Photo Challenge

The Diagnosis: Prayer Callus

After our patient demonstrated his routine position for prayer (Figure), the

The position of the patient’s left foot while praying.

diagnosis of prayer callus was confirmed. We suggested he use a foam pad under his foot during prayer. The patient did not return for follow-up.

Prayer calluses have been documented in the literature as prayer nodules^1-3 or marks.⁴ Callus is a preferred term, as it implies the act of repeated pressure or friction to an area of the skin. It is more descriptive than marks and avoids the inclusion of nodules caused by different prayer or religious activities such as ritual scarring.⁵

Abanmi et al⁴ studied a large group (N=349) of Muslim patients with regular praying habits and found a high prevalence of what they referred to as prayer marks, defined by lichenification and/or hyperpigmentation, on the knees, forehead, ankles, and dorsal aspects of the feet. They studied the histopathology of 33 marks. No statistical analysis was performed. They reported common histologic findings of compact orthokeratosis, hypergranulosis, dermal papillary fibrosis, and dermal vascularization. In contrast to lichen simplex chronicus, the authors found that the dermal fibrosis did not exhibit collagen bundles perpendicular to the epidermis.⁴

This study found that a higher prevalence of lichenification was observed on the left foot (males, 57%; females, 39%) than the right foot (males, 14%; females, 19%), which was attributed to a more typical prayer position that placed pressure on the left foot.⁴ Our patient only had calluses on his left foot, which was consistent with the prayer position.

Treatment options for prayer calluses include debridement, either mechanical or with topical keratolytic preparations. There is a high likelihood of recurrence if prayer practices are not changed. Optimally, more definitive treatment, which can be combined with initial debridement, would be position adjustment to lessen pressure or friction to the area or protection over the area with a cushion, as we attempted with our patient in the form of a foam pad.

The Diagnosis: Prayer Callus

After our patient demonstrated his routine position for prayer (Figure), the

The position of the patient’s left foot while praying.

diagnosis of prayer callus was confirmed. We suggested he use a foam pad under his foot during prayer. The patient did not return for follow-up.

Prayer calluses have been documented in the literature as prayer nodules^1-3 or marks.⁴ Callus is a preferred term, as it implies the act of repeated pressure or friction to an area of the skin. It is more descriptive than marks and avoids the inclusion of nodules caused by different prayer or religious activities such as ritual scarring.⁵

Abanmi et al⁴ studied a large group (N=349) of Muslim patients with regular praying habits and found a high prevalence of what they referred to as prayer marks, defined by lichenification and/or hyperpigmentation, on the knees, forehead, ankles, and dorsal aspects of the feet. They studied the histopathology of 33 marks. No statistical analysis was performed. They reported common histologic findings of compact orthokeratosis, hypergranulosis, dermal papillary fibrosis, and dermal vascularization. In contrast to lichen simplex chronicus, the authors found that the dermal fibrosis did not exhibit collagen bundles perpendicular to the epidermis.⁴

This study found that a higher prevalence of lichenification was observed on the left foot (males, 57%; females, 39%) than the right foot (males, 14%; females, 19%), which was attributed to a more typical prayer position that placed pressure on the left foot.⁴ Our patient only had calluses on his left foot, which was consistent with the prayer position.

Treatment options for prayer calluses include debridement, either mechanical or with topical keratolytic preparations. There is a high likelihood of recurrence if prayer practices are not changed. Optimally, more definitive treatment, which can be combined with initial debridement, would be position adjustment to lessen pressure or friction to the area or protection over the area with a cushion, as we attempted with our patient in the form of a foam pad.

The Diagnosis: Prayer Callus

After our patient demonstrated his routine position for prayer (Figure), the

The position of the patient’s left foot while praying.

diagnosis of prayer callus was confirmed. We suggested he use a foam pad under his foot during prayer. The patient did not return for follow-up.

