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Crusted scabies in a patient with chronic mucocutaneous candidiasis
Letters io the Editor
affected. An infective aetiology with an underlying immune dysfunction is postulated but no agent has yet been found. Most casespresent with painless cervical lymphadenopathy. About 30% have extranodal involvement and skin is one of the commonest sites [2]. Solitary skin involvement is rare [3]. The cutaneous manifestations of this disease have been reviewed previously and are typically xanthomatous but this appearanceis by no means invariable [4]. Treatment of skin lesions is difficult and assessment of treatment intervention is also difficult as the diseasemay undergo spontaneousremissions. Treatment with topical steroids is ineffective and radiotherapy with or without surgery has met with mixed success [5]. Therapeutic success as defined by an 80% flattening in skin lesions, has been reported with vinblastine loaded platelets [6]. Etoposide, cyclophosphamide and combination regimens have also met with some reported success [5,7,8]. In this case PUVA treatment was successful in producing a sustainedflattening of the skin lesions. The exact mechanism of action is unknown, however PUVA can deplete the skin of Langerhans cells [9] and its action on the foamy histiocytes in sinus histiocytosis with massive lymphadenopathy may be similar. Mortality from this disorder is more likely to be iatrogenic than through diseaserelated complications, though this should change with the perception of this as a reactive condition rather than a malignancy. It is thus welcome that the often disfiguring and recalcitrant skin lesions of this disorder may be treatedwith relative safety by PUVA therapy.
Crusted scabies in a patient with chronic mucocutaneous candidiasis To the Editor: One patient with association of chronic mucocutaneous candidiasis (CMC) and crusted scabies has been described previously [l]. In both diseasescell-mediated immunity alteration is the most common immune defect. An 18-year-old Caucasian woman consulted our department for the first time because she presented crusty, hyperkeratotic and pruriginous lesions on the
R. Parslew*, MS. Lewis-Jones, P.S. Friedmann Department of Dermatology, 3rdJloor UCD building, Royal Liverpool Universiw Hospital, Prescot street, Liverpool, L69 38, UK [l] Rosai J, Dorfman RF. Sinus histiocytosis with massive lymphadenopathy, a newly recognised benign clinicopathologic entity. Arch Path01 1969;87:63-70. [2] Foucar E, Rosai I, Dorfman R. Sinus histiocytosis with massive lymphadenopathy (Rosai-Dorfman disease): review of the entity. Semin Diagn Path01 1990;7(1):19-73. [3] Perrin C, Michiels F, Lxour JP et al. Sinus histiocytosis (Rosai-Dorfman disease) clinically limited to the skin. An immunohistochemical and ultrastructural study. J Cutaneous Path01 1993;20(4):368-374. [4] Thawerani H, Sanchez RL, Rosai J et al. The cutaneous manifestations of sinus histiocytosis with massive lymphadenopathy. Arch Dermatol 1978;114:191-197. 151 Foucar E, Rosai J, Dorfman RF. The ophthalmologic manifestations of sinus histiocytosis with massive lymphadenopathy. Am J Ophthalmol 1979;87:354-367. [6] Penneys NS, Ahn YS, McKinney EC et al. Sinus histiocytosis with massive lymphadenopathy: a case with unusual skin involvement and a therapeutic response to vinblastine-loaded platelets. Cancer 1984;54: 1834- 1840. [7] Newman SB, Sweet DL, Vardiman JW. Sinus histiocytosis with massive lymphadenopathy: response to cyclophosphamide therapy. Cancer Treat Rep 1984;68:901-902. [8] Suarez CR, Zeller WPS, Silberman S et al. Sinus histiocytosis with massive lymphadenopathy: remission with chemotherapy. Am J Paediatr Haematol Oncol 1983;3:235-241. [9] Friedmann PS. Disappearance of epidexmal Langerhans cells during PUVA therapy. Br J Dermatol 1981;105:219.
