[P246] A case of nail dystrophy with selective IgA deficiency

( Youngkyoung Lim ),( Hyun Jung Byun ),( Chan Seong Park ),( Jae Ho Lee ),( Ji-hye Park ),( Jong-hee Lee ),( Dong-yoon Lee ),( Joo-heung Lee ),( Jun-mo Yang ) 대한피부과학회 2017 대한피부과학회 학술발표대회집 Vol.69 No.1

Nail is a mirror of our health. Nail dystrophy is very common and associated with various diseases. The causes of nail dystrophies are related to infections (fungus, bacteria, and virus), congenital disorders, environmental inputs (such as trauma and drugs), various cutaneous diseases (psoriasis, lichen planus, eczema, etc.), and systemic conditions. A 4-year-old boy visited our clinic with nail dystrophy which started 4 months ago. Nail changes were observed including onycholysis with oil-drop discoloration, superficial fragility and onychomadesis of the proximal nail plate, Beau’s line, koilonychia, and onychorrhexis. Nail clipping examination for fungus was negative. There has been frequent history of infections such as pneumonia and sinusitis since 4 years ago. Laboratory findings showed decreased serum immunoglobulin A (IgA) level (4mg/dl, reference: 27~195mg/dl) in the presence of normal serum levels of IgG and IgM. He was diagnosed as selective IgA deficiency which is a type of hypogammaglobulinemia. He died of a disseminated enterovirus infection 16 months later. Herein, we present a rare case of nail dystrophy with selective IgA deficiency and discuss the possible significance of this finding.

Two cases of metastatic calcinosis cutis

( Youngkyoung Lim ),( Chan Seong Park ),( Jae Ho Lee ),( You Jin Lee ),( Ji Hye Park ),( Jong Hee Lee ),( Dong Youn Lee ),( Joo Heung Lee ),( Jun Mo Yang ) 대한피부과학회 2016 대한피부과학회 학술발표대회집 Vol.68 No.1

Calcinosis cutis (CC) represents the deposition of calcium in the skin. CC can be divided into four categories; dystrophic, metastatic, idiopathic and iatrogenic CCs. Dystrophic CC occurs on damaged tissue by various underlying conditions with normal serum level of calcium (Ca) and phosphate (P). Idiopathic CC is not related to any tissue defects or metabolic abnormalities of Ca and P. Iatrogenic CC occurs secondarily by medical procedures such as intravenous administration of Ca or P. Lastly, metastatic CC is related to abnormalities of Ca and P metabolism, and generally associated with hyperphosphatemia and hypercalcemia. Metastatic CC is most commonly associated with chronic renal failure (CRF). We present two cases of metastatic CC. Both patients had underlying CRF with hemodialysis. The first patient was 64-year-old woman having multiple yellowish nodules on her hands which was initially confused with the non-tuberculous mycobacterium (NTM) infection. The second patient, a 53-year-old man, developed a rigid nodule on his finger. The skin specimens presented calcium deposits in the dermis and both of them showed significantly elevated serum P levels. We therefore diagnosed these two cases as metastatic calcinosis cutis.

The nail matrix nevi: Clinicopathological analysis of 28 cases

( Youngkyoung Lim ),( Jae Ho Lee ),( You Jin Lee ),( Jong Yoon Chung ),( Ji Hye Park ),( Jong Hee Lee ),( Jun Mo Yang ),( Joo Heung Lee ),( Dong Youn Lee ) 대한피부과학회 2015 대한피부과학회 학술발표대회집 Vol.67 No.2

Background: Longitudinal melanonychia (LM) is a clinicalcondition that brown-black discoloration extends from the proximal to the distal nail plate. The nail matrix nevi may show similar clinical features to subungual melanoma. Objectives: We retrospectively analyzed clinicopathologic features of 28 cases of nail matrix nevi to find out diagnostic clues.Methods: Based on clinical photography, width of melanonychia and presence of nail dystrophy(ND) and pseudo-Hutchinson``s sign(PHS) were recorded. Dermoscopic examinations were done in 17 cases. For the pathological evaluation, 27 nail matrix biopsy specimens and a hyponychial skin biopsy specimen were reviewed. Results: Among 28 patients, total melanonychia was observed in two cases, and the others were partial melanonychia without background pigmentation. The ND and PHS were observed in 17 and 5 cases, respectively. Childhood nail matrix nevi were tended to be wider than adults, and melanonychia wider than 20% of nail plate were likely to have ND and cytologic atypia. On dermoscopy, brushy linear pigmentations were found on PHS. In all cases, well demarcated junctional melanocytic nests with abundant cytoplasm were observed. Conclusion: Pediatric wide nail matrix nevi can be adiagnostic pitfall because they share clinicopathological features with subungual melanoma, especially the Hutchinson’s sign. We suggest partial melanonychia, brushy linear pigmentation and characteristic junctional nests are useful in differentiation.

