Parry-Romberg Syndrome with Band-like Linear Alopecia / 대한피부과학회지

Parry and Romberg described progressive facial hemiatrophy, characterized by progressive unilateral wasting of the skin, fat, muscle and bone in 1825 and 1846, respectively. Involvement of the central nervous system, with impairment of neurologic function, occurs infrequently. Linear scleroderma on frontal and parietal scalp, named 'en coup de sabre', is considered an overlapping condition. An 11 year-old girl was presented to our office with left facial hemiatrophy and transverse linear band-like alopecia on her posterior parietal scalp. Histopathologically, there was a sclerotic change of the collagen fibers and dermal lymphocytic infiltrations around the skin appendages. Her skin lesion has improved by intralesional corticosteroid injection. Linear scleroderma frequently coexists with Parry-Romberg syndrome. But in this case, she had a unique pattern of scleroderma, which is distinct from the previously reported cases.

The Study of the Clinical, Histopathological Features of Pilomatricoma and the Differentiation to Normal Hair Follicles / 대한피부과학회지

BACKGROUND: Pilomatricoma is a benign appendageal tumor characterized by basaloid and shadow cells. Differentiation towards normal hair follicles is often observed in this condition. OBJECTIVE: The purpose of this study was to analyze the clinical and histopathological features of pilomatricoma and to confirm diverse types of differentiation towards normal hair follicles, and its origin. METHODS: We retrospectively reviewed medical records and histopathological findings of 64 patients who had visited our hospital in the last 7 years. The evolutional stages of tumor and differentiation were analyzed through histopathological findings. RESULTS: The majority of the tumors occurred in the first two decades (69.8%). Unlike previous reports, the male to female ratio was 1.1:1. The mean duration was 11.8 months. The predilection sites were the upper extremities (40.8%), face (33.8%), neck (15.5%), back (2.8%), scalp (2.8%), and thigh (2.8%) in that order. Histopathologically, most of the tumors were located in the lower dermis (76%) and enclosed by pseudocapsules. The evolution of tumor was divided into four groups- early, fully developed, early regressive, and late regressive stage, as previously reported. Our 71 cases were classified as: 2 early, 16 fully developed, 34 early regressive, and 19 late regressive stage. Calcification (60.5%) and ossification (8.4%) were observed, and the ratio increased as the tumor aged. Inflammatory cell infiltration and multinucleated giant cells were observed disorderly. Differentiation to normal hair follicles was observed: 14 cases to infundibulum, 10 isthmus, 17 outer root sheath, and 11 inner root sheath. CONCLUSION: Pilomatricoma is a benign tumor that differentiates toward the hair cortex. Basaloid and shadow cells correspond to hair matrix cells and immature hair cortex cells, respectively. Basaloid cell can also differentiate to normal hair follicles.

A Nevus Comedonicus Symmetrically Developed on Both Eyelids in Middle Aged Man / 대한피부과학회지

Nevus comedonicus is a relatively rare hamartoma, which originates from pilosebaceous unit. The disease usually occurs at birth or during childhood and most commonly unilateral or along with Blascko line. A 59-year-old man presented with black grouped dilated pores, with keratin plug on both eyelids. A histopathologic finding showed multiple keratin filled epidermal invaginations. We herein report an unusual case of bilateral nevus comedonicus developed in middle aged persons.

A Case of Type 1 Pachyonychia Congenita (Jadassohn-Lewandowsky Syndrome) / 대한피부과학회지

Pachyonychia congenita (PC) is characterized by hypertrophic nail dystrophy, focal palmoplantar keratoderma and blistering, oral leukokeratosis, cyst formation, palmoplantar hyperhydrosis, and follicular keratoses on the trunk and extremities. PC is diagnosed by clinical findings and molecular genetic studies. A 26-year-old man presented with hypertrophic nail dystrophy and subungual debris of all 20 nails, hyperkeratotic plaques on the heels of both soles, and oral leukokeratosis. He had no family history of similar clinical findings. The patient's clinical presentation and history were consistent with PC. Herein we report on a rare case of pachyonychia congenita with a review of literature.

