1026
Journal of the American Academy of Dermatology
Brief communications
blood coagulation factor X a in the tick Ornithodoros moubata. This factor, tick anticoagulant peptide, is a serine protease inhibitor, specific to factor Xa. It might be the cause of the response in our patients who had been bitten by a similar tick species. Galun and Ben-Chetrit lo prophylactically treated 13 persons with cave tick bites with tetracycline, 1 gin/day, for 3 to 5 days; none of them had symptoms of tick-borne relapsing fever. They suggested that a short-term, low-dose course of tetracycline might prevent the disease. Our treatment with tetracycline was not followed by relapsing fever. REFERENCES
1. PawlowskyEN, Stein AK. Ueber die Wirkung des Stiehes yon Ornithodoros papillipes Bir auf den Mensehen. Leningrad: Festsehr Nachrt, 1927:401-408.
2. Katzenellenbogen I. On stings and bites causing skin diseases in Israel. Dapim Refuim 1962;19:673-81. 3. Arthur DR, ed. Ticks and disease. Oxford: Pergamon, 1962:130-1.
4. RonnenM, Kahana M, Schewach M. Ornithodoriasis precedingBorreliainfection.Arch Dermatol 1984;120:1520-1. 5. AviviA, Warburg M, Galun R. Ecologicalstudies on the cave tick Ornithodoros tholozani and its distribution in Israel. Isr J Entomol 1973;8:109-29. 6. Theodor O, Costa M. A survey on the parasites of wild mammals and birds in Israel. I. Ectoparasites. Jerusalem: Israel Academy of Sciences and Humanities, 1967:92-5. 7. Lidror R. Relapsing fever in Israel (in Hebrew). Nikrot Zurim 1991;16:147-57. 8. BeaverPC, Jung RC, Cupp EW, eds.Clinical parasitology. 9th ed. Philadelphia: Lea & Febiger, 1984:589-90. 9. Waxman L, Smith DE, Arcuri KE, et al. Tick anticoagulant peptide (TAP) is a novelinhibitor of blood coagulation factor Xa. Science 1990;248:593-6. 10. Galun E, Ben-Chetrit E. Possibleprevention of tick-borne relapsing fever. J Infect Dis 1984;150:617.
Acral persistent papular mucinosis Scott W. Fosko, MD, Maritza I. Perez, MD, and B. Jack Longley, M D New Haven, Connecticut In 1986 Rongioletti et al. 1first described a distinct and rare mucinosis, acral persistent papular mucinosis ( A P P M ) . W e report the case of an additional patient with this mucinosis and summarize the patients previously reported. CASE REPORT A 40-year-old white woman had a 1-year history of asymptomatic papules on her hands and wrists. The patient was not taking any medications when her skin lesions developed. Physical examination revealed five ivory-white, translucent, smooth papules, measuring 2 to 4 ram, on the back of her hands and extensor wrists (Fig. 1). A skin biopsy specimen revealed mucin deposition in the papillary and upper reticular dermis, thin interspersed collagen fibers, and an increased number of fibroblasts (Fig. 2). The mucin stained positive with aleian blue at pH 2.5 and with colloidal iron. It stained negative with alcian blue at pH 0.5, which suggests the presence of hyaluronic acid. From the Department of Dermatology, Yale University School of Medicine. Reprint requests:Maritza I. Perez, MD, Departmentof Dermatology, YaleUniversitySchoolofMedicine,333CedarSt., NewHaven,CT 06510. 16/54/40026
Results of all laboratory studies were normal, including serum protein electrophoresis and thyroid function. DISCUSSION APPM, recently reviewed by Rongioletti and Rebora, 2 has been previously reported in nine patients (TableI). A P P M is characterized by multiple, usually asymptomatic, ivory to flesh-colored, translucent papules, located exclusively on the back of the hands, the extensor surface of the wrists, and, occasionally, the distal forearms. The female/male ratio is 9:1. Translucent viscous fluid is present in some lesions. The lesions persist and may enlarge slowly without resolution. N o associated systemic findings have been reported. Histopathologically, an accumulation of mucin (mainly hyaluronic acid) is seen in the upper reticular dermis, mostly focal but rarely diffuse. A grenz zone is a characteristic finding; an increased number of fibroblasts is an inconstant finding. Discrete papular lichen myxedematosus (DPLM) can be distinguished from A P P M clinically and histologically. In DPLM, the lesions m a y be larger, erythematous, and may involve sites other than the back of the hands, extensor surface of t he wrists, and
Volume 27 Number 6, Part I December 1992
Brief communications
1027
Fig. 1. Ivory-white, translucent, 2 to 4 mm, smooth papules on back of hand. Fig. 2. Photomicrograph of histopathologic specimen of papule stained with colloidal iron.