Prayer calluses have been documented in the literature as prayer nodules^1-3 or marks.⁴ Callus is a preferred term, as it implies the act of repeated pressure or friction to an area of the skin. It is more descriptive than marks and avoids the inclusion of nodules caused by different prayer or religious activities such as ritual scarring.⁵

Abanmi et al⁴ studied a large group (N=349) of Muslim patients with regular praying habits and found a high prevalence of what they referred to as prayer marks, defined by lichenification and/or hyperpigmentation, on the knees, forehead, ankles, and dorsal aspects of the feet. They studied the histopathology of 33 marks. No statistical analysis was performed. They reported common histologic findings of compact orthokeratosis, hypergranulosis, dermal papillary fibrosis, and dermal vascularization. In contrast to lichen simplex chronicus, the authors found that the dermal fibrosis did not exhibit collagen bundles perpendicular to the epidermis.⁴

This study found that a higher prevalence of lichenification was observed on the left foot (males, 57%; females, 39%) than the right foot (males, 14%; females, 19%), which was attributed to a more typical prayer position that placed pressure on the left foot.⁴ Our patient only had calluses on his left foot, which was consistent with the prayer position.

Treatment options for prayer calluses include debridement, either mechanical or with topical keratolytic preparations. There is a high likelihood of recurrence if prayer practices are not changed. Optimally, more definitive treatment, which can be combined with initial debridement, would be position adjustment to lessen pressure or friction to the area or protection over the area with a cushion, as we attempted with our patient in the form of a foam pad.

The Diagnosis: Atrophic Lupus Erythematosus

Cutaneous lupus erythematosus is divided into acute, subacute, and chronic cutaneous lupus erythematosus (CCLE). There are more than 20 subtypes of CCLE mentioned in the literature including atrophic lupus erythematosus (ALE).¹ The most typical presentation is CCLE with discoid lesions. Most commonly, discoid CCLE is an entirely cutaneous process without systemic involvement.Discoid lesions appear as scaly red macules or papules primarily on the face and scalp.² They may evolve into hyperkeratotic plaques with irregular hyperpigmented borders and develop a central hypopigmented depression with atrophy and scarring.^2,3 Discoid CCLE has a female predominance and commonly occurs between 20 and 30 years of age. Triggers of discoid lesions include UV exposure, trauma, and infection.² 

Our case of multiple atrophic plaques of the face, scalp, trunk, and upper extremities demonstrated a diagnostic challenge. Our patient presented with atrophic facial plaques, which are not typical of discoid lesions of CCLE. Our patient’s findings appeared clinically similar to acne scarring or atrophoderma. Histology showed common features of CCLE, including basal liquefactive degeneration, thickening of the basement membrane zone, increased melanin, and a lymphocytic inflammatory infiltrate (Figure).^2,3 There was no evidence of hyperkeratosis, which often is seen in discoid lesions of CCLE.

Clinicopathologically, our case was consistent with ALE. A review of the literature revealed similar cases documented by Christianson and Mitchell⁴ in 1969; they described annular atrophic plaques of the skin of unknown diagnostic classification. Chorzelski et al⁵ reiterated the difficulty of defining diagnostically similar atrophic plaques of the face showing histopathologic features consistent with lupus and suggested these cases may represent an uncharacteristic presentation of discoid lupus erythematosus. Our patient demonstrated this rare subtype of discoid lupus erythematosus, known as ALE. There are few reports in the literature of ALE; thus we have managed our patient similar to other CCLE patients. Management of CCLE patients includes strict sun protection. Treatment options include corticosteroids, calcineurin inhibitors, antimalarial agents, and thalidomide.² Our patient started using tacrolimus ointment 0.1% daily and hydroxychloroquine 200 mg twice daily. She also was practicing strict photoprotection. The patient was lost to follow-up. Topical steroids are not an option in ALE. It is important for dermatologists to recognize this rare variant of CCLE to prevent disfigurement.

