Generalized purpuric lichen nitidus. Report of a case and review of the literature
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Generalized purpuric lichen nitidus. Report of a case and review of the literatureDepartment of Dermatology1 and Histopathology2, Army General Hospital, Athens, Greece. email@example.com
Efstathios Rallis MD1, Constantinos Verros MD1, Vassiliki Moussatou MD1, Dimitrios Sambaziotis MD2, Pavlos Papadakis MD1
Dermatology Online Journal 13 (2): 5
Generalized purpuric lichen nitidus is an extremely rare form of lichen nitidus. We present the third documented case of this uncommon dermatosis, which was treated successfully with orally administered cyclosporine. During a 12-month follow-up after the cessation of the drug, no recurrence was noticed.
Lichen nitidus is an uncommon, chronic dermatosis of unknown origin. It is usually asymptomatic and clinically characterized by the presence of pin-point to pin-head-sized, skin-colored papules with a flat, shiny surface . The disorder is often localized, and only a few cases of generalized lichen nitidus have been reported [2, 3].
Purpuric lichen nitidus seems to represent a rare variant of lichen nitidus with a red-brown appearance of the papules due to underlying hemorrhage within the lesions. The generalized form of this entity is considered unusual . To our knowledge we present the third documented case of generalized purpuric lichen nitidus. The patient responded sufficiently to oral administration of cyclosporine.
A 42-year-old man was referred to our clinic because of the appearance of a 22-month history of a progressive, pruritic, papular eruption. At first, the lesions were located on the ankle and the distal third of the legs with symmetric distribution (Figs. 1,2). During the next 9 months, the lesions extended to the thighs, groins, genitalia, abdomen, and trunk; later they became generalized (Figs. 3,4,5).
The lesions were monomorphic, pruritic papules with a flat, shiny red surface that did not blanch with diascopy. They were bright red to orange in color, each 1-2mm in diameter tending to form plaques (Fig. 6). The papular lesions had showed purpura from the time of onset of the eruption. The lesions were most densely clustered on the feet, ankles, lateral sides of abdomen and forearms. The face, nails and mucosa were spared. The patient's past history and family history were unremarkable. Laboratory tests, including hepatitis-C virus antibodies, revealed no abnormalities.
|Figure 1||Figure 2|
|Figures 1,2. Pruritic, monomorphic papules were seen on the ankles and the distal third of the legs with symmetric distribution.|
|Figure 3||Figure 4|
|Figures 3 and 4. Pruritic, purpuric papules extended to knees (3), thighs (4)|
|Figure 5||Figure 5|
|Figure 5. Involvement of the forearms|
|Figure 6. Bright red to orange, pin-head sized papules, 1-2 mm in diameter each, most densely clustered on the feet.|
Three punch skin biopsies were taken from three different lesions on the dorsum of the left foot, abdomen, and waist. Histological examination revealed a dense, inflammatory infiltrate of the upper dermis. The infiltrate consisted of lymphocytes and epithelioid histiocytes. Mild focal subepidermal hemorrhages were observed in two of the three biopsy specimens. The overlying epidermis showed acanthosis, exocytosis and basal cell vacuolization. Mild elongated rete ridges but no hemosiderin accumulation or hyalinization of the capillary walls were seen. These findings were compatible with purpuric lichen nitidus (Figs. 7-12).
|Figure 7||Figure 8|
|Figure 9||Figure 10|
|Figure 11||Figure 12|
|Biopsy specimen revealed mild focal subepidermal hemorrhages and infiltration of lymphocytes and epithelioid histiocytes. (Hematoxylin - eosin stain; original magnification x 40).|
The treatment was initiated with topical corticosteroids and methylprednisolone 40 mg for 1 month without any significant response. Acitretin 35 mg /day was added and systemic corticosteroids were tapered off, but no improvement was noticed after 2 months of treatment. The administration of cyclosporine at a dose of 4 mg/kg proved quite effective. During the first month of treatment a remarkable improvement was observed and complete resolution of the eruption was seen by the end of the second month. The medication was tapered off gradually after 4 months of continuous administration. In a follow-up of 12 months after the cessation of the drug, no recurrence was noticed.
Generalized purpuric lichen nitidus is a rare form of purpuric lichen nitidus, an unusual variant of lichen nitidus. Only two cases of generalized purpuric lichen nitidus have been documented in the literature [4, 5]. However, as Yanez et al. have mentioned, Michelson in 1931—in a paper with no illustrations—described a third case of clinically pigmented purpuric dermatosis with a microscopic picture of lichen nitidus .
In all reported cases including ours, the purpuric lesions initially developed on the lower legs of the patients (dorsa of feet, ankles, distal thirds of legs) simulating pigmented purpuric dermatosis. New papules developed and the eruption generalized during the subsequent months. There is no obvious explanation for this evolution of the eruption but probably represents a characteristic clinical feature of the disease.
The histopathologic findings described in all cases of generalized purpuric lichen nitidus has been that of lichen nitidus with the additional presence of subepidermal hemorrhages. Krook stated that the purpuric nature of the lesions developed due to stasis of the upper arms and caused hemorrhage in the papules of the forearm and palm [5, 7]. Coulson et al. described the appearance of purpuric lesions on the palms of a patient supporting Krook's case [7, 8]. Jetton et al. reported a case of vesicular and hemorrhagic lichen nitidus and suggested that scratching or rubbing changed some papular lesions to small hemorrhagic papulovesicles after several hours [7, 9]. Yanez et al. attributed these subepidermal hemorrhages to the fragility of the capillaries as determined by hyalinization of their walls . Endo et al. showed the same histological features; changes were clearly observed in the blood vessels of the lesions suggesting the term purpuric lichen nitidus .
Cyclosporine is a well-known immunosuppressive drug that is proven effective for the treatment of autoimmune diseases of the skin such as psoriasis, alopecia areata, and autoimmune bullous diseases [11, 12, 13]. It has been used with some success in lichen planus and proved beneficial in our case. To our knowledge this is the first case of successful administration of cyclosporine in purpuric lichen nitidus.
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