- Email: [email protected]
Penile calciphylaxis
736 Letters
J AM ACAD DERMATOL APRIL 2006
2. Mutasim DF, Cummings MP. Linear IgA disease with clinical and immunopathological features of epidermolysis bullosa acquisita. Pediatr Dermatol 1997;14:303-6. 3. Caux F, Kirtschig G, Lemarchand-Venencie F, Venecie PY, HoangXuan T, Robin H, et al. IgA-epidermolysis bullosa acquisita in a child resulting in blindness. Br J Dermatol 1997;137:270-5. 4. Bauer JW, Schaeppi H, Metze D, Muss W, Pohla-Gubo G, Hametner R, et al. Ocular involvement in IgA-epidermolysis bullosa acquisita. Br J Dermatol 1999;141:887-92. 5. Nousari HC, Sragovich A, Kimyai-Asadi A, Orlinsky D, Anhalt GJ. Mycophenolate mofetil in autoimmune and inflammatory skin disorders. J Am Acad Dermatol 1999;40:265-8. 6. Farley-Li J, Mancini AJ. Treatment of linear IgA bullous dermatosis of childhood with mycophenolate mofetil. Arch Dermatol 2003;139:1121-4. 7. Trebing D, Ziemer A. Acquired epidermolysis bullosa with a highly varied clinical picture and successful treatment with mycophenolate mofetil [in German]. Hautarzt 2001;52:717-21. doi:10.1016/j.jaad.2005.07.009
Penile calciphylaxis To the Editor: A 35-year-old man with hemodialysisdependent end-stage renal disease secondary to diabetes mellitus presented with a 1-week history of a painful penile lesion. The patient denied trauma to the area. On physical examination there was a single, wellmarginated, 2-cm, indurated, black, necrotic plaque over the lateral aspect of the glans penis (Fig 1). There was no exudate or inguinal lymphadenopathy. Medical history was significant for poorly controlled hypertension, diabetic gastropathy, retinopathy, peripheral vascular disease, and dependence on hemodialysis 3 times per week for 18 months. Pertinent laboratory results included calcium of 8.4 mg/dL (normal 8.5-10.6), phosphorus of 7.4 mg/dL (2.5-4.5), calcium-phosphate product of 62.16 mg2/dL2 (21.3-47.7), blood urea nitrogen of 52 mg/dL (10-26), creatinine of 4.5 mg/dL (0.6-1.3), and parathyroid hormone (PTH) of 38 pg/mL (15-75). Punch biopsy was performed, which revealed necrosis of the dermis with narrowing and occlusion of the arterioles from medial calcification. A diagnosis of penile calciphylaxis was made and the patient was referred to an urologist for further evaluation and management of the penile lesion. Because of increasing pain and difficulty with urination, a partial penectomy of the glans was performed 1 month after development of the initial lesion. The patient subsequently continued to develop further necrosis of the remaining penis and total penectomy was performed. Although the patient had no recurrence after the penectomy, the psychologic morbidity was such that he elected to discontinue hemodialysis within 2 months. He died shortly thereafter. Nearly all cases of calciphylaxis are associated with end-stage renal disease, which may also lead to
Fig 1. A single, well-marginated, 2-cm, indurated, black, necrotic plaque over the lateral aspect of the glans penis.
