Chapter 8 8 Minimal Variants of Atopic Eczema

B. Wüthrich

In the diagnosis of atopic eczema (atopic ), 8.1 the criteria established by Hanifin and Rajka [1] are Localized Minimal Variants of Atopic Eczema those most often referred to. However, besides full- blown cases of atopic eczema, there are minor or atypi- Such localized variants include: cal disease manifestations. The description of these ) Lower lid eczema, frequently occurring in the forms can be attributed primarily to French authors in spring as a pollinosis equivalent [11] (Figs. 8.1, 8.2) 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 typi- cal 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, Fig. 8.1. Atopic lower lid eczema (31-year-old female with grass their classification as a minimal form of atopic eczema pollen allergy) may be disputable, especially in the intrinsic type or in non-IgE-associated atopic eczema/dermatitis syn- drome in which no demonstrable sensitization to atop- ic 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 consti- tution, positive skin reactions (e.g., white dermogra- phism), exclusion of a contact allergy to haptens, and histology, which demonstrates eczematous, inflamma- tory 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. Fig. 8.2. Atopic eyelid eczema (35-year-old man with pollinosis) 8.1 Localized Minimal Variants of Atopic Eczema 75

) Exfoliating cheilitis with perl`eche (Figs. 8.3, 8.4) ) Rhagades of the nasal orifices, often with chronic ) Earlobe rhagades or retroauricular nasal obstruction as the expression of a perennial (Figs. 8.5, 8.6) allergic rhinitis

Fig. 8.3. Exfoliating cheilitis with perl`eche (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) 76 8 Minimal Forms of Atopic Eczema

) Nipple eczema (Fig. 8.7) ) Tylotic, rhagadiform, finger pad eczema (pulpite ) Genital eczema (vulva, penis [Fig. 8.8], or scrotum) s`eche or pulpite digitale keratosique craquel´ee r´eci- divante;“atopichands”)(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 rha- Fig. 8.11. “Atopic feet” or “atopic winter feet,” dorsal aspect gades (17-year-old man) (5-year-old boy)

Fig. 8.9. “Atopic hands”: tylotic, rhagadiform, fingerpad ecze- ma (pulpite s`eche or pulpitedigitalekeratosiquecraquel´ee r´eci- Fig. 8.12. “Atopic feet” or “atopic winter feet,” plantar aspect divante (23-year-old female) (same 5-year-old boy as in Fig. 8.11) 8.2 Juvenile Plantar Dermatosis 77

observed on the anterior part of the sole and is charac- ) The “juvenile plantar dermatosis” (see below) terized by erythema, hyperkeratosis, and rhagades (Figs. 8.13, 8.14). (Figs. 8.13, 8.14). Histologically, there is an eczematous According to Herzberg [7–9], over 70% of patients with dermatitis with spongiosis and lymphohistiocytic, fingerpad eczema are women, in whom the role of skin subepidermal, and perivascular infiltration. As early as traumatization in influencing localization is emphasized 1966, Racouchot [4] had explicitly alluded to its rela- by the fact that the pads of the first to third fingers are tionship with the group of atopic forms: “La k´eratose initially affected and those of the fourth and fifth fingers plantaire et les fissures hivernales des bords des talons only later. Furthermore in the right-handed person, the sontaretenir ` ” (Plantar hyperkeratosis and rhagades right hand is more affected than in the left. Apart from occurring in winter at the edges of the heels must be the clinical signs of a pale erythema, keratosis, scaling, observed). In 1968 Silvers and Glickman [20] gave a and fissuring, concomitant pain leads subjectively to thorough description of the special features of atopic impairment of function in the involved hand. As a prac- eczema of the feet in children. On 1972, this disorder tical sign, delicate materials such as nylon stockings are became well known due to a publication by Moller [21], torn by the sharp edges of the fissures when being put on who proposed the name of “atopic winter feet.” Howev- or taken off. Atopic hands and atopic feet occur more er, it also occurs in the summer months, so that in prac- ofteninchildren,andoftenimproveduringsummer tice it is often misdiagnosed as a foot mycosis and thus holidays at the seaside, and after puberty. erroneously treated with antimycotics. In our follow-up study of 47 patients who had suf- Verbov [22] as well as Hambly and Wilkinson [23] feredfromatopiceczemaininfancy(<2yearsofage), described “forefoot eczema” in the context of atopic which was conducted using a questionnaire and per- eczema, Mackie and Husain [24] considered “juvenile sonal 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 perl`eches (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 test- ing to possible contact allergens (standard series, peri- oral 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] Fig. 8.13. “Juvenile plantar dermatosis,” lateral aspect (10-year- came to the conclusion that retroauricular rhagades, old boy) cheilitis, nipple eczema, and are statisti- cally 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].

