- Email: [email protected]
Acquired perforating dermatosis: A report on 4 cases associated with scabies infection
J AM ACAD DERMATOL VOLUME 51, NUMBER 4
Fig 1. A 32-year-old woman with an asymmetric 25 3 28 mm macular tumor. Note the irregular borders and the light and dark brown colors.
Letters 665
bended breast. The colors were homogenously light to dark brown, partly slate gray. Because of this dermoscopic pattern no malignancy was assumed. The lesion was totally removed and, in histopathology, a melanosis of the areola was diagnosed. The cobblestone pattern in dermoscopy, which has not been described in literature, but is now seen in several cases, represents the texture of the normal skin of the areola. The melanosis of the areola has this cobblestone pattern combined with hyperpigmentation and this knowledge can change the management of these lesions. Therefore, this pattern should be added to the list of nonmelanocytic tumors of the skin.
Andreas Blum, MD Gisela Metzler, MD Ulrich Caroli, MD Department of Dermatology University of Tuebingen 72076 Tuebingen, Germany
Fig 2. In dermoscopy, an asymmetric, pigmented lesion with cobblestone pattern and slightly narrow parallel lines (small arrow). The cobblestone pattern was also seen in the non-pigmented area of the areola (large arrows). Note the homogenously light to dark brown and partly slate gray coloration.
can help in the diagnosis of pigmented tumors of the areola. A 32-year-old woman had noticed a new pigmentation on her left areola since the third month of her pregnancy. She was admitted to the hospital 5 months later to have a malignant melanoma ruled out. Clinically, she presented with an asymmetric macular tumor with irregular borders, light and dark brown colors, and a size of 25 3 28 mm (Fig 1). In dermoscopy, an asymmetric, pigmented lesion was present with cobblestone pattern and slightly narrow parallel lines (Fig 2). Cobblestone pattern was also seen in the nonpigmented area of the areola (Fig 2); the cracked oil appearance was a result of the
Correspondence to: Andreas Blum, MD Department of Dermatology University of Tuebingen Liebermeisterstrasse 25 72076 Tuebingen, Germany E-mail: [email protected]
REFERENCES 1. Pittis JD, Barber FA. Melanosis of the areola. Arch Dermatol 1990;126:542-3. 2. Kittler H, Pehamberger H, Wolff K, Binder M. Diagnostic accuracy of dermoscopy. Lancet Oncol 2002;3:159-65. 3. Argenziano G, Soyer HP, Chimenti S, Talamini R, Corona R, Sera F, et al. Dermoscopy of pigmented skin lesions: results of a consensus meeting via the Internet. J Am Acad Dermatol 2003;48:679-93. doi:10.1016/j.jaad.2004.04.011
Acquired perforating dermatosis: A report on 4 cases associated with scabies infection To the Editor: Acquired perforating dermatosis (APD) belongs to a group of perforating skin disorders with an obligatory transepidermal elimination of dermal
666 Letters
J AM ACAD DERMATOL OCTOBER 2004
Table I. Clinical features Patient No.
1 2 3 4
Age (y)/sex
Localization
Duration of APD (mo)
Pruritus
Diabetes mellitus duration (y)
Relation to HD
64/F 78/M 86/F 76/F
a, ba, bu, c, l a, ba, c ba, bu a, ba, c
2 7 8 6
Severe Severe Severe Moderate
5 8 10 26
No No No 4 y after HD
A, Arms; APD, acquired perforating dermatosis; ba, back; bu, buttocks; c, chest; F, female; HD, hemodialysis; l, legs; M, male.
Fig 1. Umbilicated papule with adherent central keratotic plug on lower aspect of back.
