Pediatric Dermatology Vol. 9 No. 3 283-287
Congenital Miliaria Crystallina: Case Report and Literature Review Christopher J. Arpey, M.D.,* Lauren S. Nagashima-Whalen, M.D.,* Mary-Margaret Chren, M.D.,*t and M. Tarif Zaim, M.D.± Departments of *Dermatology and tPathology, University Hospitals of Cleveland, and '•'Veterans Affairs Medical Center, Case Western Reserve University School of Medicine
Abstract: Generalized congenital miliaria crystallina occurred in a black newborn boy. Although miliaria crystaliina occurring in infancy and beyond is well established, congenital occurrence is very rare. The pathogenesis of the disorder is not well understood. We discuss some hypotheses ot pathogenesis In the context of our patient, as well as a differential diagnosis and a comparison with a previously reported case. Miliaria crystallina should be considered in the differential diagnosis of vesicutobullous eruptions in newborns.
Miliaria encompasses a family of transient occlusive disorders of eccrine sweat ducts resulting in leakage of sweat into the epidermis or papillary dermis. These disorders may be distinguished by the level of obstruction within the ducts. Miliaria crystallina is the most superficial, having intracorneal obstruction and subcorneal vesicle formation as its hallmarks. Miliaria rubra is intermediate in depth and results from intraepidermal duct obstruction, with outpouring of sweat into the lower epidermis. Miliaria profunda is the deepest, with blockage of the ducts in the region of the dermoepidermal junction and subsequent leakage of sweat into the papillary dermis. Although miliaria rubra (prickly heat) and miliaria crystallina are relatively common in children and infants, particularly under hot and humid conditions or after a febrile illness, they are rarely reported in newborns. Moreover, their pathogenesis remains speculative.
CASE REPORT A 1-hour-old, full-term black boy was seen for a generalized vesicular eruption noted at birth. The mother was a 21-year-old gravida 6, para 2 woman whose pregnancy was notable for poor prenatal care, intermittent cocaine use, and a high fever with shaking chills during the week prior to delivery. Family history was negative for skin disease; there was no maternal history of genital herpes or syphilis. The mother was admitted in active labor, with temperature 38.8°C, blood pressure 100/50 mm Hg, heart rate 116 beats/minute, and respiratory rate 36/ minute. Her physical examination was otherwise unremarkable. Laboratory data were remarkable for a white blood cell count of 21,800 cell/(il with 66% neutrophils and 25% band forms, and a urine toxicology screen positive for cocaine metabolites. Her chest radiograph was normal. Blood and urine cultures, and serology for syphilis and human im-
Address correspondence lo Mary-Margaret Chren, M,D,, Department of Dermatology, Veterans Affairs Medical Center. 10701 East Boulevard, Cleveland, OH 44106.
283
284 Pediatric Dermatology Vol. 9 No. 3 September 1992 munodeficiency virus were negative with the exception of a positive urine culture for Escherichia coli. At birth, the infant was meconium stained, pale, and hypotonic with shallow respirations. His axillary temperature was 35.5°C, blood pressure 70/41 mm Hg, heart rate 112 beats/minute, and respiratory rate 62/minute. Apgar scores were 3 at 1 minute and 5 at 5 minutes. He weighed 2555 g. Physical examination was remarkable for small, superficial, flaccid vesicles containing clear fluid. The vesicles were 1 to 4 mm in diameter, on a noninflammatory base, and distributed over the face, trunk, and extremities (Figs. 1 and 2). Palms, soles, and mucosae were uninvolved. The baby's laboratory data were remarkable for hemoglobin 9.7 g/dl, and white blood cell count 15,100 cells/jxl with 36% neutrophils, 12% band forms, and 30% lymphocytes. Chemistry panel was normal except for a bilirubin of 5.1 mg/dl. Urine toxicology screen was positive for cocaine metabolites. Blood cultures, urine cytomegaiovirus culture, and toxoplasma titers were negative. Syphilis