Chapter 8
8 Minimal Variants of Atopic Eczema
B. Wüthrich
In the diagnosis of atopic eczema (atopic dermatitis),
the criteria established by Hanifin and Rajka [1] are
those most often referred to. However, besides fullblown cases of atopic eczema, there are minor or atypical disease manifestations. The description of these
forms can be attributed primarily to French authors in
the mid-1960s and early 1970s [2 – 6] and to Herzberg
[7 – 9] in the German-speaking sphere. Special credit is
due to this author for having alluded to atopic eczema
found usually in two but occasionally even in three and
four generations of patients, based on his clinical
observations and skin reaction studies.
These special forms and minimal variants may
occur alone, together, or alternate with the more typical eczematous, lichenoid, pruriginous, and seborrheic
forms whose occurrence is related to age, individual
predisposition, and disease duration, and is indicative
of their status as atopic skin manifestations [1]. When
occurring alone, without the major features of atopic
eczema, and without other atopic manifestations, such
as allergic rhinitis, allergic asthma, or food allergy,
their classification as a minimal form of atopic eczema
may be disputable, especially in the intrinsic type or in
non-IgE-associated atopic eczema/dermatitis syndrome in which no demonstrable sensitization to atopic allergens exists [10 – 15]. Thus, the diagnosis can
only be finally accepted on the basis of a family and
personal history, progress monitoring, further clinical
features related to other stigmata of the atopic constitution, positive skin reactions (e.g., white dermographism), exclusion of a contact allergy to haptens, and
histology, which demonstrates eczematous, inflammatory changes.
Some of these variants attract attention because of
the particular morphological characteristics, others
because of the particular location, such as the eyelids,
lips, nipples, vulva, finger pads and toes.
8.1
Localized Minimal Variants of Atopic Eczema
Such localized variants include:
) Lower lid eczema, frequently occurring in the
spring as a pollinosis equivalent [11] (Figs. 8.1, 8.2)
Fig. 8.1. Atopic lower lid eczema (31-year-old female with grass
pollen allergy)
Fig. 8.2. Atopic eyelid eczema (35-year-old man with pollinosis)
8.1 Localized Minimal Variants of Atopic Eczema
) Exfoliating cheilitis with perlèche (Figs. 8.3, 8.4)
) Earlobe rhagades or retroauricular intertrigo
(Figs. 8.5, 8.6)
) Rhagades of the nasal orifices, often with chronic
nasal obstruction as the expression of a perennial
allergic rhinitis
Fig. 8.3. Exfoliating cheilitis with perlèche (6-year-old boy)
Fig. 8.4. Cheilitis with perioral eczema (8-year-old girl)
Fig. 8.5. Earlobe rhagades (6-year-old boy)
Fig. 8.6. Retroauricular intertrigo (20-year-old man)
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8 Minimal Forms of Atopic Eczema
) Nipple eczema (Fig. 8.7)
) Genital eczema (vulva, penis [Fig. 8.8], or scrotum)
) Tylotic, rhagadiform, finger pad eczema (pulpite
sèche or pulpite digitale keratosique craquelée récidivante; “atopic hands”) (Figs. 8.9, 8.10)
) Similar manifestations on the toes (“atopic feet” or
“atopic winter feet”), often misdiagnosed as foot
mycosis (Figs. 8.11, 8.12),
Fig. 8.7. Nipple eczema (17-year-old female)
Fig. 8.10. Atopic fingerpad eczema (6-year-old boy)
Fig. 8.8. Isolated eczema of the dorsum penis with painful rhagades (17-year-old man)
Fig. 8.11. “Atopic feet” or “atopic winter feet,” dorsal aspect
(5-year-old boy)
Fig. 8.9. “Atopic hands”: tylotic, rhagadiform, fingerpad eczema (pulpite sèche or pulpite digitale keratosique craquelée récidivante (23-year-old female)
Fig. 8.12. “Atopic feet” or “atopic winter feet,” plantar aspect
(same 5-year-old boy as in Fig. 8.11)
8.2 Juvenile Plantar Dermatosis
) The “juvenile plantar dermatosis” (see below)
(Figs. 8.13, 8.14).
According to Herzberg [7 – 9], over 70 % of patients with
fingerpad eczema are women, in whom the role of skin
traumatization in influencing localization is emphasized
by the fact that the pads of the first to third fingers are
initially affected and those of the fourth and fifth fingers
only later. Furthermore in the right-handed person, the
right hand is more affected than in the left. Apart from
the clinical signs of a pale erythema, keratosis, scaling,
and fissuring, concomitant pain leads subjectively to
impairment of function in the involved hand. As a practical sign, delicate materials such as nylon stockings are
torn by the sharp edges of the fissures when being put on
or taken off. Atopic hands and atopic feet occur more
often in children, and often improve during summer
holidays at the seaside, and after puberty.
In our follow-up study of 47 patients who had suffered from atopic eczema in infancy (< 2 years of age),
which was conducted using a questionnaire and personal examination at the mean age of 23 years, it was
found that 72.3 % of them were still suffering from
atopic skin problems [16]. In 66 % of the 47 patients,
minor manifestations were present, most frequently
perlèches (40.4 %), retroauricular intertrigo (34 %),
eyelid eczema (21 %), and fingerpad eczema (21.3 %).