* Corresponding author. Tel.: +44 151 7062000. PII: SO926-9959(97)00108-6
back of her fingers, finger web spaces(Fig. l), buttocks, thighs and feet. Physical examination revealed a patient small in stature who also presentedgingival hyperplasia, scrotal tongue, oral thrush and nail dystrophy with paronychial involvement in some of her finger nails (Fig. 2). Some depressedscarred lesions were seen on her forehead, cheeks and scalp; these were the consequenceof hyperkeratotic lesions in infancy, not appraisedby us at the time of exploration. From infancy the patient had referred a history of
Letters
Fig. 1. Hyperkeratotic
crusted lesions on the back of the fingers.
recurrent outbreaks of whitish plaques on oral mucosa, angular cheilitis and periungueal inflammation with involvement of the nail plates. She had also been hospitalized on numerous occasions because of repetitive respiratory infections due to the presence of bronchiestasias. A skin biopsy specimen from a hyperkeratotic lesion on the back of one of the patient’s hands revealed the presence of several mites in the stratum comeum. Culture samples of a whitish lesion of the oral mucosa and the nail plate showed Cundidu albicans in both. The diagnosis was CMC and crusted scabies. Treatment consisting of keratolytics (5% salicylic acid) associated with 0.3% lindane and 30% benzyl benzoate resulted in the disappearance of the lesions of crusted scabies. Lesions of CMC improved with the application of topical clotrimazole and the administration of oral ketoconazole (200 mg daily) for 6 months, alternating with rest periods. At that time (198 1) ketoconazole was the only oral imidazole available commercially. The endocrinologic study (thyroid function, parathyroid hormone, growth hormone and basal cortisol tests) was normal. Sella turcica global enlargement, without any localized erosions, was seen in a skull Xray. An unspecific immunity deficiency, of both T and B cells, was detected in the immunologic study, with decrease of the total number of lymphocytes,
to the Editor
alteration of T-lymphocyte function with decreased proliferative response to mitogen (phytohemagglutinin) and IgG and IgM production deficit. Skin tests to trichophytin, Candida antigen, tuberculin and streptokinase-streptodornase, and sensitization test to dinitrochlorobenzene were negative. The patient’s evolution was torpid, presenting numerous pneumonias secondary to bronchiestasias until her death at the age of 30 years, caused by acute lung edema due to a secondary amyloidosis. CMC has been described associated with a variety of immune defects [2,3]. The most constant anomaly is decreased or absent T-lymphocyte response to Candida antigen [2,4]. Most patients also exhibit a generalized decrease in cell-mediated immunity and often show normal in vitro T-lymphocyte responses to mitogens like phytohemagglutinin [5]. Patients usually have normal total lymphocyte, T cell and B cell counts, although individuals with reduced numbers of T cells [6] and a combination of cell-mediated and humoral immune deficiencies, as in our case, have been reported [7]. In recent years crusted scabies has been reported in immunodepressed patients, and more commonly in diseases with cell-mediated immunity alteration [8]. AIDS is one of the diseases recently described as having a predisposition to it [9]. Cell-mediated immunity plays an important role in ectoparasitic infestations like Sarcoptes scabiei [lo], and both qualitative and quantitative T-lymphocyte alteration could explain the florid clinical picture. Our case is the second one described in the literature of association of crusted scabies and CMC [l].
Fig. 2. White membranous plaques on the tongue.
