Correspondence
Non-Itchy Lichen Amyloidosus
We would like to stress the fact that the absence of pruritus may not lead to the exclusion of the diagnosis of LA, provided a given case fulfills the other criteria regarded as characteristic for this entity, Michael E, Kuligowski, M.D. Ann Chang, M.D, Nijmegen, The Netherlands
To the Editor: We read with interest the report about localized forms of tbe primary cutaneous amyloidosus. From tbeir group of 15 patients witb lichen amyloidosus (LA), four were free of pruritus. We find it important in this context to underline that LA need not be associated with itching, which is still regarded a cardinal clinical feature of this dermatosis.
Reference 1, Kibbi AG, Rubeiz NG, Zaynoun ST, Kurban AK, Primary localized cutaneous amyloidosis, Int J Dermatol 1992; 31:95-98.
Case Report A 50-year-old man presented with a history of symmetrical skin eruption located predominantly over the shins (Fig. 1) and in a lesser degree on the forearms and thighs. The first lesions had appeared approximately 30 years earlier and had been then slightly pruritic. The itch diminished gradually until it completely disappeared in the first months of the disease. Since then, the skin lesions remained unchanged and caused the patient no further inconvenience aside from esthetic problems, Histologic examination showed focally hyperkeratotic epidermis with hypergranulosis and elongated rete ridges. In the papillary dermis discrete homogeneous eosinophilic masses were observed, wbich gave a positive reaction in the Congo red staining and with the polarized light. Laboratory investigation including protein electropboresis, immuno-electrophoresis, urine screening for the BenceJones proteins, sternal bone marrow aspiration biopsy, and x-ray of the thorax revealed no abnormalities. Based upon the clinical and histologic findings, the diagnosis of LA was made. Consequently various therapeutic modalities (topically: corticosteroids under occlusion, tretinoin, dermabrasion and cryotherapy; and systemic: I'UVA and acitretin) were tried without success.
Pentoxifylline Treatment for Beta-Thalassemia Intermedia Leg Ulcer To the Editor: Ulcers of the leg are a well-recognized complication of thalassemia; they are recurrent and extremely resistant to treatment. Several factors may be involved in tbeir patbogenesis. Tbe abnormal shape of the red blood cells and the presence in them of inclusions may limit their deformability and thus hinder the normal blood flow in the capillaries,' These ulcers occur in tissues with a possible hypoxia from chronic anemia and where orthostatic pressure creates a slow return of blood,^ Patients with thalassemia intermedia may have large amounts of HbF, which has high affinity for oxygen and may also contribute to tissue hypoxia,' Pentoxifylline, a methylxanthine derivative, has been shown to affect the red cell membrane microrheologic pat-
Discussion The described case fulfills all of the clinicopathologic features required of LA except for the absence of itch. Because there was no associated pruritus the lichenification of the skin lesions cannot be convincingly ascribed to mechanical factors as scratching. We concur in this respect with the opinion of Kibbi et al, that the occurrence of pruritus in many, but certainly not all, cases of LA cannot be presently sufficiently explained.
Figure 1. Discrete confluent reddish-brown hard papules over the shins.
Figure 1. 747
Ulcer on the left internal malleolus.
International Journal of Dermatology Vol, 31, No, 10, October 1992
tern and improves the delivery of oxygen in ischemic tissues.^ Pentoxifylline has been proven to be useful in the treatment of venous,"* ischemic,-' and sickle cell anemia leg ulcers,^ On the basis of this experience, we have tried pentoxifylline in the treatment of a leg ulcer in a patient with beta-thalassemia intermedia.
There had been no improvement of the ulcer with bed rest, elevation of the feet, topical application of aluminium acetate solution, and topical fusidic acid. After pentoxifylline, 400 mg t,i,d,, witb bed rest was started, the ulcer became smaller, and 4 months later, it healed (Fig. 2). Six months later it is still cleared.
Case Report A 40-year-old woman with known beta-thalassemia intermedia since age 17, who had required blood transfusions on nine different occasions, bad presented five leg ulcers previously, and she had a painful leg ulcer for the last 4 years. The ulcer was located over the left internal malleolus, it was oval in shape, 8x6 cm, with sharp borders, and a reddisb base (Fig.l), Histopathology showed groups of small vessels with mixed inflammatory infiltrate in the superficial and mid-dermis. The lumina of the vessels were filled with fibrin and thrombi. Smears and cultures from the ulcer were negative. Laboratory findings: red blood cell count 3,700,000/mm'; hemoglobin, 7.0 g/100 mL; hematocrit, 24.5 %; Mcv, 66.2 fl; MCHb, 18.5 pg; MCHC, 27.2 %; reticulocytes, 8,7 %; HbAz, 5,5 %; HbAi, 84,5%; HbF, 10%, Blood smears showed anisocytosis, hypochromia, microcytosis, and polychromasia.
