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Case Report
Received: December 17, 2001Accepted: February 24, 2002 Meyerson Phenomenon within a
Nevus flammeus
The Different Eczematous Reactions within Port-Wine Stains
Key Words
on the anteromedial aspect of the left lower localized to the neck and face. They are rare- leg (fig. 1). Further physical examination re- ly found in the context of a Klippel-Trenau- vealed no other skin abnormalities. The pa- nay syndrome [3]. The pathogenesis of this tient does not have any varicose veins. The investigation with color duplex sonography (Sonoline Versa Pro®, Siemens, Linear Ar- Abstract
first time a papulosquamous halo dermatitis ray 5,0L45) did not reveal any insufficient overlying pigmented nevi. Unlike the more perforating vein under the nevus flammeus.
tions overlying nevi flammei exist. All of frequently observed halo nevus or nevus Sut- On histological examination focal acantho- ton, the nevus remains unchanged after the sis, parakeratosis and spongiosis were found scription of an eczematous reaction within a overlying some capillary ectasias. There was congenital nevus flammeus on the left lower 1988, Nicholls and Mason [5] proposed the no inflammatory infiltrate (fig. 2). Topically leg of a male adult gives reason to discuss eponym ‘Meyerson’s nevus’. Since then, this, also called ‘Meyerson phenomenon’ [6], has eczema within 2 weeks. Until today no re- rarely been seen in relation with other skin may mostly be a collision dermatosis with tumors [7, 8]. In those cases, the pathogene- an atopic dermatitis, especially when they arise in children and are localized to the neck Discussion
and face. When they are observed within a reported who developed an eczematous reac- grossly visible vascular malformation, as for tion within a congenital nevus flammeus on his left lower leg. A possible relation between vascular structures are common. They most- drome, they may have a pathogenesis simi- this observation and the Meyerson phenom- ly arise as stasis dermatitis from varicose lar to stasis dermatitis. In rare cases, an veins. They can electively be located to der- mal varicose venectasias, but are more often less circumscribed. Histological examina- cism and is interpreted as a variant of the Case Report
tion shows either a subacute or chronic der- matitis with hemosiderin scattered through the dermis, and the dilated vessels are em- bedded in a fibrotic dermis [9]. The patho- flammeus on his left lower leg for 1 year. He genesis of stasis dermatitis is not yet com- Introduction
hardly complained about itchiness. His per- sonal medical history was uneventful. There All the recently reported cases involving was no history of eczema or allergy. Physical matory or eczematous skin changes limited examination revealed a brown reddish area, flammeus, occurred in children [1–3]. They to the area of port-wine stains or congenital about 12 cm in diameter with a pronounced are mostly localized to the neck (otherwise nevi flammei [1, 2]. These observations are and scaly margin, within a nevus flammeus also called salmon patch of the midline) and Tel. +41 56 427 27 22, Fax +41 56 427 00 12, E-Mail [email protected] face. Sidwell et al. [3] suggest that it may becaused by an abnormal production of cyto-kines, resulting in inflammatory changeswithin the skin. It is also possible that theendothelial cells could play an importantrole in the inflammatory process. Since theeczema improves by laser therapy to theport-wine stains [1, 3], Sidwell et al. [3] sup-pose that the increased vasculature predis-poses to the development of eczema. AlsoBonifazi and Mazzotta [2] suppose, that‘.the vascular malformation, with the rele-vant vasodilatation, may be the localizationfactor of the inflammatory changes.’. If thiswere the explanation for this observation,should it not also be possible to find an ecze-ma more frequently within nevi flammei ofadults or within port-wine stains otherwiselocalized than the head, or even also overly-ing the ‘couperose’ of patients with rosacea?Furthermore nuchal nevus flammeus isfound in 15.6% of newborns [11]. Atopiceczema in children and adolescents is local-ized in 10.2% on the nape and in 30.8% onthe neck [12]. Therefore the coincidence of Fig. 1. Eczema (
could also be interpreted as the result of astatistical probability. Eczema taking placewithin a grossly visible vascular malforma-tion [3], as in the Klippel-Trenaunay syn-drome with arteriovenous anastomoses andvaricose vein segments, may have a patho-genesis comparable to stasis dermatitis. Un-fortunately the reports of Sidwell et al. [3]and Bonifazi and Mazzota [2] lack any infor-mation about a histological examination.