Letters to the Editor
We suspectthat in both casesthe causeis due to cellmediated immunity alteration. However more studies are necessaryand it is possible that in the future the specific immune defect that predisposes CMC patients to develop crusted scabies may be determined precisely. Magdalena Gonzrilez-Gtiemes, Ignacio Yanguas, J. Jaime Goday, Ricardo Soloeta Department of Dermatology, Hospital Santiago Apdstol, Vitoria, Spain
111Ververeli KO, Rabinowitz LG, Knapp S et al. Hyperkeratotic papular rash in a patient with chronic mucocutaneouscandidiasis. Ann Allergy 1994;72:198-202. PI Kirkpatrick CH, Rich RR, Bennett SE. Chronic mucocutaneous candidiasis: model building in cellular immunity. Ann Intern Med 1971;74:955-978. [31 Ammann AJ, Hong R. Disorders of the T cell system. In: Stiehm ER, Fulginiti VA, Eds. Immunologic Disorders in Infants and Children. 3rd edn. Philadelphia: WB Saunders, 1989;257-315. [41 SamsWM, Jorizzo JL, SyndermanR et al. Chronic mucocutaneouscandidiasisimmunologic studiesof three generations of a single family. Am J Med 1979;67:948-959.
Acquired smooth muscle hamartoma To the Editor: Smooth muscle hamartoma(SMH) representsa proliferation of randomly oriented dermal smooth muscles,noted at birth or in young adults [l-6]. To date, only two casesof SMH acquired after the age of 30 years have been reported [3,4]. We describe another acquired SMH with facial location with onset at the age of 35 years. A 45-year-old female patient presented with an indurated and discolored plaque located on the preauricular region extending from the zygomatic processto the inferior border of the mandible, measuring 4 x 6 cm in diameter (Fig. 1). This lesion startedas a brown line 3 cm in length 10 years ago. The plaque was firm, slightly depressedand yellowish in color. Bright white follicular papules were detected in the middle of the lesion. There was no hair on the plaque. A pseudo-Darier’s sign could not be elicited. Two skin biopsies obtained from the center of the plaque revealed increased smooth muscle bundles arranged haphazardly in the superficial and deep dermis (Fig. 2). The smooth muscle did not have a prominent
[51 StaverenAM, Stiehm ER. Chronic mucocutaneouscandidia-
sis: clinical immnnologic and therapeutic considerations.In: Moss AJ, Ed. Pediatrics Update. New York: Elsevier, 1986;93-110.
161Ammann AJ, Hong R. Disorders of the T-cell system. In: Stiehm ER, Fulginiti VA, Eds. Immunologic Disorders in Infants and Children. 3rd edn. Philadelphia: WB Saunders, 1989;286-290. 171 Gill FF, Portnoy JM. An unusual combination of immunologic abnormalities in a patient with chronic mucocutaneous candidiasis.Ann Allergy 1989;63:98-100. PI Dick GF, Burgdorf WHC, Gentry WC. Norwegian scabiesin Bloom’s syndrome.Arch Dermatol 1979;115:212-213. [91 Espaila Alonso A, Hermida Donate JM, Montilla de Mora P et al. Sarnanomegay sindromede imnunodeficienciaadquirida. Actas Dermosifiliogr 1990,81:174-175. 1101Dahl MV. The immunology of scabies. Ann Allergy 1983;51:560-564.
