Hae-Woong Lee

Two cases of angiomyxolipoma (vascular myxolipoma) of subcutaneous tissue

Abstract:  Angiomyxolipoma (vascular myxolipoma) is a recently described rare variant of lipoma, four cases of which have been reported to date. Microscopically, the lesion consists of adipose tissue without lipoblasts, extensive myxoid areas, and numerous blood vessels. The main differential diagnosis of this lesion is myxoid liposarcoma, and other adipocytic lesions such as myxolipoma, myxoid spindle cell lipoma should be included. We report two cases of angiomyxolipoma located in the subcutaneous tissue of the forearm and the wrist.

Pseudomelanoma following laser therapy.

1 Gimènez Garcìa R, de la Lama Lòpez-Areal J, Avellabeda Martinez C. Scabies in the elderly. J Eur Acad Dermatol Venereol 2004; 18 : 105–107. 2 Bergin O, O’Reilly M, Goha J, O’Neill M. Incidence of sexually transmitted diseases amongst a elderly cohort attending a genito-urinary clinic. J Eur Acad Dermatol Venereol 1995; 5 : 218–221. 3 Gordon SM, Thompson S. The changing epidemiology of human immunodeficiency virus infection in older persons. J Am Geriatric Soc 1995; 43 : 7–9. 4 Tan HH, Goh CL. Parasitic skin infestations in the elderly: recognition and drug treatment. Drugs and Aging 2001; 18 : 165–176. 5 Morin C, Joly P, Courville P et al. Chronic eczematiform eruption in the elderly. Ann Dermatol Venereol 2002; 129 : 19–22. 6 Iehl-Robert M, Monnier F, North C. Scabies, a pathology still present in the elderly. Soins Gerontol 2001; 18 : 165–176.

Hyperkeratosis of the nipple and areola as a sign of malignant acanthosis nigricans

A 34-year-old woman presented with a 2-year history of generalized hyperpigmented, verrucous cutaneous thickening and a 9-month history of hyperkeratotic lesions over her nipples and areolae. Two years previously, she had experienced recurrent epigastric soreness and cutaneous truncal thickening, especially in the axillae. Diagnostic investigation at that time revealed a poorly differentiated gastric adenocarcinoma with multiple lymph-node metastases. Distal gastrectomy and nine courses of monthly chemotherapy were performed, and the patient experienced significant improvement of her skin lesions. She was then lost to follow-up, until she returned to the oncology department for generalized weakness, abdominal pain and orthopnoea. She was subsequently referred to us because of worsening of her cutaneous conditions, which she reported had recurred approximately 9 months previously, including involvement of her nipples and areolae. Physical examination revealed hyperkeratotic thickening with brownish hyperpigmentation on the nipples and areolae (Fig. 1a), an almost generalized hyperpigmented velvety thickening of the skin, most pronounced in the axillae, neck, groin and perineum, papillomatous lesions on her eyelids and perioral area, and tylosis with pachydermatoglyphy (Fig. 1b). Skin biopsies from axilla and areola showed hyperkeratosis and epidermal papillomatosis; acanthosis was more prominent in the areolar lesion (Fig. 2a, b). The patient was diagnosed with malignant

Two Cases of Telangiectasia Macularis Eruptiva Perstans Demonstrated by Immunohistochemistry for C‐Kit (CD 117)

Telangiectasia macularis eruptiva perstans (TMEP) is an uncommon form of cutaneous mastocytosis that occurs exclusively in adults. Histologically, TMEP presents with scattered mast cells lined up around the dilated capillaries and venules of the superficial vascular plexus. In some cases, the number of mast cells falls within the range observed in normal skin and therefore cannot be detected by routine histologic examination. We used immunohistochemical staining for c‐kit (CD 117) for the definitive diagnosis in two patients with TMEP. One of them was successfully treated with topical application of pimecrolimus.

A case of pseudolymphomatous folliculitis

230 JEADV 2006, 20, 214–238

Segmental lichen aureus: combination therapy with pentoxifylline and prostacyclin.

1 Naeyaert JM. Xanthomes. Ann Dermatol Venereol 2003; 130: 65–68. 2 Seymour CA. Xanthomas and abnormalities of lipid metabolism and storage. In: Champion RH, Burton JL, Burns DA, Breathnach SM, eds. Textbook of Dermatology, vol. 3, 6th edn. Blackwell Science Ltd., Oxford, 1998: 2600–2615. 3 Garcia CK, Wilund K, Arca M et al. Autosomal recessive hypercholesterolemia caused by mutations in a putative LDL receptor adaptor protein. Science 2001; 292: 1394–1398. 4 Zuliani G, Vigna GB, Corsini A et al. Severe hypercholesterolaemia: unusual inheritance in an Italian pedigree. Eur J Clin Invest 1995; 25: 322–331. 5 Arca M, Zuliani G, Kenneth W et al. Autosomal recessive hypercholesterolaemia in Sardinia, Italy, and mutation in ARH: a clinical and molecular genetic analysis. Lancet 2002; 359: 841–847. 6 Cohen JC, Kimmel M, Polanski A, Hobbs HH. Molecular mechanisms of autosomal recessive hypercholesterolemia. Curr Opin Lipidol 2003; 14: 121–127.