The Diagnosis: Atrophic Lupus Erythematosus

Cutaneous lupus erythematosus is divided into acute, subacute, and chronic cutaneous lupus erythematosus (CCLE). There are more than 20 subtypes of CCLE mentioned in the literature including atrophic lupus erythematosus (ALE).¹ The most typical presentation is CCLE with discoid lesions. Most commonly, discoid CCLE is an entirely cutaneous process without systemic involvement.Discoid lesions appear as scaly red macules or papules primarily on the face and scalp.² They may evolve into hyperkeratotic plaques with irregular hyperpigmented borders and develop a central hypopigmented depression with atrophy and scarring.^2,3 Discoid CCLE has a female predominance and commonly occurs between 20 and 30 years of age. Triggers of discoid lesions include UV exposure, trauma, and infection.² 

Our case of multiple atrophic plaques of the face, scalp, trunk, and upper extremities demonstrated a diagnostic challenge. Our patient presented with atrophic facial plaques, which are not typical of discoid lesions of CCLE. Our patient’s findings appeared clinically similar to acne scarring or atrophoderma. Histology showed common features of CCLE, including basal liquefactive degeneration, thickening of the basement membrane zone, increased melanin, and a lymphocytic inflammatory infiltrate (Figure).^2,3 There was no evidence of hyperkeratosis, which often is seen in discoid lesions of CCLE.

Clinicopathologically, our case was consistent with ALE. A review of the literature revealed similar cases documented by Christianson and Mitchell⁴ in 1969; they described annular atrophic plaques of the skin of unknown diagnostic classification. Chorzelski et al⁵ reiterated the difficulty of defining diagnostically similar atrophic plaques of the face showing histopathologic features consistent with lupus and suggested these cases may represent an uncharacteristic presentation of discoid lupus erythematosus. Our patient demonstrated this rare subtype of discoid lupus erythematosus, known as ALE. There are few reports in the literature of ALE; thus we have managed our patient similar to other CCLE patients. Management of CCLE patients includes strict sun protection. Treatment options include corticosteroids, calcineurin inhibitors, antimalarial agents, and thalidomide.² Our patient started using tacrolimus ointment 0.1% daily and hydroxychloroquine 200 mg twice daily. She also was practicing strict photoprotection. The patient was lost to follow-up. Topical steroids are not an option in ALE. It is important for dermatologists to recognize this rare variant of CCLE to prevent disfigurement.

The Diagnosis: Atrophic Lupus Erythematosus

Cutaneous lupus erythematosus is divided into acute, subacute, and chronic cutaneous lupus erythematosus (CCLE). There are more than 20 subtypes of CCLE mentioned in the literature including atrophic lupus erythematosus (ALE).¹ The most typical presentation is CCLE with discoid lesions. Most commonly, discoid CCLE is an entirely cutaneous process without systemic involvement.Discoid lesions appear as scaly red macules or papules primarily on the face and scalp.² They may evolve into hyperkeratotic plaques with irregular hyperpigmented borders and develop a central hypopigmented depression with atrophy and scarring.^2,3 Discoid CCLE has a female predominance and commonly occurs between 20 and 30 years of age. Triggers of discoid lesions include UV exposure, trauma, and infection.² 

Our case of multiple atrophic plaques of the face, scalp, trunk, and upper extremities demonstrated a diagnostic challenge. Our patient presented with atrophic facial plaques, which are not typical of discoid lesions of CCLE. Our patient’s findings appeared clinically similar to acne scarring or atrophoderma. Histology showed common features of CCLE, including basal liquefactive degeneration, thickening of the basement membrane zone, increased melanin, and a lymphocytic inflammatory infiltrate (Figure).^2,3 There was no evidence of hyperkeratosis, which often is seen in discoid lesions of CCLE.