(x4o.) T a b l e I. Summary of clinical and laboratory features of 10 patients
Sex/ Authors
Rongioletti et al.l
Age (yr)
No./ Location
F/40 Extensor wrists, back of hands 2-5 mm, asymptomatic white papules, translucent, dense fluid expressed when punctured
Duration (yr)
Histopathologie findings
Focal accumulation At 6 mo slight increase in size of mucin in upper reticular dermis Staining (+) aloian blue (pH 4.0, 2.5); metachromatic toluidine blue (-) alcian blue (pH 0.4) F/41 Extensor surface 5 "Subepidermal pap- NR wrist/hands, flesh ule" of mucin in upper reticular colored 2-7 mm, dermis, with splitasymptomatic ting of collagen papules fibers and proliferation of fibroblasts Staining (+) alcian blue (pH 2.5); (-) mucicarmine (pH 0.5); hyaluronidase-labile "Many" Mucin in mid der- NR F/66 I0 on dorsum of Naeyaert hands, distal foreand Kint 3 mis Staining (+) alcian arms, 10, 2 mm, blue (pH 2.5); waxy papules, metachromatic skin-colored, pruritic toluidine blue NR, Not reported.
2
Associated findings
Course
DIF granular IgG at basal zone, IgM bodies in papillary dermis, linear [gG eccrine glands
None
Elevated cholesterol and triglycerides
Continued on p. 1028
1028
Journal of the American Academy of Dermatology
Brief communications
Table I. Cont'd.
Authors RaveUa et al.4
Sex/ Age (yr)
No./ , Location,.
Duration { (yr)
F/56 Asymptomatic, ivory-white, 3-5 mm papules, extensor forearms, back of hands M/55 Asymptomatic 2-4 ram, ivory papules, extensor surface of forearms and back of hands
6
Crovato et al. 6
F/46 Hundreds, discrete 2-5 mm, fleshcolored papules, extensor surface of distal forearms, back of hands
3
Cribier et al. 7
F/77 Multiple, asymptomatic, ivory papules, back of one hand and wrist, small amount of liquid expressed from some papules
10
Naeyaert et al. 8
F/60 40 asymptomatic, yellow to fleshcolored 1-3 ram, papules over extensor side forearms and back of hands
5
Royer et al. s
Aho et al. 9 F/24
10 asymptomatic, pale, 2-4 ram, discrete papules over extensor surface hands and wrists
1
Histolmthologic findings
Associated findings
9
Course
Well-defined super- No change ficial dermal deposits of acid mucopolysaccharides
None
Increased mucin in Developed new lesuperficial dermis, sions over next and splitting the 2 yr collagen fibers Staining (+) alcian blue (pH 4.5); metachromatic toluidine blue
Ultrastructural studies showed microfibrillar network aggregated to collagen fibrils enclosing finely granular amorphous material; some fibroblasts had dilated endoplasmie reticulum Died of breast cancer 1 yr after presentation of APPM
Well-defined deposits of mucin in upper reticular dermis, with splitting of collagen fibers and proliferation of fibroblasts Staining (+) alcian blue (pH 2.5); ( - ) alcian blue (pU 4.0) Accumulation of No change mucin in between collagen fibers of the reticular dermis; some biopsies with increased fibroblasts in reticular dermis Staining (+) alcian blue Well-circumscribed amorphous material in upper reticular dermis and slight increase in number of fibroblasts Staining (+) alcian blue (pH 2.5) Accumulation of No change hyaluronidase labile ground substance Staining (+) alcian blue (pH 2.5)
None
Died 3 yr after presentation, cause unknown
Factor XIIa + cells in papillary dermis; electronmicroscopy showed intervening network of spindle
Volume 27 Number 6, Part 1 December 1992
Brief communications 1029
T a b l e I. Cont'd, Sex/
Age
Authors
(yr)
No./ Location
Duration
Aho et al. 9 (eont'd)
Present case
F/40 5 asymptomafic, 2-4 mm, ivory-white, translucent, smooth, firm, papules on back of hands and extensor wrists
I
Histopathologic findings
Course
Thin, delicate collagen bundles and slight increase in stellate fibroblasts in reticular dermis Accumulation of No change over 4 mucin in papilmo of observation lary and upper reticular dermis, admixed with thin collagen fibers and increased number of fibroblasts Staining (+) alcian blue (pH 2.5); colloidal iron; ( - ) alcian blue (pH
I
Associated findings
fibroblasts with long cytoplasmic projections
None
0.5)
distal forearms. 10 In D P L M mucin deposits are diffusely distributed in the reticular dermis together w i t h an irregular arrangement of collagen bundles a n d striking fibroblast proliferation.