screen (VDRL) was nonreactive. Chest radiograph and liver-kidney ultrasound were normal. Tzanck smear was negative. Gram's stain from a vesicle showed rare white blood cells and a few grampositive cocci singly and in pairs. A skin biopsy specimen obtained from the back on the second hospital day demonstrated intracorneal vesicles containing red blood cells and a few neutrophils (Fig. 3). Serial sections exhibited contiguity between acrosyringia and vesicles. Overlying the acrosyringia were small caps of parakeratosis admixed with red blood cells, occasional lymphocytes, and a few neutrophils (Fig. 4). The
Figure 2. Early superficial desquamation of the arms, with scattered intact vesicles. dermis showed a superficial perivascular and interstitial sparse, predominantly neutrophilic, mixed infiammatory eel! infiltrate. The patient was treated with intravenous ampicillin, gentamicin, and nafcillin. He was intubated shortly after delivery, but within 12 hours he improved greatly and was extubated. Three days later antibiotics were discontinued. Blood, urine, and skin viral and bacterial cultures remained negative. The infant developed no new vesicles, and his skin underwent superficial desquamation over days 3 through 8. By discharge at 2 weeks of age he had normal-appearing skin. At 2 months of age he continues in good health without cutaneous findings. DISCUSSION
Miliaria crystallina is the most superficial of a group of relatively common transient occlusive disorders
. "*:-U-:
Figure 1. Generalized small, flaccid vesicles on a noninflammatory base.
?i
Figure 3. Biopsy specimen from the back demonstrating a subcorneai vesicle containing red blood cells and a few neutrophils. (Hematoxylin & eosin; magnification 10x.j
Arpey et al: Congenital Miliaria Crystallina
Figure 4. Acrosyringium with overlying parakeratotic cap, red blood cells, and a few lymphocytes and neutrophils. (Hematoxylin & eosin; magnification 40x.)
of eccrine sweat ducts. Elucidation of the pathogenesis of the obstruction in al! types of miliariacrystallina, rubra, and profunda—has been difficult, despite interest and investigation beginning in the late nineteenth century (1,2). Miliaria is seen in all age groups and in both sexes worldwide. Clinically, miliaria crystaliina typically follows a febrile illness, and is characterized by 1- to 3-mm translucent, flaccid vesicles on a noninflammatory base that resemble drops of water (3-5). The level of obstruction is very superficial, with resultant intracorneal vesicles (6). The vesicles are usually asymptomatic and resolve quickly, with superficial branny desquamation and no long-term sequelae. Recurrences are possible with repeated episodes of fever and profuse sweating. The trunk and intertriginous areas are most frequently affected, although in neonates the face and neck also may be involved (7). In a retrospective study of 5387 Japanese infants over a 10-year period (7), miliaria crystallina was found to occur in 4.5%, with a peak frequency at 1 week of age; onset before day 4 was "exceptional." Indeed, to our knowledge, only one previous case of congenital miliaria crystallina has been reported (8). Miliaria rubra most commonly occurs in hot, humid conditions, and usually affects areas of friction and occlusion. The level of duct obstruction is intraepidermal, with sweat leakage and vesicle formation around the ducts. In neonates, the face, neck, and truck are most commonly involved (7), whereas in adults the face is usually spared. Lesions typically consist of pruritic, erythematous, nonfollicular, 1- to 2-mm papules or papulovesicles (3,5), and occasionally pustules (miliaria pustulosa). Miliaria
285