A Case of Trauma-Induced Single Xanthelasma Palpebrarum

A 33-year old Korean man visited our department complaining of single mildly pruritic yellowish flat-topped papule on left upper eyelid. Lipid profile including low density lipoprotein-cholesterol and triglyceride level was above normal range. He remembered that the site was scalded by boiling oil about eight months ago. The patient was diagnosed with xanthelasma palpebrarum (XP) based on the clinical and histopathological findings, including diffuse infiltrate of large, pale-staining cells which have abundant foamy cytoplasm in the dermis. He was treated with CO2 laser ablation followed by application of 70% trichloroacetic acid with wooden stick. After a month, the skin lesion showed moderate improvement with disappearance of yellowish color and flat-topped elevation. So far, case of XP developed after minor trauma has not been reported. This case illustrates that minor trauma could be a trigger factor in the development of the disorder.

A Case of Cutaneous Horn Originating from Keratoacanthoma

Cutaneous horn is the clinical description of a hyperproliferation of compact keratin in response to a wide array of underlying benign and malignant pathologic changes. We report here on a case of cutaneous horn that originated from keratoacanthoma in a 76-year-old woman. Grossly, a 2.5x0.7 cm sized yellow-white colored scaly fungating mass from an erythematous nodule was observed on the right temporal area. Histopathologically, it was reported as keratoacanthoma with cutaneous horn. The lesion was totally excised after the diagnosis.

Imatinib Mesylate (Gleevec(TM))-induced Lichenoid Drug Eruption Improved by Tentative Dose-reduction and Topical Steroid / 대한피부과학회지

Imatinib mesylate (Gleevec(TM)) is an oral anticancer drug. It works as a selective and competitive inhibitor of tyrosine kinases such as bcr-abl protein, c-kit, and platelet-derived growth factor receptors (PDGFR). Gleevec(TM) is a first-line therapeutic agent for chronic myelogenous leukemia (CML) and gastrointestinal stromal tumors, but causes various adverse cutaneous reactions. We herein report on a case of lichenoid drug eruption induced by Gleevec(TM) in a patient with a malignant gastrointestinal stromal tumor.

A Case of White Fibrous Papulosis of the Neck / 대한피부과학회지

White fibrous papulosis of the neck is a rare skin disease that manifests with tiny papules that are distributed on the neck. Thick collagen bundles in the papillary dermis and diminution of the elastic fiber are characteristic on histologic examination. It is thought to be a normal aging process of the skin or an associated disorder. We report here on a case of white fibrous papulosis of the neck of a 49-year-old Korean woman.

A Case of Cutaneous Mucormycosis Mimicking Sporotrichosis / 대한피부과학회지

Mucormycosis is a rare invasive fungal infection that chiefly occurs in immunocompromized patients and the outcome is generally fatal. Primary cutaneous mucormycosis is a rare phenotype of mucormycosis, and it usually follows direct inoculation of fungi on damaged skin. Many cutaneous infections mimic sprotrichosis, including virus, bacteria, acid-fast bacilli, fungi and protozoa, yet mucormycosis is very rare. We report on a case of cutaneous mucormycosis that mimicked sporotrichosis in a 77 years old woman.

Blastoid Mantle Cell Lymphoma: A Case Report / 대한피부과학회지

Mantle cell lymphoma (MCL) is a low-grade malignant lymphoma derived from a subset of B-cells localized in the mantle zone of the lymphoid follicle. MCL commonly involves extranodal sites, usually as a manifestation of disseminated disease. The immunophenotype of MCL is characterized as CD20+, CD5+ and Cyclin D1+. Blastoid mantle cell lymphoma (BV-MCL) is a rare variant and has a more aggressive clinical course. In the blastoid variant, the genetic profile is often more complex and includes tetraploid chromosomes, CCND1 amplification, a higher incidence of p53 gene mutations, and p16(INK4a) deletions. We report a case of a 62-year-old male patient who presented with erythematous subcutaneous nodules on the lower back. He had been treated for BV-MCL of the testis which had occurred 1 year ago. The analysis of histopathological and immunohistochemical examinations of the skin lesions also revealed a BV-MCL.

Usefulness of Current Withdrawal Threshold on Evaluation of the Neuropathic Pain in Animal Model

OBJECTIVE: To evaluate usefulness of the current withdrawal threshold (CWT) on evaluation of the neuropathic pain in animal model. METHOD: Surgical neuropathy was induced in 40 Sprague-Dawley rats. Fourteen days after the surgery, neuropathic rats were evaluated by von Frey hair. The CWT was determined by various stimulus intensities which induce the tail-withdrawal response or vocalization of rats. The experimental group was compared with the control group by CWT. Then the experimental group was subdevided to two groups. The experimental group 1 was injected with beta-methasone 0.1 mg/kg, intra-peritoneally and experimental group 2 was injected with normal saline with the same amount as steroid. The CWT of two experimental subgroups were measured before and 30 minutes after injections. RESULTS: The experimental group showed significant decrease of the CWT compared with the control group after the neuropathic pain was induced. The CWT of experimental group 1 was increased after steroid administration (p<0.01). CONCLUSION: This preliminary study suggests that the measurement of CWT would be an useful tool to study the neuropathic pain in experimental animal model.