REFERENCES t. Rongioletti F, Rebora A, Crovato F. Acral persistent papular mucinosis: A new entity? Arch Dermatol 1986; 122:1237-9. 2. Rongioletti F, Rebora A. The new cutaneous mucinoses: a review with an up-to-date classificationof cutaneous mucinoses. J AM ACADDERMATOL1991;24:265-70. 3. Naeyaert JM, Kint A. Papular mucinosis [Abstract]. Dermatologica 1985;171:508. 4. Ravella A, Garcia P, Moreno A, et al. Mucinosepapuleuse persistante acrale, Ann Dermatol Venereol 1987;114:1438-
40.
5. Royer P, Beylot C, Garabiol B, et al. Acromucinose papuleuse persistante. Ann Dermatol Venereol 1988;115:82731. 6. Crovato F, Nazzari G, Desirello G. Acral persistent papular mucinosis. J AM ACAD DERMATOL] 990;23:121-2. 7. Cribier B, Heid E, Sass U. Mueinose papuleuse acrale persistante. Nouv Dermatol 1990;9:333-4. 8. Naeyaert JM, Geerts ML, Kudsi S, et al. Acral persistent papular mucinosis: a peculiar variant of the discrete papular form of lichen myxedematosus. Arch Dermatol 1990; 126:1372-4. 9. Aho HJ, Forsten Y, Hopsu-Havu V. Ultrastructural signs of altered intracellular metabolism in acral persistent papular mucinosis. J Cutan Pathol 1991;18:347-52. 10. Montgomery H, Underwood LJ. Lichen myxedematosus (differentiation from cutaneous myxedemas or mucoid states). J Invest Dermatol 1953;20:213-33.
Journal of the American Academy of Dermatology
Brief communications
blood coagulation factor X a in the tick Ornithodoros moubata. This factor, tick anticoagulant peptide, is a serine protease inhibitor, specific to factor Xa. It might be the cause of the response in our patients who had been bitten by a similar tick species. Galun and Ben-Chetrit lo prophylactically treated 13 persons with cave tick bites with tetracycline, 1 gin/day, for 3 to 5 days; none of them had symptoms of tick-borne relapsing fever. They suggested that a short-term, low-dose course of tetracycline might prevent the disease. Our treatment with tetracycline was not followed by relapsing fever. REFERENCES
1. PawlowskyEN, Stein AK. Ueber die Wirkung des Stiehes yon Ornithodoros papillipes Bir auf den Mensehen. Leningrad: Festsehr Nachrt, 1927:401-408.
2. Katzenellenbogen I. On stings and bites causing skin diseases in Israel. Dapim Refuim 1962;19:673-81. 3. Arthur DR, ed. Ticks and disease. Oxford: Pergamon, 1962:130-1.
4. RonnenM, Kahana M, Schewach M. Ornithodoriasis precedingBorreliainfection.Arch Dermatol 1984;120:1520-1. 5. AviviA, Warburg M, Galun R. Ecologicalstudies on the cave tick Ornithodoros tholozani and its distribution in Israel. Isr J Entomol 1973;8:109-29. 6. Theodor O, Costa M. A survey on the parasites of wild mammals and birds in Israel. I. Ectoparasites. Jerusalem: Israel Academy of Sciences and Humanities, 1967:92-5. 7. Lidror R. Relapsing fever in Israel (in Hebrew). Nikrot Zurim 1991;16:147-57. 8. BeaverPC, Jung RC, Cupp EW, eds.Clinical parasitology. 9th ed. Philadelphia: Lea & Febiger, 1984:589-90. 9. Waxman L, Smith DE, Arcuri KE, et al. Tick anticoagulant peptide (TAP) is a novelinhibitor of blood coagulation factor Xa. Science 1990;248:593-6. 10. Galun E, Ben-Chetrit E. Possibleprevention of tick-borne relapsing fever. J Infect Dis 1984;150:617.