rubra affects 4% of neonates (7), with a peak frequency at J1 to 14 days of life. If generalized, it may lead to several weeks of hypohidrosis and heat intolerance; thus, its potential sequelae are more significant than those of miliaria crystallina. Miliaria profunda almost always follows repeated bouts of miliaria rubra, and occurs most commonly in adults living in hot climates. It is rare in neonates (9). The level of eccrine duct obstruction is at the dermoepidermal junction and results in firm, pale to flesh-colored papules of 1 to 3 mm. The patients are at high risk for hyperthermia resulting from widespread inactivation of eccrine glands, so-called tropical anhidrotic asthenia (10). Miliaria has been reproduced experimentally by a variety of means; for example, ethyl chloride spray, iontophoresis of various chemicals, ultraviolet irradiation, aluminum chloride application, and occlusive pads of various types, including plastic wraps (11-14). After such manipulations, induction of sweating results in miliaria crystallina or rubra. Reviews of previous investigations showed that the pathogenesis of miiiada remains elusive (1,15). Initially, the primary event was thought to be the formation of a keratotic plug in the sweat duct. Others theorized that somehow profuse sweating leads to maceration and abnormal keratinization of the homy layer, and histologicaliy demonstrated keratotic plugs in patients with miiiada rubra (16). Subsequently, similar hyperkeratotic and parakeratotic plugs were demonstrated in a variety of patients with experimentally induced miliaria crystallina and miliaria rubra (10,13,14). In the 1950s and 1960s, however, other investigators were unable to demonstrate homy plugs in such patients during the earlier stage of anhidrosis that precedes the miiiada (17-19). These investigations led to the hypothesis that an initial functional or ultrastructura! obstruction might occur, resulting in damage to the acrosyringium. The parakeratotic plug forms as a reparative process in this case, with the resultant histologically visible anatomic obstruction perpetuating the miliaria. A later study confirmed the early absence of plugs in experimentally induced miliaria after various periods of occlusion (20). Instead, only pedductal inflammation was seen in 1200 serial sections. A role for bacteria was postulated, perhaps by a toxin that could damage luminal cells (21,1). Bacterial proliferation was demonstrated (I) with the occlusive methods used to induce miliaria, and sweat inhibition appeared to be prevented by some, but
286 Pediatric Dermatology Vol. 9 No. 3 September 1992
not all, topical antibacterial agents used. However, a causal relationship between these findings and creation or inhibition of miliaria could not be proved. Of interest, Holzle and Kligman were able to demonstrate periodic acid-Schiff-positive debds in the acrosyringium just above the dermoepidermal junction, revitalizing the notion that a visible plug might play a role in the pathogenesis. In our patient, the presence of miiiada crystallina may be attributed to the mother's febrile illness pdor to delivery, and the moist occlusive environment created by amniotic fluid and vernix. Fever and occlusion often occur in neonates and adults with the condition, and are used to create experimentally induced miiiada. It is interesting to postulate that the parakeratotic caps are indicators of transiently abnormal keratinization by the affected ducts, which leads to duct obstruction and subsequent miliaria. It is also plausible to postulate that the first episode of miliaria in our patient occurred several days before delivery, and that the parakeratotic mounds may indicate the onset of repair of the acrosydngium in utero. Thus, the miiiada seen at birth may have been the latest of several crops occurring in utero, with the parakeratosis perpetuating duct obstruction, and maternal fever providing a recurrent stimulus for the fetus to sweat. One of the challenges in assessing and managing the patient was the possibility of congenital infection or primary blistering disease. The lesions were noninflammatory, translucent, and vesicular, rather than erythematous, turbid, or pustular. However, we believed congenital Candida, syphilis, and staphylococcal scalded-skin syndrome should be considered in the differential diagnosis and were ruled out be appropriate laboratory studies. Herpes simplex and vadcella