Case Report Access this article online Website: www.ijtrichology.com DOI: 10.4103/0974-7753.138590 Quick Response Code:
Single Lesion of Sarcoidosis Presenting as Cicatricial Alopecia: A Rare Report from India Aparajita Ghosh, Sujata Sengupta, Arijit Coondoo, Ramesh Chandra Gharami1 Department of Dermatology, KPC Medical College and Hospital, 1Medical College and Hospital, Kolkata, West Bengal, India ABSTRACT
Address for correspondence: Dr. Aparajita Ghosh, Flat No. 2, Building No. W‑2A (R) 4, Phase 4A, Golf Green, Kolkata ‑ 700 095, West Bengal, India. E‑mail: dr.aparajitaghosh@ gmail.com
Sarcoidosis affects different systems of the body including the skin where it can affect various cutaneous sites. Among these sites, the scalp is a very unusual location for lesions of sarcoidosis. Sarcoidosis of the scalp can very rarely be accompanied by cicatricial alopecia. We report here a rare case of sarcoidosis of scalp with cicatricial alopecia. To the best of our knowledge, this is the first such report from India.
Key words: Cicatricial alopecia, sarcoidosis, scalp
INTRODUCTION
CASE REPORT
arcoidosis is a multi‑system disorder of unknown etiology which can involve any organ of the body, more commonly affecting the lymph nodes, lungs, liver, spleen, skin, eyes, and bones. Diagnosis of this condition is made primarily by histopathology wherein characteristic noncaseating epitheloid granulomas are observed in all or most of the affected tissues.[1]
A 43‑year‑old woman presented with a complaint of loss of scalp hair for preceding 7 months. The condition had started insidiously and slowly progressed to involve almost the whole of the scalp. The patient also complained of dryness of the affected area without any associated itching. There was no history of exposure to radiation or intake of any medications. On clinical examination we observed patches of scarring alopecia, which had become confluent to progressively involve a major portion of the vertex of the scalp [Figures 1 and 2]. Normal hair bearing skin was present among the patches and toward the margins of the scalp [Figure 3]. Other terminal hair bearing areas were not affected. No other cutaneous findings suggestive of any other dermatologic condition were found at any other cutaneous site. Systemic examination revealed no abnormalities in any other organ. A provisional diagnosis of cicatricial alopecia was made, and a punch biopsy was done to differentiate between the common causes of ciatricial alopecia such as lichen planopilaris, discoid lupus erythematosus, psudopelade of Brocq, folliculitis decalvans, dissecting cellulitis of the scalp frontal fibrosing alopecia, and acne keloidalis.
S
Cutaneous sarcoidosis can occur in almost a quarter of cases of systemic sarcoidosis. Morphologically the disease exhibits varied morphological presentations thus imitating many unrelated dermatologic conditions. [2] Hence, it has been described as a great mimicker in dermatology.[3] Though sarcoidosis may affect the skin of various areas of the body, the scalp is a rare site of presentation of the disease. Rarer still is the incidence of sarcoidosis of scalp without affection of other cutaneous sites. Sarcoidosis of the scalp may lead to cicatricial alopecia, a distressing complication for the patient.[4] We report here a case of a middle‑aged female who presented with scarring alopecia of the scalp as the only cutaneous manifestation of sarcoidosis. International Journal of Trichology / Apr-Jun 2014 / Vol-6 / Issue-2
63
Ghosh, et al.: Sarcoidosis presenting as cicatricial alopecia
Figure 1: Patches of scarring alopecia on the scalp showing dryness and scaling
Figure 3: Normal hair-bearing lesions at the occipital area
Figure 2: Lesions over temporal and frontal areas
Figure 4: Noncaseating epitheloid cell granuloma in the dermis with a complete absence of any adnexal structure (H and E, ×100)
