Journal of the American Academy of Dermatology
688 Brie] communications
Fig. 1. Case 1. Longitudinal pigmented nail bands with bluish discoloration of nail bed. Fig. 2. Large melanin granules (G) and dendritic melanocytes (M) throughout nail matrix (MX}. NP, Nail plate. (Ammoniacal silver nitrate stain; X400.)
REFERENCES 1. Furth PA, Kazakis AM. Nail pigmentation changes associated with azidothymidine (zidovudine) [Abstract]. Ann Intern Med 1987;107:350. 2. Pannwalker AP. Nail pigmentation in the acquired immunodeficiency syndrome (AIDS). Ann Intern Med 1987; 107:943-4. 3. Gonzalcz-Lahoz JM, Garcia Aguado C, MartinezFernandez R, et al. Pigmentation azulada en las unas de los pacientes con SIDA: efecto colateral de la zidovudina? [Letter] Rev Clin Espan 1988;183:278-9. 4. Azon-Masoliver A, Mallolas J, Gatell J, et al. Zidovudineinduced nail pigmentation. Arch Dermatol 1988;124: 1570-1. 5. Riechman DD, Fieschl MA, Grieco MH, et al. The toxicity of azidothymidine (AZT) in the treatment of patients with AIDS and AIDS-related complex: a double blind controlled trial. N Engl J Med 1987;317:192-7. 6. McEvoy GK, ed. American Hospital Formulary Service: drug information. Bethesda, Md.: American Society of Hospital Pharmacists, 1988:392-7.
7. Daniel CR, Scher RK. Nail changes secondary to systemic drugs or ingestants.J AMACAD DERMATOL 1984;10:250-8.
Acquired port-wine stain Mark W. Cobb, MD, and Leon Goldman, MD* Bethesda, Maryland, and San Diego, California Nevus flammeus is a common congenital malformation of mature capillaries that occur in up to 75% of newborns.' The most common type is the salmon patch, From the Department of Dermatology, Naval Hospital. The views expressed herein are those of the authors and do not reflect the officialpolicyor position of the Department of the Navy, Department of Defense, or the U.S. Government. No reprints available. *Dr. Goldman is at the Naval Hospital, San Diego, Calif.
Volume 22 Number 4 April 1990
Brief communications
689
found on the nape of the neck, glabella, forehead, eyelids, or upper lip.2 Those on the face tend to involutebythe first year. Nuchal lesions resolve less frequently and approximately 50% persist into adulthood. Port-wine stains, like salmon patches, are almost always present at birth; however, rather than involutingor fading, they persistand often become raised and nodular as the patient ages. Rarely port-wine stains are acquired, most of which are posttraumatic.' We report a case of an acquired port-wine stain in a patient with no history of antecedent trauma. Case report. A 41-year-old man had an asymptomatic discoloration of the right sideof the face and scalp that began approximately 8 yearsearlier. The lesion had enlarged for the first several years, thenstabilized. He denied anyhistory ofprior trauma to thearea.His general healthwas good andhe took no medications. Physical examination revealed several erythematousto violaceous patches ontherighttemporal scalp, preauricular area, chin, and neck (Fig. 1). The lesion only partially blanched on diascopy. The clinical impression was a port-wine stain.An incisional biopsy was done and thespecimen revealed ectaticvessels in the papillary dermis, consistent with port-wine stain (Fig. 2). Discussion. Port-wine stain represents a progressive ectasia of vesselsin the superficialvascular plexusand not a proliferative processas in hemangioma.v 5 Barskyet al. 5 believed that collagen degeneration of the dermal structures supporting the vessels was involvedin this progressive ectasia. Recently it has been postulated that the port-wine stains may be due to a local alteration in neural modulation of vascular tone. Specifically, Rosen and Smeller" hypothesized that sympathetic innervation of cutaneous vasculature may be functionally deficient in port-wine stain; congenital lesions demonstrate a maturation defect in innervation and acquired lesions result from a lossin sympathetic function. The resultingcontinuous unregulated blood flow would then predispose the cutaneous vessels to progressive ectasia.' The same authors' have demonstrated a significantdecreasein both nerve density and vessels with associated nerves in portwine stain when compared with normal skin and hemangiomas. Acquired port-wine stains are rare; most reportedcases occur after some form of local trauma. The reported antecedent injury varies from being struck by a cricket ball' to intense cold exposure at the Russian Front during World War H.9 These diverse insults may adversely affect the sympathetic vasoconstrictive innervation to superficial cutaneous vessels and result in ectasia. Brinkmann'!' reported two cases of acquired port-wine stain in female patients; the first was pregnant and the second was pubertal at the time the lesion developed. There was no history of antecedent trauma and increased estrogen levels were suggested as being a factor. Cases of acquired port-wine stain associated with use of oral contraceptive pills have also been reported. II Our case is re-