Clinicopathologically, our case was consistent with ALE. A review of the literature revealed similar cases documented by Christianson and Mitchell⁴ in 1969; they described annular atrophic plaques of the skin of unknown diagnostic classification. Chorzelski et al⁵ reiterated the difficulty of defining diagnostically similar atrophic plaques of the face showing histopathologic features consistent with lupus and suggested these cases may represent an uncharacteristic presentation of discoid lupus erythematosus. Our patient demonstrated this rare subtype of discoid lupus erythematosus, known as ALE. There are few reports in the literature of ALE; thus we have managed our patient similar to other CCLE patients. Management of CCLE patients includes strict sun protection. Treatment options include corticosteroids, calcineurin inhibitors, antimalarial agents, and thalidomide.² Our patient started using tacrolimus ointment 0.1% daily and hydroxychloroquine 200 mg twice daily. She also was practicing strict photoprotection. The patient was lost to follow-up. Topical steroids are not an option in ALE. It is important for dermatologists to recognize this rare variant of CCLE to prevent disfigurement.

The Diagnosis: Eosinophilic Fasciitis (Shulman Syndrome)

In 1984, Shulman¹ reported 2 cases of diffuse fasciitis with hypergammaglobulinemia and eosinophilia, which was later termed eosinophilic fasciitis (EF) by Rodnan et al.² Eosinophilic fasciitis also is commonly known as diffuse fasciitis with eosinophilia³ or Shulman syndrome.^3,4 It is an inflammatory condition of unknown etiology and pathogenesis, though an underlying autoimmune process has been proposed.^3,5 The disorder is characterized by inflammation and subsequent fibrosis of deep fascia² with variable involvement of the underlying muscle. It often is referred to as a sclerodermalike condition^2,5-8 and is considered to be one of a group of fibrosing connective tissue disorders that may be confused with systemic sclerosis.^3,4,6,9 Although the cause of EF is unknown, more than 50% of cases are associated with vigorous exercise or trauma.³ Other reported triggers and associations include Borrelia burgdorferi infection,^5,6,10 arthropod bites,^5,10 lymphoproliferative and hematologic disorders,^5,6,10 inflammatory arthritis,⁶ morphea,³ thyroid dysfunction,^5,6,10 statins,^5,10 and phenytoin.^5,10

There have been more than 250 cases of EF reported in the literature.³ Some authors report more frequent occurrence in males,^3,4 while others report equal occurrence of disease in both males and females.^5,10 Eosinophilic fasciitis has the highest incidence during the second to sixth decades of life,^3,10 though childhood cases have been reported.^3,11 White individuals appear to be affected most often, but sporadic cases in other ethnic groups also have been reported.³

The clinical presentation of EF frequently is characterized by the rapid onset of erythema, pain, and nonpitting edema of the involved areas.^5,7,10,11 Fatigue and itching also are reported symptoms.¹² A cobblestone texture of the skin may be noted, commonly referred to as the peau d’orange effect.^6,10 Woody induration subsequently develops, which may lead to contracture or limited range of motion.^5,7,11-13 The distal extremities, particularly the forearms and calves, are the most common areas affected by EF.³ There usually is symmetric involvement; however, unilateral cases of EF also have been reported.^3,6,10 Linear indentations may form along the vasculature of the involved areas when the extremities are lifted or extended. These indentations serve to demarcate the underlying muscle groups, forming the groove sign or venous furrowing.^3,5,14 The epidermis is not characteristically involved in EF and wrinkling of the skin can be elicited by pinching,³ which may be helpful in clinically distinguishing EF from scleroderma. Sparing of the digits is another characteristic feature of adult-onset EF.^3,10 However, involvement of the digits resulting in painless contracture has been reported in multiple childhood cases and may represent a phenotype specific to children.¹¹ Involvement of the trunk is less frequently observed but may serve as a poor prognostic indicator; indeed, patients demonstrating young age of onset, morphealike skin sclerosis, and trunk involvement are more likely to develop refractory disease.¹⁵

Eosinophilic fasciitis is characterized by various laboratory abnormalities. Peripheral eosinophilia, hypergammaglobulinemia, elevated sedimentation rate,^{3,6,9,10,13,15} and elevated C-reactive protein^3,6 are commonly reported; however, antinuclear antibody, rheumatoid factor, or antibodies associated with systemic sclerosis are not frequently found.^3,6 There are no laboratory abnormalities required to render a diagnosis of EF, and absence of peripheral eosinophilia has been noted in up to 20% of cases.^3,4 An elevation of creatine kinase or aldolase levels may be present if muscle is involved.^3,6 In general, abnormal laboratory findings have not been found to correlate with disease activity^13,15 and do not appear to have prognostic significance.¹⁵