8.2 Juvenile Plantar Dermatosis

Juvenile plantar dermatosis is a painful variant of atop- Fig. 8.14. “Juvenile plantar dermatosis,” plantar aspect (same ic eczema, frequently occurring in children. It is chiefly 10-year-old boy as in Fig. 13) 78 8 Minimal Forms of Atopic Eczema

plantar dermatosis” to be a new entity since only nine of dren” [39], “summer lichenoid dermatitis of the elbows the 102 children they studied had manifest atopy. They in children” [40], “dermatite du toboggan” [41], “Sut- attributedthecausetonylonsocks.Subsequently,other ton’s summer prurigo of the elbows” [42], “dermatitis termswereproposedsuchas“dermatitisplantarissic- papulosa juvenilis” [43], “papular neurodermatitis” ca” [25, 26], “peridigital dermatitis” [27], or “wet and [44], and “recurrent papular eruption of childhood” dryfootsyndrome”[28],butthetermmostcommonly [45]. Changes observed in biopsy specimens show used today is the simple descriptive “juvenile plantar hyperkeratosis, a moderate degree of acanthosis and a dermatosis” (JPD). Association with atopy has been lymphocytic perivascular infiltrate in the upper der- found by various authors, ranging from 50.3% of mis [37, 44]. The pathogenetic influence of such rough patients [29] to 61% [23], and 74% [30]. Other authors materialsassandandwoolandofaphotosensitivityin found no preponderance of atopy [24, 31]. Rajka [32] patients with atopic predisposition is suggested by suggested distinguishing between an “atopy-associat- many authors [44–46]. Already in 1983, we assigned ed” and a “nonatopic” variant. In both types, along with these acro-located, small papular lesions to atopic a possible constitutional weakness of the skin, various eczema based on family and personal history, demon- other endogenous and exogenous factors have been dis- stration of specific IgE to inhaled or food allergens, and cussed as pathogenetic, such as frequently repeated an eczematous histologic picture [44] (Figs. 8.15–8.18). microtraumata (“frictional ”), 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 epicu- taneous testing in JPD in the following circumstances: ) 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 out- er 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]. Fölster- Holst et al. [37] reported a 9-year-old boy who devel- oped 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 dis- ease 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]. Fig. 8.15. Papular form of atopic eczema: disseminated flat pap- Others terms are “frictional lichenoid eruption in chil- ules on elbows (7-year-old boy) 8.4 Patchy Pityriasiform Lichenoid Eczema: The Follicular Form of Atopic Eczema 79

Fig. 8.16. Papular form of atopic eczema: several itchy flat pap- Fig. 8.17. Papular form of atopic eczema: flat papules on exten- ules on the hollow of the knee (same patient as in Fig. 8.15) sor aspect of knees (5-year-old girl)

The occurrence mainly in the spring and summer, that together with Takahashi and Sasagawa he had often in association with pollinosis, is pathogenetically described a plaque-shaped, lichenoid, scaly eczema as suggestive of an inhaled precipitant such as a hay fever an independent dry type of childhood eczema. This equivalent [11, 47], in addition to a mechanical compo- type, evidently common in Japan, was allocated by nent (friction, chafing) and photosensitivity. 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]. 8.4 Thus, clinically there are partly confluent areas with Patchy Pityriasiform Lichenoid Eczema: densely juxtaposed, mildly bran-like, scaly, nonhyper- The Follicular Form of Atopic Eczema keratotic, skin-colored papules (Figs. 8.19–8.21). They are found, with a generally dry integument, especially Patchy pityriasiform lichenoid eczema is a dry, only onthetrunkbutalsoonthenapeoftheneckandthe slightly itching, follicular form of atopic eczema occur- knees. Histologically, these itchy “goose-flesh spots” ring in childhood. Its first description is attributable to exhibit a spongiosis of the follicular epithelium and an Japanese authors as far back as 1950 [48]. In a 1966 intra- and perifollicular lymphohistiocytic infiltration, paper published in German, Kitamura [49] mentioned- which indicated the eczematous nature of this condi- 80 8 Minimal Forms of Atopic Eczema

Fig. 8.18. Histologic findings in papule of right knee: spongio- Fig. 8.20. Follicular form of atopic eczema: closely packed, sis in the epidermis, lymphohistiocytic perivascular infiltrate skin-colored follicular papules on the trunk (3-year-old boy) in the dermis (same patient as in Fig. 8.17)

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 backsofthehandsorelbowsareoccasionallypresent. 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 improve- ment 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 Fig. 8.19. Follicular form of atopic eczema (patchy pityriasiform lichenoid eczema, Kitamura-Takahashi-Sasagawa): plaque- probably constitute the primary efflorescence of atopic shaped “goose-flesh spots” on infrascapular region of back eczema. (1-year-old boy, dark skinned) 8.5 Comments 81

Fig. 8.21. Histologic findings showed the follicular form of Fig. 8.22. Histologic findings: spongiosis of follicular epitheli- atopic eczema in a 10-year-old girl: follicular papule with um with lymphohistiocytic infiltration (same patient as in spongiosis of the epidermis and lymphohistiocytic intra- and Fig. 8.21) perifollicular infiltration

Differential diagnosis requires distinction from supra- and appropriate therapy. An atopic background should follicular keratosis, follicular hyperkeratosis in the be carefully assessed by allergy investigations (skin context of an autosomal dominant ichthyosis vulgaris, prick tests and IgE determination to environmental lichen planopilaris, lichen nitidus, and the various allergens and fungi, e.g., Malassezia sympodialis [56]). lichenoid id-reactions, e.g., mykid or Gianotti-Crosti Moreover, it would be of interest to perform atopy syndrome [55]. patch tests to standard aeroallergens, food, and fungi in these conditions, which are often non-IgE-associat- ed, to evaluate cell-mediated immunity. There is also a 8.5 need for setting up cohort studies relating to the natu- Comments ral history of such atypical or minimal forms of atopic eczema, especially of the so-called intrinsic or non- The knowledge of the minimal variants of atopic ecze- IgE-associated type [57, 58]. ma, together with the evaluation of constitutional ato- py stigmata, is a prerequisite for the correct diagnosis 82 8 Minimal Forms of Atopic Eczema

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