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.
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.