high levels of calcium, phosphorus, and PTH. In those not associated with end-stage renal disease, there is often primary or tertiary hyperparathyroidism, or other causes of hypercalcemia. Trauma has been associated with some cases of calciphylaxis.1 Patients with penile calciphylaxis present with painful violaceous papules with underlying induration, usually on the glans.2,3 Initial lesions have a mottled appearance and can mimic livedo reticularis. Lesions subsequently ulcerate and become necrotic, often within a few days. Laboratory values can be helpful in supporting the diagnosis of calciphylaxis. The calcium-phosphate product is elevated in up to 80% of patients and PTH is also often elevated.4 However, normal serum levels of the calcium-phosphate product or PTH do not preclude the diagnosis of calciphylaxis.4 Pathologic evaluation should be used to confirm clinical suggestion. Histologic findings include medial calcification and intimal fibrosis of small to medium blood vessels without associated vasculitic change. The differential diagnosis of penile calciphylaxis includes sexually transmitted diseases or other infections, primary neoplasms such as squamous cell carcinoma, trauma, fixed drug eruption, cutaneous Crohn’s disease, pyoderma gangrenosum, erosive lichen planus, and contact dermatitis. A biopsy is often helpful in distinguishing these conditions from calciphylaxis. Treatment of penile calciphylaxis remains unsatisfactory and is largely supportive. Current treatment modalities include surgical debridement, parathyroidectomy, calcium and phosphate binders, and dietary restriction of phosphorus.1 Improved survival with parathyroidectomy has been noted by some, particularly with evidence of hyperparathyroidism. However, mortality remains between 60% and 69%, often as a result of infection from gangrenous tissue.2,3 Matthew Woods, MD Sean F. Pattee, MD Norman Levine, MD
Letters 737
J AM ACAD DERMATOL VOLUME 54, NUMBER 4
Section of Dermatology University of Arizona Reprints requests: Norman Levine, MD Section of Dermatology, University of Arizona 535 N Wilmot Rd, Suite 101 Tucson, AZ 85711 E-mail: [email protected] REFERENCES 1. Ivker RA, Woosley J, Briggaman RA. Calciphylaxis in three patients with end-stage renal disease. Arch Dermatol 1995;131:63-8. 2. Karpman E, Das S, Kurzrock E. Penile calciphylaxis: analysis of risk factors and mortality. J Urol 2003;169:2206-9. 3. Boccaletti VP, Ricci R, Sebastio N, Cortellini P, Alinovi A. Penile necrosis. Arch Dermatol 2000;136:259-64. 4. Bour J, Steinhardt G. Penile necrosis in patients with diabetes mellitus and end-stage renal disease. J Urol 1984;132:560-2. doi:10.1016/j.jaad.2005.07.025
The psoriasis variant palmoplantar pustulosis can be improved after cessation of smoking To the Editor: The psoriasis variant palmoplantar pustulosis (PPP) is closely associated with smoking; 95% of patients are smokers at the onset of the disease.1 In addition, 90% are women and the risk that a woman who is a smoker will develop PPP is 74 times higher than the risk for a nonsmoking healthy woman of the same age.2 Nicotine is excreted in the eccrine palmar duct,3 which is the target for the inflammation in PPP.1
Despite the possible role of smoking as a precipitating or aggravating factor for PPP, there are no reports on the clinical course of PPP in relation to cessation of smoking. We have tried to perform a prospective study with the aim of gaining more information about the clinical course of PPP in patients who were able to stop smoking in comparison with those who were not. In all, 63 consecutive patients with PPP (59 women and 4 men, mean age 54 6 10 years, range 25-73 years), all smokers, answered a questionnaire concerning their medical history and smoking habits. All had been smokers (in the majority about 20 years) at onset of PPP. The mean duration of their PPP was 9 6 8 years (range 1-30 years). All were examined by the same dermatologist (G. M.). The number of fresh pustules, the degree of erythema and scaling (0-3), and the percentage area affected on each palm and sole were recorded and a severity score calculated. All patients were informed about a possible role of smoking in PPP and detailed written and oral information about PPP was given.1,2,4,5 After being given this information, the patients were asked whether they were willing to try to stop smoking and, if so, when (within 1-2 months or later). Irrespective of their intention to stop smoking, the patients were also asked whether they were willing to fill in a protocol once weekly for at least 3 months concerning the activity of their PPP. The patients were instructed how to count fresh (yellow) pustules and were also asked to mark on a 10-cm visual analog scale how severe they considered
Table I. Patient evaluation n
Patient evaluation Total no. pustules Stopped smoking Did not stop smoking Total VAS Stopped smoking Did not stop smoking Dematologist evaluation Total no. fresh pustules Stopped smoking Did not stop smoking Total score Stopped smoking Did not stop smoking
Before
After
P
9 8
23 6 32 20 6 22
10 6 22 52 6 44
.0117 .2367
9 8
11.3 6 5.7 9.8 6 4.6
5.1 6 6.8 10.1 6 4.8
.0173 .3980
15 13
21 6 9 22 6 27
2 6 2 37 6 42
.0185 .1361
15 13
9.6 6 8.3 7.0 6 4.1
2.3 6 2.0 7.8 6 5.8
.007 .7837
Patient evaluation of number of pustules and severity of palmoplantar pustulosis based on diaries before and after 3-month attempts to stop smoking and dermatologist evaluation of number of pustules and total score (based on erythema, scaling, and area involved) at baseline and at follow-up after 3 to 6 months in patients who stopped and did not stop smoking. VAS, Visual analog scale.