Acral persistent papular mucinosis Scott W. Fosko, MD, Maritza I. Perez, MD, and B. Jack Longley, M D New Haven, Connecticut In 1986 Rongioletti et al. 1first described a distinct and rare mucinosis, acral persistent papular mucinosis ( A P P M ) . W e report the case of an additional patient with this mucinosis and summarize the patients previously reported. CASE REPORT A 40-year-old white woman had a 1-year history of asymptomatic papules on her hands and wrists. The patient was not taking any medications when her skin lesions developed. Physical examination revealed five ivory-white, translucent, smooth papules, measuring 2 to 4 ram, on the back of her hands and extensor wrists (Fig. 1). A skin biopsy specimen revealed mucin deposition in the papillary and upper reticular dermis, thin interspersed collagen fibers, and an increased number of fibroblasts (Fig. 2). The mucin stained positive with aleian blue at pH 2.5 and with colloidal iron. It stained negative with alcian blue at pH 0.5, which suggests the presence of hyaluronic acid. From the Department of Dermatology, Yale University School of Medicine. Reprint requests:Maritza I. Perez, MD, Departmentof Dermatology, YaleUniversitySchoolofMedicine,333CedarSt., NewHaven,CT 06510. 16/54/40026
Results of all laboratory studies were normal, including serum protein electrophoresis and thyroid function. DISCUSSION APPM, recently reviewed by Rongioletti and Rebora, 2 has been previously reported in nine patients (TableI). A P P M is characterized by multiple, usually asymptomatic, ivory to flesh-colored, translucent papules, located exclusively on the back of the hands, the extensor surface of the wrists, and, occasionally, the distal forearms. The female/male ratio is 9:1. Translucent viscous fluid is present in some lesions. The lesions persist and may enlarge slowly without resolution. N o associated systemic findings have been reported. Histopathologically, an accumulation of mucin (mainly hyaluronic acid) is seen in the upper reticular dermis, mostly focal but rarely diffuse. A grenz zone is a characteristic finding; an increased number of fibroblasts is an inconstant finding. Discrete papular lichen myxedematosus (DPLM) can be distinguished from A P P M clinically and histologically. In DPLM, the lesions m a y be larger, erythematous, and may involve sites other than the back of the hands, extensor surface of t he wrists, and
Volume 27 Number 6, Part I December 1992
Brief communications
1027
Fig. 1. Ivory-white, translucent, 2 to 4 mm, smooth papules on back of hand. Fig. 2. Photomicrograph of histopathologic specimen of papule stained with colloidal iron.
(x4o.) T a b l e I. Summary of clinical and laboratory features of 10 patients
Sex/ Authors
Rongioletti et al.l
Age (yr)
No./ Location
F/40 Extensor wrists, back of hands 2-5 mm, asymptomatic white papules, translucent, dense fluid expressed when punctured
Duration (yr)
Histopathologie findings
Focal accumulation At 6 mo slight increase in size of mucin in upper reticular dermis Staining (+) aloian blue (pH 4.0, 2.5); metachromatic toluidine blue (-) alcian blue (pH 0.4) F/41 Extensor surface 5 "Subepidermal pap- NR wrist/hands, flesh ule" of mucin in upper reticular colored 2-7 mm, dermis, with splitasymptomatic ting of collagen papules fibers and proliferation of fibroblasts Staining (+) alcian blue (pH 2.5); (-) mucicarmine (pH 0.5); hyaluronidase-labile "Many" Mucin in mid der- NR F/66 I0 on dorsum of Naeyaert hands, distal foreand Kint 3 mis Staining (+) alcian arms, 10, 2 mm, blue (pH 2.5); waxy papules, metachromatic skin-colored, pruritic toluidine blue NR, Not reported.
2
Associated findings
Course
DIF granular IgG at basal zone, IgM bodies in papillary dermis, linear [gG eccrine glands
None
Elevated cholesterol and triglycerides
Continued on p. 1028
1028
Journal of the American Academy of Dermatology
Brief communications
Table I. Cont'd.
Authors RaveUa et al.4
Sex/ Age (yr)
No./ , Location,.