Biopsy of involved tissue characteristically reveals fascial thickening and fibrosis.^10,13,15 A lymphoplasmacytic infiltrate normally is found in the subcutaneous tissues and fascia and may extend to involve the deeper dermis¹⁵ but usually spares the epidermis.³ It is important to note that increased numbers of eosinophils are not always found in the affected fascia, especially in the later stages of the disease and after steroid treatment.^3,15 Muscle involvement is variable. When muscle is involved, histologic features range from mild inflammation with no evidence of necrosis³ to substantial fibrinoid necrosis.¹³

Magnetic resonance imaging has become a valuable tool in the diagnosis and treatment of EF. Magnetic resonance imaging findings in EF patients typically reveal fascial thickening on T1-weighted images, enhancement after the use of contrast, and hyperintensity on T2-weighted images.⁷ Magnetic resonance imaging has proven useful in supporting clinical diagnosis, monitoring disease activity, identifying optimal biopsy location, and determining response to treatment.^3,7,8,11,13 Magnetic resonance imaging also has been proven to be particularly useful with atypical clinical presentations.⁸ Although deep tissue biopsy remains the gold standard for diagnosis,¹³ magnetic resonance imaging may provide equal or superior diagnostic information.

In general, the prognosis is very good, and the majority of patients will achieve complete remission and cure.³ Up to one-third of patients with EF may experience spontaneous remission without any treatment intervention.¹⁰ The condition generally is highly responsive to oral corticosteroids,^6,10,15 and it has been reported that up to 70% of patients will respond to corticosteroid treatment.³ Most patients respond to a daily dose of prednisone (0.5–1.5 mg/kg), which is continued until clinical response is observed and then followed by a slow steroid taper over a period of several months.³ Patients with persistent fibrosis often require adjunctive therapy in addition to corticosteroids. A variety of adjunctive therapies have been reported to be effective, including hydroxychloroquine sulfate,^{5,10,13,15,16} azathioprine,^15,16 ibuprofen,^10,15 D-penicillamine,^5,15 cyclophosphomide,¹⁵ methotrexate,^6,13,15 cyclosporine,^13,15 psoralen plus UVA,^5,6,15 extracorporeal photochemotherapy,^5,6,10,16 colchicine,⁵ cimetidine,^5,6,10,16 infliximab,^5,6 griseofulvin,¹⁶ ketotifen,¹⁶ sulfasalazine,¹³ and dapsone.¹⁶ Physical therapy has been reported to be useful in preventing permanent joint contractures.^3,5,10,13,16 If treatment fails, the possibility of underlying malignancy must be considered³; however, long courses of treatment are not uncommon. Some patients may need 12 to 18 months of treatment for full response, and even refractory cases are likely to eventually achieve full remission.³

The Diagnosis: Eosinophilic Fasciitis (Shulman Syndrome)

In 1984, Shulman¹ reported 2 cases of diffuse fasciitis with hypergammaglobulinemia and eosinophilia, which was later termed eosinophilic fasciitis (EF) by Rodnan et al.² Eosinophilic fasciitis also is commonly known as diffuse fasciitis with eosinophilia³ or Shulman syndrome.^3,4 It is an inflammatory condition of unknown etiology and pathogenesis, though an underlying autoimmune process has been proposed.^3,5 The disorder is characterized by inflammation and subsequent fibrosis of deep fascia² with variable involvement of the underlying muscle. It often is referred to as a sclerodermalike condition^2,5-8 and is considered to be one of a group of fibrosing connective tissue disorders that may be confused with systemic sclerosis.^3,4,6,9 Although the cause of EF is unknown, more than 50% of cases are associated with vigorous exercise or trauma.³ Other reported triggers and associations include Borrelia burgdorferi infection,^5,6,10 arthropod bites,^5,10 lymphoproliferative and hematologic disorders,^5,6,10 inflammatory arthritis,⁶ morphea,³ thyroid dysfunction,^5,6,10 statins,^5,10 and phenytoin.^5,10

