A Case of Linear Lupus Erythematosus Tumidus / 대한피부과학회지

Lupus erythematosus tumidus (LET) is a variant of chronic cutaneous lupus erythematosus (CCLE). Clinically, the lesions show erythematous, succulent, urticaria-like, nonscarring papules and plaques with a smooth surface, and are usually related to sun exposure. This condition resembles polymorphous light eruption, cutaneous lupus erythematosus, and reticular erythematosus mucinosis. Histopathological findings of the lesion showed perivascular and periadnexal lymphocytic cell infiltration with interstitial mucin deposition, but there is little or no epidermal or dermo-epidermal involvement. Only a few cases of LET have been described in the literature because no definitive clinical and histopathologic diagnostic criteria for LET have been accepted. Linear cutaneous lupus erythematous is an unusual presentation of CCLE. Moreover linear LET is extremely rare. Therefore we present herein a case of unilateral linear lupus erythematosus tumidus on left upper extremity in a 37-year-old Korean woman.

A Case of Granulomatous Reactions to a Permanent Red Tattoo / 대한피부과학회지

Complications of tattooing include infection, hypersensitivity reaction and scarring. Hypersensitivity reaction is most common. Components of the red pigments used in some tattoos are the most commonly known cause of delayed tattoo reactions. A 22-year-old woman presented with 3~4 mm sized, well-defined indurated scaly plaques confined to red portion of rose tattoo on the dorsal surface of the left foot. She complained of mild pain and severe pruritus on the lesion. Histopathologic examination showed granulomatous reactions from a permanent red tattoo. The patient was transferred to the plastic surgery for surgical intervention. We document an unusual tattoo reaction presenting as plaques from red portion of tattoo, which on histopathologic examination showed granulomatous reaction.

A Case of Proliferating Trichilemmal Cyst on the Elbow / 대한피부과학회지

Proliferating trichilemmal cyst is a rare benign tumor of external root sheath origin that usually occurs on the scalp of elderly women. It is rarely found on the face, neck, trunk and back. A 17 year-old man presented with a skin-colored, subcutaneous, tender nodule on the elbow with a history of bee sting. A histological examination revealed a well demarcated multilobular cystic mass characterized by trichilemmal keratinization in the dermis. The skin lesion was totally excised and recurrence has not been found. We present a case of proliferating trichilemmal cyst of unusual predilection site.

Two Pilosebaceous Cysts with Apocrine Hidrocystoma in One Biopsy Site: A Spectrum of the Same Disease Process?

A 28-year-old woman presented with multiple, asymptomatic, erythematous to bluish papules located on the chest. Histopathologically, three round, well defined cystic structures were seen on the upper and lower dermis. The first cyst was milia, the second was apocrine hidrocystoma and the other, largest cyst was an eruptive vellus hair cyst (EVHC). A diagnosis of multiple pilosebaceous cysts combined with apocrine hidrocystoma was made. Since the milia and EVHC originate from the pilosebaceous unit, and the apocrine duct opens to the pilosebaceous orifice, we suggest that they can occur simultaneously in the same unit.

Treatment of Severe Alopecia Areata: Combination Therapy Using Systemic Cyclosporine A with Low Dose Corticosteroids

BACKGROUND: Combination therapy using cyclosporine A (CsA) together with low-dose corticosteroids has adequate efficacy with little toxicity for the treatment of severe alopecia areata (AA). OBJECTIVE: We wanted to evaluate the clinical efficacy of combination therapy using CsA with low-dose corticosteroid for the treatment of severe AA and we also wanted to determine the safe therapeutic concentration of CsA in the peripheral blood. METHODS: We treated 34 cases of severe AA with combination therapy for 24 weeks and we evaluated the efficacy at 12 and 24 weeks. We monitored the peripheral blood concentration of CsA to determine the therapeutic range of CsA that has the fewest side effects. RESULTS: Of the patients, 77.4% (n=24) and 22.6% (n=10) were classified in the responder and poor-responder groups, respectively. The mean trough concentration of CsA was 95.1 and 101.2 ng/ml in the responder and poor-responder groups, respectively. For the patients with side effects associated with CsA, the mean CsA concentration was 195.8 ng/ml. CONCLUSION: We found that combination therapy with systemic CsA and low-dose corticosteroids effectively treats severe AA and this therapy results in a safe, therapeutic concentration of CsA in the peripheral blood.

