Deok-Woo Lee

Clinical and pathological characteristics of extradigital and digital glomus tumours: a retrospective comparative study.

Background  Most glomus tumours are located in the digits, especially in subungual areas. Less is known, however, about the clinical characteristics of extradigital glomus tumours.

Erythema annulare centrifugum following herpes zoster infection: Wolf's isotopic response?

1 Yamaguchi K. Human T-lymphotropic virus type I in Japan. Lancet 1994; 343:213–6. 2 Johno M, Ohishi M, Kojo Y et al. Cutaneous manifestations of adult T-cell leukemia ⁄ lymphoma. Gann Monogr Cancer Res 1992; 39:33–41. 3 Bazarbachi A, Ghez D, Lepelletier Y et al. New therapeutic approaches for adult T-cell leukaemia. Lancet Oncol 2004; 5:664–72. 4 Setoyama M, Katahira Y, Kanzaki T. Clinicopathologic analysis of 124 cases of adult T-cell leukemia ⁄ lymphoma with cutaneous manifestations: the smouldering type with skin manifestations has a poorer prognosis than previously thought. J Dermatol 1999; 26:785–90. 5 Shimoyama M. Diagnostic criteria and classification of clinical subtypes of adult T-cell leukaemia-lymphoma. A report from the Lymphoma Study Group (1984–87). Br J Haematol 1991; 79:428–37. 6 Pawson R, Mufti GJ, Pagliuca A. Management of adult T-cell leukaemia ⁄ lymphoma. Br J Haematol 1998; 100:453–8. 7 Zhang C, Duvic M. Retinoids: therapeutic applications and mechanisms of action in cutaneous T-cell lymphoma. Dermatol Ther 2003; 16:322–30. 8 Maeda Y, Miyatake J, Sono H et al. New therapeutic effects of retinoid for adult T-cell leukemia. Blood 1996; 88:4726–7. 9 Maeda Y, Naiki Y, Sono H et al. Clinical application of all-trans retinoic acid (tretinoin) for adult T-cell leukaemia. Br J Haematol 2000; 109:677–8. 10 Maeda Y, Yamaguchi T, Ueda S et al. All-trans retinoic acid reduced skin involvement of adult T-cell leukemia. Leukemia 2004; 18:1159–60.

Intravascular histiocytosis without rheumatoid arthritis

Dear Editor, Intravascular histiocytosis (IVH) is a rare condition characterized by the proliferation of histiocytes in a vessel lumen, and is thought to arise as a benign reaction to certain stimuli, such as rheumatoid arthritis (RA). We report a case of IVH without evident systemic disease, presenting as erythematous papulonodules on the neck and demonstrating distinctive histopathological features. A 29-year-old Korean woman presented with a 1-year history of two painful papulonodules on her right neck (Fig. 1). The lesions had a fluctuating course, and were accompanied by mild fever, throat soreness of the right side and ear pain of the right side radiating to the neck when the lesions had been aggravated. Treatment with anti-inflammatory drugs for several weeks relieved the symptoms, but did not prevent recurrence. Physical examination of her ears and throat and past medical history were unremarkable. Laboratory tests were generally within normal limits, except for a mild decrease in C3 (73 mg/dL; normal range, 88–201). Histopathological examination of a skin biopsy from one papule showed increased dilated and thrombosed vessels in the dermis, proliferating in a glomeruloid pattern. Some vessels contained free-floating, medium-sized mononuclear cells without evident nuclear atypia (Fig. 2a,b). Dermal hemorrhage was also observed. Most mononuclear cells in the vascular lumen were immunohistochemically positive for CD68 (KP-1) (Fig. 2c), but not for leukocyte-common antigens CD3 or CD20. Some endothelial cells lining the vessels, but not the mononuclear cells themselves, were positive for CD31 (Fig. 2d) and CD34. The vessels were negative for D2-40. The final diagnosis of IVH was made. The other nodular lesion was later found to have similar histopathological properties. Following complete removal of both lesions, there have been no recurrences in a 1-year follow-up period. Intravascular histiocytosis was first observed as the intraluminal proliferation of cells bearing histiocytic markers, with only 11 cases reported to date in the English language published work. IVH was likely to arise in association with coexistent systemic diseases, with 8 cases associated with RA, as evidenced by specific eruptions such as chronic irregularly-shaped or livedoid erythema or papules over and around the involved joints. The other cases which were not associated with RA showed erythematous to violaceous, sometimes hemorrhagic or ecchymotic macules, patches, papules and nodules, consistent with our patient’s erythematous papulonodules. The pathogenesis of IVH is unclear. In previous cases of IVH with RA, cutaneous lesions have been thought to be caused by lymphatic drainage of inflammatory components away from the inflamed synovium, as suggested by the lymphatic nature of the vessels. In contrast, the vessels observed in our case exhibited negative expression of the lymphatic marker, D2-40, suggesting that different etiological factors may be involved in the development of IVH