Interstitial granulomatous dermatitis with plaques associated with antiphospholipid syndrome

formation in BCC. For mutation analysis, DNA was isolated from peripheral blood leucocytes from both patients, and the entire coding regions and exon–intron borders of PTCH were amplified by polymerase chain reaction (PCR) using genomic DNA as the template. Oligonucleotide primers for amplification of all 23 exons of the gene have been reported elsewhere. Then DNA sequencing of the PCR products for all the exons was carried out with ABI Prism 377, version 3Æ0 (Applied Biosystems, Foster City, CA, U.S.A.). We found a novel 7-bp insertion mutation in exon 14 (2263insGGTGATC) that led to premature termination at codon 763 (Fig. 2) in patients 1 and 2. According to our review of the pertinent literature and to the PTCH database (http://www.cybergene.se/cgi-bin/ w3-msg1/ptchbase.html), the mutation we identified has not been reported previously. We could not find this mutation in other members of the family or in 50 healthy control subjects. Although the proband and his mother had the same mutation, the clinical features of the mother were much milder than those of the son. Patient 2 had no clinical features of NBCCS except for multiple BCCs on her trunk (Fig. 1f). Anderson et al. have suggested that cutaneous findings of BCCs and pitting increase in frequency with age, and reported that some patients with a mild phenotype of NBCCS are not diagnosed as having NBCCS unless the development of multiple BCCs is sufficiently evident to allow diagnosis, as in patient 2. These findings do not support the phenotype–genotype correlation for mutations of PTCH and NBCCS suggested by Boutet et al. We therefore hypothesize that the different phenotypes observed in individuals with identical PTCH mutations may be due to modifier genes which affect the SHH signalling pathway as previously identified in neurofibromatosis type 1. The association with segmental vitiligo in patient 1 seems to be unique: this is the only case associated with NBCCS of which we are aware. It is of interest that the appearance of the depigmented lesion was preceded by a history of expanding cysts in the patient’s left mandible and maxillary sinus. Although the pathogenesis of segmental vitiligo is still unknown, our findings suggest that expansion of the lesions may cause some physiological abnormality that contributes to the development of segmental vitiligo.

Giant dermatofibroma with granular cell changes: side‐effect of bee‐venom acupuncture?

Dermatofibroma (DF) is a common benign fibrohistiocytic tumour with a predilection for the legs in middle‐aged women. Giant DF, a rare clinical variant of DF, is characterized by its unusually large size. Granular cell change is typical of granular cell tumour, but can be observed in diverse cell lineages. Traumatic factors may be involved in the pathogenesis of giant DF and cellular granularity. We describe a 49‐year‐old Korean man with a giant DF showing granular cell differentiation, which may have been caused in part by multiple treatments with bee‐venom acupuncture.

Acquired ichthyosis associated with an overlap syndrome of systemic sclerosis and systemic lupus erythematosus.

Acquired ichthyosis is a condition accompanying many systemic illnesses such as lymphoma, sarcoidosis, dermatomyositis and systemic lupus erythematosus (SLE). Overlap syndromes are defined as clinical entities which satisfy each of the diagnostic criteria of two different connective tissue diseases concurrently or consecutively. The coexistence of SLE with systemic sclerosis has been very rarely reported. We describe a 33‐year‐old woman with an overlap syndrome consisting of systemic sclerosis and SLE who developed ichthyosis on her extremities.

Lipodystrophia centrifugalis abdominalis in Korea

Abstract:  Lipodystrophia centrifugalis abdominalis infantilis is a rare disorder described exclusively in Asians. Thirty Korean patients (4 women, 20 girls, and 6 boys) with a clinical and/or histopathologic diagnosis of this disorder were evaluated. The mean age of onset was 6.2 years. Infantile or congenital onset was seen in only three girls and two boys. In 50% of females, onset was after 5 years of age. All patients had involvement of the lower abdomen and groin both as the site of onset and as the main site. Histopathologic analysis showed loss of fat with or without an infiltrate of lymphocytes and histiocytes in the subcutis. Only one patient responded to treatment, an adult female who received photochemotherapy with softening of the lesions and symptomatic improvement. In previous reports, the age of onset of this disorder was at an age younger than 5 years but older than infants, making the term lipodystrophia centrifugalis abdominalis juvenilis more appropriate. In our series, 50% of females had onset after 5 years of age and 12.5% after 18 years of age. In one woman, the lesion that developed at 4 years of age progressed until adulthood. Our study of affected Korean patients showed a fourfold female predilection and later onset in female patients.