D). The patient received acitretin (20 mg/day) for maintenance after a single dose of infliximab. No relapse of the pustules has been noted, but mild psoriatic plaques have followed. Fig. 2 Generalized pustular papules on the entire body in case 2 (A, B). Near remission of a pustular eruption was seen 48 hours after infliximab infusion (C, D). Infliximab is a chimeric immunoglobulin G1 anti-tumor necrosis factor-α monoclonal antibody that is effective in treating moderate to severe psoriasis and psoriatic arthritis1. Several studies have documented a rapid improvement in recalcitrant GPP with infliximab (5 mg/kg)2,3. Because infliximab has a faster onset of action than that of other modalities including biologics, some researchers recommended that infliximab be used as the first-line treatment modality in patients with severe and acute GPP4. Both patients in the current study experienced dramatic resolution of pustules within 48~72 hours of infliximab infusion without any serious adverse events. Although the optimal infliximab dose and maintenance regimen for GPP has not yet been established, low dose (3 mg/kg) of infliximab showed excellent response in the current cases. In an earlier report, there was no significant difference between the 3 mg/kg treatment group and the 5 mg/kg treatment group for moderate to severe psoriasis5. However, more research is needed and we believed that low dose infliximab with or without acitretin may have an effect on acute GPP. Since infliximab appears to offer the advantage of rapid response and reduced morbidity, we may consider it extremely effective and well tolerated in adult patients with severe and acute GPP. However, since GPP tends to relapse frequently, large population studies are needed to evaluate the efficacy of maintenance infliximab therapy for GPP with recurrent pustular episodes.

Erythrodermic Psoriasis Treated with Golimumab: A Case Report.

Erythrodermic psoriasis (EP) is a very severe variant of psoriasis whose management poses a challenge to physicians, as currently available therapies often provide unsatisfactory results. Many biologics have been used to treat chronic plaque psoriasis, the most common form of psoriasis; however, their effectiveness for EP is poorly understood. A recently developed biologic, golimumab, has been extensively studied for the treatment of moderate-to-severe active rheumatoid arthritis, psoriatic arthritis, active ankylosing spondylitis, and chronic plaque psoriasis. However, no clinical trials have been performed for EP. Here, we report the case of a 32-year-old man who presented with severe psoriasis that previously failed to respond satisfactorily to methotrexate, cyclosporine, retinoid, narrow-band ultraviolet B phototherapy, and topical agents (i.e., steroids and calcipotriol). Skin lesions worsened progressively and developed into erythroderma. Psoriatic arthritis was also detected. Conventional therapies lacked efficacy. Therefore, we administered golimumab 50 mg. The skin lesions improved significantly according to the Psoriasis Area and Severity Index score after the first administration; lesions improved further throughout the treatment course. Although additional studies are required to fully evaluate the efficacy and safety of golimumab, this agent may be an alternative treatment strategy for some patients with recalcitrant EP.

Clinical features and course of generalized pustular psoriasis in Korea

The clinical course of generalized pustular psoriasis (GPP) is variable and unpredictable. Sufficient data on the clinical course of the disease has not been reported due to its rarity. To investigate the clinical features and course of GPP according to its subtypes, medical records of patients diagnosed with GPP from 2002 to 2012 at two tertiary hospitals were reviewed. The data included patient demographics, associated symptoms, aggravating factors, patterns of relapse and prognosis. Thirty‐three patients with GPP were included in our study, with a mean age of 45.6 years and a male : female ratio of 1:1.2. Patients were categorized based on the following subtypes: acute GPP, 21 (63.6%); GPP of pregnancy, two (6.1%); juvenile GPP, three (9.1%); and annular GPP, seven (21.2%). In the acute GPP population, skin lesions cleared within 2 months in 11 (73.3%) patients, and six (40.0%) of these had no relapse. Severe complications, abortion or death, were observed in two patients (100.0%) with GPP of pregnancy. Nineteen (76.0%) of the GPP patients experienced persistence or relapse of skin lesions. The patterns of skin lesions upon relapse included plaques in six patients (31.6%), pustules in eight patients (42.1%), and plaques and pustules in five patients (26.3%). Among acute GPP patients, 16.7% of patients with no relapse had a history of plaque psoriasis. However, 77.8% of patients with persistence and relapse in their clinical course had a history of plaque psoriasis. In conclusion, our study presents the detailed clinical course of GPP by subtype in Korean patients.

Screening for Psoriatic Arthritis in Korean Psoriasis Patients Using the Psoriatic Arthritis Screening Evaluation Questionnaire.

Background Psoriatic arthritis (PsA) is chronic seronegative inflammatory arthritis that causes irreversible joint damage. Early recognition of PsA in patients with psoriasis is important for preventing physical disability and deformity. However, diagnosing PsA in a busy dermatology outpatient clinic can be difficult. Objective This study aimed to validate the Psoriatic Arthritis Screening and Evaluation (PASE) questionnaire for the detection of PsA in Korean patients with psoriasis. Methods The PASE questionnaire was prospectively given to 148 patients diagnosed with psoriasis but without a previous diagnosis of PsA. All patients underwent radiologic and laboratory examinations, and a subsequent clinical evaluation by a rheumatologist. Results Eighteen psoriasis patients (12.2%) were diagnosed with PsA according to the Classification Criteria for Psoriatic Arthritis. The PASE questionnaire scores of differed significantly between PsA and non-PsA patients. Receiver operator characteristic analysis showed an area under the curve of 0.82 (95% confidence interval: 0.72, 0.92) for PASE score. A PASE score cut-off of 37 points had a sensitivity of 77.8% and specificity of 82.3% for the diagnosis of PsA. Conclusion The PASE questionnaire is a simple and convenient screening tool for detecting PsA in Korean dermatology clinics. A PASE questionnaire score of 37 points appears to be an appropriate cut-off for screening Korean psoriasis patients.

A Case of Soft Fibroma of the Nipple with a Cauliflower-Like Appearance

Dear Editor: Soft fibroma (acrochordon, fibroepithelial polyp, or skin tag) is a common pedunculated skin neoplasm and usually appears as a furrowed papule, filiform lesion, or large bag-like protrusion. Soft fibromas primarily occur on the neck, axillae, and groin. However, it may present at unusual sites of the body such as the penis, urethra, and vulva. Moreover, a few reports on soft fibromas occurring on the breast and nipple have been published1. Herein, we report a rare and interesting case of a soft fibroma arising from the nipple, showing an unusual cauliflower-like appearance. A 51-year-old obese woman presented with a 25-year history of a painless pedunculated polyp originating from her right nipple. The lesion measured 2.4×2.3×1.4 cm and demonstrated a verrucous and cauliflower-like surface (Fig. 1A, B). Neither acanthosis nigricans nor epidermal nevus was observed on the adjacent skin. Dermoscopy revealed irregular epidermal projections and focal dotted vessels (Fig. 1C). Total surgical excision of the polyp, sparing the right nipple, was performed. Histopathological analysis showed papillomatosis, hyperkeratosis, and regular acanthosis of the epidermis with fibrocollagenous tissue in the dermis (Fig. 1D). On the basis of these findings, we diagnosed the lesion as a soft fibroma. Fig. 1 (A) A large solitary pedunculated polyp originating from the right nipple. (B) The polypoid lesion measuring 2.4×2.3×1.4 cm and demonstrating a cauliflower-like surface