Pigmentation Disorders

Pyogenic granuloma, often referred to by many other names, including lobular capillary hemangioma, is a benign vascular hyperplasia that tends to occur on the gingiva, lips, nasal mucosa, face, and hands.1,2 The lesion often is preceded by minor trauma, has a history of bleeding easily, and appears as a pedunculated or sessile papule with a rough red surface. There is a well-known tendency toward local recurrence after treatment.3 Results of a histopathologic examination shows lobular aggregates of capillaries and venules with plump endothelial cells.4 The pathogenesis of this lesion is poorly understood.

Port-wine stains are a type of nevus flammeus that present as a deep red or purple macule and are typically unilateral.4 Unlike salmon patches, the other type of nevus flammeus, port-wine stains tend to persist into adulthood.5 These congenital malformations are composed of capillary and venule ectasia in the dermis, and the ectasias progress over the course of a lifetime with increasing erythrocyte stasis.6-8 As Finley6 pointed out, in time, the port-wine stain can develop a thickened "cobblestone pattern," as well as nodules that are thought to be arteriovenous malformations or "localized tumor formations." Klapman and Yao8 recently noted that the thickening and nodules are most likely to occur in the areas of the face innervated by the second and third branches of the trigeminal nerve than in other parts of the body. Port-wine stains have been associated with other abnormalities such as nevus anemicus, glaucoma, choroidal angiomas, and Sturge-Weber syndrome.4 The pathogenesis of the port-wine stain has been a controversial issue, but one possibility is that it represents a deficiency of sympathetic innervation of blood vessels.4

The occurrence of vascular neoplasms arising in capillary malformations is a rare, but documented, event.9 Pyogenic granulomas infrequently have been reported to occur in association with other vascular abnormalities such as port-wine stains or hemangiomas, particularly after laser treatment.10,11 A number of other vascular neoplasms, such as capillary hemangiomas, tufted angiomas, and cavernous hemangiomas, have been reported in association with port-wine stains, as well.6,12,13 Very few pyogenic granulomas have arisen in port-wine stains without a history of trauma, laser treatment, or pregnancy. We now report a pyogenic granuloma occurring within a port-wine stain without a history of trauma or laser treatment in a man, and we contend that this occurrence offers unique insight into the pathogenesis of these poorly understood but commonly encountered entities. 

Case Report

A 35-year-old Asian man with a history of a port-wine stain on his left upper back presented for evaluation of a "mushroom" growing within his birthmark (Figure 1). He noted that some lesion had been present in this location for several months but that it had grown rapidly in the previous 3 weeks and begun to bleed easily. There was no history of trauma or treatment of the area. On physical examination, the patient had a 1.4-cm pedunculated, moist tumor with a rough surface within a port-wine stain with surrounding contact dermatitis from the bandaging he had used to protect the friable papule. Saucerization was performed, and the extremely vascularized base was cauterized. Results of histopathologic examination revealed lobules of vascular hyperplasia consistent with pyogenic granuloma (Figure 2). Vascular ectasia was seen below the pyogenic granuloma that represented the underling port-wine stain.

Comment

The Table documents the reported cases of pyogenic granulomas arising within port-wine stains.3,10,11,13-23 Many have occurred after laser treatment of port-wine stains.11,15-17,24,25 Several cases have occurred after other manipulation of port-wine stains, such as grenz-ray therapy15 or local trauma.20 Three cases occurred during pregnancy, which is a hormonal state that is known to predispose the patient to the formation of pyogenic granulomas, including one patient who previously was treated with the 577-nm pulsed dye laser and subsequently became pregnant.14,15,21 Holloway et al18 noted the development of a proliferation that had a superficial component of pyogenic granuloma and a deeper component of cavernous hemangioma. Other authors have seen the occasional association of port-wine stains and cavernous hemangiomas.6 Not surprisingly, pyogenic granulomas have occurred within port-wine stains in the setting of other syndromes, such as phacomatosis pigmentovascularis and Sturge-Weber syndrome.19

Only rarely does the pyogenic granuloma seem to emerge from the port-wine stain without a history of manipulation or pregnancy, as it did in the present case. When there is no history of predisposing factors, the presentation can be very alarming, simulating other processes such as amelanotic melanoma.22,23 Interestingly, as opposed to the thickening and nodules that have shown to be more likely to develop in port-wine stains in the distribution of the trigeminal nerve, most of the pyogenic granulomas that have arisen in port-wine stains did so outside of this distribution, occurring more often on the neck, trunk, or extremities.