were considered unlikely given the superficial nature of the vesicles and the absence of inflammation. Epidermolysis bullosa also seemed unlikely for similar reasons, together with the negative family history. Biopsy and the patient's clinical course confirmed the impression of miiiada crystallina. As a precaution, broadspectrum antibiotics were administered, but discontinued three days later, as further diagnostic evaluation, including bacterial cultures, was negative. Our patient had many features similar to the single previously reported case of congenital miliaria crystallina, and was managed similarly (8). The clinical appearance, histology, and clinical course were all consistent with miiiada crystallina in their patient, a newborn black girl, although no history of
maternal fever or ambient temperature elevation was reported. The in utero environment, particularly in the setting of maternal fever, may serve as an initiator of miliaria; however, the pathogenesis remains speculative. Miiiada crystallina should be considered in the differential diagnosis of congenital vesiculobullous dermatoses. REFERENCES 1. Holzle E. Kligman AM. The pathogenesis of miliaria rubra. Role of the resident microflora. Br J Dermatol 1978;99:117-137. 2. Pollitzer S. Prickly heat—lichen tropicus, miliaria papulosa, miliaria rubra, etc. J Cutan Genito-Urin Dis !893;n:50-56. 3. Dobson RL. Villareal D. Miliaria. In: Demis DJ. ed. Clinical dermatology, vol 2, 18th ed. Philadelphia: JB Lippincott. 199!: 1-7. 4. Cage GW. Sato K. Schwachman H. Eccrine glands. In: Fitzpatrick TB, Eisen AZ. Wolff K. Freedberg IM. Austen KF, eds. Dermatology in general medicine, vol I. 3rd ed. New York: McGraw-Hill, 1987: 701-703. 5. Champion RH. Disorders of sweat glands. In: Rook A, Wilkinson DS. Ebling FJG, Champion RH, Burtoo JL. eds. Textbook of dermatology, vol 3. 4th ed. Oxford: Biackwell. 1986:1891-1893." 6. Lever WF, Schaumburg-Lever G. Histopathology of the skin, 7th ed. Philadelphia: JB Lippincott, 1990: 114. 7. Hidano A, Purwoko R, Jitsukawa K. Statistical survey of skin changes in Japanese neonates. Pediatr Dermatol 1986;3:140-144. 8. Straka BF, Cooper PH. Greer KE. Congenital miliaria crystallina. Cutis 1991 ;47:103-106. 9. Silverman R. Nail and appendageal abnormalities. In: Schachner LA. Hansen RC. eds. Pediatric dermatology, vol 1. New York: Churchill Livingstone, 1988: 643-645. 10. O'Brien JP. A study of miliaria rubra, tropical anhidrosis, and anhidrotic asthenia. Br i Dermatol 1947;59:125-158. 11. Sulzberger MB, Griffin TB, Wiley HS. Miliaria and anhidrosis 1. Experimental production in volunteers. Dermatologica I967;135:414-42O. 12. Stoughton RB. Suppression of miliaria rubra (prickly heat) by a topical anticholinergic agent. J Invest Dermatol 1964;42:287. 13. Shelley WB, Horvath PN. Experimental miliaria in man. II. Production of sweat retention anhidrosis. i Invest Dermatol 1950;14:9-20. 14. Shelley WB, Horvath PN. Experimental miiiada in man. III. Production of miliaria rubra (prickly heal). J Invest Dermatol 1950;14:193-204. 15. Rogers M, Kan A, Stapleton K, Kamp A. Giant centrifugal miliaria profunda. Pediatr Dennatoi 1990;7: 140-146.
Arpey et al: Congenital Miliaria Crystallina
16. Sulzberger MB, Zimmerman HM. Studies on prickly heat. II. Experimental and histological findings. J Invest Dermatol 1946;7:61-68. 17. Hambrick GW, Blank H. The microanatomy of miliaria crystallina. J Invest Dermatol !956;26:327-336. 18. Dobson RL, Lobitz WC. Some histologic observations on the human eccdne sweat glands. II. The pathogenesis of miliaria. Arch Dermato! 1957:75:653666. 19. Papa CM, Kligman AM. Mechanisms of eccrine an-
287
hidrosis. I. High level blockage. J Invest Dermatol 1966;47:l-9. 20. Sulzberger MB, Harris DR. Miliaria and anhidrosis. in. Multiple small patches and the eifects of different periods of occlusion. Arch Dermatol 1972;105:845850. 21. Henning DR, Griffin TB, Maibach HI. Studies on changes in skin surface bacteria in induced miliaria and associated hypohidrosis. Acta Dermatol Venereol 1972 ;52:371-375.