Fig. 1. Erythematous, nonblanchable patch involves right temporal scalp and preauricular area. Fig. 2. Several ectatic vessels present in papillary dermis. (Hematoxylin-eosin stain; XlOO.) markable for lack of any history of preceding injury or source of increased estrogens. We were able to find only two such cases in the liter ature. I I, 12 REFERENCES 1. CaroWA,Bronstein BR.Tumorsof theskin.In:Moschella SL, Hurley HJ, eds. Dermatology, 2nd ed. Philadelphia: WB Saunders, 1985:1599-600. 2. Atherton DJ, Rook A. Naeviand otherdevelopmental defects. In: Rook A, Wilkinson DS, Ebling FJG, et al., eds. Textbook of dermatology. 4th ed. Oxford: Blackwell, 1986:194-5. 3. Colver GB,RyanTJ. Acquired port-wine stain.Arch DermatoI1986;122:1415 c6. 4. Lever WF, Schaumburg-Lever G, ed. Histopathology of the skin. Philadelphia: JB Lippincott, 1983:623-4. 5. Barsky SH, Rosen S, Geer DE, et al. The nature and evolution ofport-wine stains:a computer-assisted study. J Invest Dermatol 1980;74: 154-7. 6. Rosen S, Smaller BR. Port-wine stains: a newhypothesis. JAM ACAD DERMATOL 1987;17:164-6. 7. Rosen S, Smoller B. Pathogenesis of port-wine stains. A new hypothesis. Med Hypotheses 1987;22:365-8.
690 Brief communications
Journal of the American Academy of Dermatology
8. Smaller BR, Rosen S. Port-wine stains. Arch Dermatol 1986;122:177-9. 9. Niemand-Anderssen 1. Naevus f1ammeus tardivus (attributable to cold?). Z Hauth 1952;12:251-6. 10. Brinkmann W. Acquired nevus flammeus. Z Hautkr 1981;56:1334-40. 11. Goldman L. Oral contraceptives and vascular anomalies. Lancet 1970;2:108-9. 12. Traub EF. Naevus flammeus appearing at the age of 23. Arch Dermatol Syphilo1 1939;39:752.
Papular dermatitis of pregnancy: A case report Luat Q. Nguyen, MD, and O. Robert Sarmini, MD
Arlington, Texas Numerous physiologic and pathologic changes can occur in the skin of pregnant women. 1, 2 These include a disorder first described in 1962 by Spangler et al. 3 They termed it "papular dermatitis of pregnancy" and reported an increase in fetal mortality. We report a case of papular dermatitis of pregnancy with abnormal laboratory findings, good maternal outcome, and no fetal loss. Case report. A 27-year-old woman, gravida 4, para 2, had lower abdominal contractions and bleeding. The estimated gestational age of the fetus on the patient's admission was 26 weeks 4 days. Physical examination revealed a fundal height of26 em with uterine contractions every 5 minutes. An ultrasound study ruled out placenta previa. The patient's premature labor was controlled with ritodrine hydrochloride (Yutopar). At the twenty-eighth week of pregnancy, a pruritic, eczematous rash developed on the chest and back. The eruption rapidly cleared with topical steroid therapy and 6 mg of beta methasone sodium phosphate intramuscularly. Two weeks later, numerous severely itching, erythematous papules appeared on the abdomen and back (Fig. 1). Because the eruption continued to worsen despite repeated doses of betamethasone sodium phosphate, a skin biopsy was performed. The specimen revealed a superficial lyrnphocytic perivascular infiltrate. Immunofluorescence study demonstrated no immunoglobulin or complement deposition. Results of routine laboratory studies were either within normal limits or negative. A hormonal evaluation revealed lowvalues of cortisol at 1.8 Itg/dl (normal 5.1 to 20.1 ,ug/dl). Values of urinary estriols were 0.8 gm/24 hr (normal 0.6 to 1.8 gm/24 hr). Urinary chorionic gonadotropin levels were moderately high at 27,756 TU/24 hr (normal 6000 to 25,000 IU/24 hr). A diagnosisof papular dermatitis of pregnancy was considered, and the patient was treated with oral prednisone (60 mg/day). At 32 weeks 2 days of pregnancy, results of the patient's ultrasound examination revealed oligohydramnios and signs of placental degeneration and calcification. Amniocentesis showed a high
From the Departments ofDermatology and Obstetrics and Gynecology, HCA South Arlington Medical Center, Financial support for color reproduction was received from Dermik Laboratories. Reprint requests: Luat Q. Nguyen, MD, 3132 Matlock Rd., Suite 307, Arlington, TX 76015.