Clearly, the co-occurrence of these 2 vascular abnormalities must offer some information on the pathogenesis of these poorly understood conditions. Several years ago, Swerlick and Cooper10 speculated that the co-occurrence supported the possibility of pyogenic granulomas developing at the sites of microscopic arteriovenous anastomoses. More recently, as discussed by Garzon et al,9 a number of studies indicate that abnormalities in embryonic vasculogenesis are involved in forming vascular malformations such as port-wine stains, and these same abnormalities may predispose patients toward forming vascular hyperplasia or tumors. The well-known hormonally driven vascular changes that happen during pregnancy also may be able to generate pyogenic granulomas in the setting of port-wine stains.21

On a basic science level, chemical mediators such as inducible nitric oxide synthase have been implicated in the formation of pyogenic granulomas, and mast cells have been found to be dramatically increased in the settings of both pyogenic granulomas and port-wine stains, though this may not be evidence of a cause-and-effect relationship.26,27 With regard to laser-induced pyogenic granulomas in port-wine stains, speculation has involved several factors including local tissue trauma in an area of increased microscopic arteriovenous anastomoses, retention of abnormal vasculature, and nonspecific laser interaction with tissue.15,16

Pyogenic granulomas tend to develop on the fingers, lips, face, and hands (places known to be rich in arteriovenous anastomoses), suggesting that this vascular phenomenon tends to occur with minor trauma in the setting of such anastomoses.13,20 This conclusion is consistent with the available data. The present case of a pyogenic granuloma arising without recognized trauma in a port-wine stain also supports this hypothesis.

Conclusion

Pyogenic granuloma is a well-known and probably underreported complication of port-wine stains. Unlike thickening and nodule formation that tend to occur in port-wine stains in the trigeminal nerve distribution, pyogenic granulomas arising in port-wine stains tend to occur more commonly on the neck, trunk, or extremities. The pyogenic granuloma should not be confused with a true malignancy because it represents hyperplasia of vascular tissue in response to trauma, but the performance of an excisional biopsy is warranted.

Pyogenic granuloma, often referred to by many other names, including lobular capillary hemangioma, is a benign vascular hyperplasia that tends to occur on the gingiva, lips, nasal mucosa, face, and hands.1,2 The lesion often is preceded by minor trauma, has a history of bleeding easily, and appears as a pedunculated or sessile papule with a rough red surface. There is a well-known tendency toward local recurrence after treatment.3 Results of a histopathologic examination shows lobular aggregates of capillaries and venules with plump endothelial cells.4 The pathogenesis of this lesion is poorly understood.

Port-wine stains are a type of nevus flammeus that present as a deep red or purple macule and are typically unilateral.4 Unlike salmon patches, the other type of nevus flammeus, port-wine stains tend to persist into adulthood.5 These congenital malformations are composed of capillary and venule ectasia in the dermis, and the ectasias progress over the course of a lifetime with increasing erythrocyte stasis.6-8 As Finley6 pointed out, in time, the port-wine stain can develop a thickened "cobblestone pattern," as well as nodules that are thought to be arteriovenous malformations or "localized tumor formations." Klapman and Yao8 recently noted that the thickening and nodules are most likely to occur in the areas of the face innervated by the second and third branches of the trigeminal nerve than in other parts of the body. Port-wine stains have been associated with other abnormalities such as nevus anemicus, glaucoma, choroidal angiomas, and Sturge-Weber syndrome.4 The pathogenesis of the port-wine stain has been a controversial issue, but one possibility is that it represents a deficiency of sympathetic innervation of blood vessels.4

The occurrence of vascular neoplasms arising in capillary malformations is a rare, but documented, event.9 Pyogenic granulomas infrequently have been reported to occur in association with other vascular abnormalities such as port-wine stains or hemangiomas, particularly after laser treatment.10,11 A number of other vascular neoplasms, such as capillary hemangiomas, tufted angiomas, and cavernous hemangiomas, have been reported in association with port-wine stains, as well.6,12,13 Very few pyogenic granulomas have arisen in port-wine stains without a history of trauma, laser treatment, or pregnancy. We now report a pyogenic granuloma occurring within a port-wine stain without a history of trauma or laser treatment in a man, and we contend that this occurrence offers unique insight into the pathogenesis of these poorly understood but commonly encountered entities. 

Case Report

A 35-year-old Asian man with a history of a port-wine stain on his left upper back presented for evaluation of a "mushroom" growing within his birthmark (Figure 1). He noted that some lesion had been present in this location for several months but that it had grown rapidly in the previous 3 weeks and begun to bleed easily. There was no history of trauma or treatment of the area. On physical examination, the patient had a 1.4-cm pedunculated, moist tumor with a rough surface within a port-wine stain with surrounding contact dermatitis from the bandaging he had used to protect the friable papule. Saucerization was performed, and the extremely vascularized base was cauterized. Results of histopathologic examination revealed lobules of vascular hyperplasia consistent with pyogenic granuloma (Figure 2). Vascular ectasia was seen below the pyogenic granuloma that represented the underling port-wine stain.