giant cells of Langhans and foreign body type were seen [Figure 5]. The dermis showed marked fibrosis. No follicular structures were evident. On the basis of these histological findings, a diagnosis of sarcoidosis of scalp was made. No abnormalities were found on the chest roentgenogram, but serum calcium levels were mildly elevated. All other routine investigations were within normal limits. Based on the histological finding of naked epithelioid granulomas with multinucleated giant cells of the Langhasns type, a diagnosis of sarcoidsis was confirmed. Figure 5: Granuloma showing Langhan’s giant cell (H and E, ×400)
A punch biopsy specimen obtained from the affected area for histopathology showed multiple naked epitheloid cell granuloma in the dermis [Figure 4]. A number of 64
The patient was treated with systemic corticosteroids at a dose of 30 mg prednisolone/day gradually tapered over a period of 3 months. Follow‑up up to 1 year showed no regrowth of hair occurred though the treatment successfully controlled any further hair loss. International Journal of Trichology / Apr-Jun 2014 / Vol-6 / Issue-2
Ghosh, et al.: Sarcoidosis presenting as cicatricial alopecia
DISCUSSION
Sarcoidosis is a disease affecting multiple systems of the body. Cutaneous lesions were observed in 20-30% of cases of sarcoidosis and may be specific or nonspecific. Specific lesions exhibit noncaseating granulomas on histopathological examination. However, nonspecific lesions are devoid of such granulomas. The commonest specific skin lesions observed clinically are reddish yellow maculopapules, plaques and subcutaneous nodules, but innumerable variations such as lupus pernio and scar sarcoidosis may occur. Hence, the diagnosis of the condition is often challenging.[5] Sarcoidosis of the scalp resulting in alopecia is a rare cutaneous manifestation. While reporting two cases of sarcidosis induced alopecias House et al. observed that a thorough literature search in PubMed revealed reports of 39 published cases till 2012.[6] Later one more case has been reported from Mexico[7] followed by one from Japan.[8] In most of these cases, the alopecia was cicatricial in nature though a few cases with nonscarring alopecia have also been reported.[9] The scalp involvement has been seen to be mostly local in nature. However, diffuse alopecia has also been reported, though rarely.[10] A Syrian patient with cicatrical alopecia due to sarcoidosis was found to have severely disfiguring lesions in the scalp, though the number of lesions were few.[11] The disease shows a strong gender bias with 33 of the 38 cases (where details were available) occurring in females. Our case also followed this trend. Racially, most of the reported cases were seen in Afrcans or Afro‑Americans.[6] Histologically, sarcoidosis can be distinguished from other causes of cicatricial alopecia by the presence of naked epithelioid cell granulomas.[12] Since our patient presented with scarring alopecia of 7 months duration discoid lupus erythematosus and lichen planopilaris were clinically considered as possible causes. However, the histopathology revealed the characteristic naked epithelioid cell granuloma granuloma of sarcoidosis. The absence of lichenoid features, follicular plugging, vacuolar degeneration of basal cell layer and perivascular and periappendageal infiltrates helped in excluding lichen planopilaris or discoid lupus erythematosus as possible causes histologically. Folliculitis decalvans and dissecting cellulitis were not considered as candidates for diagnosis because of the absence of suppurative folliculitis, abscesses, and sinus tracts. Though systemic involvement particularly of the thorax has been found to occur in most of the cases of scalp sarcoidosis reported thus far,[6] we did not find any evidence International Journal of Trichology / Apr-Jun 2014 / Vol-6 / Issue-2