* Correspondingauthor. Servicio de Dermatologfa,Hospital Santiago Apdstol, C/Olaguibel 29,01004 Vitoria, Spain. Tel.: +34 945 253600; fax: +34 945 256181. PZL SO926-9959(97)00106-2
association with hair follicles. Trichrome stain was performed to confirm the presenceof smooth muscle cells. The overlying epidermis showed a slight increasein pigmentation at the basal layer; however, there was no increase in the number of melanocytes. A review of the literature showed that most SMH were patches or slightly indurated plaques mostly with prominent overlying hair [4,7]. In a smaller percentage of cases, the plaques contained follicular papules without prominent hair, as in our case [7,8]. Hyperpigmentation of various amounts was detectedin some lesions [4,7,8]. SMHs were usually located on the trunk or proximal extremities and varied from 3- 10 cm in diameter. Scrotal and eyelideyebrow involvement were recently reported [3,7]. In our case the lesion was located on the face in the preauricular region, which was also an unusual location. Differentiating SMH from Becker’s nevus can be difficult. Many authorsbelieve that thesetwo entities belong at opposite poles of the same developmental spectrum of hamartomatouschange [9]. When SMH is acquired, it may be easily confused with Becker’s
affected. An infective aetiology with an underlying immune dysfunction is postulated but no agent has yet been found. Most casespresent with painless cervical lymphadenopathy. About 30% have extranodal involvement and skin is one of the commonest sites [2]. Solitary skin involvement is rare [3]. The cutaneous manifestations of this disease have been reviewed previously and are typically xanthomatous but this appearanceis by no means invariable [4]. Treatment of skin lesions is difficult and assessment of treatment intervention is also difficult as the diseasemay undergo spontaneousremissions. Treatment with topical steroids is ineffective and radiotherapy with or without surgery has met with mixed success [5]. Therapeutic success as defined by an 80% flattening in skin lesions, has been reported with vinblastine loaded platelets [6]. Etoposide, cyclophosphamide and combination regimens have also met with some reported success [5,7,8]. In this case PUVA treatment was successful in producing a sustainedflattening of the skin lesions. The exact mechanism of action is unknown, however PUVA can deplete the skin of Langerhans cells [9] and its action on the foamy histiocytes in sinus histiocytosis with massive lymphadenopathy may be similar. Mortality from this disorder is more likely to be iatrogenic than through diseaserelated complications, though this should change with the perception of this as a reactive condition rather than a malignancy. It is thus welcome that the often disfiguring and recalcitrant skin lesions of this disorder may be treatedwith relative safety by PUVA therapy.
Crusted scabies in a patient with chronic mucocutaneous candidiasis To the Editor: One patient with association of chronic mucocutaneous candidiasis (CMC) and crusted scabies has been described previously [l]. In both diseasescell-mediated immunity alteration is the most common immune defect. An 18-year-old Caucasian woman consulted our department for the first time because she presented crusty, hyperkeratotic and pruriginous lesions on the
R. Parslew*, MS. Lewis-Jones, P.S. Friedmann Department of Dermatology, 3rdJloor UCD building, Royal Liverpool Universiw Hospital, Prescot street, Liverpool, L69 38, UK [l] Rosai J, Dorfman RF. Sinus histiocytosis with massive lymphadenopathy, a newly recognised benign clinicopathologic entity. Arch Path01 1969;87:63-70. [2] Foucar E, Rosai I, Dorfman R. Sinus histiocytosis with massive lymphadenopathy (Rosai-Dorfman disease): review of the entity. Semin Diagn Path01 1990;7(1):19-73. [3] Perrin C, Michiels F, Lxour JP et al. Sinus histiocytosis (Rosai-Dorfman disease) clinically limited to the skin. An immunohistochemical and ultrastructural study. J Cutaneous Path01 1993;20(4):368-374. [4] Thawerani H, Sanchez RL, Rosai J et al. The cutaneous manifestations of sinus histiocytosis with massive lymphadenopathy. Arch Dermatol 1978;114:191-197. 151 Foucar E, Rosai J, Dorfman RF. The ophthalmologic manifestations of sinus histiocytosis with massive lymphadenopathy. Am J Ophthalmol 1979;87:354-367. [6] Penneys NS, Ahn YS, McKinney EC et al. Sinus histiocytosis with massive lymphadenopathy: a case with unusual skin involvement and a therapeutic response to vinblastine-loaded platelets. Cancer 1984;54: 1834- 1840. [7] Newman SB, Sweet DL, Vardiman JW. Sinus histiocytosis with massive lymphadenopathy: response to cyclophosphamide therapy. Cancer Treat Rep 1984;68:901-902. [8] Suarez CR, Zeller WPS, Silberman S et al. Sinus histiocytosis with massive lymphadenopathy: remission with chemotherapy. Am J Paediatr Haematol Oncol 1983;3:235-241. [9] Friedmann PS. Disappearance of epidexmal Langerhans cells during PUVA therapy. Br J Dermatol 1981;105:219.