Comment

The Table documents the reported cases of pyogenic granulomas arising within port-wine stains.3,10,11,13-23 Many have occurred after laser treatment of port-wine stains.11,15-17,24,25 Several cases have occurred after other manipulation of port-wine stains, such as grenz-ray therapy15 or local trauma.20 Three cases occurred during pregnancy, which is a hormonal state that is known to predispose the patient to the formation of pyogenic granulomas, including one patient who previously was treated with the 577-nm pulsed dye laser and subsequently became pregnant.14,15,21 Holloway et al18 noted the development of a proliferation that had a superficial component of pyogenic granuloma and a deeper component of cavernous hemangioma. Other authors have seen the occasional association of port-wine stains and cavernous hemangiomas.6 Not surprisingly, pyogenic granulomas have occurred within port-wine stains in the setting of other syndromes, such as phacomatosis pigmentovascularis and Sturge-Weber syndrome.19

Only rarely does the pyogenic granuloma seem to emerge from the port-wine stain without a history of manipulation or pregnancy, as it did in the present case. When there is no history of predisposing factors, the presentation can be very alarming, simulating other processes such as amelanotic melanoma.22,23 Interestingly, as opposed to the thickening and nodules that have shown to be more likely to develop in port-wine stains in the distribution of the trigeminal nerve, most of the pyogenic granulomas that have arisen in port-wine stains did so outside of this distribution, occurring more often on the neck, trunk, or extremities.

Clearly, the co-occurrence of these 2 vascular abnormalities must offer some information on the pathogenesis of these poorly understood conditions. Several years ago, Swerlick and Cooper10 speculated that the co-occurrence supported the possibility of pyogenic granulomas developing at the sites of microscopic arteriovenous anastomoses. More recently, as discussed by Garzon et al,9 a number of studies indicate that abnormalities in embryonic vasculogenesis are involved in forming vascular malformations such as port-wine stains, and these same abnormalities may predispose patients toward forming vascular hyperplasia or tumors. The well-known hormonally driven vascular changes that happen during pregnancy also may be able to generate pyogenic granulomas in the setting of port-wine stains.21

On a basic science level, chemical mediators such as inducible nitric oxide synthase have been implicated in the formation of pyogenic granulomas, and mast cells have been found to be dramatically increased in the settings of both pyogenic granulomas and port-wine stains, though this may not be evidence of a cause-and-effect relationship.26,27 With regard to laser-induced pyogenic granulomas in port-wine stains, speculation has involved several factors including local tissue trauma in an area of increased microscopic arteriovenous anastomoses, retention of abnormal vasculature, and nonspecific laser interaction with tissue.15,16

Pyogenic granulomas tend to develop on the fingers, lips, face, and hands (places known to be rich in arteriovenous anastomoses), suggesting that this vascular phenomenon tends to occur with minor trauma in the setting of such anastomoses.13,20 This conclusion is consistent with the available data. The present case of a pyogenic granuloma arising without recognized trauma in a port-wine stain also supports this hypothesis.

Conclusion

Pyogenic granuloma is a well-known and probably underreported complication of port-wine stains. Unlike thickening and nodule formation that tend to occur in port-wine stains in the trigeminal nerve distribution, pyogenic granulomas arising in port-wine stains tend to occur more commonly on the neck, trunk, or extremities. The pyogenic granuloma should not be confused with a true malignancy because it represents hyperplasia of vascular tissue in response to trauma, but the performance of an excisional biopsy is warranted.

Pyogenic granuloma, often referred to by many other names, including lobular capillary hemangioma, is a benign vascular hyperplasia that tends to occur on the gingiva, lips, nasal mucosa, face, and hands.1,2 The lesion often is preceded by minor trauma, has a history of bleeding easily, and appears as a pedunculated or sessile papule with a rough red surface. There is a well-known tendency toward local recurrence after treatment.3 Results of a histopathologic examination shows lobular aggregates of capillaries and venules with plump endothelial cells.4 The pathogenesis of this lesion is poorly understood.