of underlying systemic involvement in our case other than a mildly raised serum calcium level. Therapy of scalp sarcoidosis with alopecia has been quite unsatisfactory. Earlier reports of therapy of sarcoidosis with alopecia with corticosteroids (topical and intralesional and systemic) antimalarials and methotrexate have shown poor to moderate response.[6] There are case reports of therapy with systemic corticosteroids where the progress of the disease has been arrested without any new hair growth.[13] In our case, too, systemic corticosteroids were able to check the progress of the disease. The lack of improvement in the existing patches of cicatricial alopecia, in spite of therapy, was but expected as the hair follicles had been permanently destroyed by the disease process. Not much is known about the overall prognosis of scalp sarcoidosis primarily because of the small number of reported cases. It might be possible that cases of alopecia due to sarcoidosis are often misdiagnosed in the absence of proper histopathological evaluation. This is unfortunate as recognition of the cutaneous lesions of sarcoidosis which are easily accessible for histopathology, can provide a clue to the underlying systemic disease and help in proper management of the patient. Sarcoidosis of scalp has been reported earlier from India.[14] However, to the best of our knowledge our case is the first report of cicatricial alopecia due to scalp sarcoidosis from this country. This report also emphasizes the E role of histopathology as an important diagnostic tool in the evaluation of cases of both scarring and nonscarring alopecia presenting to the dermatologist. REFERENCES 1. Iannuzzi MC, Rybicki BA, Teirstein AS. Sarcoidosis. N Engl J Med 2007;357:2153‑65. 2. Mañá J, Marcoval J. Skin manifestations of sarcoidosis. Presse Med 2012;41:e355‑74. 3. Katta R. Cutaneous sarcoidosis: A dermatologic masquerader. Am Fam Physician 2002;65:1581‑4. 4. Takahashi H, Mori M, Muraoka S, Sato T, Dempo R, Shiratori A, et al. Sarcoidosis presenting as a scarring alopecia: Report of a rare cutaneous manifestation of systemic sarcoidosis. Dermatology 1996;193:144‑6. 5. H a i m o v i c A , S a n c h e z M , J u d s o n M A , P r y s t o w s k y S. Sarcoidosis: A comprehensive review and update for the dermatologist: Part I. Cutaneous disease. J Am Acad Dermatol 2012;66:699.e1‑18. 6. House NS, Welsh JP, English JC 3rd. Sarcoidosis‑induced alopecia. Dermatol Online J 2012;18:4. 7. Fernández‑Sánchez M, Saeb‑Lima M. Cutaneous sarcoidosis. Case report. Rev Med Inst Mex Seguro Soc 2012;50:541‑4. 8. Nonomura Y, Otsuka A, Miyachi Y, Kabashima K. Sarcoidosis of the scalp presenting as patchy alopecia; analysis of transforming growth factor‑ß expression in the affected area by immunostaining. Eur J Dermatol 2013;23:115‑6. 65
Ghosh, et al.: Sarcoidosis presenting as cicatricial alopecia 9. Katta R, Nelson B, Chen D, Roenigk H. Sarcoidosis of the scalp: A case series and review of the literature. J Am Acad Dermatol 2000;42:690‑2. 10. Baker H. Atrophic alopecia due to granulomatous infiltration of the scalp in systemic sarcoidosis. Proc R Soc Med 1965;58:243‑4. 11. Douri T, Chawaf AZ, Alrefaee BA. Cicatricial alopecia due to sarcoidosis. Dermatol Online J 2003;9:16. 12. Ball NJ, Kho GT, Martinka M. The histologic spectr um of cutaneous sarcoidosis: A study of twenty‑eight cases. J Cutan Pathol 2004;31:160‑8. 13. Smith SR, Kendall MJ, Kondratowicz GM. Sarcoidosis - A cause of steroid
66
responsive total alopecia. Postgrad Med J 1986;62:205‑7. 14. Mahajan VK, Sharma NL, Sharma RC, Sharma VC. Cutaneous sarcoidosis: Clinical profile of 23 Indian patients. Indian J Dermatol Venereol Leprol 2007;73:16‑21.
How to cite this article: Ghosh A, Sengupta S, Coondoo A, Gharami RC. Single lesion of sarcoidosis presenting as cicatricial alopecia: A rare report from India. Int J Trichol 2014;6:63-6. Source of Support: Nil, Conflict of Interest: None declared.
International Journal of Trichology / Apr-Jun 2014 / Vol-6 / Issue-2
Copyright of International Journal of Trichology is the property of Medknow Publications & Media Pvt. Ltd. and its content may not be copied or emailed to multiple sites or posted to a listserv without the copyright holder's express written permission. However, users may print, download, or email articles for individual use.