components (TEE). They are divided into primary and secondary forms: primary perforating dermatoses include idiopathic, distinct entities such as elastosis perforans serpiginosa, perforating folliculitis, Kyrle’s disease, and reactive perforating collagenosis. A secondary form is APD, formerly known as acquired reactive perforating collagenosis.1 APD has been reported to be associated with diabetes mellitus, chronic renal failure, malignant neoplasia, liver disorders, and HIV infection.2 Pruritus is the most prominent feature, and linear configuration and Koebner phenomenon can be observed occasionally. The TEE tissue is variable in APD, where collagen and/or elastic fibers can be found.3 Clinically, umbilicated papules and nodules with a central adherent, brownish-greenish keratotic plug characterize APD, histologically showing TEE of altered fibers into a cup-shaped epidermal depression. These findings combined with an onset after the age of 18 years have been described by Faver et al4 as obligatory diagnostic criteria for APD. Topical steroids, systemic retinoids, phototherapy, h1-antihistamines, allopurinol, doxycycline, cryotherapy, and liquid nitrogen have been used for treatment with varying degree of success. Recently, Kurschat et al5 reported one case of APD triggered by scabies
infection. Accordingly, we here describe 4 cases of scabies-provoked APD. APD was diagnosed in 4 patients with scabies infection between 1999 and 2003; their age ranged from 64 and 86 years. Scabies for all patients was confirmed by positive skin scrapings for eggs, larva, mites, or fecal pellets by light microscopy. Three patients had a history of diabetes mellitus and one had chronic renal insufficiency undergoing hemodialysis. All 4 patients presented typical umbilicated papules predominantly on the trunk, lower aspect of back, and/or the lower extremities’ extensor surfaces (Fig 1). Patients’ relevant clinical characteristics are summarized in Table I. The papules existed for a period ranging from 2 to 8 months. Initial pruritus was evident in every case. Scabies was treated according to a standard protocol. Pruritus in scabies infection can persist over weeks; thus, improvement was only seen in the regressing development of new papules and slow healing of the pre-existing lesions. In two patients, the problematic noncompliance with regard to their diabetes mellitus might have influenced the delayed improvement as well. The pathogenesis of APD is poorly understood. Generally accepted is the trigger function of superficial microtraumata afflicted to the skin through scratching. In addition, insufficient blood supply as a result of microangiopathy and polymorphonuclear leucocytes seems to be a predisposing factor for focal necrobiosis of tissue. Why the histologically altered, but ultrastructurally intact collagen bundles are found in vertical continuation in dermoepidermal tunnels remains unclear.6 In this respect, current investigations look at the significance of matrixdegrading metalloproteins. Their assumed influence on transepithelial channel formation by loosening interkeratinocyte bonds, disruption of the basement membrane, and detaching of collagen fibers is supported by increased levels after epidermal injury (eg, scratching) after hypoxic conditions or in diabetes state.
Letters 667
J AM ACAD DERMATOL VOLUME 51, NUMBER 4
We confirm the association between scabies infection and APD in 4 more cases, and underline the significant trigger function of superficial skin microtraumata in the initiation of TEE.
Wiebke Hinrichs, MD Frank Breuckmann, MD Peter Altmeyer, MD Alexander Kreuter, MD Department of Dermatology and Allergology Ruhr-University Bochum Germany Correspondence to: Alexander Kreuter, MD Department of Dermatology Ruhr-University Bochum Gudrunstrasse 56 D-44791 Bochum, Germany E-mail: [email protected]
REFERENCES 1. Kru¨ger K, Tebbe B, Krengel S, Goerdt S, Orfanos C. Acquired reactive perforating dermatosis. Successful treatment in 2 cases. Hautarzt 1999;50:115-20. 2. Schmults CA. Acquired reactive perforating collagenosis. Dermatol Online J 2002;8:8. 3. Fujimoto N, Akagi A, Tajima S, Ishibashi A, Nomura K, Matsushita A, et al. Expression of 67-kDa elastin receptor in perforating skin disorders. Br J Dermatol 2002;146:74-9. 4. Faver IR, Daoud MS, Daniel Su WP. Acquired reactive perforating collagenosis: report of six cases and review of literature. J Am Acad Dermatol 1994;30:575-80. 5. Kurschat P, Kroger A, Scharffetter-Kochanek K, Hunzelmann N. Acquired reactive perforating collagenosis triggered by scabies infection. Acta Derm Venereol 2000;80:384-5. 6. Brinkmeier T, Schaller J, Herbst RA, Frosch PJ. Successful treatment of acquired reactive perforating collagenosis with doxycycline. Acta Derm Venerol 2002;82:393-5.