Comment The improvement of the ulcer by pentoxifylline in this patient might be induced by its several reported properties. The enhancement in oxygen delivery in ischemic tissues and the increase erythrocyte flexibility^ are very important actions of this drug. Moreover, the inhibition of the procoagulant activity generated by activated monocytes^ and the ability to reverse cytokine activation of polymorphonuclear leucocytes" may play a role in the healing of thalassemic ulcers , Although our experience is still short, we think oral pentoxifylline may be a useful therapy for leg ulcers in beta-thalassemia intermedia. Drug Name pentoxifylline: Trental Jesus Tercedor, M.D, M, Teresa Herranz, M,D. Jose M, Rodenas, M,D. Elena Garcia-Lora, M.D, Maria Skiljo, M,D, Valentin Garcia-Mellado, M,D, Granada, Spain References 1. Stevens DM, Shupack JL, Javid J, Silber R, Ulcers of the leg in thalassemia. Arch Dermatol 1977; 113:1558-1560, 2, Samitz MH, Walforf DS, Shrager J. Leg ulcers in Mediterranean anemia. Arch Dermatol 1964; 90:567-571, 3,
Caimi G, Serra A, Francavilla G, et al. Vascular atherosclerotic disease: evaluation of the whole-blood filterability and red cell membrane microrbeological pattern after intravenous administration of a single dose of pentoxifylline, Curr Med Res Opin 1990; 12:263-267,
4.
Colgan M-P, Dormandy JA, Jones PW, et al, Oxpentifylline treatment of venous ulcers of the leg, BMJ 1990; . 300:972-975. 5. Velanovich V, Fahey MJ. Treatment of ischemic leg ulcers with pentoxifylline: a case report and theoretical considerations, Ann Plast Surg 1990; 25:58-62, 6. Prost ML, Treadwell P, Treatment of sickle cell leg ulcers with pentoxifylline. Int J Dermatol 1990; 29: 375-376. 7, De Prost D, Ollivier V, Hakim J. Pentoxifylline inhibition of procoagulant activity generated by activated mononuclear phagocytes. Mol Pharmacol 1990; 38:562-566. Novick WJ Jr, Sullivan G, Mandell G. New pharmacological studies with pentoxifylline. Biorheology 1990; 27:449^54. .
Figure 2. Left internal malleolus 4 months after Figure 1 was obtained.
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Non-Itchy Lichen Amyloidosus
We would like to stress the fact that the absence of pruritus may not lead to the exclusion of the diagnosis of LA, provided a given case fulfills the other criteria regarded as characteristic for this entity, Michael E, Kuligowski, M.D. Ann Chang, M.D, Nijmegen, The Netherlands
To the Editor: We read with interest the report about localized forms of tbe primary cutaneous amyloidosus. From tbeir group of 15 patients witb lichen amyloidosus (LA), four were free of pruritus. We find it important in this context to underline that LA need not be associated with itching, which is still regarded a cardinal clinical feature of this dermatosis.
Reference 1, Kibbi AG, Rubeiz NG, Zaynoun ST, Kurban AK, Primary localized cutaneous amyloidosis, Int J Dermatol 1992; 31:95-98.
Case Report A 50-year-old man presented with a history of symmetrical skin eruption located predominantly over the shins (Fig. 1) and in a lesser degree on the forearms and thighs. The first lesions had appeared approximately 30 years earlier and had been then slightly pruritic. The itch diminished gradually until it completely disappeared in the first months of the disease. Since then, the skin lesions remained unchanged and caused the patient no further inconvenience aside from esthetic problems, Histologic examination showed focally hyperkeratotic epidermis with hypergranulosis and elongated rete ridges. In the papillary dermis discrete homogeneous eosinophilic masses were observed, wbich gave a positive reaction in the Congo red staining and with the polarized light. Laboratory investigation including protein electropboresis, immuno-electrophoresis, urine screening for the BenceJones proteins, sternal bone marrow aspiration biopsy, and x-ray of the thorax revealed no abnormalities. Based upon the clinical and histologic findings, the diagnosis of LA was made. Consequently various therapeutic modalities (topically: corticosteroids under occlusion, tretinoin, dermabrasion and cryotherapy; and systemic: I'UVA and acitretin) were tried without success.
