Kimura\'s disease

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Lenk et al.

Kimura's disease Cameo

Treatment is not warranted and is usually not necessary in NLS other than for cosmetic reasons, in which case simple surgical excision is enough. It is said that this lesion does not recur after excision.^ As far as we know, only one case reported by Wilson-Jones et al.^ showed slow recurrence after excision of the tumor 5 years previously. In our case, after surgical removal of the lesion, no evidence of recurrence has yet to be found. To the best of our knowledge this is the first reported case of multiple lesions of NLS presented on the facial area. : u.n . :.• v, r ,• R e f e r e n c e s






I Hoffmann E, Zurhelle E. Uber einen Naevus lipomatodes cutaneus superficialis der linken Glutaalgegend. Arch Dermatol Syphiloi 1921; 130: 3Z7-333.


z Wilson-Jones E, Marks R, Pongsehirun D. Naevus superficialis lipomatosus: a clinicopathological report of twenty cases. Br J Dermatol 1975; 93: 121-133. 3 Ghanoki M, Sugamoto I, Suzuki S, Hamada T. Nevus lipomatosus cutaneus superficialis of the scalp. Cutis ^9^9;

43: 143-144-

4 Dotz W, Prioleau PG. Nevus lipomatosus cutaneous superficialis: a light and electron microscopic study. Arch Dermatol 1984; 120: 376-379. 5 Einley AG, Musso LA. Naevus lipomatosus cutaneus superficialis (Hoffmann-Zurhelle). Br J Dermatol 197Z; 87: 557-564-


6 Abel R, Dougherty JW. Nevus lipomatosus cutaneus superficialis (Hoffmann-Zurhelle): report of two cases. Arch Dermatol 1962; 85: 524-526. 7 Knoth W. Uber Naevus lipomatosus cutaneus superficialis Hoffmann-Zurhelle und uber Naevus naevocellularis partim lipomatodes. Dermatologica s' '1962; 125: 161. 8 Weitzner S. Solitary naevus lipomatosus cutaneus superficialis of scalp. Arch Dermatol 1968; 97: 540-542. 9 Sawada Y. Solitary nevus lipomatosus superficialis on the forehead. Ann Plastic Surgery 1986; 16: 356-358. 10 Hann SK, Yang DS, Lee SH. Giant nevus lipomatosus superficialis associated with carvernous hemangioma. ] Dermatol 1988; 15: 543-545. ; 11 Sathyanarayana V, Weitzner S. Solitary nevus lipomatosus cutaneus superficialis of the knee. Arch Dermatol 1978; 114: 1226-1227. ' 12 Nikolowski W. Uber Naevus lipomatodes cutaneus superficialis (Hoffmann-Zurhelle). Dermatol Wocheiischr 1950; 122: 735. '> 13 Holtz KH. Beitrage zur Histologie des Naevus lipomatodes cutaneus superficialis (Hoffmann-Zurhelle). Arch Dermatol Syphiloi 1955; I99- Z75-Z86. 14 Robinson HM, Ellis FA. Naevus lipomatosus subepidermalis seu superficialis cutis. Arch Dermatol 1937; 35: 48515 Reymond JL, Stoebner P, Amblard P. Nevus lipomatosus cutaneous superficialis: an electron microscopic study of four cases. / Cutan Pathol 1980; 7: 295-301.


Kimura's disease Nurdan Lenk, MD, Ferda Artiiz, MD, Sezer Kula^oglu, MD, and Nuran AUi, MD From the Departments of Dermatology and Pathology, Ankara Numune Hospital, Ankara, Turkey Correspondence Nurdan Lenk, MD Gulhane Lojmanlari Serter Apt. No: 30 Etiik/Ankara Turkey

© 1997 Blackwell Science Ltd

A 40-year-old white Caucasian man presented with a 6-month history of a 1.5 x 1.5 cm nodular lesion on"his left arm. No history of trauma preceeded the lesion and the patient was in good general health. Dermatologic examination revealed a solitary, red, painless nodule located on the left arm (Fig. 1). Histopathologic examination of punch biopsy revealed lymphoid follicles, some of which had active germinal centers in the deep dermis and subcutaneous tissue. Mixed infiltration of lymphocytes, plasma cells and many eosinophils were present in the interfollicular region. Infiltration of the germinal centers by eosinophils, causing partial destruction, was observed (Fig. 2A). There was also proliferation of thin-walled small vessels and fibrosis in some parts of the interfollicular areas (Fig. 2B). Although Kimura's disease (KD) and angiolymphoid hyperplasia with eosinophilia (ALHE) were considered in the differential diagnosis, it was diagnosed as KD based on these histologic features. Further laboratory examinations were performed. Serum IgE was normal and peripheral eosinophilia was absent. The lesion regressed after biopsy. Complete healing was achieved with intralesional corticosteroid (triamcinolone acetonide) treatment.

International Journal of Dermatology 1997, 36, 435-452



Cameo Kimura's disease

Discussion Kimura's disease (KD) is an uncommon, chronic inflammatory condition of unknown etiology.''^ It is endemic in Orientals and may also occur sporadically in Caucasians.'"' The disease involves subcutaneous tissues, major salivary glands, and lymph nodes, chiefly in the head and neck region.'"^ However, other sites such as the oral cavity, axilla, groin, hmbs, and trunk may also be involved.^ It is often associated with peripheral blood eosinophilia and elevated serum IgE.'"^ KD, described firstly in Ghina by Kim and Szeto in 1937, is an angiolymphoid proliferative disorder of soft tissue.^'5 It is very similar to ALHE but is no longer used synonymously. Features common to both conditions include male predominance, predilection for the head and neck, tendency to recur, and lymphoid and eosinophilic infiltration. Kimura's disease is usually seen in younger individuals, has a longer duration, and occurs as a deeply seated, large soft tissue mass, without significant change of the overlying skin initially. Lymphadenopathy, peripheral eosinophiha.

