Kyung Jeh Sung

Clinicopathologic Analysis of 66 Cases of Erythema Annulare Centrifugum

Erythema annulare centrifugum (EAC) denotes a group of eruptions characterized by slowly migrating annular or configurate erythematous lesions. It can be classified histopathologically as the deep or superficial type. Although there are many case reports of EAC associated with various underlying conditions, no recent clinicopathologic studies exist. The purpose of this study was aimed at analyzing the clinical and histopathologic features of EAC. Sixty‐six patients who have been diagnosed as EAC by clinical and histopathological examination were collected. The medical records and skin biopsy specimens of these patients were reviewed retrospectively. There were 24 male patients and 42 female ones. The mean age was 39.7 years, and the mean durtion of the disease was 2.8 years. The lower extremities, particularly the thigh, were the most frequently involved locations. The most common clinical manifestation was large (>1 cm), scaly, erythematous, indurated plaques. Forty‐eight patients (72%) had combined diseases including cutaneous fungal infection (48%), such as tinea pedis, other skin diseases (18%), internal malignancies (13%), and other systemic diseases (21%). By histopathologic examination, 33 of 42 patients (78%) were identified as superficial type, and 9 patients (22%) were deep type. Therapeutic trials with systemic or topical corticosteroid and oral antihistamine did not affect the chronic and recurrent course of these patients. EAC is a chronic and recurrent disease despite treatment. EAC is thought to be highly associated with internal disease as well as with superificial fungal infection. However, it was difficult to prove a causal association. The recognition and exact diagnosis of EAC is important, because it may be a quite stressful condition and lead to unnecessary over‐treatments.

Apoptosis Is Induced by Anti‐Fas Antibody Alone in Cultured Human Keratinocytes

Fas is a well‐known cell surface receptor whose main function is the induction of apoptosis in many cell types including human keratinocytes. Several reports indicate that anti‐Fas antibody can induce apoptosis in cultured keratinocytes after interferon gamma (IFNγ) pretreatment.

A case of necrobiotic xanthogranuloma without paraproteinemia presenting as a solitary tumor on the thigh

A 82‐year‐old Korean woman had had a 6‐month history of an asymptomatic, flat, hard, red to brown tumor on her right thigh. This lesion had been slowly enlarging with an advancing margin. She had noted gradually developing pain associated with necrosis and ulceration on the lesion. Examination revealed a solitary, 8 × 7.5 cm, yellow to dark red, telangiectatic tumor with multiple areas of punched out ulceration and a peripheral elevated yellowish margin on the right inner upper thigh ( Fig. 1 ). No clinically similar lesions on the periorbital area or other sites were seen. Histologic examination revealed a massive palisading granulomatous infiltration with several layers of extensive bands of necrobiotic zone in the entire dermis and deep subcutaneous tissue ( Fig. 2a ). In the granulomatous infiltrate in the dermis and subcutis, many various‐shaped, some bizarre, angulated, foreign‐body type multinucleated giant cells, many Touton giant cells, and a few Langhans giant cells were found to be scattered ( Fig. 2b ). There were numerous xanthomatized histiocytes. Dense infiltration of lymphoplasma cells was seen in the periphery of the granuloma and perivascularly. Conspicuous granulomatous panniculitis composed of lymphoplasma cells, polymorphonuclear cells, foam cells, and Touton and foreign‐body giant cells was also seen. However, cholesterol clefts and lymphoid follicles were not seen. Subcutaneous septae were widened by necrobiotic change and fibrosis with thrombosed large vessels. Gram, Gomeri‐methenamine silver and acid‐fast stains were negative. The necrobiotic areas were positive to alcian blue. Laboratory investigation revealed elevated white blood cell counts, anemia and elevated erythrocyte sedimentation rate. The following parameters were within the normal range: lipids, glucose, renal and liver function tests, serum complements, serum immunoglobulins, cryoglobulins and antinuclear antibodies. The findings of chest X‐ray, skull X‐ray and ectorcardiography were normal. Serum electrophoresis and serum immunoelectrophoresis revealed no abnormality. The patient was diagnosed as having necrobiotic xanthogranuloma without paraproteinemia. She was treated with oral steroid (0.5–0.6 mg/kg) and NSAIDS for 1 month with partial improvement of pain and the lesion ceased to enlarge. In the following 1 year of follow‐up, with only intermittent NSAIDS, her lesion did not progress and there were no signs of systemic involvement or new skin lesions.