Pentoxifylline Treatment for Beta-Thalassemia Intermedia Leg Ulcer To the Editor: Ulcers of the leg are a well-recognized complication of thalassemia; they are recurrent and extremely resistant to treatment. Several factors may be involved in tbeir patbogenesis. Tbe abnormal shape of the red blood cells and the presence in them of inclusions may limit their deformability and thus hinder the normal blood flow in the capillaries,' These ulcers occur in tissues with a possible hypoxia from chronic anemia and where orthostatic pressure creates a slow return of blood,^ Patients with thalassemia intermedia may have large amounts of HbF, which has high affinity for oxygen and may also contribute to tissue hypoxia,' Pentoxifylline, a methylxanthine derivative, has been shown to affect the red cell membrane microrheologic pat-
Discussion The described case fulfills all of the clinicopathologic features required of LA except for the absence of itch. Because there was no associated pruritus the lichenification of the skin lesions cannot be convincingly ascribed to mechanical factors as scratching. We concur in this respect with the opinion of Kibbi et al, that the occurrence of pruritus in many, but certainly not all, cases of LA cannot be presently sufficiently explained.
Figure 1. Discrete confluent reddish-brown hard papules over the shins.
Figure 1. 747
Ulcer on the left internal malleolus.
International Journal of Dermatology Vol, 31, No, 10, October 1992
tern and improves the delivery of oxygen in ischemic tissues.^ Pentoxifylline has been proven to be useful in the treatment of venous,"* ischemic,-' and sickle cell anemia leg ulcers,^ On the basis of this experience, we have tried pentoxifylline in the treatment of a leg ulcer in a patient with beta-thalassemia intermedia.
There had been no improvement of the ulcer with bed rest, elevation of the feet, topical application of aluminium acetate solution, and topical fusidic acid. After pentoxifylline, 400 mg t,i,d,, witb bed rest was started, the ulcer became smaller, and 4 months later, it healed (Fig. 2). Six months later it is still cleared.
Case Report A 40-year-old woman with known beta-thalassemia intermedia since age 17, who had required blood transfusions on nine different occasions, bad presented five leg ulcers previously, and she had a painful leg ulcer for the last 4 years. The ulcer was located over the left internal malleolus, it was oval in shape, 8x6 cm, with sharp borders, and a reddisb base (Fig.l), Histopathology showed groups of small vessels with mixed inflammatory infiltrate in the superficial and mid-dermis. The lumina of the vessels were filled with fibrin and thrombi. Smears and cultures from the ulcer were negative. Laboratory findings: red blood cell count 3,700,000/mm'; hemoglobin, 7.0 g/100 mL; hematocrit, 24.5 %; Mcv, 66.2 fl; MCHb, 18.5 pg; MCHC, 27.2 %; reticulocytes, 8,7 %; HbAz, 5,5 %; HbAi, 84,5%; HbF, 10%, Blood smears showed anisocytosis, hypochromia, microcytosis, and polychromasia.
Comment The improvement of the ulcer by pentoxifylline in this patient might be induced by its several reported properties. The enhancement in oxygen delivery in ischemic tissues and the increase erythrocyte flexibility^ are very important actions of this drug. Moreover, the inhibition of the procoagulant activity generated by activated monocytes^ and the ability to reverse cytokine activation of polymorphonuclear leucocytes" may play a role in the healing of thalassemic ulcers , Although our experience is still short, we think oral pentoxifylline may be a useful therapy for leg ulcers in beta-thalassemia intermedia. Drug Name pentoxifylline: Trental Jesus Tercedor, M.D, M, Teresa Herranz, M,D. Jose M, Rodenas, M,D. Elena Garcia-Lora, M.D, Maria Skiljo, M,D, Valentin Garcia-Mellado, M,D, Granada, Spain References 1. Stevens DM, Shupack JL, Javid J, Silber R, Ulcers of the leg in thalassemia. Arch Dermatol 1977; 113:1558-1560, 2, Samitz MH, Walforf DS, Shrager J. Leg ulcers in Mediterranean anemia. Arch Dermatol 1964; 90:567-571, 3,
Caimi G, Serra A, Francavilla G, et al. Vascular atherosclerotic disease: evaluation of the whole-blood filterability and red cell membrane microrbeological pattern after intravenous administration of a single dose of pentoxifylline, Curr Med Res Opin 1990; 12:263-267,
4.
Colgan M-P, Dormandy JA, Jones PW, et al, Oxpentifylline treatment of venous ulcers of the leg, BMJ 1990; . 300:972-975. 5. Velanovich V, Fahey MJ. Treatment of ischemic leg ulcers with pentoxifylline: a case report and theoretical considerations, Ann Plast Surg 1990; 25:58-62, 6. Prost ML, Treadwell P, Treatment of sickle cell leg ulcers with pentoxifylline. Int J Dermatol 1990; 29: 375-376. 7, De Prost D, Ollivier V, Hakim J. Pentoxifylline inhibition of procoagulant activity generated by activated mononuclear phagocytes. Mol Pharmacol 1990; 38:562-566. Novick WJ Jr, Sullivan G, Mandell G. New pharmacological studies with pentoxifylline. Biorheology 1990; 27:449^54. .
Figure 2. Left internal malleolus 4 months after Figure 1 was obtained.
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