doi:10.1016/j.jaad.2004.02.025
Mycophenolate mofetil treatment of a patient with hyperglobulinemic purpura To the Editor: Hyperglobulinemic purpura of Waldenstro¨m is characterized by recurring nonthrombocytopenic purpura, hypergammaglobulinemia, and an increased sedimentation rate. Hyperglobulinemic purpura of Waldenstro¨m may
Table I. Cutaneous disorders treated with mycophenolate mofetil Psoriasis Chronic dermatitis Vesiculobullous dermatoses Bullous pemphigoid Cicatricial pemphigoid Pemphigus—vulgaris, foliaceus, paraneoplastic Collagen vascular diseases Systemic and cutaneous lupus erythematosus Dermatomyositis Vasculitic syndromes Wegener’s granulomatosis Behc¸et’s disease Polyarteritis nodosa Cutaneous Crohn’s disease
present alone as a primary entity or as a secondary form associated with diseases, such as Sjo¨gren’s syndrome or systemic lupus erythematosus. Multiple therapies including corticosteroids, antimalarials, plasmapheresis, and other anti-inflammatory and/ or immunosuppressive agents have led to variable results. We report a 70-year-old woman with primary hyperglobulinemic purpura of Waldenstro¨m who responded to treatment with mycophenolate mofetil. Hyperglobulinemic purpura of Waldenstro¨m (HGPW) is characterized by recurring nonthrombocytopenic purpura, hypergammaglobulinemia, an increased sedimentation rate, and, often, a positive rheumatoid factor.1-5 HGPW may occur as primary form or secondary to an associated disorder, most commonly an autoimmune disease.1,2,5-9 Previously, multiple therapies including oral corticosteroids,1,10 antimalarials,1 colchicine,1,10 indomethacin,1,10 azathioprine,1 chlorambucil,5 and plasmapheresis11,12 have had variable results. Mycophenolate mofetil (MMF), an antimetabolite with immunosuppressive properties, has been reported in the treatment of several cutaneous diseases, such as autoimmune blistering disorders, psoriasis, cutaneous Crohn’s disease, and connective tissue disorders.13-17 We present a case of primary HGPW successfully treated with MMF. A 73-year-old woman was hospitalized in May 1999 for acute onset mental status changes. Her medical history included a diagnosis of a nonoperable right frontal meningioma. Her evaluation at that time was normal or negative for the following tests: complete blood cell count; comprehensive metabolic panel; rheumatoid factor; HIV screen;
Fig 1. A 32-year-old woman with an asymmetric 25 3 28 mm macular tumor. Note the irregular borders and the light and dark brown colors.
Letters 665
bended breast. The colors were homogenously light to dark brown, partly slate gray. Because of this dermoscopic pattern no malignancy was assumed. The lesion was totally removed and, in histopathology, a melanosis of the areola was diagnosed. The cobblestone pattern in dermoscopy, which has not been described in literature, but is now seen in several cases, represents the texture of the normal skin of the areola. The melanosis of the areola has this cobblestone pattern combined with hyperpigmentation and this knowledge can change the management of these lesions. Therefore, this pattern should be added to the list of nonmelanocytic tumors of the skin.
Andreas Blum, MD Gisela Metzler, MD Ulrich Caroli, MD Department of Dermatology University of Tuebingen 72076 Tuebingen, Germany
Fig 2. In dermoscopy, an asymmetric, pigmented lesion with cobblestone pattern and slightly narrow parallel lines (small arrow). The cobblestone pattern was also seen in the non-pigmented area of the areola (large arrows). Note the homogenously light to dark brown and partly slate gray coloration.
can help in the diagnosis of pigmented tumors of the areola. A 32-year-old woman had noticed a new pigmentation on her left areola since the third month of her pregnancy. She was admitted to the hospital 5 months later to have a malignant melanoma ruled out. Clinically, she presented with an asymmetric macular tumor with irregular borders, light and dark brown colors, and a size of 25 3 28 mm (Fig 1). In dermoscopy, an asymmetric, pigmented lesion was present with cobblestone pattern and slightly narrow parallel lines (Fig 2). Cobblestone pattern was also seen in the nonpigmented area of the areola (Fig 2); the cracked oil appearance was a result of the
Correspondence to: Andreas Blum, MD Department of Dermatology University of Tuebingen Liebermeisterstrasse 25 72076 Tuebingen, Germany E-mail: [email protected]
REFERENCES 1. Pittis JD, Barber FA. Melanosis of the areola. Arch Dermatol 1990;126:542-3. 2. Kittler H, Pehamberger H, Wolff K, Binder M. Diagnostic accuracy of dermoscopy. Lancet Oncol 2002;3:159-65. 3. Argenziano G, Soyer HP, Chimenti S, Talamini R, Corona R, Sera F, et al. Dermoscopy of pigmented skin lesions: results of a consensus meeting via the Internet. J Am Acad Dermatol 2003;48:679-93. doi:10.1016/j.jaad.2004.04.011
Acquired perforating dermatosis: A report on 4 cases associated with scabies infection To the Editor: Acquired perforating dermatosis (APD) belongs to a group of perforating skin disorders with an obligatory transepidermal elimination of dermal
666 Letters
J AM ACAD DERMATOL OCTOBER 2004
Table I. Clinical features Patient No.