Glomeruloid hemangioma as a very early presenting sign of POEMS syndrome

To the Editor, Glomeruloid hemangioma (GH) is a distinctive cutaneous angioma with unique histological features resembling those of renal glomeruli. GH has been considered as a specific marker for POEM (polyneuropathy, organomegaly, endocrinopathy, M-protein and skin changes) syndrome.1 However, four reports have claimed that GH is not always associated with POEMS syndrome.2– 5 Here, we report on a 40-year-old Korean woman with multiple GHs who was diagnosed with POEMS syndrome 6 years after first GH onset. A 40-year-old Korean woman presented with 6year history of multiple eruptive purple red papules on her face and neck (Fig. 1). As she also had generalized edema, 5 kg of weight loss over the preceding month and hypertrichosis, she was admitted and generally evaluated. Examination revealed bilateral pleural effusions, ascites, hypothyroidism [T3<50 ng/dl (normal, 98–180 ng/dl); thyroidstimulating hormone 11.0 mIU/l (normal, 0.4–5.0 mIU/l); free T40.71 ng/dl (normal, 0.8–1.9 ng/dl)], hepatosplenomegaly, retroperitoneal lymphadenopathy, resting pulmonary hypertension and sensorimotor polyneuropathy. Serum protein electrophoresis was positive for lambda-type immunoglobulin A, and urine immunofixation electrophoresis revealed Bence-Jones proteins of the lambda type. Skin biopsies revealed a vascular tumor composed of capillaries and sinusoid-like spaces resembling renal glomeruli (Fig. 2). Sinusoidal endothelial cells had eosinophilic globules, which stained with periodic acid-Schiff (PAS) reagent (Fig. 3A). Capillary endothelial cells were positive for CD31 (Fig. 3B) and CD34 (Fig. 3C) but negative for CD68 (Fig. 3D). Sinusoidal endothelial cells were positive for CD31 (Fig. 3B) and CD68 (Fig. 3D) but negative for CD34 (Fig. 3C). The histopathologic and immunohistochemical findings were consistent with GH. (A)

A Case of Tumor of the Follicular Infundibulum with Sebaceous Differentiation

Tumor of the follicular infundibulum (TFI) is an uncommon benign adnexal tumor that usually presents as a solitary keratotic papule on the face or scalp of elderly patients. Histopathologically, it typically manifests as a plate-like fenestrated proliferation of monomorphic pale-staining cells. A 76-year-old male presented with about a 2 cm, well-defined, yellowish to brownish, slightly elevated, twisted and bent, interrupted, cord-like plaque on his left lower abdomen. Microscopic examination revealed a sharply demarcated plate-like proliferation of pale cells localized in the papillary dermis with multiple connections to the overlying epidermis. The histopathological features were compatible with TFI, except for foci of sebaceous differentiation. There has been one previous case report of TFI with sebaceous differentiation in the English medical literature. Herein, we report on a singular case of TFI with sebaceous differentiation.

An iatrogenic dermatosis with ulceration

A 36-year-old woman presented with a 1-year history of denuded nodules on the perianal area. She complained of skin laxity in the axilla, groin and abdomen of 7 years’ duration, and she had multiple, yellowish, 2–3 mm sized papules on her neck, giving a plucked chicken appearance (Fig. 1). She had been on longterm penicillamine therapy at a dose of 1 g daily since the age of 10 years for treatment of Wilson’s disease. Skin examination demonstrated several denuded nodules with marginal induration on the perianal area (Fig. 2).

A case of incontinentia pigmenti associated with multiorgan abnormalities.

Incontinentia pigmenti is a systemic disorder affecting the skin, teeth, eyes, nervous tissue, hair, nails, musculoskeletal system, and heart. We describe an 11-month-old girl with incontinentia pigmenti associated with a ventricular septal defect, left hemiatrophy, hemangiomas, an abnormal labial frenum, and spastic cerebral palsy manifested as left hemiplegia and developmental delay. We believe this patient illustrates that incontinentia pigmenti is a systemic disorder necessitating a multidisciplinary approach to management.