16/4/149"6
Fig. 1. Disseminated papular eruption on chest and abdomen.
lecithin/sphingomyelin ratio with positive phosphatidylglycerol, demonstrating fetal lung maturity. The patient underwent cesarean section, which resulted in the delivery of a growthretarded infant at 33 weeks' gestation with no skin involvement. The cortisone was gradually tapered and was discontinued, and within a week after delivery the eruption had completely subsided. Discussion. The natural history, underlying mechanisms, and causes of some dermatoses of pregnancy are fairly well understood." A few conditions remain controversial (e.g., toxemic rash of pregnancy, prurigo gestationis of Besnier, and papular dermatitis of pregnancy) and are not unanimously accepted as distinct clinical entities.' Spangler et al.;' however, believed that among the several dermatoses characterized by pruritic papules was a subgroup of patients with identifiable clinical and biochemical features. Papular dermatitis of pregnancy is rare and occurs in 0.026% of births." As of 1988 a literature search revealed only about 23 reported cases. The eruption may begin at any time during pregnancy' and consists of intensely pruritic erythematous papules, 3 to 5 mm in diameter, superimposed by a central crust. It usually begins on the trunk and spreads to the extremities." The lesions gradually fade to reddish brown and heal with postinflammatory hyperpigmentation. The biochemical hallmarks are increased urinary chorionic gonadotropin levels in the last trimester of pregnancy and reduced plasma cortisol and urinary estriol levels." No specific histopathologic find-
688 Brie] communications
Fig. 1. Case 1. Longitudinal pigmented nail bands with bluish discoloration of nail bed. Fig. 2. Large melanin granules (G) and dendritic melanocytes (M) throughout nail matrix (MX}. NP, Nail plate. (Ammoniacal silver nitrate stain; X400.)
REFERENCES 1. Furth PA, Kazakis AM. Nail pigmentation changes associated with azidothymidine (zidovudine) [Abstract]. Ann Intern Med 1987;107:350. 2. Pannwalker AP. Nail pigmentation in the acquired immunodeficiency syndrome (AIDS). Ann Intern Med 1987; 107:943-4. 3. Gonzalcz-Lahoz JM, Garcia Aguado C, MartinezFernandez R, et al. Pigmentation azulada en las unas de los pacientes con SIDA: efecto colateral de la zidovudina? [Letter] Rev Clin Espan 1988;183:278-9. 4. Azon-Masoliver A, Mallolas J, Gatell J, et al. Zidovudineinduced nail pigmentation. Arch Dermatol 1988;124: 1570-1. 5. Riechman DD, Fieschl MA, Grieco MH, et al. The toxicity of azidothymidine (AZT) in the treatment of patients with AIDS and AIDS-related complex: a double blind controlled trial. N Engl J Med 1987;317:192-7. 6. McEvoy GK, ed. American Hospital Formulary Service: drug information. Bethesda, Md.: American Society of Hospital Pharmacists, 1988:392-7.
7. Daniel CR, Scher RK. Nail changes secondary to systemic drugs or ingestants.J AMACAD DERMATOL 1984;10:250-8.
Acquired port-wine stain Mark W. Cobb, MD, and Leon Goldman, MD* Bethesda, Maryland, and San Diego, California Nevus flammeus is a common congenital malformation of mature capillaries that occur in up to 75% of newborns.' The most common type is the salmon patch, From the Department of Dermatology, Naval Hospital. The views expressed herein are those of the authors and do not reflect the officialpolicyor position of the Department of the Navy, Department of Defense, or the U.S. Government. No reprints available. *Dr. Goldman is at the Naval Hospital, San Diego, Calif.