Congenital Miliaria Crystallina: Case Report and Literature Review Christopher J. Arpey, M.D.,* Lauren S. Nagashima-Whalen, M.D.,* Mary-Margaret Chren, M.D.,*t and M. Tarif Zaim, M.D.± Departments of *Dermatology and tPathology, University Hospitals of Cleveland, and '•'Veterans Affairs Medical Center, Case Western Reserve University School of Medicine
Abstract: Generalized congenital miliaria crystallina occurred in a black newborn boy. Although miliaria crystaliina occurring in infancy and beyond is well established, congenital occurrence is very rare. The pathogenesis of the disorder is not well understood. We discuss some hypotheses ot pathogenesis In the context of our patient, as well as a differential diagnosis and a comparison with a previously reported case. Miliaria crystallina should be considered in the differential diagnosis of vesicutobullous eruptions in newborns.
Miliaria encompasses a family of transient occlusive disorders of eccrine sweat ducts resulting in leakage of sweat into the epidermis or papillary dermis. These disorders may be distinguished by the level of obstruction within the ducts. Miliaria crystallina is the most superficial, having intracorneal obstruction and subcorneal vesicle formation as its hallmarks. Miliaria rubra is intermediate in depth and results from intraepidermal duct obstruction, with outpouring of sweat into the lower epidermis. Miliaria profunda is the deepest, with blockage of the ducts in the region of the dermoepidermal junction and subsequent leakage of sweat into the papillary dermis. Although miliaria rubra (prickly heat) and miliaria crystallina are relatively common in children and infants, particularly under hot and humid conditions or after a febrile illness, they are rarely reported in newborns. Moreover, their pathogenesis remains speculative.
CASE REPORT A 1-hour-old, full-term black boy was seen for a generalized vesicular eruption noted at birth. The mother was a 21-year-old gravida 6, para 2 woman whose pregnancy was notable for poor prenatal care, intermittent cocaine use, and a high fever with shaking chills during the week prior to delivery. Family history was negative for skin disease; there was no maternal history of genital herpes or syphilis. The mother was admitted in active labor, with temperature 38.8°C, blood pressure 100/50 mm Hg, heart rate 116 beats/minute, and respiratory rate 36/ minute. Her physical examination was otherwise unremarkable. Laboratory data were remarkable for a white blood cell count of 21,800 cell/(il with 66% neutrophils and 25% band forms, and a urine toxicology screen positive for cocaine metabolites. Her chest radiograph was normal. Blood and urine cultures, and serology for syphilis and human im-
Address correspondence lo Mary-Margaret Chren, M,D,, Department of Dermatology, Veterans Affairs Medical Center. 10701 East Boulevard, Cleveland, OH 44106.
283
284 Pediatric Dermatology Vol. 9 No. 3 September 1992 munodeficiency virus were negative with the exception of a positive urine culture for Escherichia coli. At birth, the infant was meconium stained, pale, and hypotonic with shallow respirations. His axillary temperature was 35.5°C, blood pressure 70/41 mm Hg, heart rate 112 beats/minute, and respiratory rate 62/minute. Apgar scores were 3 at 1 minute and 5 at 5 minutes. He weighed 2555 g. Physical examination was remarkable for small, superficial, flaccid vesicles containing clear fluid. The vesicles were 1 to 4 mm in diameter, on a noninflammatory base, and distributed over the face, trunk, and extremities (Figs. 1 and 2). Palms, soles, and mucosae were uninvolved. The baby's laboratory data were remarkable for hemoglobin 9.7 g/dl, and white blood cell count 15,100 cells/jxl with 36% neutrophils, 12% band forms, and 30% lymphocytes. Chemistry panel was normal except for a bilirubin of 5.1 mg/dl. Urine toxicology screen was positive for cocaine metabolites. Blood cultures, urine cytomegaiovirus culture, and toxoplasma titers were negative. Syphilis screen (VDRL) was nonreactive. Chest radiograph and liver-kidney ultrasound were normal. Tzanck smear was negative. Gram's stain from a vesicle showed rare white blood cells and a few grampositive cocci singly and in pairs. A skin biopsy specimen obtained from the back on the second hospital day demonstrated intracorneal vesicles containing red blood cells and a few neutrophils (Fig. 3). Serial sections exhibited contiguity between acrosyringia and vesicles. Overlying the acrosyringia were small caps of parakeratosis admixed with red blood cells, occasional lymphocytes, and a few neutrophils (Fig. 4). The
