Correspondence 81

electron microscopic, and monoclonal antikeratin antibody findings suggest that syringomas are of ec- crine duct differentiation (I). Although they usually present as numerous small, skin-colored papules, many clinical variants have been recognized. Ac- cording to morphologic features and associations, Friedman and Butler suggested a classification of syringomas into four principal types, namely locat- ized (solitary or multiple), generalized, associated with Down syndrome, and familial (1 3 ) .

Localized syringomas can be solitary or more of- ten multiple. Usually they are confined to the lower eyelids in otherwise healthy in~ividuals; other sites of predilection are the face, neck, axillae, and abdo- men. Although the genital area can be involved as part of a more generalized distribution, it rarely rep- resents the sole localization. Vulvar syringomas have mostly been reported in association with ex- tragenital lesions (2-4).

To the best of our knowledge only a few cases of syringomas limited to the vulva have been reported (2,5-10), mostly in adults. The first patients were described by Carneiro et al. in 1971 in two white women age, respectively, 35 and 43 years (2). A case of localized vulvar syringomas in a 9-year-old girl was reported by Brown and Freeman in 1988 (5) . Localized vulvar syringomas have been ob- served underneath a seborrheic wart (14) or associ- ated with a nevocellular nevus and abortive hair (IS). In all reports they appeared as multiple papu- lar lesions that may or may not have been pruritic, involving both sides of the vulva.

Syringomas should be considered in the differen- tial diagnosis of papular lesions arising in the vulvar region in both adults and children. Fox-Fordyce disease, epidermal cyst, lichen simplex chroni- cus, steatocystoma multiplex, and lymphangioma should also be considered.

Our patient is of interest both for the unusual lo- calization of her lesions and her age. Localized vul- var syringomas have rarely been reported in pread- olescent girls (5,6).

REFERENCES

1. Hashimoto K, Gross BG, Lever WF. Syringoma: his- tochemical and electron microscopy studies. J Invest Dermatol 1966;46: 150-166.

2. Carneiro SJ, Gardner HL, Knox JM. Syringoma of the vulva. Arch Dermatol 1971;103:494-496.

3. Scherbenske JM, Lupton GP, James WD, Kirkle DB. Vulvar syringomas occurring in a 9-year-old child. J Am Acad Derrnatol 1988;19:575-577.

4. Young AW. Syringoma of the vulva. Obstet Gynecol 198035 :5 15-5 18.

5. Brown SM, Freeman RG. Syringoma limited to the vulva. Arch Dermatol 1971;104:331.

6. Dekio S, Maehama Y. Syringoma limited to genitalia of a preadolescent girl. J Dermatol 1981;8:423426.

7. King DT, Hirose FM, King LA. Simultaneous occur- rence of familial benign chronic pemphigus (Hailey- Hailey disease) and syringoma of the vulva. Arch Dermatol 1978;114:801.

8. Thomas J, Majmudar B. Syringoma localized to the vulva. Arch Dermatol 1979;115:95-96.

9. Zhu W. Vulvar syringoma. Int J Dermatol 1988;28: 1 42- 143.

10. Isaacson D, Turner M. Localized vulvar syringomas. J Am Acad Dermatol 1979;1:325-326.

11. Butterworth T, Strean L., Beerman H , et al. Syrin- goma and mongolism. Arch Dermatol 1964;90:483- 487.

12. Schepis C, Siragusa M, Palazzo R, Ragusa RM, Masi G, Fabrizi G. Palpebral syringomas and Down’s syn- drome. Dermatology 1994; 189:248--250.

13. Friedman SJ, Butler DF. Syringomas presenting as milia. J Am Acad Dermatol 1987;16:310-314.

14. Aguilar Martinez A, Requena Caballero L, Ambrojo Antunez PS, Sanchez Yus E. Syringoma of the vulva underneath a seborrheic wart. Ann Dermatol Ve- nereol 1989;116:323-324.

15. Gianotti R, Alessi E. Siringoma vulvare associato a nevo nevocellulare e displasia pilare. G Ital Dermatol Venereol 1992; 127:365-367.