Two cases of phakomatosis pigmentovascularis

( Youngkyoung Lim ),( Chan Seong Park ),( Jae Ho Lee ),( You Jin Lee ),( Ji Hye Park ),( Jong Hee Lee ),( Dong Youn Lee ),( Joo Heung Lee ),( Jun Mo Yang ) 대한피부과학회 2016 대한피부과학회 학술발표대회집 Vol.68 No.1

Phakomatosis pigmentovascularis (PPV) is a rare syndrome which showed both vascular nevus like nevus flammeus or port-wine syndrome (PWS) and pigmentary nevus such as Mongolian spot or Ota nevus. PPV can be related other nevi including nevus anemicus, epidermal nevus, nevus spilus and cutis marmorata telangiectatica congenita. This syndrome can be classified into 5 major types according to the combination of clinical manifestations, the most common type is type II which presents nevus flammeus plus dermal melanosis with or without nevus anemicus. Each major types are divided into two subtypes, subtype A and B, according to whether there are only skin manifestations or systemic involvements coexist. Herein, we report two cases of PPV. The first case of 41-year-old woman with PWS on her left arm and pigmentary lesions of the back and shoulders. The second patient, a 15-year-old boy, presented with PWS on his right face and Ota nevus of the right ocular area. Moreover, he showed PWS with mild hypertrophy of both hands and truncular type of congenital vascular malformation-inferior vena cava (IVC) agenesis.

A case of multiple cutaneous leiomyomas

( Youngkyoung Lim ),( Chan Seong Park ),( Jae Ho Lee ),( You Jin Lee ),( Ji Hye Park ),( Jong Hee Lee ),( Dong Youn Lee ),( Joo Heung Lee ),( Jun Mo Yang ) 대한피부과학회 2016 대한피부과학회 학술발표대회집 Vol.68 No.1

Cutaneous leiomyomas (CL) are benign smooth muscle tumors, which are also called as piloleiomyomas. CL may occur sporadically or as a cutaneous manifestation of an autosomal dominant genetic syndrome, hereditary leiomyomatosis and renal cell cancer (HLRCC), which was revealed to be associated with fumarate hydratase (FH) gene. HLRCC is associated with multiple CL and uterine leiomyomas in females, and renal cancers. CL may occur more often (76 to 100%) in individuals with the mutation of FH gene than renal cell cancer (about 15 to 20%). HLRCCassociated renal cancers are aggressive, so that dermatological diagnosis of CL is important for renal cancer screening. If possible, genetic study of FH mutation should be considered in individuals with multiple CL. Herein, we report a case of 59-year-old woman who presented painful multiple nodules and papules on her chest and right shoulder. She had a history of hysterectomy 16 years ago due to uterine leiomyoma. From skin biopsy, the specimen showed a wellcircumscribed nodular tumor within the reticular dermis. It presented interlacing bundles of tumor cells with blunt-ended or cigar-shaped nuclei. Tumor cells were positive for SMA, but they were S-100 negative. Finally, the cutaneous lesion was diagnosed as leiomyoma.

Cutaneous anaplastic large cell lymphoma: clinical features and prognostic factors of 25 Korean patients

( Youngkyoung Lim ),( Chan Seong Park ),( Jae Ho Lee ),( You Jin Lee ),( Ji-hye Park ),( Jong Hee Lee ),( Joo-heung Lee ),( Jun-mo Yang ),( Dong-youn Lee ) 대한피부과학회 2016 대한피부과학회 학술발표대회집 Vol.68 No.2

Background: Cutaneous anaplastic large cell lymphoma (ALCL) can be divided into two groups, primary cutaneous ALCL (PC-ALCL) which initially presents in the skin and cutaneous involvement of systemic ALCL (CIS-ALCL) which is secondary to nodal disease. Objectives: We attempted to assess clinical presentation and prognostic factors of patients with cutaneous ALCL. Methods: This was a retrospective chart review of patients with cutaneous ALCL which had been confirmed with skin biopsy between 1996 and 2016. Results: A total of 25 patients with cutaneous ALCL were included. Fourteen patients had PC-ALCL and eleven showed skin involvement of systemic ALCL. CIS-ALCL was more likely to present with extensive and multiple skin lesions than PC-ALCL, but it was not statistically significant. The prognosis of CIS-ALCL patients was poor. Seven of eleven patients (63.6%) died within a mean survival time of 9.7 months after the confirmation of skin involvement. Leukocyte or C-reactive protein (CRP) elevation in the blood were associated with poorer outcome in CIS-ALCL. Conclusion: In addition to well-known prognostic factors of lymphoma such as B-symptom and increased LDH, having leukocytosis or elevated CRP level may predict poor survival in patient with cutaneous ALCL.