Congenital CD34-positive granular cell dendrocytosis

Granular cell tumors involving the skin are mostly acquired lesions. The Schwann cell origin of these lesions is supported by positive Immunostaining for S‐100 protein and myelin basic protein. S‐100–granular cell lesions rarely have been described in association with fibrous papules or dermatofibromas. The congenital variety of S‐100‐granular cell tumors occurs almost exclusively in the gingiva. The cell origin of these lesions is not well delineated. We report a hitherto undescribed case of a congenital cutaneous lesion which is histologically characterized by diffuse dermal infiltrates of S‐100‐ but CD34+ granular dermal dendrocytes. The granular appearance of these CD34+ dendrocytes is attributed to an abundance of phagolysosomes. The pathogenetic mechanism of this unusual lesion remains to be elucidated.

A Case of Pretibial Mucinosis without Thyroid Disease

To the Editor: Pretibial mucinosis associated with venous stasis or other underlying conditions must be differentiated from pretibial myxedema, which is characterized by localized mucin deposition in patients with thyroid disease. Pretibial mucinosis shares many similar clinical findings with pretibial myxedema but has different histologic features. We present a case of pretibial mucinosis which was combined with chronic, recurrent cellulitis and venous insufficiency after trauma. A 44-year-old female visited the dermatologic clinic for evaluation of an erythematous swollen patch on her right leg. Nine months earlier, she had been traumatized on the right leg during a traffic accident. Thereafter, she had suffered from recurrent painful edema and erythematous patches on the right leg four times and been diagnosed with recurrent, bacterial cellulitis, confirmed by tissue culture in another orthopedic clinic. A physical examination revealed relatively ill-defined, erythematous, swollen patches with some erythematous macules on the right shin (Fig. 1). A Roentgenologic examination of the right leg revealed no abnormal findings other than mild soft tissue swelling. Histopathology revealed a loosely edematous superficial dermis and no discernible grenz zone of normal collagen in the papillary dermis. Colloidal iron staining demonstrated mucin deposition filling the papillary dermis and extending to the upper reticular dermis (Fig. 2A). There was remarkable hemosiderin deposition on the upper reticular dermis (Fig. 2B). There were thickened and degenerated veins in the deep dermis with mild perivascular inflammatory infiltrates. She had no past medical history of thyroid disease, and her thyroid function test and TSH level were within normal limits. Pretibial mucinosis associated with venous insufficiency due to recurrent cellulitis after trauma was the final diagnosis. Pretibial mucinosis without thyroid disease is a very rare disease frequently associated with stasis dermatitis (1, 2). Its clinical features can be difficult to distinguish from those of pretibial myxedema associated with thyroid disease. The histopathologic findings of pretibial mucinosis that are distinguishable from those of pretibial myxedema are deposition of mucin in the upper dermis, no discernable grenz zone of normal collagen in the superficial papillary dermis, angioplasia, and hemosiderin deposition (1, 2). In contrast, the consistent histologic features of pretibial myxedema included a grenz zone The Journal of Dermatology Vol. 29: 383–385, 2002

Metastatic cutaneous leiomyosarcoma from primary neoplasm of the mesentery.

A 31‐year‐old South Korean woman was referred to the dermatology department from the oncology department for the evaluation of a subcutaneous nodular lesion on the back. Three years before, she noted a palpable, fingertip‐sized, nontender mass on her right lower abdomen. The mass had increased in size slowly. One year ago, she visited a local clinic and physical examination revealed a 7 × 8 × 7 cm, slightly tender, deep‐seated mass on the right lower quadrant of the abdomen. The mass on the ilial mesentery was resected by surgical exploration and tissue examination revealed leiomyosarcoma. She refused adjuvant chemotherapy.