VITO DI LERNIA, M.D. GIUSEPPE BISIGHINI, M.D. Reggio Emilia, Italy

ATOPIC DIATHESIS

To the Editors: The diagnosis of atopic dermatitis (AD) depends

on fulfillment of three basic and three minor clinical features as suggested by Hanifin and Rajka ( I ) . However, many of the minor clinical features lack specificity (2-41, and the significance of the major clinical features is also questionable (5). Therefore, a strict mathematical calculation of three major and three minor clinical features may not always be cor- rect to diagnose AD (6). It is the constellation of certain features, be they major or minor, in a partic- ular setting that points toward a diagnosis of AD. However, the presence of dermatitis (acute, sub- acute, chronic) is the prerequisite for making the di- agnosis.

Quite frequently we observe in some patients a particular group of atopic features without any der- matitis. To be more precise, we often see patients with personal and/or family history of atopy , xero- sis, and the presence of five or six other minor features, such as keratosis pilaris, ichthyosis, Dennie-Morgan (D-M) infraorbital fold, palmar hy- perlinearity , facial erythema, and diffuse scaling of

82 Pediatric Dermatology Vol. 13 No. 1 January/February 1996

the scalp. No evidence of any dermatitis is found on careful and thorough examination of these patients. Therefore, we cannot label their cutaneous abnor- mality as AD, although to our mind they have a strong predisposition to develop AD. We like to use the term atopic diathesis for this group of patients. Diathesis is a word of Greek origin meaning “dispo- sition.” It is defined as “a constitution or condition of the body which makes the tissue react in special ways to certain stimuli and thus tends to make a person unduly susceptible to certain diseases” (7). The term prurigo diathesique, used by Besnier as early as in 1892, has become redundant in subse- quent literature on AD. However, the word diathe- sis as used by Besnier had a different connotation than what we want to imply.

Whether the skin of patients with atopic diathesis can be called symptom-free skin as designated by Rajka (8), instead of uninvolved skin is a matter of conjecture. It is possible that the barrier function and irritant threshold values of the skin are abnor- mal in these patients (8). Therefore, a thorough in- vestigation should be carried out, incorporating to- tal circulating eosinophil count, evaluation of bacterial flora on the skin, serum IgE, radioaller- gosorbent test, interleukins, leukotrienes, ultra- structure, estimation of irritant threshold, and bar- rier functions of the skin to note any difference between these patients and those with AD. It is also essential to find out the difference in HLA specific- ities, if any, between these two groups of patients.

Whether environment, diet, choice of clothing, psychologic, or any other factor has a role in the

development of dermatitis in a predisposed (hence- forth called atopic diathesis) individual in the same manner as it modifies severity of AD (9), requires further study. However, as yet the exact factors re- sponsible for the progression of atopic diathesis to AD is unknown.

REFERENCES

1. Hanifin JM, Rajka G. Diagnostic features of atopic dermatitis. Acta Dermatol Venereol (Stockh) 198O;suppl 92:44-47.

2. Kang K, Tian R. Atopic dermatitis-an evaluation of clinical and laboratory findings. Int J Dermatol 1987; 26:27-32.

3. Mevorah 3 , Frenk E, Wietlisbach V, et al. Minor clinical features of atopic dermatitis-evaluation of their diagnostic significance. Dermatologica 1988; 177: 360-364.

4. Kanwar AJ, Dhar S, Kaur S. Evaluation of minor clinical features of atopic dermatitis. Pediatr Derma- to1 1991;8: 114-1 16.

5. Kanwar AJ, Dhar S. How specific are major criteria for the diagnosis of atopic dermatitis? Dermatology 1994; 189: 102.

6. Kanwar AJ, Dhar S. Frequency and significance of major and minor clinical features of atopic dermatitis. Dermatology 1995; 190(4):3 17.

7. Friel PJ, ed. Dorland’s illustrated medical dictionary. Philadelphia: WB Saunders, 1982.

8. Rajka G . Essential aspects of atopic dermatitis. Ber- lin: Springer-Verlag, 1989.

9. Kanwar AJ, Dhar S. Severity of atopic dermatitis in India. Br J Dermatol 1994;131:733-734.

S A ~ D I P A N DHAR, M.D., D.N.B. AMRINDER J. KANWAR, M.D. Department of Dermatology Chandigarh, India