Single Lesion of Sarcoidosis Presenting as Cicatricial Alopecia: A Rare Report from India Aparajita Ghosh, Sujata Sengupta, Arijit Coondoo, Ramesh Chandra Gharami1 Department of Dermatology, KPC Medical College and Hospital, 1Medical College and Hospital, Kolkata, West Bengal, India ABSTRACT
Address for correspondence: Dr. Aparajita Ghosh, Flat No. 2, Building No. W‑2A (R) 4, Phase 4A, Golf Green, Kolkata ‑ 700 095, West Bengal, India. E‑mail: dr.aparajitaghosh@ gmail.com
Sarcoidosis affects different systems of the body including the skin where it can affect various cutaneous sites. Among these sites, the scalp is a very unusual location for lesions of sarcoidosis. Sarcoidosis of the scalp can very rarely be accompanied by cicatricial alopecia. We report here a rare case of sarcoidosis of scalp with cicatricial alopecia. To the best of our knowledge, this is the first such report from India.
Key words: Cicatricial alopecia, sarcoidosis, scalp
INTRODUCTION
CASE REPORT
arcoidosis is a multi‑system disorder of unknown etiology which can involve any organ of the body, more commonly affecting the lymph nodes, lungs, liver, spleen, skin, eyes, and bones. Diagnosis of this condition is made primarily by histopathology wherein characteristic noncaseating epitheloid granulomas are observed in all or most of the affected tissues.[1]
A 43‑year‑old woman presented with a complaint of loss of scalp hair for preceding 7 months. The condition had started insidiously and slowly progressed to involve almost the whole of the scalp. The patient also complained of dryness of the affected area without any associated itching. There was no history of exposure to radiation or intake of any medications. On clinical examination we observed patches of scarring alopecia, which had become confluent to progressively involve a major portion of the vertex of the scalp [Figures 1 and 2]. Normal hair bearing skin was present among the patches and toward the margins of the scalp [Figure 3]. Other terminal hair bearing areas were not affected. No other cutaneous findings suggestive of any other dermatologic condition were found at any other cutaneous site. Systemic examination revealed no abnormalities in any other organ. A provisional diagnosis of cicatricial alopecia was made, and a punch biopsy was done to differentiate between the common causes of ciatricial alopecia such as lichen planopilaris, discoid lupus erythematosus, psudopelade of Brocq, folliculitis decalvans, dissecting cellulitis of the scalp frontal fibrosing alopecia, and acne keloidalis.
S
Cutaneous sarcoidosis can occur in almost a quarter of cases of systemic sarcoidosis. Morphologically the disease exhibits varied morphological presentations thus imitating many unrelated dermatologic conditions. [2] Hence, it has been described as a great mimicker in dermatology.[3] Though sarcoidosis may affect the skin of various areas of the body, the scalp is a rare site of presentation of the disease. Rarer still is the incidence of sarcoidosis of scalp without affection of other cutaneous sites. Sarcoidosis of the scalp may lead to cicatricial alopecia, a distressing complication for the patient.[4] We report here a case of a middle‑aged female who presented with scarring alopecia of the scalp as the only cutaneous manifestation of sarcoidosis. International Journal of Trichology / Apr-Jun 2014 / Vol-6 / Issue-2
63
Ghosh, et al.: Sarcoidosis presenting as cicatricial alopecia
Figure 1: Patches of scarring alopecia on the scalp showing dryness and scaling
Figure 3: Normal hair-bearing lesions at the occipital area
Figure 2: Lesions over temporal and frontal areas
Figure 4: Noncaseating epitheloid cell granuloma in the dermis with a complete absence of any adnexal structure (H and E, ×100)
giant cells of Langhans and foreign body type were seen [Figure 5]. The dermis showed marked fibrosis. No follicular structures were evident. On the basis of these histological findings, a diagnosis of sarcoidosis of scalp was made. No abnormalities were found on the chest roentgenogram, but serum calcium levels were mildly elevated. All other routine investigations were within normal limits. Based on the histological finding of naked epithelioid granulomas with multinucleated giant cells of the Langhasns type, a diagnosis of sarcoidsis was confirmed. Figure 5: Granuloma showing Langhan’s giant cell (H and E, ×400)
A punch biopsy specimen obtained from the affected area for histopathology showed multiple naked epitheloid cell granuloma in the dermis [Figure 4]. A number of 64
The patient was treated with systemic corticosteroids at a dose of 30 mg prednisolone/day gradually tapered over a period of 3 months. Follow‑up up to 1 year showed no regrowth of hair occurred though the treatment successfully controlled any further hair loss. International Journal of Trichology / Apr-Jun 2014 / Vol-6 / Issue-2