There have been more than 250 cases of EF reported in the literature.³ Some authors report more frequent occurrence in males,^3,4 while others report equal occurrence of disease in both males and females.^5,10 Eosinophilic fasciitis has the highest incidence during the second to sixth decades of life,^3,10 though childhood cases have been reported.^3,11 White individuals appear to be affected most often, but sporadic cases in other ethnic groups also have been reported.³

The clinical presentation of EF frequently is characterized by the rapid onset of erythema, pain, and nonpitting edema of the involved areas.^5,7,10,11 Fatigue and itching also are reported symptoms.¹² A cobblestone texture of the skin may be noted, commonly referred to as the peau d’orange effect.^6,10 Woody induration subsequently develops, which may lead to contracture or limited range of motion.^5,7,11-13 The distal extremities, particularly the forearms and calves, are the most common areas affected by EF.³ There usually is symmetric involvement; however, unilateral cases of EF also have been reported.^3,6,10 Linear indentations may form along the vasculature of the involved areas when the extremities are lifted or extended. These indentations serve to demarcate the underlying muscle groups, forming the groove sign or venous furrowing.^3,5,14 The epidermis is not characteristically involved in EF and wrinkling of the skin can be elicited by pinching,³ which may be helpful in clinically distinguishing EF from scleroderma. Sparing of the digits is another characteristic feature of adult-onset EF.^3,10 However, involvement of the digits resulting in painless contracture has been reported in multiple childhood cases and may represent a phenotype specific to children.¹¹ Involvement of the trunk is less frequently observed but may serve as a poor prognostic indicator; indeed, patients demonstrating young age of onset, morphealike skin sclerosis, and trunk involvement are more likely to develop refractory disease.¹⁵

Eosinophilic fasciitis is characterized by various laboratory abnormalities. Peripheral eosinophilia, hypergammaglobulinemia, elevated sedimentation rate,^{3,6,9,10,13,15} and elevated C-reactive protein^3,6 are commonly reported; however, antinuclear antibody, rheumatoid factor, or antibodies associated with systemic sclerosis are not frequently found.^3,6 There are no laboratory abnormalities required to render a diagnosis of EF, and absence of peripheral eosinophilia has been noted in up to 20% of cases.^3,4 An elevation of creatine kinase or aldolase levels may be present if muscle is involved.^3,6 In general, abnormal laboratory findings have not been found to correlate with disease activity^13,15 and do not appear to have prognostic significance.¹⁵

Biopsy of involved tissue characteristically reveals fascial thickening and fibrosis.^10,13,15 A lymphoplasmacytic infiltrate normally is found in the subcutaneous tissues and fascia and may extend to involve the deeper dermis¹⁵ but usually spares the epidermis.³ It is important to note that increased numbers of eosinophils are not always found in the affected fascia, especially in the later stages of the disease and after steroid treatment.^3,15 Muscle involvement is variable. When muscle is involved, histologic features range from mild inflammation with no evidence of necrosis³ to substantial fibrinoid necrosis.¹³

Magnetic resonance imaging has become a valuable tool in the diagnosis and treatment of EF. Magnetic resonance imaging findings in EF patients typically reveal fascial thickening on T1-weighted images, enhancement after the use of contrast, and hyperintensity on T2-weighted images.⁷ Magnetic resonance imaging has proven useful in supporting clinical diagnosis, monitoring disease activity, identifying optimal biopsy location, and determining response to treatment.^3,7,8,11,13 Magnetic resonance imaging also has been proven to be particularly useful with atypical clinical presentations.⁸ Although deep tissue biopsy remains the gold standard for diagnosis,¹³ magnetic resonance imaging may provide equal or superior diagnostic information.