Port-wine stains are a type of nevus flammeus that present as a deep red or purple macule and are typically unilateral.4 Unlike salmon patches, the other type of nevus flammeus, port-wine stains tend to persist into adulthood.5 These congenital malformations are composed of capillary and venule ectasia in the dermis, and the ectasias progress over the course of a lifetime with increasing erythrocyte stasis.6-8 As Finley6 pointed out, in time, the port-wine stain can develop a thickened "cobblestone pattern," as well as nodules that are thought to be arteriovenous malformations or "localized tumor formations." Klapman and Yao8 recently noted that the thickening and nodules are most likely to occur in the areas of the face innervated by the second and third branches of the trigeminal nerve than in other parts of the body. Port-wine stains have been associated with other abnormalities such as nevus anemicus, glaucoma, choroidal angiomas, and Sturge-Weber syndrome.4 The pathogenesis of the port-wine stain has been a controversial issue, but one possibility is that it represents a deficiency of sympathetic innervation of blood vessels.4

The occurrence of vascular neoplasms arising in capillary malformations is a rare, but documented, event.9 Pyogenic granulomas infrequently have been reported to occur in association with other vascular abnormalities such as port-wine stains or hemangiomas, particularly after laser treatment.10,11 A number of other vascular neoplasms, such as capillary hemangiomas, tufted angiomas, and cavernous hemangiomas, have been reported in association with port-wine stains, as well.6,12,13 Very few pyogenic granulomas have arisen in port-wine stains without a history of trauma, laser treatment, or pregnancy. We now report a pyogenic granuloma occurring within a port-wine stain without a history of trauma or laser treatment in a man, and we contend that this occurrence offers unique insight into the pathogenesis of these poorly understood but commonly encountered entities. 

Case Report

A 35-year-old Asian man with a history of a port-wine stain on his left upper back presented for evaluation of a "mushroom" growing within his birthmark (Figure 1). He noted that some lesion had been present in this location for several months but that it had grown rapidly in the previous 3 weeks and begun to bleed easily. There was no history of trauma or treatment of the area. On physical examination, the patient had a 1.4-cm pedunculated, moist tumor with a rough surface within a port-wine stain with surrounding contact dermatitis from the bandaging he had used to protect the friable papule. Saucerization was performed, and the extremely vascularized base was cauterized. Results of histopathologic examination revealed lobules of vascular hyperplasia consistent with pyogenic granuloma (Figure 2). Vascular ectasia was seen below the pyogenic granuloma that represented the underling port-wine stain.

Comment

The Table documents the reported cases of pyogenic granulomas arising within port-wine stains.3,10,11,13-23 Many have occurred after laser treatment of port-wine stains.11,15-17,24,25 Several cases have occurred after other manipulation of port-wine stains, such as grenz-ray therapy15 or local trauma.20 Three cases occurred during pregnancy, which is a hormonal state that is known to predispose the patient to the formation of pyogenic granulomas, including one patient who previously was treated with the 577-nm pulsed dye laser and subsequently became pregnant.14,15,21 Holloway et al18 noted the development of a proliferation that had a superficial component of pyogenic granuloma and a deeper component of cavernous hemangioma. Other authors have seen the occasional association of port-wine stains and cavernous hemangiomas.6 Not surprisingly, pyogenic granulomas have occurred within port-wine stains in the setting of other syndromes, such as phacomatosis pigmentovascularis and Sturge-Weber syndrome.19

Only rarely does the pyogenic granuloma seem to emerge from the port-wine stain without a history of manipulation or pregnancy, as it did in the present case. When there is no history of predisposing factors, the presentation can be very alarming, simulating other processes such as amelanotic melanoma.22,23 Interestingly, as opposed to the thickening and nodules that have shown to be more likely to develop in port-wine stains in the distribution of the trigeminal nerve, most of the pyogenic granulomas that have arisen in port-wine stains did so outside of this distribution, occurring more often on the neck, trunk, or extremities.

Clearly, the co-occurrence of these 2 vascular abnormalities must offer some information on the pathogenesis of these poorly understood conditions. Several years ago, Swerlick and Cooper10 speculated that the co-occurrence supported the possibility of pyogenic granulomas developing at the sites of microscopic arteriovenous anastomoses. More recently, as discussed by Garzon et al,9 a number of studies indicate that abnormalities in embryonic vasculogenesis are involved in forming vascular malformations such as port-wine stains, and these same abnormalities may predispose patients toward forming vascular hyperplasia or tumors. The well-known hormonally driven vascular changes that happen during pregnancy also may be able to generate pyogenic granulomas in the setting of port-wine stains.21

On a basic science level, chemical mediators such as inducible nitric oxide synthase have been implicated in the formation of pyogenic granulomas, and mast cells have been found to be dramatically increased in the settings of both pyogenic granulomas and port-wine stains, though this may not be evidence of a cause-and-effect relationship.26,27 With regard to laser-induced pyogenic granulomas in port-wine stains, speculation has involved several factors including local tissue trauma in an area of increased microscopic arteriovenous anastomoses, retention of abnormal vasculature, and nonspecific laser interaction with tissue.15,16

Pyogenic granulomas tend to develop on the fingers, lips, face, and hands (places known to be rich in arteriovenous anastomoses), suggesting that this vascular phenomenon tends to occur with minor trauma in the setting of such anastomoses.13,20 This conclusion is consistent with the available data. The present case of a pyogenic granuloma arising without recognized trauma in a port-wine stain also supports this hypothesis.