J AM ACAD DERMATOL APRIL 2006
2. Mutasim DF, Cummings MP. Linear IgA disease with clinical and immunopathological features of epidermolysis bullosa acquisita. Pediatr Dermatol 1997;14:303-6. 3. Caux F, Kirtschig G, Lemarchand-Venencie F, Venecie PY, HoangXuan T, Robin H, et al. IgA-epidermolysis bullosa acquisita in a child resulting in blindness. Br J Dermatol 1997;137:270-5. 4. Bauer JW, Schaeppi H, Metze D, Muss W, Pohla-Gubo G, Hametner R, et al. Ocular involvement in IgA-epidermolysis bullosa acquisita. Br J Dermatol 1999;141:887-92. 5. Nousari HC, Sragovich A, Kimyai-Asadi A, Orlinsky D, Anhalt GJ. Mycophenolate mofetil in autoimmune and inflammatory skin disorders. J Am Acad Dermatol 1999;40:265-8. 6. Farley-Li J, Mancini AJ. Treatment of linear IgA bullous dermatosis of childhood with mycophenolate mofetil. Arch Dermatol 2003;139:1121-4. 7. Trebing D, Ziemer A. Acquired epidermolysis bullosa with a highly varied clinical picture and successful treatment with mycophenolate mofetil [in German]. Hautarzt 2001;52:717-21. doi:10.1016/j.jaad.2005.07.009
Penile calciphylaxis To the Editor: A 35-year-old man with hemodialysisdependent end-stage renal disease secondary to diabetes mellitus presented with a 1-week history of a painful penile lesion. The patient denied trauma to the area. On physical examination there was a single, wellmarginated, 2-cm, indurated, black, necrotic plaque over the lateral aspect of the glans penis (Fig 1). There was no exudate or inguinal lymphadenopathy. Medical history was significant for poorly controlled hypertension, diabetic gastropathy, retinopathy, peripheral vascular disease, and dependence on hemodialysis 3 times per week for 18 months. Pertinent laboratory results included calcium of 8.4 mg/dL (normal 8.5-10.6), phosphorus of 7.4 mg/dL (2.5-4.5), calcium-phosphate product of 62.16 mg2/dL2 (21.3-47.7), blood urea nitrogen of 52 mg/dL (10-26), creatinine of 4.5 mg/dL (0.6-1.3), and parathyroid hormone (PTH) of 38 pg/mL (15-75). Punch biopsy was performed, which revealed necrosis of the dermis with narrowing and occlusion of the arterioles from medial calcification. A diagnosis of penile calciphylaxis was made and the patient was referred to an urologist for further evaluation and management of the penile lesion. Because of increasing pain and difficulty with urination, a partial penectomy of the glans was performed 1 month after development of the initial lesion. The patient subsequently continued to develop further necrosis of the remaining penis and total penectomy was performed. Although the patient had no recurrence after the penectomy, the psychologic morbidity was such that he elected to discontinue hemodialysis within 2 months. He died shortly thereafter. Nearly all cases of calciphylaxis are associated with end-stage renal disease, which may also lead to
Fig 1. A single, well-marginated, 2-cm, indurated, black, necrotic plaque over the lateral aspect of the glans penis.