Volume 22 Number 4 April 1990
Brief communications
689
found on the nape of the neck, glabella, forehead, eyelids, or upper lip.2 Those on the face tend to involutebythe first year. Nuchal lesions resolve less frequently and approximately 50% persist into adulthood. Port-wine stains, like salmon patches, are almost always present at birth; however, rather than involutingor fading, they persistand often become raised and nodular as the patient ages. Rarely port-wine stains are acquired, most of which are posttraumatic.' We report a case of an acquired port-wine stain in a patient with no history of antecedent trauma. Case report. A 41-year-old man had an asymptomatic discoloration of the right sideof the face and scalp that began approximately 8 yearsearlier. The lesion had enlarged for the first several years, thenstabilized. He denied anyhistory ofprior trauma to thearea.His general healthwas good andhe took no medications. Physical examination revealed several erythematousto violaceous patches ontherighttemporal scalp, preauricular area, chin, and neck (Fig. 1). The lesion only partially blanched on diascopy. The clinical impression was a port-wine stain.An incisional biopsy was done and thespecimen revealed ectaticvessels in the papillary dermis, consistent with port-wine stain (Fig. 2). Discussion. Port-wine stain represents a progressive ectasia of vesselsin the superficialvascular plexusand not a proliferative processas in hemangioma.v 5 Barskyet al. 5 believed that collagen degeneration of the dermal structures supporting the vessels was involvedin this progressive ectasia. Recently it has been postulated that the port-wine stains may be due to a local alteration in neural modulation of vascular tone. Specifically, Rosen and Smeller" hypothesized that sympathetic innervation of cutaneous vasculature may be functionally deficient in port-wine stain; congenital lesions demonstrate a maturation defect in innervation and acquired lesions result from a lossin sympathetic function. The resultingcontinuous unregulated blood flow would then predispose the cutaneous vessels to progressive ectasia.' The same authors' have demonstrated a significantdecreasein both nerve density and vessels with associated nerves in portwine stain when compared with normal skin and hemangiomas. Acquired port-wine stains are rare; most reportedcases occur after some form of local trauma. The reported antecedent injury varies from being struck by a cricket ball' to intense cold exposure at the Russian Front during World War H.9 These diverse insults may adversely affect the sympathetic vasoconstrictive innervation to superficial cutaneous vessels and result in ectasia. Brinkmann'!' reported two cases of acquired port-wine stain in female patients; the first was pregnant and the second was pubertal at the time the lesion developed. There was no history of antecedent trauma and increased estrogen levels were suggested as being a factor. Cases of acquired port-wine stain associated with use of oral contraceptive pills have also been reported. II Our case is re-
Fig. 1. Erythematous, nonblanchable patch involves right temporal scalp and preauricular area. Fig. 2. Several ectatic vessels present in papillary dermis. (Hematoxylin-eosin stain; XlOO.) markable for lack of any history of preceding injury or source of increased estrogens. We were able to find only two such cases in the liter ature. I I, 12 REFERENCES 1. CaroWA,Bronstein BR.Tumorsof theskin.In:Moschella SL, Hurley HJ, eds. Dermatology, 2nd ed. Philadelphia: WB Saunders, 1985:1599-600. 2. Atherton DJ, Rook A. Naeviand otherdevelopmental defects. In: Rook A, Wilkinson DS, Ebling FJG, et al., eds. Textbook of dermatology. 4th ed. Oxford: Blackwell, 1986:194-5. 3. Colver GB,RyanTJ. Acquired port-wine stain.Arch DermatoI1986;122:1415 c6. 4. Lever WF, Schaumburg-Lever G, ed. Histopathology of the skin. Philadelphia: JB Lippincott, 1983:623-4. 5. Barsky SH, Rosen S, Geer DE, et al. The nature and evolution ofport-wine stains:a computer-assisted study. J Invest Dermatol 1980;74: 154-7. 6. Rosen S, Smaller BR. Port-wine stains: a newhypothesis. JAM ACAD DERMATOL 1987;17:164-6. 7. Rosen S, Smoller B. Pathogenesis of port-wine stains. A new hypothesis. Med Hypotheses 1987;22:365-8.