The allocation of these localized variants to atopic
eczema obviously postulates negative epicutaneous testing to possible contact allergens (standard series, perioral group, shoe eczema group, etc.). A prospective
study of 195 patients with typical atopic eczema (average
age, 8.5 years) and 113 controls without eczema, [17]
came to the conclusion that retroauricular rhagades,
cheilitis, nipple eczema, and pityriasis alba are statistically highly significant (p< 0.005) associated with atopic
eczema, but that Dennie-Morgan infraorbital fold and
anterior neck creases are not. Also controversial are the
allocation to atopic eczema of the “dirty neck” sign, a
reticular pigmentation of the neck, that makes it appear
unwashed [18], and the correlation between “geographic
tongue” and atopy [19].
observed on the anterior part of the sole and is characterized by erythema, hyperkeratosis, and rhagades
(Figs. 8.13, 8.14). Histologically, there is an eczematous
dermatitis with spongiosis and lymphohistiocytic,
subepidermal, and perivascular infiltration. As early as
1966, Racouchot [4] had explicitly alluded to its relationship with the group of atopic forms: “La kératose
plantaire et les fissures hivernales des bords des talons
sont à retenir” (Plantar hyperkeratosis and rhagades
occurring in winter at the edges of the heels must be
observed). In 1968 Silvers and Glickman [20] gave a
thorough description of the special features of atopic
eczema of the feet in children. On 1972, this disorder
became well known due to a publication by Moller [21],
who proposed the name of “atopic winter feet.” However, it also occurs in the summer months, so that in practice it is often misdiagnosed as a foot mycosis and thus
erroneously treated with antimycotics.
Verbov [22] as well as Hambly and Wilkinson [23]
described “forefoot eczema” in the context of atopic
eczema, Mackie and Husain [24] considered “juvenile
Fig. 8.13. “Juvenile plantar dermatosis,” lateral aspect (10-yearold boy)
8.2
Juvenile Plantar Dermatosis
Juvenile plantar dermatosis is a painful variant of atopic eczema, frequently occurring in children. It is chiefly
Fig. 8.14. “Juvenile plantar dermatosis,” plantar aspect (same
10-year-old boy as in Fig. 13)
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8 Minimal Forms of Atopic Eczema
plantar dermatosis” to be a new entity since only nine of
the 102 children they studied had manifest atopy. They
attributed the cause to nylon socks. Subsequently, other
terms were proposed such as “dermatitis plantaris sicca” [25, 26], “peridigital dermatitis” [27], or “wet and
dry foot syndrome” [28], but the term most commonly
used today is the simple descriptive “juvenile plantar
dermatosis” (JPD). Association with atopy has been
found by various authors, ranging from 50.3 % of
patients [29] to 61 % [23], and 74 % [30]. Other authors
found no preponderance of atopy [24, 31]. Rajka [32]
suggested distinguishing between an “atopy-associated” and a “nonatopic” variant. In both types, along with
a possible constitutional weakness of the skin, various
other endogenous and exogenous factors have been discussed as pathogenetic, such as frequently repeated
microtraumata (“frictional contact dermatitis”), a
clammy microclimate due to the wearing of synthetic
socks and high shoes, or contact allergy to parts of
shoes. Pirkl et al. [33] suggested broad-spectrum epicutaneous testing in JPD in the following circumstances:
dren” [39], “summer lichenoid dermatitis of the elbows
in children” [40], “dermatite du toboggan” [41], “Sutton’s summer prurigo of the elbows” [42], “dermatitis
papulosa juvenilis” [43], “papular neurodermatitis”
[44], and “recurrent papular eruption of childhood”
[45]. Changes observed in biopsy specimens show
hyperkeratosis, a moderate degree of acanthosis and a
lymphocytic perivascular infiltrate in the upper dermis [37, 44]. The pathogenetic influence of such rough
materials as sand and wool and of a photosensitivity in
patients with atopic predisposition is suggested by
many authors [44 – 46]. Already in 1983, we assigned
these acro-located, small papular lesions to atopic
eczema based on family and personal history, demonstration of specific IgE to inhaled or food allergens, and
an eczematous histologic picture [44] (Figs. 8.15 – 8.18).
) The appearance of JPD after wearing new shoes
) Characteristic JPD which spreads secondarily over
the heels, or dorsum of the foot or toes, or the outer border of the foot
) Severe JPD with primary involvement of the heels.
If the rare cases of allergic contact eczema are excluded,
follow-up studies have shown that complete recovery
may be expected with puberty in the majority of patients
after a course of 7 – 8 years on average [28, 34, 35].