high levels of calcium, phosphorus, and PTH. In those not associated with end-stage renal disease, there is often primary or tertiary hyperparathyroidism, or other causes of hypercalcemia. Trauma has been associated with some cases of calciphylaxis.1 Patients with penile calciphylaxis present with painful violaceous papules with underlying induration, usually on the glans.2,3 Initial lesions have a mottled appearance and can mimic livedo reticularis. Lesions subsequently ulcerate and become necrotic, often within a few days. Laboratory values can be helpful in supporting the diagnosis of calciphylaxis. The calcium-phosphate product is elevated in up to 80% of patients and PTH is also often elevated.4 However, normal serum levels of the calcium-phosphate product or PTH do not preclude the diagnosis of calciphylaxis.4 Pathologic evaluation should be used to confirm clinical suggestion. Histologic findings include medial calcification and intimal fibrosis of small to medium blood vessels without associated vasculitic change. The differential diagnosis of penile calciphylaxis includes sexually transmitted diseases or other infections, primary neoplasms such as squamous cell carcinoma, trauma, fixed drug eruption, cutaneous Crohn’s disease, pyoderma gangrenosum, erosive lichen planus, and contact dermatitis. A biopsy is often helpful in distinguishing these conditions from calciphylaxis. Treatment of penile calciphylaxis remains unsatisfactory and is largely supportive. Current treatment modalities include surgical debridement, parathyroidectomy, calcium and phosphate binders, and dietary restriction of phosphorus.1 Improved survival with parathyroidectomy has been noted by some, particularly with evidence of hyperparathyroidism. However, mortality remains between 60% and 69%, often as a result of infection from gangrenous tissue.2,3 Matthew Woods, MD Sean F. Pattee, MD Norman Levine, MD
Letters 737
J AM ACAD DERMATOL VOLUME 54, NUMBER 4
Section of Dermatology University of Arizona Reprints requests: Norman Levine, MD Section of Dermatology, University of Arizona 535 N Wilmot Rd, Suite 101 Tucson, AZ 85711 E-mail: [email protected] REFERENCES 1. Ivker RA, Woosley J, Briggaman RA. Calciphylaxis in three patients with end-stage renal disease. Arch Dermatol 1995;131:63-8. 2. Karpman E, Das S, Kurzrock E. Penile calciphylaxis: analysis of risk factors and mortality. J Urol 2003;169:2206-9. 3. Boccaletti VP, Ricci R, Sebastio N, Cortellini P, Alinovi A. Penile necrosis. Arch Dermatol 2000;136:259-64. 4. Bour J, Steinhardt G. Penile necrosis in patients with diabetes mellitus and end-stage renal disease. J Urol 1984;132:560-2. doi:10.1016/j.jaad.2005.07.025
The psoriasis variant palmoplantar pustulosis can be improved after cessation of smoking To the Editor: The psoriasis variant palmoplantar pustulosis (PPP) is closely associated with smoking; 95% of patients are smokers at the onset of the disease.1 In addition, 90% are women and the risk that a woman who is a smoker will develop PPP is 74 times higher than the risk for a nonsmoking healthy woman of the same age.2 Nicotine is excreted in the eccrine palmar duct,3 which is the target for the inflammation in PPP.1
Despite the possible role of smoking as a precipitating or aggravating factor for PPP, there are no reports on the clinical course of PPP in relation to cessation of smoking. We have tried to perform a prospective study with the aim of gaining more information about the clinical course of PPP in patients who were able to stop smoking in comparison with those who were not. In all, 63 consecutive patients with PPP (59 women and 4 men, mean age 54 6 10 years, range 25-73 years), all smokers, answered a questionnaire concerning their medical history and smoking habits. All had been smokers (in the majority about 20 years) at onset of PPP. The mean duration of their PPP was 9 6 8 years (range 1-30 years). All were examined by the same dermatologist (G. M.). The number of fresh pustules, the degree of erythema and scaling (0-3), and the percentage area affected on each palm and sole were recorded and a severity score calculated. All patients were informed about a possible role of smoking in PPP and detailed written and oral information about PPP was given.1,2,4,5 After being given this information, the patients were asked whether they were willing to try to stop smoking and, if so, when (within 1-2 months or later). Irrespective of their intention to stop smoking, the patients were also asked whether they were willing to fill in a protocol once weekly for at least 3 months concerning the activity of their PPP. The patients were instructed how to count fresh (yellow) pustules and were also asked to mark on a 10-cm visual analog scale how severe they considered
Table I. Patient evaluation n
Patient evaluation Total no. pustules Stopped smoking Did not stop smoking Total VAS Stopped smoking Did not stop smoking Dematologist evaluation Total no. fresh pustules Stopped smoking Did not stop smoking Total score Stopped smoking Did not stop smoking
Before
After
P
9 8
23 6 32 20 6 22
10 6 22 52 6 44
.0117 .2367
9 8
11.3 6 5.7 9.8 6 4.6
5.1 6 6.8 10.1 6 4.8
.0173 .3980
15 13
21 6 9 22 6 27
2 6 2 37 6 42
.0185 .1361
15 13
9.6 6 8.3 7.0 6 4.1
2.3 6 2.0 7.8 6 5.8
.007 .7837
Patient evaluation of number of pustules and severity of palmoplantar pustulosis based on diaries before and after 3-month attempts to stop smoking and dermatologist evaluation of number of pustules and total score (based on erythema, scaling, and area involved) at baseline and at follow-up after 3 to 6 months in patients who stopped and did not stop smoking. VAS, Visual analog scale.