Effects of Clonidine and Yohimbine on Neuropathic Pain in a Rat Model

OBJECTIVE: In a rat model of peripheral neuropathy, to determine whether neuropathic pain is related to the alpha-2 adrenergic receptor. METHOD: The neuropathic pain was produced by unilateral transection of the superior caudal trunk between the S3 and S4 spinal nerves. These animals showed the behavioral signs of neuropathic pain in the tail. Two weeks after the neuropathic surgery, tail withdrawal responses to the mechanical stimuli with von Frey hair (2.0 g) were examined 1, 2 and 24 hrs following the administration of clonidine, alpha-2 receptor agonist. One week after the clonidine test, the same behavioral test was done after the administration of clonidine along with yohimbine, alpha-2 receptor antagonist. RESULTS: Clonidine significantly reduced the frequency of tail response and yohimbine reversed the clonidine-induced anti-allodynic effect. CONCLUSION: These results suggest that neuropathic pain is related to the sympathetic nervous system via alpha-2 adrenergic receptor.

Effects of Exercise on Neuropathic Pain in an Experimental Model of Peripheral Neuropathy

OBJECTIVE: The purpose of this study was to evaluate the short- and long-term effects of exercise on neuropathic pain. METHOD: Pain responses between rats in the exercise and control groups were compared to evaluate the effects of exercise in neuropathic pain. Materials consisted of 30 male Sprague-Dawley rats (8 weeks old, 180~200 g), which were divided into an exercise group (n=15) and a control group (n=15). Neuropathic pain was produced by partially injuring the nerve innervating the tail. Running exercise was given on a Rota-rod treadmill exercise machine for 3 weeks (3.1 Km/day, 6 cycle of 9 minutes exercise and 1 minute rest). Behavioral reactions to mechanical allodynia were checked using a von Frey hairs of 2.0 g (19.6 mN) bending force at 10 minutes, 1 hour and 24 hours post-exercise to evaluate the short term effects of exercise. Behavioral reactions to mechanical and thermal allodynia with 4 degrees C or 40 degrees C were evaluated 7, 14, 21 and 28 days following exercise. RESULT: The exercise group exhibited less tail-flick frequencies to mechanical stimulation from 58.8+/-6.8% to 41.1+/-5.4%, 37.6+/-13.2% at 1 and 24 hours post-exercise compared to the control group, but there was no significant difference between the groups at weeks 1 through 4. In the exercise group, the decrease of tail-flick frequencies were blocked by naloxone (2 mg/kg i.p.). It is suggested that long-lasting muscle exercise (e.g. running) which influences central endorphin mechanisms giving analgetic effects. CONCLUSION: The results of this study support the hypothesis that the exercise can reduce neuropathic pain in the acute stage.

Auditory Brainstem Evoked Potentials in Preterm Infants

OBJECTIVE: To investigate the change of peak latency, interpeak latency and amplitude of auditory brainstem evoked potentials (AEPs) in normal preterm infants in accordance with the age, and to find out the correlation between reproducibility of AEPs and high risk of premature infants. METHOD: AEP studies were performed on 266 premature infants (male 143, female 123) within a month of the birth. Acquired potentials were grouped by the reproducibility of waveforms, and latency, interpeak latency and amplitude were measured in each group of potentials to interpret age appropriate changes of AEPs. RESULTS: 1) Peak latency of peak I, III and V were shortened in accordance with the age, especially latency of peak V was significantly decreased from 7.42 msec to 6.84 msec. 2) There was no significant change in interpeak latency or amplitude of AEPs according to the postmenstrual age. 3) Reproducibility of AEPs was worse in premature infants with history of asphyxia. CONCLUSION: Considering the results, the latency of peak V can be used as one of the useful parameter to investigate and follow up the premature infants. Significant negative correlation between low grade reproducibility and history of neonatal asphyxia was found.