Four Cases of Pustulotic Arthro‐Osteitis

We describe four cases of pustulotic arthro‐osteitis. All of them suffered from anterior chest wall symptoms, such as swelling and pain on the sternoclavicular joint, sternal angle area, sternocostal joint, and costochondral junction. Although chest X‐ray findings were all normal, 90mTc‐scintigraphy revealed increased uptake in the affected areas in all cases. Laboratory examinations including rheumatoid factor were negative or normal except for elevation of the erythrocyte sedimentation rate and C‐reactive protein in 2 cases. After treatment with non‐steroidal anti‐inflammatory drugs and etretinate or topical PUVA, the skin lesions on the palms and soles and the arthro‐osteitis were moderately improved. Pustulotic arthro‐osteitis should be considered when a patient with pustulosis palmoplantaris complains of anterior chest wall pain and other joint pain.

A CASE OF HIDROACANTHOMA SIMPLEX

There are many conditions characterized by nests of cells within the epidermis. One of them, hidroacanthoma simplex, has been regarded as an epidermal tumor differentiating to intraepidermal eccrine duct cell. We report a case of hidroacanthoma simplex with the results of immunohistochemical study. Staining for 35βH11 (reacting with keratin No. 8), 35βE12 (reacting with keratin No. 1, 5, 10, 11), S‐100 protein, and CEA was negative in the tumor cell nests; these monoclonal antibodies stained the nests of eccrine poroma.

A case of congenital tufted angioma mimicking cavernous hemangioma.

To the Editor: Since Nakagawa first described tufted angioma (angioblastoma) in 1949, many cases have been reported. Recently, new cases with uncommon locations or unusual clinical presentations have been added to the list. These include tufted angioma with spontaneous regression, metastatic or intravenous cases, and one case arising in a portwine stain (1–3). We herein report a newborn baby with tufted angioma on the thigh that presented as cavernous hemangioma. This 14-day-old female infant had had a huge mass on the right posterior thigh from birth. Clinically, the lesion presented as a localized, 6 × 6 cm, firm, rubbery, subcutaneous mass with overlying telangiectatic redto bluish skin and a pale dusky border (Fig. 1). The lesion was warm to the touch without thrill. Initially, the clinical impression was congenital cavernous hemangioma. A biopsy specimen from the skin lesion showed multiple nodular aggregations of blood vessels from the upper dermis to near the dermal-subcutaneous junction. The vessels appeared in discrete rounded tufts forming what has been referred to as a “cannonball” pattern (Fig. 2). In the tufts, there were dilated, cleft-like spaces lined by somewhat large endothelial cells without cytologic atypia (Fig. 3). Most of the tumor cells were stained positively for CD34. All histopathologic findings suggested tufted angioma. Tufted angioma is a rare benign vascular abnormality named for the characteristic histopathologic findings of dermal capillary tufts (4). The lesion usually begins in infancy or early childhood and sometimes appears in adults. Tufted angioma presenting at birth, as in our case, is not an unusual event. Igarashi et al. (5) insisted that the congenital form was approximately one forth of cases. The commonly involved sites are the trunk, neck, shoulder, upper chest, back, lower limbs and upper limbs in descending order (5, 6). Clinically, tufted angiomas most comThe Journal of Dermatology Vol. 28: 514–515, 2001

A Case of Neutrophilic Eccrine Hidradenitis in an Infant

relatively rare neutrophilic dermatosis and first described as a transient and benign complication of various chemotherapy regimens for acute leukemia. More recently, NEH has been observed in other conditions, such as infections or drugs, before the diagnosis of malignancy and even generally healthy individuals. Furthermore, there were several reports of idiopathic palmoplantar hidradenitis, which had similar clinical and histopathologic findings with NEH, in children without history of chemotherapy. We herein describe a nine-month-old female infant with NEH on the extremities including palms and soles. Although she had acute myelogenous leukemia (AML), the lesions of NEH appeared before starting chemotherapy, suggestive of other etiology. CASE REPORT