Soo Bin Son

A case of cutaneous inflammatory myofibroblastic tumor

Pseudo-inflammatory tumors are also known as plasma cell granuloma, inflammatory pseudo-tumor and inflammatory myofibroblastic tumor, and these tumors are a group of highly variable proliferations of myofibroblastic cells that are associated with a prominent inflammatory infiltrate. This tumor is known to most commonly occur in the lungs, bladder and gastrointestinal system with only a few cases having been reported in the skin. A previously healthy 26-year-old man presented with a 6-year history of an intermittently pruritic lesion on his back. On the histologic examination, there were spindle cells in fascicles and a mixed inflammatory cellular infiltrate of plasma cells and lymphocytes. A diagnosis of inflammatory fibroblastic tumor was made and the nodule was surgically removed. We report here on an additional case of this rare cutaneous entity, and it is probably the first such report from Korea.

Birt-Hogg-Dubé Syndrome, a Rare Case in Korea Confirmed by Genetic Analysis

Simple benign tumors can present as part of a syndrome with substantial mortality. Fibrofolliculomas are benign skin tumors most often associated with the Birt-Hogg-Dubé syndrome (BHDS). The most life-threatening complication of this syndrome is renal cancer and other major features include multiple lung cysts and spontaneous pneumothorax. We present the case of a 54 year-old man with multiple flesh-colored papules on his face confirmed histologically as fibrofolliculomas. He had a history of recurrent pneumothorax and chest computed tomography showed multiple lung cysts. To confirm the diagnosis of BHDS, we conducted gene analysis that revealed a single nucleotide duplication in the folliculin (FLCN) gene (Exon 11, C.1285dupC). BHDS confirmed by the FLCN gene mutation is rarely reported in Korea. Appropriate investigation is recommended whenever a patient with benign skin tumors is encountered.

Successful treatment of palmoplantar arsenical keratosis with a combination of keratolytics and low‐dose acitretin

Despite the fact that Australia has a reputation as a land of sunshine, studies have found that vitamin D deficiency is common in the normal Australian population, even in the subtropical region of the continent. Deficiency is usually corrected by large doses of cholecalciferol. Patients are given 300,000–500,000 IU of cholecalciferol over 1– 10 weeks, and this has been shown to be a safe, effective way of replenishing vitamin D stores. As ultraviolet (UV) light therapy is now generally given as narrowband UVB (nUVB) therapy rather than broadband UVB, we aimed to determine if nUVB therapy could correct vitamin D deficiency as effectively as high doses of cholecalciferol. The study was carried out during the winter of 2006 in Melbourne, Australia, which is located at 37 south. The study population was divided into two groups. The two groups were not closely matched because the aim of this small pilot study was to see if nUVB could raise serum 25-hydroxy vitamin D (25-OHD) and to evaluate its efficacy compared with vitamin D supplements. The UVB group comprised outpatients (seven men) with extensive psoriasis or dermatitis requiring nUVB therapy to clear the condition, and the cholecalciferol group comprised people (five men, two women) with pronounced vitamin D deficiency as assessed by the 25-OHD level. This group were either outpatients with skin diseases that did not need UVB therapy or doctors working in the outpatient department. In the UVB group, serum 25-OHD was measured before the start of UVB therapy. Patients were given nUVB three times ⁄ week for 6 weeks and the serum 25-OHD level measured again at the end of that period. The entire body, apart from the area covered by underpants, or by goggles to protect the eyes, was exposed. The starting dose was 0.1 J ⁄ cm, which this was increased by 0.05 J ⁄ cm at each visit. No patient received burns during therapy. Participants in the cholecalciferol group were given cholecalciferol 50,000 IU ⁄ mL. They took a dose of 1 mL ⁄ week for 10 weeks and serum 25OHD level was measured at the end of this period. Serum 25-OHD level increased in all patients. The cholecalciferol group had an average serum 25-OHD of 20 mmol ⁄ L (range 13–25) before therapy, increasing to an average of 55 mmol ⁄ L (range 42–67) after treatment. The nUVB group had an average serum 25 OHD of 31 mmol ⁄ L (range 23–37) before therapy, increasing to an average of 95 mmol ⁄ L (range 66–126) after therapy. One dose of 50,000 IU of cholecalciferol raised the serum level by an average of 3.5 mmol ⁄ L, which was similar to the effect of one dose of nUVB to the entire body. In summary, nUVB therapy is an effective way to correct vitamin D deficiency in outpatients, as effective as high doses of cholecalciferol. D. Czarnecki Repatriation Campus, Austin Health, Melbourne, Australia E-mail: dandsec@bigpond.net.au Conflict of interest: none declared. Accepted for publication 29 June 2007 Correspondence: Dr D. Czarnecki, Suite 1, 333 Wantirna Road, Wantirna, 3152 Australia.

A Case of Kikuchi's Disease Presenting with Unique Facial Involvement

Kikuchis disease (KD), or histiocytic necrotizing lymphadenitis, is a rare, self-limited lymphadenopathy, typically in young women, that usually remits spontaneously and does not recur. KD is clinically characterized by cervical lymphadenopathy and a high fever. Extranodal involvement (skin, arthritis, meningitis) rarely occurs. When KD is involved in a skin lesion, it presents with various shapes, but rarely manifests with symmetrically distributed, erythematous, firm nodules only on the face. In this report, we describe a patient with KD and unique skin manifestations.

Aggravation of Relapsing Polychondritis due to the Infection and Its Manifestation on a Nasal Tip Graft

Relapsing polychondritis (RP) is an uncommon systemic disease that is characterized by episodic and progressive inflammation of the cartilaginous structures, which can be very debilitating and in some instances life-threatening. The pathogenic pathways of RP are largely unknown. However, several hypothesis have been suggested. We had an interesting case of aggravation of RP due to the infection. Graft cartilage on the nasal tip was affected by RP also. This case can give a clue of revealing the pathogenesis of RP. We introduce a case with a review of the literature.