* Corresponding author. Tel.: +44 151 7062000. PII: SO926-9959(97)00108-6
back of her fingers, finger web spaces(Fig. l), buttocks, thighs and feet. Physical examination revealed a patient small in stature who also presentedgingival hyperplasia, scrotal tongue, oral thrush and nail dystrophy with paronychial involvement in some of her finger nails (Fig. 2). Some depressedscarred lesions were seen on her forehead, cheeks and scalp; these were the consequenceof hyperkeratotic lesions in infancy, not appraisedby us at the time of exploration. From infancy the patient had referred a history of
Letters
Fig. 1. Hyperkeratotic
crusted lesions on the back of the fingers.
recurrent outbreaks of whitish plaques on oral mucosa, angular cheilitis and periungueal inflammation with involvement of the nail plates. She had also been hospitalized on numerous occasions because of repetitive respiratory infections due to the presence of bronchiestasias. A skin biopsy specimen from a hyperkeratotic lesion on the back of one of the patient’s hands revealed the presence of several mites in the stratum comeum. Culture samples of a whitish lesion of the oral mucosa and the nail plate showed Cundidu albicans in both. The diagnosis was CMC and crusted scabies. Treatment consisting of keratolytics (5% salicylic acid) associated with 0.3% lindane and 30% benzyl benzoate resulted in the disappearance of the lesions of crusted scabies. Lesions of CMC improved with the application of topical clotrimazole and the administration of oral ketoconazole (200 mg daily) for 6 months, alternating with rest periods. At that time (198 1) ketoconazole was the only oral imidazole available commercially. The endocrinologic study (thyroid function, parathyroid hormone, growth hormone and basal cortisol tests) was normal. Sella turcica global enlargement, without any localized erosions, was seen in a skull Xray. An unspecific immunity deficiency, of both T and B cells, was detected in the immunologic study, with decrease of the total number of lymphocytes,
to the Editor
alteration of T-lymphocyte function with decreased proliferative response to mitogen (phytohemagglutinin) and IgG and IgM production deficit. Skin tests to trichophytin, Candida antigen, tuberculin and streptokinase-streptodornase, and sensitization test to dinitrochlorobenzene were negative. The patient’s evolution was torpid, presenting numerous pneumonias secondary to bronchiestasias until her death at the age of 30 years, caused by acute lung edema due to a secondary amyloidosis. CMC has been described associated with a variety of immune defects [2,3]. The most constant anomaly is decreased or absent T-lymphocyte response to Candida antigen [2,4]. Most patients also exhibit a generalized decrease in cell-mediated immunity and often show normal in vitro T-lymphocyte responses to mitogens like phytohemagglutinin [5]. Patients usually have normal total lymphocyte, T cell and B cell counts, although individuals with reduced numbers of T cells [6] and a combination of cell-mediated and humoral immune deficiencies, as in our case, have been reported [7]. In recent years crusted scabies has been reported in immunodepressed patients, and more commonly in diseases with cell-mediated immunity alteration [8]. AIDS is one of the diseases recently described as having a predisposition to it [9]. Cell-mediated immunity plays an important role in ectoparasitic infestations like Sarcoptes scabiei [lo], and both qualitative and quantitative T-lymphocyte alteration could explain the florid clinical picture. Our case is the second one described in the literature of association of crusted scabies and CMC [l].
Fig. 2. White membranous plaques on the tongue.
Letters to the Editor
We suspectthat in both casesthe causeis due to cellmediated immunity alteration. However more studies are necessaryand it is possible that in the future the specific immune defect that predisposes CMC patients to develop crusted scabies may be determined precisely. Magdalena Gonzrilez-Gtiemes, Ignacio Yanguas, J. Jaime Goday, Ricardo Soloeta Department of Dermatology, Hospital Santiago Apdstol, Vitoria, Spain
111Ververeli KO, Rabinowitz LG, Knapp S et al. Hyperkeratotic papular rash in a patient with chronic mucocutaneouscandidiasis. Ann Allergy 1994;72:198-202. PI Kirkpatrick CH, Rich RR, Bennett SE. Chronic mucocutaneous candidiasis: model building in cellular immunity. Ann Intern Med 1971;74:955-978. [31 Ammann AJ, Hong R. Disorders of the T cell system. In: Stiehm ER, Fulginiti VA, Eds. Immunologic Disorders in Infants and Children. 3rd edn. Philadelphia: WB Saunders, 1989;257-315. [41 SamsWM, Jorizzo JL, SyndermanR et al. Chronic mucocutaneouscandidiasisimmunologic studiesof three generations of a single family. Am J Med 1979;67:948-959.