Conclusion

Pyogenic granuloma is a well-known and probably underreported complication of port-wine stains. Unlike thickening and nodule formation that tend to occur in port-wine stains in the trigeminal nerve distribution, pyogenic granulomas arising in port-wine stains tend to occur more commonly on the neck, trunk, or extremities. The pyogenic granuloma should not be confused with a true malignancy because it represents hyperplasia of vascular tissue in response to trauma, but the performance of an excisional biopsy is warranted.

Lichen planus is a papulosquamous inflammatory dermatitis that affects less than 1% of the population. Classic lichen planus typically presents as erythematous to violaceous flat-topped papules that are pruritic and polygonal. The lesions usually involve the flexural areas of the arms, legs, trunk, and lower back._¹ Multiple clinical variants of lichen planus have been described.¹ We report a case of lichen planus actinicus, a subtype of lichen planus that characteristically affects sun-exposed areas of the face, forearms, and dorsa of the hands in individuals with dark complexions living in subtropical climates.^2-8

Case Report

A 55-year-old Indian man presented with a 7-year history of asymptomatic hyperpigmentation on his face, neck, and forearms. The patient had tried treating the condition with hydroquinone preparations and topical steroids without improvement in the pigmentation. He had no other medical problems and was not taking any systemic medications.

On physical examination, there were hyperpigmented macules and patches on his forehead (Figure 1), nose, cheeks, and chin. He also had thin, flat-topped, hyperpigmented papules on the dorsal surfaces of his forearms and hands (Figure 2). All areas of hyperpigmentation were in areas of sun exposure. He had no associated mucous membrane or nail involvement. Histologic examination demonstrated an atrophic epidermis with mild hyperkeratosis and orthokeratosis (Figure 3). Basilar vacuolization, a bandlike lymphocytic infiltrate, and marked pigmentary incontinence were present. A diagnosis of lichen planus actinicus was made.

Please refer to the PDF to view the figures

The patient was treated with topical tacrolimus 0.1% ointment and oral acitretin 25 mg daily for 2 months. The acitretin was discontinued because of lack of improvement and unacceptable side effects (dryness of skin, peeling of hands, and scalp hair loss). He was then started on hydroxychloroquine 400 mg daily with improvement (decreased thickening and pigmentation) after one month of therapy. 

Comment

Lichen planus actinicus is a photodistributed variant of lichen planus that most often occurs in dark-complexioned young adults of Middle Eastern descent.^3,8-10 Other names for this disorder include: summertime actinic lichenoid eruption,^2,6 lichen planus atrophicus annularis,² lichen planus in subtropical countries,^3,4 lichen planus subtropicus,⁵ lichen planus tropicus, and lichenoid melanodermatitis.⁷ Although the etiology of this disease has not been determined, sunlight appears to be the triggering factor in most cases.^2-11 Lichen planus actinicus has been induced with artificial UV light sources. In one patient, repeated doses of UVB radiation induced lichen planus–like lesions, whereas UVA radiation did not invoke this cutaneous response.¹² Subtropical climates and nutritional deficiencies also may be contributing factors.²

The lesions of lichen planus actinicus usually occur on the forehead, face, neck, and the extensor surfaces of the arms and hands. The lateral aspect of the forehead is the most common site of presentation.⁵ Several morphologic patterns have been reported including annular hyperpigmented plaques, melasmalike patches, dyschromic papules, and classic lichenoid papules/plaques (Table 1).^2,3,10,11 The lesions often are exacerbated by sun exposure and may improve spontaneously in the winter months.^3,4 Rarely, the nonexposed skin of the legs, trunk, genitals, and oral mucosa may be affected.^3,4 Unlike classic lichen planus, lichen planus actinicus has an earlier age of onset, a longer course, a predilection for dark-complexioned females, and a seasonal occurrence.^2-4 Additionally, pruritus, scaling, nail involvement, and the Köbner reaction frequently are absent (Table 2).^2-4

Please refer to the PDF to view the tables

Differential diagnosis of lichen planus actinicus includes discoid lupus erythematosus, granuloma annulare, melasma, secondary syphilis, fixed drug eruption, polymorphous light eruption, and erythema dyschronicum perstans.⁵ The clinician also must exclude the possibility of a drug-induced photosensitive lichenoid eruption by taking a complete medication history.