first time a papulosquamous halo dermatitisoverlying pigmented nevi. Histological ex-amination shows an irregular acanthosis, pa-rakeratosis and focal spongiosis overlying anotherwise unchanged nevus. There are noinflammatory cells invading the pigmentednevus. A lymphocytic perivascular infiltrateis present just peripheral to the nevus. Un-like the more frequently observed halo nevusor nevus Sutton, the nevus remains un-changed, after the inflammatory process hasdisappeared. In 1988, Nicholls and Mason[5] proposed the eponym ‘Meyerson’s ne-vus’. Since then, the ‘Meyerson phenome-non’ [6], also called ‘nevocentric’ halo der-matitis [13], has rarely been seen in relationwith other skin tumors [7, 8] or inflammato-ry skin diseases such as atopic eczema (ownobservation), pityriasis rosea [14], erythemamultiforme [13] and psoriasis [15]. Even if Fig. 2. On histological examination, focal acanthosis, parakeratosis and spongiosis overlying
an immunological process is suggested [16– some capillary ectasias are seen. There is no inflammatory infiltrate. HE. !100.
18], the exact pathogenesis of this phenome-non has yet to be clarified.
the more pronounced linear lesions reflect that endothelial cells, arranged in monoclon- flammeus does not loose its hair, whereas the al zones, ‘. may be involved in the patho- surrounding skin is undergoing an autoim- genesis of hitherto poorly understood dis- tion of what we call a nevus: ‘Nevi are visi- mune reaction giving rise to alopecia areata.
eases . for example, vascular clones in the ble, circumscribed, long-lasting lesions of the dermis regulate the behavior of epidermal skin or the neighboring mucosa, reflecting quired skin diseases, inflammatory dermati- melanocytes’. Their hypothesis tried to ex- tis or drug eruptions following the Blaschko plain a harlequin-like pattern of skin pig- melanocytic nevi, they do not show neoplas- lines – skin reactions like the Meyerson phe- mentation in a Negress, which we nowadays tic growth. They never show malignant neo- nomenon occurring in the context of nevi, would describe as a patient with chimerism, plasia’ [24]. He supposes that nevi, as a are also the result of genetic mosaicism. The result of their genetic mosaicism, show a here presented 46-year-old man has an ecze- checkerboard, but also including ovoid lenti- matous reaction, strongly limited to the area ginous macules, with their long axes orien- that of the surrounding skin. This genetic of a congenital nevus flammeus on his left tated along the lines of Blaschko. Congenital, mosaic may for instance explain the protec- nevoid and acquired skin diseases following tion of a hairy pigmented nevus from hair show any further abnormalities. There is no the Blaschko lines are thought to be caused loss, whereas the surrounding skin is un- causative varicose vein detectable. The his- by genetic mosaicism, resulting from lyoni- dergoing an autoimmune reaction giving rise tological picture resembles the description zation in X-linked disorders, postzygotic so- to alopecia areata [25]. In 1972, Burgoyne matic mutations in sporadic conditions and [26] also described a female patient with alo- reaction is interpreted as a new variant of the gametic half-chromatid mutations [20]. In pecia areata: treatment with local injection 2000, Lipsker et al. [21] demonstrated for of triamcinolone diacetate was followed by the first time that also an acquired inflam- regrowth of hair at the sites injected, but changes within a port-wine stain may mostly matory dermatitis in Blaschko-linear distri- after a time the hair in these areas fell out, be a collision dermatosis with an atopic der- except in the area of a nuchal nevus flam- matitis, especially when they arise in chil- genetic concept, loss of heterozygosity in hu- meus. Port-wine stains fulfil the new defini- dren and are localized to the neck and face. If man skin, plays a role not only in cutaneous tion of a nevus, even if their genetic mosai- they are observed within a grossly visible malignant growth but also in the develop- cism is not yet proved: they are visible, cir- vascular malformation, as for example the ment of benign skin disorders [22]. It may cumscribed, long-lasting lesions. They may also be the explanation for the coexistence of have a pathogenesis similar to stasis derma- a linear and disseminated drug eruption: the [27] and enhance vascular proliferation [28, titis. In both cases, histological examination loss of heterozygosity in a somatic cell, dur- 29] but they do not show neoplastic growth.
has yet to be done. Further in rare cases, as in ing early embryogenesis, gives rise to a clonal population of cells being either homozygous flammei, similar to melanocytic nevi, as a within a nevus flammeus may be the result result of their genetic mosaicism, show a of genetic mosaicism and is interpreted as a variant of the so-called Meyerson phenome- would reflect a heterozygous state, whereas that of the surrounding skin. This presump- References
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