1 2 3 4
Age (y)/sex
Localization
Duration of APD (mo)
Pruritus
Diabetes mellitus duration (y)
Relation to HD
64/F 78/M 86/F 76/F
a, ba, bu, c, l a, ba, c ba, bu a, ba, c
2 7 8 6
Severe Severe Severe Moderate
5 8 10 26
No No No 4 y after HD
A, Arms; APD, acquired perforating dermatosis; ba, back; bu, buttocks; c, chest; F, female; HD, hemodialysis; l, legs; M, male.
Fig 1. Umbilicated papule with adherent central keratotic plug on lower aspect of back.
components (TEE). They are divided into primary and secondary forms: primary perforating dermatoses include idiopathic, distinct entities such as elastosis perforans serpiginosa, perforating folliculitis, Kyrle’s disease, and reactive perforating collagenosis. A secondary form is APD, formerly known as acquired reactive perforating collagenosis.1 APD has been reported to be associated with diabetes mellitus, chronic renal failure, malignant neoplasia, liver disorders, and HIV infection.2 Pruritus is the most prominent feature, and linear configuration and Koebner phenomenon can be observed occasionally. The TEE tissue is variable in APD, where collagen and/or elastic fibers can be found.3 Clinically, umbilicated papules and nodules with a central adherent, brownish-greenish keratotic plug characterize APD, histologically showing TEE of altered fibers into a cup-shaped epidermal depression. These findings combined with an onset after the age of 18 years have been described by Faver et al4 as obligatory diagnostic criteria for APD. Topical steroids, systemic retinoids, phototherapy, h1-antihistamines, allopurinol, doxycycline, cryotherapy, and liquid nitrogen have been used for treatment with varying degree of success. Recently, Kurschat et al5 reported one case of APD triggered by scabies
infection. Accordingly, we here describe 4 cases of scabies-provoked APD. APD was diagnosed in 4 patients with scabies infection between 1999 and 2003; their age ranged from 64 and 86 years. Scabies for all patients was confirmed by positive skin scrapings for eggs, larva, mites, or fecal pellets by light microscopy. Three patients had a history of diabetes mellitus and one had chronic renal insufficiency undergoing hemodialysis. All 4 patients presented typical umbilicated papules predominantly on the trunk, lower aspect of back, and/or the lower extremities’ extensor surfaces (Fig 1). Patients’ relevant clinical characteristics are summarized in Table I. The papules existed for a period ranging from 2 to 8 months. Initial pruritus was evident in every case. Scabies was treated according to a standard protocol. Pruritus in scabies infection can persist over weeks; thus, improvement was only seen in the regressing development of new papules and slow healing of the pre-existing lesions. In two patients, the problematic noncompliance with regard to their diabetes mellitus might have influenced the delayed improvement as well. The pathogenesis of APD is poorly understood. Generally accepted is the trigger function of superficial microtraumata afflicted to the skin through scratching. In addition, insufficient blood supply as a result of microangiopathy and polymorphonuclear leucocytes seems to be a predisposing factor for focal necrobiosis of tissue. Why the histologically altered, but ultrastructurally intact collagen bundles are found in vertical continuation in dermoepidermal tunnels remains unclear.6 In this respect, current investigations look at the significance of matrixdegrading metalloproteins. Their assumed influence on transepithelial channel formation by loosening interkeratinocyte bonds, disruption of the basement membrane, and detaching of collagen fibers is supported by increased levels after epidermal injury (eg, scratching) after hypoxic conditions or in diabetes state.