Figure 2. Early superficial desquamation of the arms, with scattered intact vesicles. dermis showed a superficial perivascular and interstitial sparse, predominantly neutrophilic, mixed infiammatory eel! infiltrate. The patient was treated with intravenous ampicillin, gentamicin, and nafcillin. He was intubated shortly after delivery, but within 12 hours he improved greatly and was extubated. Three days later antibiotics were discontinued. Blood, urine, and skin viral and bacterial cultures remained negative. The infant developed no new vesicles, and his skin underwent superficial desquamation over days 3 through 8. By discharge at 2 weeks of age he had normal-appearing skin. At 2 months of age he continues in good health without cutaneous findings. DISCUSSION
Miliaria crystallina is the most superficial of a group of relatively common transient occlusive disorders
. "*:-U-:
Figure 1. Generalized small, flaccid vesicles on a noninflammatory base.
?i
Figure 3. Biopsy specimen from the back demonstrating a subcorneai vesicle containing red blood cells and a few neutrophils. (Hematoxylin & eosin; magnification 10x.j
Arpey et al: Congenital Miliaria Crystallina
Figure 4. Acrosyringium with overlying parakeratotic cap, red blood cells, and a few lymphocytes and neutrophils. (Hematoxylin & eosin; magnification 40x.)
of eccrine sweat ducts. Elucidation of the pathogenesis of the obstruction in al! types of miliariacrystallina, rubra, and profunda—has been difficult, despite interest and investigation beginning in the late nineteenth century (1,2). Miliaria is seen in all age groups and in both sexes worldwide. Clinically, miliaria crystaliina typically follows a febrile illness, and is characterized by 1- to 3-mm translucent, flaccid vesicles on a noninflammatory base that resemble drops of water (3-5). The level of obstruction is very superficial, with resultant intracorneal vesicles (6). The vesicles are usually asymptomatic and resolve quickly, with superficial branny desquamation and no long-term sequelae. Recurrences are possible with repeated episodes of fever and profuse sweating. The trunk and intertriginous areas are most frequently affected, although in neonates the face and neck also may be involved (7). In a retrospective study of 5387 Japanese infants over a 10-year period (7), miliaria crystallina was found to occur in 4.5%, with a peak frequency at 1 week of age; onset before day 4 was "exceptional." Indeed, to our knowledge, only one previous case of congenital miliaria crystallina has been reported (8). Miliaria rubra most commonly occurs in hot, humid conditions, and usually affects areas of friction and occlusion. The level of duct obstruction is intraepidermal, with sweat leakage and vesicle formation around the ducts. In neonates, the face, neck, and truck are most commonly involved (7), whereas in adults the face is usually spared. Lesions typically consist of pruritic, erythematous, nonfollicular, 1- to 2-mm papules or papulovesicles (3,5), and occasionally pustules (miliaria pustulosa). Miliaria
285