The histologic features of lichen planus actinicus are consistent with findings in classic lichen planus.^2-5 Hyperkeratosis of the stratum corneum, hypergranulosis, basal cell vacuolization, and Civatte bodies usually are present.¹⁰ The epidermis may demonstrate either sawtoothed hyperplasia or atrophy. Numerous melanophages, marked pigmentary incontinence, and a bandlike lymphocytic inflammatory infiltrate also are observed in the papillary dermis.^2-4,7,10 Although clinically similar to cutaneous lupus and polymorphous light eruption, lichen planus actinicus can be distinguished histologically from these photodermatoses. Lichen planus actinicus lacks follicular plugging, thickening of the basement membrane, and the periadnexal inflammatory infiltrate seen in cutaneous lupus erythematosus.² Papillary dermal edema, which is present in polymorphous light eruption, is not observed.² The lupus erythematosus–lichen planus overlap syndrome and lichen planus actinicus may be similar histologically, but they can be easily distinguished clinically. Unlike lichen planus actinicus, the lupus erythematosus–lichen planus overlap syndrome features ulcerated, atrophic, violaceous plaques with telangiectasias on the palms and soles.^1,2

Variable treatment responses have been reported with bismuth, grenz rays, arsenic compounds, and topical corticosteroids under occlusion.^4,6 Hydroxychloroquine, intralesional corticosteroids, and topical sunscreens have been used with success.^5,8 Acitretin, used in combination with topical steroids and sun avoidance, also has resulted in complete resolution of lesions without recurrence.¹³ Although psoralen-UVA, isotretinoin, systemic corticosteroids, cyclosporine, and dapsone have been used to treat classic lichen planus, there are currently no reports of their use in lichen planus actinicus.¹⁴ Some cases may remit spontaneously with sun avoidance and use of sunblock.⁶ 

Lichen planus is a papulosquamous inflammatory dermatitis that affects less than 1% of the population. Classic lichen planus typically presents as erythematous to violaceous flat-topped papules that are pruritic and polygonal. The lesions usually involve the flexural areas of the arms, legs, trunk, and lower back._¹ Multiple clinical variants of lichen planus have been described.¹ We report a case of lichen planus actinicus, a subtype of lichen planus that characteristically affects sun-exposed areas of the face, forearms, and dorsa of the hands in individuals with dark complexions living in subtropical climates.^2-8

Case Report

A 55-year-old Indian man presented with a 7-year history of asymptomatic hyperpigmentation on his face, neck, and forearms. The patient had tried treating the condition with hydroquinone preparations and topical steroids without improvement in the pigmentation. He had no other medical problems and was not taking any systemic medications.

On physical examination, there were hyperpigmented macules and patches on his forehead (Figure 1), nose, cheeks, and chin. He also had thin, flat-topped, hyperpigmented papules on the dorsal surfaces of his forearms and hands (Figure 2). All areas of hyperpigmentation were in areas of sun exposure. He had no associated mucous membrane or nail involvement. Histologic examination demonstrated an atrophic epidermis with mild hyperkeratosis and orthokeratosis (Figure 3). Basilar vacuolization, a bandlike lymphocytic infiltrate, and marked pigmentary incontinence were present. A diagnosis of lichen planus actinicus was made.

Please refer to the PDF to view the figures

The patient was treated with topical tacrolimus 0.1% ointment and oral acitretin 25 mg daily for 2 months. The acitretin was discontinued because of lack of improvement and unacceptable side effects (dryness of skin, peeling of hands, and scalp hair loss). He was then started on hydroxychloroquine 400 mg daily with improvement (decreased thickening and pigmentation) after one month of therapy. 

Comment

Lichen planus actinicus is a photodistributed variant of lichen planus that most often occurs in dark-complexioned young adults of Middle Eastern descent.^3,8-10 Other names for this disorder include: summertime actinic lichenoid eruption,^2,6 lichen planus atrophicus annularis,² lichen planus in subtropical countries,^3,4 lichen planus subtropicus,⁵ lichen planus tropicus, and lichenoid melanodermatitis.⁷ Although the etiology of this disease has not been determined, sunlight appears to be the triggering factor in most cases.^2-11 Lichen planus actinicus has been induced with artificial UV light sources. In one patient, repeated doses of UVB radiation induced lichen planus–like lesions, whereas UVA radiation did not invoke this cutaneous response.¹² Subtropical climates and nutritional deficiencies also may be contributing factors.²