In general, the prognosis is very good, and the majority of patients will achieve complete remission and cure.³ Up to one-third of patients with EF may experience spontaneous remission without any treatment intervention.¹⁰ The condition generally is highly responsive to oral corticosteroids,^6,10,15 and it has been reported that up to 70% of patients will respond to corticosteroid treatment.³ Most patients respond to a daily dose of prednisone (0.5–1.5 mg/kg), which is continued until clinical response is observed and then followed by a slow steroid taper over a period of several months.³ Patients with persistent fibrosis often require adjunctive therapy in addition to corticosteroids. A variety of adjunctive therapies have been reported to be effective, including hydroxychloroquine sulfate,^{5,10,13,15,16} azathioprine,^15,16 ibuprofen,^10,15 D-penicillamine,^5,15 cyclophosphomide,¹⁵ methotrexate,^6,13,15 cyclosporine,^13,15 psoralen plus UVA,^5,6,15 extracorporeal photochemotherapy,^5,6,10,16 colchicine,⁵ cimetidine,^5,6,10,16 infliximab,^5,6 griseofulvin,¹⁶ ketotifen,¹⁶ sulfasalazine,¹³ and dapsone.¹⁶ Physical therapy has been reported to be useful in preventing permanent joint contractures.^3,5,10,13,16 If treatment fails, the possibility of underlying malignancy must be considered³; however, long courses of treatment are not uncommon. Some patients may need 12 to 18 months of treatment for full response, and even refractory cases are likely to eventually achieve full remission.³

The Diagnosis: Eosinophilic Fasciitis (Shulman Syndrome)

In 1984, Shulman¹ reported 2 cases of diffuse fasciitis with hypergammaglobulinemia and eosinophilia, which was later termed eosinophilic fasciitis (EF) by Rodnan et al.² Eosinophilic fasciitis also is commonly known as diffuse fasciitis with eosinophilia³ or Shulman syndrome.^3,4 It is an inflammatory condition of unknown etiology and pathogenesis, though an underlying autoimmune process has been proposed.^3,5 The disorder is characterized by inflammation and subsequent fibrosis of deep fascia² with variable involvement of the underlying muscle. It often is referred to as a sclerodermalike condition^2,5-8 and is considered to be one of a group of fibrosing connective tissue disorders that may be confused with systemic sclerosis.^3,4,6,9 Although the cause of EF is unknown, more than 50% of cases are associated with vigorous exercise or trauma.³ Other reported triggers and associations include Borrelia burgdorferi infection,^5,6,10 arthropod bites,^5,10 lymphoproliferative and hematologic disorders,^5,6,10 inflammatory arthritis,⁶ morphea,³ thyroid dysfunction,^5,6,10 statins,^5,10 and phenytoin.^5,10

There have been more than 250 cases of EF reported in the literature.³ Some authors report more frequent occurrence in males,^3,4 while others report equal occurrence of disease in both males and females.^5,10 Eosinophilic fasciitis has the highest incidence during the second to sixth decades of life,^3,10 though childhood cases have been reported.^3,11 White individuals appear to be affected most often, but sporadic cases in other ethnic groups also have been reported.³

The clinical presentation of EF frequently is characterized by the rapid onset of erythema, pain, and nonpitting edema of the involved areas.^5,7,10,11 Fatigue and itching also are reported symptoms.¹² A cobblestone texture of the skin may be noted, commonly referred to as the peau d’orange effect.^6,10 Woody induration subsequently develops, which may lead to contracture or limited range of motion.^5,7,11-13 The distal extremities, particularly the forearms and calves, are the most common areas affected by EF.³ There usually is symmetric involvement; however, unilateral cases of EF also have been reported.^3,6,10 Linear indentations may form along the vasculature of the involved areas when the extremities are lifted or extended. These indentations serve to demarcate the underlying muscle groups, forming the groove sign or venous furrowing.^3,5,14 The epidermis is not characteristically involved in EF and wrinkling of the skin can be elicited by pinching,³ which may be helpful in clinically distinguishing EF from scleroderma. Sparing of the digits is another characteristic feature of adult-onset EF.^3,10 However, involvement of the digits resulting in painless contracture has been reported in multiple childhood cases and may represent a phenotype specific to children.¹¹ Involvement of the trunk is less frequently observed but may serve as a poor prognostic indicator; indeed, patients demonstrating young age of onset, morphealike skin sclerosis, and trunk involvement are more likely to develop refractory disease.¹⁵