rubra affects 4% of neonates (7), with a peak frequency at J1 to 14 days of life. If generalized, it may lead to several weeks of hypohidrosis and heat intolerance; thus, its potential sequelae are more significant than those of miliaria crystallina. Miliaria profunda almost always follows repeated bouts of miliaria rubra, and occurs most commonly in adults living in hot climates. It is rare in neonates (9). The level of eccrine duct obstruction is at the dermoepidermal junction and results in firm, pale to flesh-colored papules of 1 to 3 mm. The patients are at high risk for hyperthermia resulting from widespread inactivation of eccrine glands, so-called tropical anhidrotic asthenia (10). Miliaria has been reproduced experimentally by a variety of means; for example, ethyl chloride spray, iontophoresis of various chemicals, ultraviolet irradiation, aluminum chloride application, and occlusive pads of various types, including plastic wraps (11-14). After such manipulations, induction of sweating results in miliaria crystallina or rubra. Reviews of previous investigations showed that the pathogenesis of miiiada remains elusive (1,15). Initially, the primary event was thought to be the formation of a keratotic plug in the sweat duct. Others theorized that somehow profuse sweating leads to maceration and abnormal keratinization of the homy layer, and histologicaliy demonstrated keratotic plugs in patients with miiiada rubra (16). Subsequently, similar hyperkeratotic and parakeratotic plugs were demonstrated in a variety of patients with experimentally induced miliaria crystallina and miliaria rubra (10,13,14). In the 1950s and 1960s, however, other investigators were unable to demonstrate homy plugs in such patients during the earlier stage of anhidrosis that precedes the miiiada (17-19). These investigations led to the hypothesis that an initial functional or ultrastructura! obstruction might occur, resulting in damage to the acrosyringium. The parakeratotic plug forms as a reparative process in this case, with the resultant histologically visible anatomic obstruction perpetuating the miliaria. A later study confirmed the early absence of plugs in experimentally induced miliaria after various periods of occlusion (20). Instead, only pedductal inflammation was seen in 1200 serial sections. A role for bacteria was postulated, perhaps by a toxin that could damage luminal cells (21,1). Bacterial proliferation was demonstrated (I) with the occlusive methods used to induce miliaria, and sweat inhibition appeared to be prevented by some, but
286 Pediatric Dermatology Vol. 9 No. 3 September 1992
not all, topical antibacterial agents used. However, a causal relationship between these findings and creation or inhibition of miliaria could not be proved. Of interest, Holzle and Kligman were able to demonstrate periodic acid-Schiff-positive debds in the acrosyringium just above the dermoepidermal junction, revitalizing the notion that a visible plug might play a role in the pathogenesis. In our patient, the presence of miiiada crystallina may be attributed to the mother's febrile illness pdor to delivery, and the moist occlusive environment created by amniotic fluid and vernix. Fever and occlusion often occur in neonates and adults with the condition, and are used to create experimentally induced miiiada. It is interesting to postulate that the parakeratotic caps are indicators of transiently abnormal keratinization by the affected ducts, which leads to duct obstruction and subsequent miliaria. It is also plausible to postulate that the first episode of miliaria in our patient occurred several days before delivery, and that the parakeratotic mounds may indicate the onset of repair of the acrosydngium in utero. Thus, the miiiada seen at birth may have been the latest of several crops occurring in utero, with the parakeratosis perpetuating duct obstruction, and maternal fever providing a recurrent stimulus for the fetus to sweat. One of the challenges in assessing and managing the patient was the possibility of congenital infection or primary blistering disease. The lesions were noninflammatory, translucent, and vesicular, rather than erythematous, turbid, or pustular. However, we believed congenital Candida, syphilis, and staphylococcal scalded-skin syndrome should be considered in the differential diagnosis and were ruled out be appropriate laboratory studies. Herpes simplex and vadcella were