The lesions of lichen planus actinicus usually occur on the forehead, face, neck, and the extensor surfaces of the arms and hands. The lateral aspect of the forehead is the most common site of presentation.⁵ Several morphologic patterns have been reported including annular hyperpigmented plaques, melasmalike patches, dyschromic papules, and classic lichenoid papules/plaques (Table 1).^2,3,10,11 The lesions often are exacerbated by sun exposure and may improve spontaneously in the winter months.^3,4 Rarely, the nonexposed skin of the legs, trunk, genitals, and oral mucosa may be affected.^3,4 Unlike classic lichen planus, lichen planus actinicus has an earlier age of onset, a longer course, a predilection for dark-complexioned females, and a seasonal occurrence.^2-4 Additionally, pruritus, scaling, nail involvement, and the Köbner reaction frequently are absent (Table 2).^2-4

Please refer to the PDF to view the tables

Differential diagnosis of lichen planus actinicus includes discoid lupus erythematosus, granuloma annulare, melasma, secondary syphilis, fixed drug eruption, polymorphous light eruption, and erythema dyschronicum perstans.⁵ The clinician also must exclude the possibility of a drug-induced photosensitive lichenoid eruption by taking a complete medication history.

The histologic features of lichen planus actinicus are consistent with findings in classic lichen planus.^2-5 Hyperkeratosis of the stratum corneum, hypergranulosis, basal cell vacuolization, and Civatte bodies usually are present.¹⁰ The epidermis may demonstrate either sawtoothed hyperplasia or atrophy. Numerous melanophages, marked pigmentary incontinence, and a bandlike lymphocytic inflammatory infiltrate also are observed in the papillary dermis.^2-4,7,10 Although clinically similar to cutaneous lupus and polymorphous light eruption, lichen planus actinicus can be distinguished histologically from these photodermatoses. Lichen planus actinicus lacks follicular plugging, thickening of the basement membrane, and the periadnexal inflammatory infiltrate seen in cutaneous lupus erythematosus.² Papillary dermal edema, which is present in polymorphous light eruption, is not observed.² The lupus erythematosus–lichen planus overlap syndrome and lichen planus actinicus may be similar histologically, but they can be easily distinguished clinically. Unlike lichen planus actinicus, the lupus erythematosus–lichen planus overlap syndrome features ulcerated, atrophic, violaceous plaques with telangiectasias on the palms and soles.^1,2

Variable treatment responses have been reported with bismuth, grenz rays, arsenic compounds, and topical corticosteroids under occlusion.^4,6 Hydroxychloroquine, intralesional corticosteroids, and topical sunscreens have been used with success.^5,8 Acitretin, used in combination with topical steroids and sun avoidance, also has resulted in complete resolution of lesions without recurrence.¹³ Although psoralen-UVA, isotretinoin, systemic corticosteroids, cyclosporine, and dapsone have been used to treat classic lichen planus, there are currently no reports of their use in lichen planus actinicus.¹⁴ Some cases may remit spontaneously with sun avoidance and use of sunblock.⁶ 

Lichen planus is a papulosquamous inflammatory dermatitis that affects less than 1% of the population. Classic lichen planus typically presents as erythematous to violaceous flat-topped papules that are pruritic and polygonal. The lesions usually involve the flexural areas of the arms, legs, trunk, and lower back._¹ Multiple clinical variants of lichen planus have been described.¹ We report a case of lichen planus actinicus, a subtype of lichen planus that characteristically affects sun-exposed areas of the face, forearms, and dorsa of the hands in individuals with dark complexions living in subtropical climates.^2-8

Case Report

A 55-year-old Indian man presented with a 7-year history of asymptomatic hyperpigmentation on his face, neck, and forearms. The patient had tried treating the condition with hydroquinone preparations and topical steroids without improvement in the pigmentation. He had no other medical problems and was not taking any systemic medications.

On physical examination, there were hyperpigmented macules and patches on his forehead (Figure 1), nose, cheeks, and chin. He also had thin, flat-topped, hyperpigmented papules on the dorsal surfaces of his forearms and hands (Figure 2). All areas of hyperpigmentation were in areas of sun exposure. He had no associated mucous membrane or nail involvement. Histologic examination demonstrated an atrophic epidermis with mild hyperkeratosis and orthokeratosis (Figure 3). Basilar vacuolization, a bandlike lymphocytic infiltrate, and marked pigmentary incontinence were present. A diagnosis of lichen planus actinicus was made.

Please refer to the PDF to view the figures

The patient was treated with topical tacrolimus 0.1% ointment and oral acitretin 25 mg daily for 2 months. The acitretin was discontinued because of lack of improvement and unacceptable side effects (dryness of skin, peeling of hands, and scalp hair loss). He was then started on hydroxychloroquine 400 mg daily with improvement (decreased thickening and pigmentation) after one month of therapy. 