A Case of Systemic Lupus Erythematosus and Secondary AntiphospholipidSyndrome Presenting as Livedo Reticularis / 대한피부과학회지

Antiphospholipid syndrome is defined as the presence of lupus anticoagulant antibodies or anticardiolipin antibodies with vascular thrombosis or specific pregnancy complications. Antiphospholipid syndrome can be associated with autoimmune, malignant or infectious diseases. Cutaneous manifestations of antiphospholipid syndrome are variable and can be a first clue to the syndrome or other associated diseases. We report a case of systemic lupus erythematosus and secondary antiphospholipid syndrome presenting as livedo reticularis on both legs of a patient. We recommend that patients with livedo reticularis should be examined for systemic lupus erythematosus or antiphospholipid syndrome.

A Case of Autosomal Recessive Type 2 Pseudoxanthoma Elasticum Presenting Generalized Cutaneous Laxity / 대한피부과학회지

Pseudoxanthoma elasticum is a rare, heritable disorder of elastic fibers characterized by yellowish, coalescing papules on the loose and wrinkled flexural skin. An 18-year-old man presented cutis laxa-like marked wrinkling on the axillae, trunk and thighs for about 10 years. There was no similar skin lesion on his family members and no other systemic involvement was found. Histopathological examination of the abdomen revealed fragmentation and calcification of elastic fibers in the middle and lower dermis. Von Kossa staining showed calcium deposition along the altered elastic fibers. Herein, we report a rare case of autosomal recessive pseudoxanthoma elasticum presenting generalized cutaneous laxity.

A Case of Linear Milia en Plaque on the Central Face

Milia en plaque (MEP) is a rarely-described benign, cutaneous dermatosis, characterized by numerous tiny milia within an erythematous base which usually occurs around the ears. We report a case of a well-defined, linear MEP arising from the right side of the forehead to the nasal bridge. This case is of particular interest as the site of occurrence is uncommon and the shape of lesion is atypical.

A Case of Restrictive Dermopathy

Restrictive dermopathy is a lethal, autosomal recessive disorder characterized by tautness of skin, multiple joint contractures, and respiratory insufficiency resulting in fetal akinesia and death during the neonatal period. Histologic findings show a flat dermoepidermal junction, overall thinned dermis with hypoplastic appendages, a dense fibrotic reticular dermis with collagen parallel to the epidermis, and a thick layer of subcutaneous adipose tissue. It is a rare disorder that has not yet been reported in the Korean literature. Herein, we present a case of restrictive dermopathy in a neonate.

Two Cases of Generalized Granuloma Annulare in Infants

Generalized granuloma annulare (GGA) is characterized by disseminated necrotic dermal papules but the pathogenesis has not been fully elucidated. A 5-month and a 3-month male infant had diffusely scattered, erythematous papules on their trunks, and upper and lower extremities. Histopathological examination revealed an infiltration of histiocytes around and between altered collagen fibers in upper and mid dermis. The collagen fibers were separated by mucin, which was positively stained with alcian blue at pH 2.5. After they were treated with topical and/or systemic corticosteroid, the lesions disappeared. To the best of our knowledge, these two cases are non-perforating GGA in the youngest patients yet reported in English literature.

A Case of Recurrent Cutaneous Eosinophilic Vasculitis / 대한피부과학회지

Recurrent cutaneous eosinophilic vasculitis (RCEV) is a rare cutaneous disease characterized by necrotizing vasculitis of small vessels, with exclusive eosinophilic infiltration and minimal, or no leukocytoclasis. It presents with recurrent erythematous or purpuric papules or plaques, or angioedema associated with peripheral eosinophilia. This disease follows a benign chronic course without evidence of systemic involvement. Systemic corticosteroid therapy is effective but the disease commonly pursues a relapsing and remitting course. Herein, we report a case of RCEV that was effectively treated with systemic corticosteroids and dapson(R)(diaminodiphenylsulphone). Furthermore, the patient did not show a relapse of the disease in spite the tapering the systemic corticosteroid dose.

A Case of Delayed Cutaneous Reaction to a Jellyfish Sting from the Korean Coastline / 대한피부과학회지

A 17-year-old woman was stung by a jellyfish on her right lower leg in waters off the Korean coastline. The lesions presented as erythematous, painful patches and cleared-up within 2 weeks. However, 2 weeks later, erythematous band-like patches and crusts developed at the previous contact site and similar lesions occurred on non-contact areas. Histologic examination revealed hyperkeratosis, focal spongiosis in the epidermis and perivascular lymphohistiocytic infiltrate, with numerous eosinophils in the upper dermis. We report a case of jellyfish dermatitis which showed immediate toxic reaction and delayed cutaneous reaction following a jellyfish sting from a species present in Korea.