considered unlikely given the superficial nature of the vesicles and the absence of inflammation. Epidermolysis bullosa also seemed unlikely for similar reasons, together with the negative family history. Biopsy and the patient's clinical course confirmed the impression of miiiada crystallina. As a precaution, broadspectrum antibiotics were administered, but discontinued three days later, as further diagnostic evaluation, including bacterial cultures, was negative. Our patient had many features similar to the single previously reported case of congenital miliaria crystallina, and was managed similarly (8). The clinical appearance, histology, and clinical course were all consistent with miiiada crystallina in their patient, a newborn black girl, although no history of
maternal fever or ambient temperature elevation was reported. The in utero environment, particularly in the setting of maternal fever, may serve as an initiator of miliaria; however, the pathogenesis remains speculative. Miiiada crystallina should be considered in the differential diagnosis of congenital vesiculobullous dermatoses. REFERENCES 1. Holzle E. Kligman AM. The pathogenesis of miliaria rubra. Role of the resident microflora. Br J Dermatol 1978;99:117-137. 2. Pollitzer S. Prickly heat—lichen tropicus, miliaria papulosa, miliaria rubra, etc. J Cutan Genito-Urin Dis !893;n:50-56. 3. Dobson RL. Villareal D. Miliaria. In: Demis DJ. ed. Clinical dermatology, vol 2, 18th ed. Philadelphia: JB Lippincott. 199!: 1-7. 4. Cage GW. Sato K. Schwachman H. Eccrine glands. In: Fitzpatrick TB, Eisen AZ. Wolff K. Freedberg IM. Austen KF, eds. Dermatology in general medicine, vol I. 3rd ed. New York: McGraw-Hill, 1987: 701-703. 5. Champion RH. Disorders of sweat glands. In: Rook A, Wilkinson DS. Ebling FJG, Champion RH, Burtoo JL. eds. Textbook of dermatology, vol 3. 4th ed. Oxford: Biackwell. 1986:1891-1893." 6. Lever WF, Schaumburg-Lever G. Histopathology of the skin, 7th ed. Philadelphia: JB Lippincott, 1990: 114. 7. Hidano A, Purwoko R, Jitsukawa K. Statistical survey of skin changes in Japanese neonates. Pediatr Dermatol 1986;3:140-144. 8. Straka BF, Cooper PH. Greer KE. Congenital miliaria crystallina. Cutis 1991 ;47:103-106. 9. Silverman R. Nail and appendageal abnormalities. In: Schachner LA. Hansen RC. eds. Pediatric dermatology, vol 1. New York: Churchill Livingstone, 1988: 643-645. 10. O'Brien JP. A study of miliaria rubra, tropical anhidrosis, and anhidrotic asthenia. Br i Dermatol 1947;59:125-158. 11. Sulzberger MB, Griffin TB, Wiley HS. Miliaria and anhidrosis 1. Experimental production in volunteers. Dermatologica I967;135:414-42O. 12. Stoughton RB. Suppression of miliaria rubra (prickly heat) by a topical anticholinergic agent. J Invest Dermatol 1964;42:287. 13. Shelley WB, Horvath PN. Experimental miliaria in man. II. Production of sweat retention anhidrosis. i Invest Dermatol 1950;14:9-20. 14. Shelley WB, Horvath PN. Experimental miiiada in man. III. Production of miliaria rubra (prickly heal). J Invest Dermatol 1950;14:193-204. 15. Rogers M, Kan A, Stapleton K, Kamp A. Giant centrifugal miliaria profunda. Pediatr Dennatoi 1990;7: 140-146.
Arpey et al: Congenital Miliaria Crystallina
16. Sulzberger MB, Zimmerman HM. Studies on prickly heat. II. Experimental and histological findings. J Invest Dermatol 1946;7:61-68. 17. Hambrick GW, Blank H. The microanatomy of miliaria crystallina. J Invest Dermatol !956;26:327-336. 18. Dobson RL, Lobitz WC. Some histologic observations on the human eccdne sweat glands. II. The pathogenesis of miliaria. Arch Dermato! 1957:75:653666. 19. Papa CM, Kligman AM. Mechanisms of eccrine an-
287
hidrosis. I. High level blockage. J Invest Dermatol 1966;47:l-9. 20. Sulzberger MB, Harris DR. Miliaria and anhidrosis. in. Multiple small patches and the eifects of different periods of occlusion. Arch Dermatol 1972;105:845850. 21. Henning DR, Griffin TB, Maibach HI. Studies on changes in skin surface bacteria in induced miliaria and associated hypohidrosis. Acta Dermatol Venereol 1972 ;52:371-375.