Comment

Lichen planus actinicus is a photodistributed variant of lichen planus that most often occurs in dark-complexioned young adults of Middle Eastern descent.^3,8-10 Other names for this disorder include: summertime actinic lichenoid eruption,^2,6 lichen planus atrophicus annularis,² lichen planus in subtropical countries,^3,4 lichen planus subtropicus,⁵ lichen planus tropicus, and lichenoid melanodermatitis.⁷ Although the etiology of this disease has not been determined, sunlight appears to be the triggering factor in most cases.^2-11 Lichen planus actinicus has been induced with artificial UV light sources. In one patient, repeated doses of UVB radiation induced lichen planus–like lesions, whereas UVA radiation did not invoke this cutaneous response.¹² Subtropical climates and nutritional deficiencies also may be contributing factors.²

The lesions of lichen planus actinicus usually occur on the forehead, face, neck, and the extensor surfaces of the arms and hands. The lateral aspect of the forehead is the most common site of presentation.⁵ Several morphologic patterns have been reported including annular hyperpigmented plaques, melasmalike patches, dyschromic papules, and classic lichenoid papules/plaques (Table 1).^2,3,10,11 The lesions often are exacerbated by sun exposure and may improve spontaneously in the winter months.^3,4 Rarely, the nonexposed skin of the legs, trunk, genitals, and oral mucosa may be affected.^3,4 Unlike classic lichen planus, lichen planus actinicus has an earlier age of onset, a longer course, a predilection for dark-complexioned females, and a seasonal occurrence.^2-4 Additionally, pruritus, scaling, nail involvement, and the Köbner reaction frequently are absent (Table 2).^2-4

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Differential diagnosis of lichen planus actinicus includes discoid lupus erythematosus, granuloma annulare, melasma, secondary syphilis, fixed drug eruption, polymorphous light eruption, and erythema dyschronicum perstans.⁵ The clinician also must exclude the possibility of a drug-induced photosensitive lichenoid eruption by taking a complete medication history.

The histologic features of lichen planus actinicus are consistent with findings in classic lichen planus.^2-5 Hyperkeratosis of the stratum corneum, hypergranulosis, basal cell vacuolization, and Civatte bodies usually are present.¹⁰ The epidermis may demonstrate either sawtoothed hyperplasia or atrophy. Numerous melanophages, marked pigmentary incontinence, and a bandlike lymphocytic inflammatory infiltrate also are observed in the papillary dermis.^2-4,7,10 Although clinically similar to cutaneous lupus and polymorphous light eruption, lichen planus actinicus can be distinguished histologically from these photodermatoses. Lichen planus actinicus lacks follicular plugging, thickening of the basement membrane, and the periadnexal inflammatory infiltrate seen in cutaneous lupus erythematosus.² Papillary dermal edema, which is present in polymorphous light eruption, is not observed.² The lupus erythematosus–lichen planus overlap syndrome and lichen planus actinicus may be similar histologically, but they can be easily distinguished clinically. Unlike lichen planus actinicus, the lupus erythematosus–lichen planus overlap syndrome features ulcerated, atrophic, violaceous plaques with telangiectasias on the palms and soles.^1,2

Variable treatment responses have been reported with bismuth, grenz rays, arsenic compounds, and topical corticosteroids under occlusion.^4,6 Hydroxychloroquine, intralesional corticosteroids, and topical sunscreens have been used with success.^5,8 Acitretin, used in combination with topical steroids and sun avoidance, also has resulted in complete resolution of lesions without recurrence.¹³ Although psoralen-UVA, isotretinoin, systemic corticosteroids, cyclosporine, and dapsone have been used to treat classic lichen planus, there are currently no reports of their use in lichen planus actinicus.¹⁴ Some cases may remit spontaneously with sun avoidance and use of sunblock.⁶ 

We describe an interesting case of a man with recurrent cutaneous and hematologic manifestations of vitamin B12 deficiency. In this deficiency, the skin, central nervous system, blood, and blood-forming tissues are commonly involved. We describe an overview of vitamin B12 deficiency and the successful treatment of a patient’s ongoing cutaneous hyperpigmentation.

We describe an interesting case of a man with recurrent cutaneous and hematologic manifestations of vitamin B12 deficiency. In this deficiency, the skin, central nervous system, blood, and blood-forming tissues are commonly involved. We describe an overview of vitamin B12 deficiency and the successful treatment of a patient’s ongoing cutaneous hyperpigmentation.

We describe an interesting case of a man with recurrent cutaneous and hematologic manifestations of vitamin B12 deficiency. In this deficiency, the skin, central nervous system, blood, and blood-forming tissues are commonly involved. We describe an overview of vitamin B12 deficiency and the successful treatment of a patient’s ongoing cutaneous hyperpigmentation.