Hyang-Suk You

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.

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.

Localized argyria: troublesome side-effect of acupuncture

events of PD-1 immunotherapy are being increasingly reported and characterized. Since our initial publication, additional cases of eruptive KAs have been reported, supporting its status as an associated adverse event. It is encouraging to see these reports so that we can better characterize the adverse event profile and gain insight into pathogenesis. It is unclear exactly why KAs paradoxically erupt secondary to a medication used to treat squamous cell carcinomas (SCCs) of the lung and head/neck. It is further confusing in the light of a recent publication reporting very similar PD-L1 expression profiles between KAs and cutaneous SCCs. Nevertheless, the eruption of KAs in PD-1 immunotherapy may prove fortuitous by providing new insight into their unique biology. Although KAs may simply represent a well-differentiated variant of SCC, many consider them a distinct entity – a notion supported on the molecular level. We know PD-1 inhibitors enhance antitumour activity by blocking the immune downregulation that occurs when PD-1 on T cells binds its ligands, often strategically overexpressed on malignant tumour cells. However, antitumour activity is not the only T-cell function ‘unchecked’. Tcell receptor signalling, cytokine production, lymphocyte motility and metabolic programming are all ‘unchecked’ by inhibiting PD-1. Perhaps then KAs are closer related to a reactive proliferation akin to exuberant squamous metaplasia, and a pro-inflammatory cytokine milieu injures predisposed keratinocytes in a way that incites a reactive proliferation that outpaces any enhanced antitumour activity. In the same vein, increased cytokine production (e.g., IL-2, IL-4) could tip a precarious inflammatory balance towards Th1 (lichenoid dermatitis, vitiligo) or Th2 (antibody-related disease) pathways. Further investigation into these immune-related adverse events is certainly needed and might include the immunohistochemical characterization of the inflammatory infiltrate before, during and after immunotherapy. Such information may provide further insight into pathogenesis as well as possible immunohistochemical methods for monitoring treatment efficacy and progress.

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

Alitretinoin can be a good treatment option for idiopathic recalcitrant trachyonychia in adults: an open‐label study

Trachyonychia can be refractory to conventional treatments including topical, intralesional or systemic corticosteroids, as well as cyclosporine and retinoids. Therefore, new treatment options are needed for recalcitrant trachyonychia.

The importance of dermoscopy for the diagnosis of acquired bilateral telangiectatic macules: the angioid streak pattern reveals underlying chronic liver disease

Acquired bilateral telangiectatic macules (ABTM) are a newly recognized disease entity, which manifest as multiple telangiectatic pigmented macules confined mostly to the upper arms.

Pigmentary mosaicism with trisomy 7.

Dear Editor, Hypomelanosis of Ito (HI) is a sporadic pigmentary anomaly along the Blaschko lines, characterized by linear and whorled hypopigmentation. In 1992, Ruiz-Maldonado et al. published a suggestion for diagnostic criteria of HI based on vast clinical experience. Nevertheless, it is important to point out that HI is not a specific diagnosis, but may occur owing to several different chromosomal abnormalities. This group of disorders is better termed “pigmentary mosaicism” (PM). A 21-month-old boy was initially examined because of hypopigmentation that appeared as linear and whorled streaks along the Blaschko lines (Fig. 1a,b). He was delivered at 41 weeks’ gestation with a birthweight of 2880 g. The pedigree showed no evidence of similar abnormalities. Early infant development was normal. By Ruiz-Maldonado’s criteria, he was diagnosed as presumptive HI. Cytogenetic analysis of peripheral blood revealed normal standard karyotype (46, XY). Microarray comparative genomic hybridization (aCGH) did not detect any microdeletion or duplication. Histological examination of the hypopigmented area revealed mildly decreased melanin pigments in the basal layer compared with normal skin (Fig. 1c–f). Karyotype analysis of fibroblast cultures from the hypopigmented lesion revealed a trisomy 7 cell line, 47, XY, +7, and that from normal skin showed 46, XY (Fig. 1g,h). Thus, a diagnosis of PM with trisomy 7 was made, and on recent examination at age 2.5 years, no extracutaneous abnormalities were observed. Although all patients with PM have been hypothesized to have underlying mosaicism, chromosomal mosaicism is found in only 20–30% of the patients. It is caused not only by submicroscopic genetic abnormality, but possibly also because conventional karyotyping of blood lymphocytes or skin fibroblasts cannot always show chromosomal abnormalities. Blood is a conventional specimen for karyotyping. However, it is recommended to karyotype skin fibroblasts as lymphocytes may not demonstrate the abnormal cell line. Such discordance between karyotypes of blood and skin is rare but has been reported. Skin pigmentation is closely related to epidermal–dermal interaction. Although pigmentation is primarily related to the melanocytes, the surrounding keratinocytes and extracellular matrix proteins and fibroblasts in the underlying dermal compartments actively contribute to cutaneous homeostasis. Thus, we suppose that skin biopsy is the best way to obtain an appropriate specimen for karyotyping, and it is better to check both fibroblasts and keratinocytes. Mosaic trisomy 7 is a rare condition. Several pigmentary genes on chromosome 7 were identified (EGFR, GPNMB, HGF, EPHA1, GPDS1) that may affect abnormal pigmentation in HI. Another trisomy mosaicism associated with HI has also been reported. No effective treatment for pigmentary mosaicism has been established; the hypopigmented macules persist throughout life, and the prognosis is influenced by the associated extracutaneous findings such as renal malformation, facial dysmorphism and growth retardation. Ours is the only case of trying to find any submicroscopic abnormalities in blood by using aCGH. However, as in our case, mosaic trisomy 7 has not yet been demonstrated in peripheral blood. Therefore, skin biopsy is crucial for karyotyping and should be performed promptly for chromosome analysis.