Lenk et. ai.

and elevated levels of serum IgE may be present with the disease. On the other hand, ALHE is characterized by multiple, small dermal papular or nodular eruptiotis which occur in older patients and have a shorter duration; they are less frequently associated with peripheral blood eosinophilia.'*'''^ The most important morphologic difference is observed in the nature of blood vessels between KD and ALHE. In ALHE, blood vessels with so-called "histiocytoid" or "epitheloid" endothelial cells are thick-walled blood vessels with concentric rings of fibrosis and edema and lined by plump eosinophilic endothelial cells that sometimes contain vacuolated cytoplasm. None the less, these cells are not present in KD. In KD, thin-walled vessels may be seen.4'7 Lymphoid follicles with active germinal centers are invariably present but are infrequently seen in ALHE. Eosino-

Figure 2 (a) Lymphoid follicle with reactive germinal center surrounded by mixed inflammatory infiltration containing many eosinophils causing foUiculolysis (hematoxylin and eosin, X40); (b) among inflammatory cells thin-walled, small calibrated vessels proliferation (hematoxylin and eosin,

Figure 1 Glinical appearance of lesionInternational Journal of Dermatology 1997, 36, 435-452


© 1997 Biackweii Science Ltd

von Recklinghausen's neurofibromatosis Cameo

Fisfier Chu, and McCalmont

pbilic deposits are frequently observed in the germinal centers of KD. Infiltration of the germinal centers by eosinophils causing progressive destruction that results in foUiculolysis is observed only in KD. Tissue eosinophilia is more variable in ALHE and infiltrates are usually more massive in KD. Fibrosis, sometimes associated witb edema, is a more constant feature of KD.-* It was also present in our case. In our case tbe left arm locatioti of the lesion is not a common anatomic region for both diseases. Moreover, the age of the patient, and the erythematous appearance of the overlying skin over the nodule, are not usual findings observed in KD. Therefore, it is hard tQ make tbe diagnosis of KD regarding the clinical findings. However, it has been suggested that the histopatbologic features, particularly eosinophilic infiltration of the gertninal centers which results in foUiculoysis, only occur in KD. This is the most prominent histologic finding of our patient during the diagnostic approach.


References I Googe PB, Harris NL, Mihm MC, Jr. Kimura's disease and angiolymphoid hyperplasia with eosinophilia: two distinct histopathological entities. / Cutan Pathol 1987; 14: 263-271.

z Hui PK, Chan JK, Ng CS, et al. Lymphadenopathy of Kimura's disease. Am J Surg Pathol 1989; 13: 177-186. 3 Kung ITM, Gibson JB, Bannatyne PM. Kimura's disease: a clinicopathological study of 21 cases and its distinction from angiolymphoid hyperplasia with eosinophilia. Pathology 1984; 16: 39-44. 4 Gung CH. Kimura's disease. Am ] Surg Pathol 1988; 12: 843-854. 5 Filo V, Ferak I, Borecka D. Multiple unilateral reddish tumors on the ear and forehead a woman with early syphilis. Arch Dermatol 1994; 130: 369-374. 6 Tham KT, Leung PC, Saw D, et al. Kimura's disease with salivary gland involvement. Br J Surg 1981; 68: 495-4977 Aguilar A, Amhrojo P, Requenal, et al. Angiolymphoid hyperplasia with eosinophilia limited to the vulva. Clin Exp Dermatol 1990; 15: 65-67.




Solitary plexiform neurofibroma is not pathognomonic of von Recklinghausen's neurofibromatosis: a report of a case David A. Fisher, MD, Paul Chu, MD, and Timothy McCalmont, MD From the Departments of Dermatology and Pathology, University of California at San Francisco, California Correspondence David A. Fisher, MD 3701 Lone Tree Way Suite 6 Antioch, CA 94509

© 1997 Biackweii Science Ltd

A 42-year-old Caucasian man presented with a 1.8-cm nodule in the proximal thigh (Fig. 1) that had been slowly enlarging for approximately 5 years. The patient had no family history of neurofibromatosis. Physical examination revealed none of the other stigmata of this neurocutaneous disorder. D.A.F. performed an elliptical excision as an out-patient procedure. Grossly, the lesion was homogeneously opalescent and gelatinous. The tissue was fixed in 10% formalin and embedded in paraffin. Routine hematoxylin and eosin stained sections were prepared. The most notable feature on low power magnification was a large, circumscribed, lobular proliferation of spindle cells in a loose fibrillar background that distorted much of the dermis and subcutaneous fat (Fig. 2). On high power magnification, a population of cells with ovoid and S-shaped nuclei admixed within densely packed eosinophilic collagenous material was seen at the center of the lobule (Pig. 3A). A small amount of mucin was also present. Within this cellular area, a dense population of S-shaped spindle cells with eosinophilic cytoplasm was arranged in a parallel array forming microfascicles. At the periphery of this cellular component, a similar population of cells was arranged against a loose fibrillar background of abundant mucin (confirmed by colloidal iron stain) and scattered mast cells (Fig. 4). Lining the lobule was a compressed rim of thin, delicate collagen bundles and perineurial cells. Pleomorphic nuclei and mitotic figures were not observed. The surrounding dermis and subcutaneous tissue were unremarkable. Thickened collagen bundles at the center of the lobule could be seen with a trichrome stain. An S-100 profein immunoperoxidase stain highlighted scattered Schwann cells within the lobule (Fig. 3B), and nerve bundles adjacent to the neurofibroma served as infernal positive controls.

International Journal of Dermatology 1997, 36, 435-452


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