Duration { (yr)
F/56 Asymptomatic, ivory-white, 3-5 mm papules, extensor forearms, back of hands M/55 Asymptomatic 2-4 ram, ivory papules, extensor surface of forearms and back of hands
6
Crovato et al. 6
F/46 Hundreds, discrete 2-5 mm, fleshcolored papules, extensor surface of distal forearms, back of hands
3
Cribier et al. 7
F/77 Multiple, asymptomatic, ivory papules, back of one hand and wrist, small amount of liquid expressed from some papules
10
Naeyaert et al. 8
F/60 40 asymptomatic, yellow to fleshcolored 1-3 ram, papules over extensor side forearms and back of hands
5
Royer et al. s
Aho et al. 9 F/24
10 asymptomatic, pale, 2-4 ram, discrete papules over extensor surface hands and wrists
1
Histolmthologic findings
Associated findings
9
Course
Well-defined super- No change ficial dermal deposits of acid mucopolysaccharides
None
Increased mucin in Developed new lesuperficial dermis, sions over next and splitting the 2 yr collagen fibers Staining (+) alcian blue (pH 4.5); metachromatic toluidine blue
Ultrastructural studies showed microfibrillar network aggregated to collagen fibrils enclosing finely granular amorphous material; some fibroblasts had dilated endoplasmie reticulum Died of breast cancer 1 yr after presentation of APPM
Well-defined deposits of mucin in upper reticular dermis, with splitting of collagen fibers and proliferation of fibroblasts Staining (+) alcian blue (pH 2.5); ( - ) alcian blue (pU 4.0) Accumulation of No change mucin in between collagen fibers of the reticular dermis; some biopsies with increased fibroblasts in reticular dermis Staining (+) alcian blue Well-circumscribed amorphous material in upper reticular dermis and slight increase in number of fibroblasts Staining (+) alcian blue (pH 2.5) Accumulation of No change hyaluronidase labile ground substance Staining (+) alcian blue (pH 2.5)
None
Died 3 yr after presentation, cause unknown
Factor XIIa + cells in papillary dermis; electronmicroscopy showed intervening network of spindle
Volume 27 Number 6, Part 1 December 1992
Brief communications 1029
T a b l e I. Cont'd, Sex/
Age
Authors
(yr)
No./ Location
Duration
Aho et al. 9 (eont'd)
Present case
F/40 5 asymptomafic, 2-4 mm, ivory-white, translucent, smooth, firm, papules on back of hands and extensor wrists
I
Histopathologic findings
Course
Thin, delicate collagen bundles and slight increase in stellate fibroblasts in reticular dermis Accumulation of No change over 4 mucin in papilmo of observation lary and upper reticular dermis, admixed with thin collagen fibers and increased number of fibroblasts Staining (+) alcian blue (pH 2.5); colloidal iron; ( - ) alcian blue (pH
I
Associated findings
fibroblasts with long cytoplasmic projections
None
0.5)
distal forearms. 10 In D P L M mucin deposits are diffusely distributed in the reticular dermis together w i t h an irregular arrangement of collagen bundles a n d striking fibroblast proliferation.
REFERENCES t. Rongioletti F, Rebora A, Crovato F. Acral persistent papular mucinosis: A new entity? Arch Dermatol 1986; 122:1237-9. 2. Rongioletti F, Rebora A. The new cutaneous mucinoses: a review with an up-to-date classificationof cutaneous mucinoses. J AM ACADDERMATOL1991;24:265-70. 3. Naeyaert JM, Kint A. Papular mucinosis [Abstract]. Dermatologica 1985;171:508. 4. Ravella A, Garcia P, Moreno A, et al. Mucinosepapuleuse persistante acrale, Ann Dermatol Venereol 1987;114:1438-
40.
5. Royer P, Beylot C, Garabiol B, et al. Acromucinose papuleuse persistante. Ann Dermatol Venereol 1988;115:82731. 6. Crovato F, Nazzari G, Desirello G. Acral persistent papular mucinosis. J AM ACAD DERMATOL] 990;23:121-2. 7. Cribier B, Heid E, Sass U. Mueinose papuleuse acrale persistante. Nouv Dermatol 1990;9:333-4. 8. Naeyaert JM, Geerts ML, Kudsi S, et al. Acral persistent papular mucinosis: a peculiar variant of the discrete papular form of lichen myxedematosus. Arch Dermatol 1990; 126:1372-4. 9. Aho HJ, Forsten Y, Hopsu-Havu V. Ultrastructural signs of altered intracellular metabolism in acral persistent papular mucinosis. J Cutan Pathol 1991;18:347-52. 10. Montgomery H, Underwood LJ. Lichen myxedematosus (differentiation from cutaneous myxedemas or mucoid states). J Invest Dermatol 1953;20:213-33.