Acquired smooth muscle hamartoma To the Editor: Smooth muscle hamartoma(SMH) representsa proliferation of randomly oriented dermal smooth muscles,noted at birth or in young adults [l-6]. To date, only two casesof SMH acquired after the age of 30 years have been reported [3,4]. We describe another acquired SMH with facial location with onset at the age of 35 years. A 45-year-old female patient presented with an indurated and discolored plaque located on the preauricular region extending from the zygomatic processto the inferior border of the mandible, measuring 4 x 6 cm in diameter (Fig. 1). This lesion startedas a brown line 3 cm in length 10 years ago. The plaque was firm, slightly depressedand yellowish in color. Bright white follicular papules were detected in the middle of the lesion. There was no hair on the plaque. A pseudo-Darier’s sign could not be elicited. Two skin biopsies obtained from the center of the plaque revealed increased smooth muscle bundles arranged haphazardly in the superficial and deep dermis (Fig. 2). The smooth muscle did not have a prominent
[51 StaverenAM, Stiehm ER. Chronic mucocutaneouscandidia-
sis: clinical immnnologic and therapeutic considerations.In: Moss AJ, Ed. Pediatrics Update. New York: Elsevier, 1986;93-110.
161Ammann AJ, Hong R. Disorders of the T-cell system. In: Stiehm ER, Fulginiti VA, Eds. Immunologic Disorders in Infants and Children. 3rd edn. Philadelphia: WB Saunders, 1989;286-290. 171 Gill FF, Portnoy JM. An unusual combination of immunologic abnormalities in a patient with chronic mucocutaneous candidiasis.Ann Allergy 1989;63:98-100. PI Dick GF, Burgdorf WHC, Gentry WC. Norwegian scabiesin Bloom’s syndrome.Arch Dermatol 1979;115:212-213. [91 Espaila Alonso A, Hermida Donate JM, Montilla de Mora P et al. Sarnanomegay sindromede imnunodeficienciaadquirida. Actas Dermosifiliogr 1990,81:174-175. 1101Dahl MV. The immunology of scabies. Ann Allergy 1983;51:560-564.
* Correspondingauthor. Servicio de Dermatologfa,Hospital Santiago Apdstol, C/Olaguibel 29,01004 Vitoria, Spain. Tel.: +34 945 253600; fax: +34 945 256181. PZL SO926-9959(97)00106-2
association with hair follicles. Trichrome stain was performed to confirm the presenceof smooth muscle cells. The overlying epidermis showed a slight increasein pigmentation at the basal layer; however, there was no increase in the number of melanocytes. A review of the literature showed that most SMH were patches or slightly indurated plaques mostly with prominent overlying hair [4,7]. In a smaller percentage of cases, the plaques contained follicular papules without prominent hair, as in our case [7,8]. Hyperpigmentation of various amounts was detectedin some lesions [4,7,8]. SMHs were usually located on the trunk or proximal extremities and varied from 3- 10 cm in diameter. Scrotal and eyelideyebrow involvement were recently reported [3,7]. In our case the lesion was located on the face in the preauricular region, which was also an unusual location. Differentiating SMH from Becker’s nevus can be difficult. Many authorsbelieve that thesetwo entities belong at opposite poles of the same developmental spectrum of hamartomatouschange [9]. When SMH is acquired, it may be easily confused with Becker’s