8.3
Juvenile Papular Dermatosis:
The Papular Form of Atopic Eczema
Juvenile papular dermatosis (Dermatitis papulosa
juvenilis) is characterized by lichenoid flat papules,
often hypopigmented, with a predilection for the dorsa
of the hands, the elbows, and the knees [36]. FölsterHolst et al. [37] reported a 9-year-old boy who developed particularly severe lesions of juvenile papular
dermatosis on the face and the back of the knee and
extreme pruritus during the summer. In fact, the disease affects children in the summer months. Sutton
first described this skin disease in 1956 under the name
of “summertime pityriasis of the elbow and knee” [38].
Others terms are “frictional lichenoid eruption in chil-
Fig. 8.15. Papular form of atopic eczema: disseminated flat papules on elbows (7-year-old boy)
8.4 Patchy Pityriasiform Lichenoid Eczema: The Follicular Form of Atopic Eczema
Fig. 8.16. Papular form of atopic eczema: several itchy flat papules on the hollow of the knee (same patient as in Fig. 8.15)
Fig. 8.17. Papular form of atopic eczema: flat papules on extensor aspect of knees (5-year-old girl)
The occurrence mainly in the spring and summer,
often in association with pollinosis, is pathogenetically
suggestive of an inhaled precipitant such as a hay fever
equivalent [11, 47], in addition to a mechanical component (friction, chafing) and photosensitivity.
that together with Takahashi and Sasagawa he had
described a plaque-shaped, lichenoid, scaly eczema as
an independent dry type of childhood eczema. This
type, evidently common in Japan, was allocated by
Sasagawa in 1967 to the group of atopic eczema [50]. In
1981, we described this at that time scarcely known
manifestation of atopic eczema in childhood [51].
Thus, clinically there are partly confluent areas with
densely juxtaposed, mildly bran-like, scaly, nonhyperkeratotic, skin-colored papules (Figs. 8.19 – 8.21). They
are found, with a generally dry integument, especially
on the trunk but also on the nape of the neck and the
knees. Histologically, these itchy “goose-flesh spots”
exhibit a spongiosis of the follicular epithelium and an
intra- and perifollicular lymphohistiocytic infiltration,
which indicated the eczematous nature of this condi-
8.4
Patchy Pityriasiform Lichenoid Eczema:
The Follicular Form of Atopic Eczema
Patchy pityriasiform lichenoid eczema is a dry, only
slightly itching, follicular form of atopic eczema occurring in childhood. Its first description is attributable to
Japanese authors as far back as 1950 [48]. In a 1966
paper published in German, Kitamura [49] mentioned-
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8 Minimal Forms of Atopic Eczema
Fig. 8.18. Histologic findings in papule of right knee: spongiosis in the epidermis, lymphohistiocytic perivascular infiltrate
in the dermis (same patient as in Fig. 8.17)
Fig. 8.19. Follicular form of atopic eczema (patchy pityriasiform
lichenoid eczema, Kitamura-Takahashi-Sasagawa): plaqueshaped “goose-flesh spots” on infrascapular region of back
(1-year-old boy, dark skinned)
Fig. 8.20. Follicular form of atopic eczema: closely packed,
skin-colored follicular papules on the trunk (3-year-old boy)
tion [48 – 51] (Fig. 8.22). Pruritus is usually present,
though not always severe. Mildly scaly depigmented
areas, rather like pityriasis alba [52, 53], and isolated,
frequently asymmetric, typical lichenified foci on the
backs of the hands or elbows are occasionally present.
In our series, a discrete perennial or seasonal allergic
rhinitis was frequently associated. Progression of the
disease is observed particularly in winter, whereas
summer visits to the seaside usually lead to improvement or healing of the rash. Ofuji and Uehara [54] also
found similar itchy follicular papules in 96 % of their
patients with atopic eczema, particularly on the lateral
parts of the trunk. They suggested that these lesions
probably constitute the primary efflorescence of atopic
eczema.
8.5 Comments
Fig. 8.21. Histologic findings showed the follicular form of
atopic eczema in a 10-year-old girl: follicular papule with
spongiosis of the epidermis and lymphohistiocytic intra- and
perifollicular infiltration
Fig. 8.22. Histologic findings: spongiosis of follicular epithelium with lymphohistiocytic infiltration (same patient as in
Fig. 8.21)
Differential diagnosis requires distinction from suprafollicular keratosis, follicular hyperkeratosis in the
context of an autosomal dominant ichthyosis vulgaris,
lichen planopilaris, lichen nitidus, and the various
lichenoid id-reactions, e.g., mykid or Gianotti-Crosti
syndrome [55].
and appropriate therapy. An atopic background should
be carefully assessed by allergy investigations (skin
prick tests and IgE determination to environmental
allergens and fungi, e.g., Malassezia sympodialis [56]).
Moreover, it would be of interest to perform atopy
patch tests to standard aeroallergens, food, and fungi
in these conditions, which are often non-IgE-associated, to evaluate cell-mediated immunity. There is also a
need for setting up cohort studies relating to the natural history of such atypical or minimal forms of atopic
eczema, especially of the so-called intrinsic or nonIgE-associated type [57, 58].
8.5
Comments
The knowledge of the minimal variants of atopic eczema, together with the evaluation of constitutional atopy stigmata, is a prerequisite for the correct diagnosis
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8 Minimal Forms of Atopic Eczema
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