A case of pigmentary mosaicism associated with diploid/triploid mixoploidy

A 21-month-old girl was referred for evaluation of irregular skin pigmentation. She was born at 37 weeks of gestation following caesarean section because of severe intrauterine growth retardation. The birth weight was 1.49 kg (below the 5th percentile). Her parents were healthy, and they denied having any family history of inherited diseases. Her skin lesions were multiple linear and whorled hyperpigmented and hypopigmented patches on the trunk and upper and lower extremities distributed along the Lines of Blaschko (Fig. 1a–e). Histopathological examination of a hyperpigmented patch revealed increased melanin pigment in the basal layer as compared to that in the perilesional normal skin. Physical examination revealed dysmorphic features including hypertelorism, microretrognathia, low-set ears, macrocephaly, brachydactyly, and clinodactyly (curved finger, Fig. 1c). Dental examination revealed hypodontia. Neurological examinations revealed generalized hypotonia, and Bailey developmental assessment detected global developmental delay. Laboratory investigations including complete blood count; liver, kidney, and thyroid function tests; and urinalysis showed normal results except low parathyroid hormone levels (6.96 pg/ ml; reference range, 10–65). A skeletal survey showed diffuse thinning of both the lower extremity bones and mild scoliosis. Karyotyping of peripheral blood lymphocytes showed a normal—46, XX—karyotype. Genetic testing for Silver-Russel and next generation sequencing were negative. In addition, karyotyping of lesional skin fibroblasts was performed, and the results revealed triploidy—69, XXX. The results of dermatological and neurological abnormalities and cytogenetic studies in our patient were consistent with those of pigmentary mosaicism associated with diploid/triploid mixoploidy (DTM). Currently, the patient is undergoing rehabilitation therapy for developmental delay and 1064 nm Nd:YAG laser treatment for pigmentary skin lesions.

Cutaneous Abscess as a Complication of Bisphosphonate-Related Osteonecrosis of the Jaw

Vol. 30, No. 2, 2018 243 Received October 21, 2016, Revised February 2, 2017, Accepted for publication April 10, 2017 Corresponding author: Byung-Soo Kim, Department of Dermatology, Pusan National University Hospital, 179 Gudeok-ro, Seo-gu, Busan 49241, Korea. Tel: 82-51-240-7338, Fax: 82-51-245-9467, E-mail: dockbs@pusan.ac.kr This is an Open Access article distributed under the terms of the Creative Commons Attribution Non-Commercial License (http://creativecommons.org/ licenses/by-nc/4.0) which permits unrestricted non-commercial use, distribution, and reproduction in any medium, provided the original work is properly cited. Copyright

Dermoscopic “Landscape Painting Patterns” as a Clue for Labial Melanotic Macules: An Analysis of 80 Cases

Background Labial melanotic macules (LMMs) are benign pigmented lesions that usually take the shape of flat asymmetrical macules with tan-brown to black color and variable size. Whereas the dermoscopic features of other pigmented skin lesions have been relatively well described, little is known about LMMs. Objective To describe the dermoscopic features and find typical and schematic dermoscopic patterns in LMMs. Methods A retrospective dermoscopic study was conducted on 80 lesions with histopathologically proved LMMs. Results We described and defined, for the first time to our knowledge, landscape painting patterns found in 65 of 80 melanotic lesions (81.3%), characterized by parallel lines or circle lines, overlapping vessels with background brown pigmentation. The background brown pigmentations were observed in 74 of 80 lesions (92.5%), the parallel lines in 62 (77.5%), the circle lines in 20 (25.0%), and overlapping vessels in 69 (86.3%). The structureless black pigmentations were only presented in 26 of 80 (32.5%). Conclusion Dermoscopy can be useful for the clinical detection of LMMs, and “Landscape painting patterns” may represent a dermoscopic clue for the diagnosis of these lesions.