690 Brief communications
Journal of the American Academy of Dermatology
8. Smaller BR, Rosen S. Port-wine stains. Arch Dermatol 1986;122:177-9. 9. Niemand-Anderssen 1. Naevus f1ammeus tardivus (attributable to cold?). Z Hauth 1952;12:251-6. 10. Brinkmann W. Acquired nevus flammeus. Z Hautkr 1981;56:1334-40. 11. Goldman L. Oral contraceptives and vascular anomalies. Lancet 1970;2:108-9. 12. Traub EF. Naevus flammeus appearing at the age of 23. Arch Dermatol Syphilo1 1939;39:752.
Papular dermatitis of pregnancy: A case report Luat Q. Nguyen, MD, and O. Robert Sarmini, MD
Arlington, Texas Numerous physiologic and pathologic changes can occur in the skin of pregnant women. 1, 2 These include a disorder first described in 1962 by Spangler et al. 3 They termed it "papular dermatitis of pregnancy" and reported an increase in fetal mortality. We report a case of papular dermatitis of pregnancy with abnormal laboratory findings, good maternal outcome, and no fetal loss. Case report. A 27-year-old woman, gravida 4, para 2, had lower abdominal contractions and bleeding. The estimated gestational age of the fetus on the patient's admission was 26 weeks 4 days. Physical examination revealed a fundal height of26 em with uterine contractions every 5 minutes. An ultrasound study ruled out placenta previa. The patient's premature labor was controlled with ritodrine hydrochloride (Yutopar). At the twenty-eighth week of pregnancy, a pruritic, eczematous rash developed on the chest and back. The eruption rapidly cleared with topical steroid therapy and 6 mg of beta methasone sodium phosphate intramuscularly. Two weeks later, numerous severely itching, erythematous papules appeared on the abdomen and back (Fig. 1). Because the eruption continued to worsen despite repeated doses of betamethasone sodium phosphate, a skin biopsy was performed. The specimen revealed a superficial lyrnphocytic perivascular infiltrate. Immunofluorescence study demonstrated no immunoglobulin or complement deposition. Results of routine laboratory studies were either within normal limits or negative. A hormonal evaluation revealed lowvalues of cortisol at 1.8 Itg/dl (normal 5.1 to 20.1 ,ug/dl). Values of urinary estriols were 0.8 gm/24 hr (normal 0.6 to 1.8 gm/24 hr). Urinary chorionic gonadotropin levels were moderately high at 27,756 TU/24 hr (normal 6000 to 25,000 IU/24 hr). A diagnosisof papular dermatitis of pregnancy was considered, and the patient was treated with oral prednisone (60 mg/day). At 32 weeks 2 days of pregnancy, results of the patient's ultrasound examination revealed oligohydramnios and signs of placental degeneration and calcification. Amniocentesis showed a high
From the Departments ofDermatology and Obstetrics and Gynecology, HCA South Arlington Medical Center, Financial support for color reproduction was received from Dermik Laboratories. Reprint requests: Luat Q. Nguyen, MD, 3132 Matlock Rd., Suite 307, Arlington, TX 76015.
16/4/149"6
Fig. 1. Disseminated papular eruption on chest and abdomen.
lecithin/sphingomyelin ratio with positive phosphatidylglycerol, demonstrating fetal lung maturity. The patient underwent cesarean section, which resulted in the delivery of a growthretarded infant at 33 weeks' gestation with no skin involvement. The cortisone was gradually tapered and was discontinued, and within a week after delivery the eruption had completely subsided. Discussion. The natural history, underlying mechanisms, and causes of some dermatoses of pregnancy are fairly well understood." A few conditions remain controversial (e.g., toxemic rash of pregnancy, prurigo gestationis of Besnier, and papular dermatitis of pregnancy) and are not unanimously accepted as distinct clinical entities.' Spangler et al.;' however, believed that among the several dermatoses characterized by pruritic papules was a subgroup of patients with identifiable clinical and biochemical features. Papular dermatitis of pregnancy is rare and occurs in 0.026% of births." As of 1988 a literature search revealed only about 23 reported cases. The eruption may begin at any time during pregnancy' and consists of intensely pruritic erythematous papules, 3 to 5 mm in diameter, superimposed by a central crust. It usually begins on the trunk and spreads to the extremities." The lesions gradually fade to reddish brown and heal with postinflammatory hyperpigmentation. The biochemical hallmarks are increased urinary chorionic gonadotropin levels in the last trimester of pregnancy and reduced plasma cortisol and urinary estriol levels." No specific histopathologic find-