A case of yellow nail syndrome with myocardial infarction and hypothyroidism

( Youngkyoung Lim ),( Jongeun Lee ),( Hyun Jeong Byun ),( Chan Seong Park ),( Jae Ho Lee ),( Ji-hye Park ),( Jong Hee Lee ),( Dong-youn Lee ),( Joo-heung Lee ),( Jun-mo Yang ) 대한피부과학회 2018 대한피부과학회 학술발표대회집 Vol.70 No.1

Yellow Nail Syndrome (YNS) is a rare disorder that almost always occurs after 50 years of age but a juvenile or familial form has also been observed. It is chracterized by yellow nail discoloration, pulmonary manifestations, and lower limb lymphedema. Nail manifestation includes slow growth, thickening, hardening, transversal ridging, onycholysis, onychorrhexis, and paronychia. Pulmonary manifestation shows bronchitis, bronchiectasis, pneumonia, and pleural effusions. Chronic sinusitis is frequently associated with the triad of YNS. A 61-year-old male visited our clinic with nail dystrophy which started 18 months ago. Nail changes were observed including nail thickening, yellow color change, and transverse ridging. Result of potassium hydroxide (KOH) preparation, and fungus culture were negative. Periodic acid-Schiff (PAS) staining of nail clipping specimen revealed no fungal hyphae. There had been history of bronchiectasia with bronchiolitis, pneumonia, and chronic sinusitis. The patient had also experienced myocardial infarction (MI) and hypothyroidism. Herein, we present a very rare case of YNS with both MI and hypothyroidism.

A case of cowden syndrome with multi-systemic tumors and thyroid cancer

( Jongeun Lee ),( Hyun Jeong Byun ),( Chan Seong Park ),( Youngkyoung Lim ),( Ji-hye Park ),( Jong Hee Lee ),( Dong-youn Lee ),( Joo-heung Lee ),( Jun-mo Yang ) 대한피부과학회 2019 대한피부과학회 학술발표대회집 Vol.71 No.1

Cowden syndrome is caused by mutation in phosphatase and tensin homolog (PTEN) gene, belonging to PTEN hamartoma tumor syndrome. A 21-year-old female presented with small papules on palms and neck. In addition, there were solitary papules under the nose and the upper lip and skin tags on both axilla. Her past medical history included intramuscular arteriovenous malformation versus hemangioma on right lower leg, cyst in the right ovary, multiple follicles in the left ovary, fibroadenoma in the left breast, and phyllodes tumor and intraductal papilloma in the right breast. Moreover, additional mass in thyroid turned out to be a papillary thyroid carcinoma. The mutation analysis for PTEN hamartoma syndrome confirmed pathogenic variant in the PTEN gene. A 3mm-punch biopsy on the left palm revealed regular orthokeratosis and hyperkeratosis consistent with palmoplantar keratosis. During the follow up visits, the patient developed hematochezia due to numerous hyperplastic and lymphoid polyps in stomach through rectum. Cowden syndrome possesses increased risk of developing malignancies including breast, thyroid, and colorectal cancers. Therefore it is important for dermatologists to recognize symptoms of the possible multi-systemic tumors in these patients. Herein, we present a case of Cowden syndrome with multi-systemic tumors and thyroid cancer.

A case of follicular mucinosis in a child at unusual location

( Jongeun Lee ),( Hyun Jeong Byun ),( Chan Seong Park ),( Youngkyoung Lim ),( Ji-hye Park ),( Jong Hee Lee ),( Dong-youn Lee ),( Joo-heung Lee ),( Jun-mo Yang ) 대한피부과학회 2019 대한피부과학회 학술발표대회집 Vol.71 No.1

Follicular mucinosis (FM) is usually presented as welldemarcated erythematous or skin-colored patches with follicular papules at head or neck. It is a rare entity in children. A healthy 4-year-old boy presented with a 1-month history of an erythematous patch at left dorsum of nose. The patch was rather uneven because of follicular papules and firm on palpation. The pathologic findings showed deposition of blue amorphous material in sebaceous gland with lymphocytic infiltration, suggesting inflammation of sebaceous gland without histopathologic features of mycosis fungoides such as epidermotropism of lymphocytes or lymphoid atypia. The lesion was completely healed after 6 weeks of topical steroid (0.25% prednicarbate). Although it has been described to follow benign courses in children, close follow up is warranted as it is difficult to distinguish idiopathic FM and lymphoma-associated FM. In English literature published so far, head accounted for around 80% in the solitary FM cases. Forehead and chin were the most frequently involved location while FM involving nose was reported only twice, each in multiple and solitary cases. Herein, we report a case of FM in a child at nose treated with topical steroid.

A case of exacerbation of psoriasis after chemotherapy (TS-1)

( Jae Ho Lee ),( Youngkyoung Lim ),( You Jin Lee ),( Jong Yoon Chung ),( Ji Hye Park ),( Jong Hee Lee ),( Dong Youn Lee ),( Joo Heung Lee ),( Jun Mo Yang ) 대한피부과학회 2015 대한피부과학회 학술발표대회집 Vol.67 No.1

Psoriasis usually regresses with chemotherapy. Axx-year-old woman with a pre-existing psoriasis was treated with TS-1 chemotherapy (120 mg/day, orally administrated) for an advanced gastric cancer. Within 8 weeks of starting therapy, she developed a psoriasis flare, proved by skin biopsy. After consulting with oncology department, we decided to continue chemotherapy and treat psoriasis with topical creams. The erythematous lesion markedly improved within 2 weeks following the treatment. In previous report on TS-1 with psoriasis, the skin lesion was improved after TS-1 intake. We report here that psoriasis can be a side effect of TS-1 therapy for cancer.