Eosinophilic fasciitis is characterized by various laboratory abnormalities. Peripheral eosinophilia, hypergammaglobulinemia, elevated sedimentation rate,^{3,6,9,10,13,15} and elevated C-reactive protein^3,6 are commonly reported; however, antinuclear antibody, rheumatoid factor, or antibodies associated with systemic sclerosis are not frequently found.^3,6 There are no laboratory abnormalities required to render a diagnosis of EF, and absence of peripheral eosinophilia has been noted in up to 20% of cases.^3,4 An elevation of creatine kinase or aldolase levels may be present if muscle is involved.^3,6 In general, abnormal laboratory findings have not been found to correlate with disease activity^13,15 and do not appear to have prognostic significance.¹⁵

Biopsy of involved tissue characteristically reveals fascial thickening and fibrosis.^10,13,15 A lymphoplasmacytic infiltrate normally is found in the subcutaneous tissues and fascia and may extend to involve the deeper dermis¹⁵ but usually spares the epidermis.³ It is important to note that increased numbers of eosinophils are not always found in the affected fascia, especially in the later stages of the disease and after steroid treatment.^3,15 Muscle involvement is variable. When muscle is involved, histologic features range from mild inflammation with no evidence of necrosis³ to substantial fibrinoid necrosis.¹³

Magnetic resonance imaging has become a valuable tool in the diagnosis and treatment of EF. Magnetic resonance imaging findings in EF patients typically reveal fascial thickening on T1-weighted images, enhancement after the use of contrast, and hyperintensity on T2-weighted images.⁷ Magnetic resonance imaging has proven useful in supporting clinical diagnosis, monitoring disease activity, identifying optimal biopsy location, and determining response to treatment.^3,7,8,11,13 Magnetic resonance imaging also has been proven to be particularly useful with atypical clinical presentations.⁸ Although deep tissue biopsy remains the gold standard for diagnosis,¹³ magnetic resonance imaging may provide equal or superior diagnostic information.

In general, the prognosis is very good, and the majority of patients will achieve complete remission and cure.³ Up to one-third of patients with EF may experience spontaneous remission without any treatment intervention.¹⁰ The condition generally is highly responsive to oral corticosteroids,^6,10,15 and it has been reported that up to 70% of patients will respond to corticosteroid treatment.³ Most patients respond to a daily dose of prednisone (0.5–1.5 mg/kg), which is continued until clinical response is observed and then followed by a slow steroid taper over a period of several months.³ Patients with persistent fibrosis often require adjunctive therapy in addition to corticosteroids. A variety of adjunctive therapies have been reported to be effective, including hydroxychloroquine sulfate,^{5,10,13,15,16} azathioprine,^15,16 ibuprofen,^10,15 D-penicillamine,^5,15 cyclophosphomide,¹⁵ methotrexate,^6,13,15 cyclosporine,^13,15 psoralen plus UVA,^5,6,15 extracorporeal photochemotherapy,^5,6,10,16 colchicine,⁵ cimetidine,^5,6,10,16 infliximab,^5,6 griseofulvin,¹⁶ ketotifen,¹⁶ sulfasalazine,¹³ and dapsone.¹⁶ Physical therapy has been reported to be useful in preventing permanent joint contractures.^3,5,10,13,16 If treatment fails, the possibility of underlying malignancy must be considered³; however, long courses of treatment are not uncommon. Some patients may need 12 to 18 months of treatment for full response, and even refractory cases are likely to eventually achieve full remission.³