Letters 667
J AM ACAD DERMATOL VOLUME 51, NUMBER 4
We confirm the association between scabies infection and APD in 4 more cases, and underline the significant trigger function of superficial skin microtraumata in the initiation of TEE.
Wiebke Hinrichs, MD Frank Breuckmann, MD Peter Altmeyer, MD Alexander Kreuter, MD Department of Dermatology and Allergology Ruhr-University Bochum Germany Correspondence to: Alexander Kreuter, MD Department of Dermatology Ruhr-University Bochum Gudrunstrasse 56 D-44791 Bochum, Germany E-mail: [email protected]
REFERENCES 1. Kru¨ger K, Tebbe B, Krengel S, Goerdt S, Orfanos C. Acquired reactive perforating dermatosis. Successful treatment in 2 cases. Hautarzt 1999;50:115-20. 2. Schmults CA. Acquired reactive perforating collagenosis. Dermatol Online J 2002;8:8. 3. Fujimoto N, Akagi A, Tajima S, Ishibashi A, Nomura K, Matsushita A, et al. Expression of 67-kDa elastin receptor in perforating skin disorders. Br J Dermatol 2002;146:74-9. 4. Faver IR, Daoud MS, Daniel Su WP. Acquired reactive perforating collagenosis: report of six cases and review of literature. J Am Acad Dermatol 1994;30:575-80. 5. Kurschat P, Kroger A, Scharffetter-Kochanek K, Hunzelmann N. Acquired reactive perforating collagenosis triggered by scabies infection. Acta Derm Venereol 2000;80:384-5. 6. Brinkmeier T, Schaller J, Herbst RA, Frosch PJ. Successful treatment of acquired reactive perforating collagenosis with doxycycline. Acta Derm Venerol 2002;82:393-5.
doi:10.1016/j.jaad.2004.02.025
Mycophenolate mofetil treatment of a patient with hyperglobulinemic purpura To the Editor: Hyperglobulinemic purpura of Waldenstro¨m is characterized by recurring nonthrombocytopenic purpura, hypergammaglobulinemia, and an increased sedimentation rate. Hyperglobulinemic purpura of Waldenstro¨m may
Table I. Cutaneous disorders treated with mycophenolate mofetil Psoriasis Chronic dermatitis Vesiculobullous dermatoses Bullous pemphigoid Cicatricial pemphigoid Pemphigus—vulgaris, foliaceus, paraneoplastic Collagen vascular diseases Systemic and cutaneous lupus erythematosus Dermatomyositis Vasculitic syndromes Wegener’s granulomatosis Behc¸et’s disease Polyarteritis nodosa Cutaneous Crohn’s disease
present alone as a primary entity or as a secondary form associated with diseases, such as Sjo¨gren’s syndrome or systemic lupus erythematosus. Multiple therapies including corticosteroids, antimalarials, plasmapheresis, and other anti-inflammatory and/ or immunosuppressive agents have led to variable results. We report a 70-year-old woman with primary hyperglobulinemic purpura of Waldenstro¨m who responded to treatment with mycophenolate mofetil. Hyperglobulinemic purpura of Waldenstro¨m (HGPW) is characterized by recurring nonthrombocytopenic purpura, hypergammaglobulinemia, an increased sedimentation rate, and, often, a positive rheumatoid factor.1-5 HGPW may occur as primary form or secondary to an associated disorder, most commonly an autoimmune disease.1,2,5-9 Previously, multiple therapies including oral corticosteroids,1,10 antimalarials,1 colchicine,1,10 indomethacin,1,10 azathioprine,1 chlorambucil,5 and plasmapheresis11,12 have had variable results. Mycophenolate mofetil (MMF), an antimetabolite with immunosuppressive properties, has been reported in the treatment of several cutaneous diseases, such as autoimmune blistering disorders, psoriasis, cutaneous Crohn’s disease, and connective tissue disorders.13-17 We present a case of primary HGPW successfully treated with MMF. A 73-year-old woman was hospitalized in May 1999 for acute onset mental status changes. Her medical history included a diagnosis of a nonoperable right frontal meningioma. Her evaluation at that time was normal or negative for the following tests: complete blood cell count; comprehensive metabolic panel; rheumatoid factor; HIV screen;