Ghosh, et al.: Sarcoidosis presenting as cicatricial alopecia
DISCUSSION
Sarcoidosis is a disease affecting multiple systems of the body. Cutaneous lesions were observed in 20-30% of cases of sarcoidosis and may be specific or nonspecific. Specific lesions exhibit noncaseating granulomas on histopathological examination. However, nonspecific lesions are devoid of such granulomas. The commonest specific skin lesions observed clinically are reddish yellow maculopapules, plaques and subcutaneous nodules, but innumerable variations such as lupus pernio and scar sarcoidosis may occur. Hence, the diagnosis of the condition is often challenging.[5] Sarcoidosis of the scalp resulting in alopecia is a rare cutaneous manifestation. While reporting two cases of sarcidosis induced alopecias House et al. observed that a thorough literature search in PubMed revealed reports of 39 published cases till 2012.[6] Later one more case has been reported from Mexico[7] followed by one from Japan.[8] In most of these cases, the alopecia was cicatricial in nature though a few cases with nonscarring alopecia have also been reported.[9] The scalp involvement has been seen to be mostly local in nature. However, diffuse alopecia has also been reported, though rarely.[10] A Syrian patient with cicatrical alopecia due to sarcoidosis was found to have severely disfiguring lesions in the scalp, though the number of lesions were few.[11] The disease shows a strong gender bias with 33 of the 38 cases (where details were available) occurring in females. Our case also followed this trend. Racially, most of the reported cases were seen in Afrcans or Afro‑Americans.[6] Histologically, sarcoidosis can be distinguished from other causes of cicatricial alopecia by the presence of naked epithelioid cell granulomas.[12] Since our patient presented with scarring alopecia of 7 months duration discoid lupus erythematosus and lichen planopilaris were clinically considered as possible causes. However, the histopathology revealed the characteristic naked epithelioid cell granuloma granuloma of sarcoidosis. The absence of lichenoid features, follicular plugging, vacuolar degeneration of basal cell layer and perivascular and periappendageal infiltrates helped in excluding lichen planopilaris or discoid lupus erythematosus as possible causes histologically. Folliculitis decalvans and dissecting cellulitis were not considered as candidates for diagnosis because of the absence of suppurative folliculitis, abscesses, and sinus tracts. Though systemic involvement particularly of the thorax has been found to occur in most of the cases of scalp sarcoidosis reported thus far,[6] we did not find any evidence International Journal of Trichology / Apr-Jun 2014 / Vol-6 / Issue-2
of underlying systemic involvement in our case other than a mildly raised serum calcium level. Therapy of scalp sarcoidosis with alopecia has been quite unsatisfactory. Earlier reports of therapy of sarcoidosis with alopecia with corticosteroids (topical and intralesional and systemic) antimalarials and methotrexate have shown poor to moderate response.[6] There are case reports of therapy with systemic corticosteroids where the progress of the disease has been arrested without any new hair growth.[13] In our case, too, systemic corticosteroids were able to check the progress of the disease. The lack of improvement in the existing patches of cicatricial alopecia, in spite of therapy, was but expected as the hair follicles had been permanently destroyed by the disease process. Not much is known about the overall prognosis of scalp sarcoidosis primarily because of the small number of reported cases. It might be possible that cases of alopecia due to sarcoidosis are often misdiagnosed in the absence of proper histopathological evaluation. This is unfortunate as recognition of the cutaneous lesions of sarcoidosis which are easily accessible for histopathology, can provide a clue to the underlying systemic disease and help in proper management of the patient. Sarcoidosis of scalp has been reported earlier from India.[14] However, to the best of our knowledge our case is the first report of cicatricial alopecia due to scalp sarcoidosis from this country. This report also emphasizes the E role of histopathology as an important diagnostic tool in the evaluation of cases of both scarring and nonscarring alopecia presenting to the dermatologist. REFERENCES 1. Iannuzzi MC, Rybicki BA, Teirstein AS. Sarcoidosis. N Engl J Med 2007;357:2153‑65. 2. Mañá J, Marcoval J. Skin manifestations of sarcoidosis. Presse Med 2012;41:e355‑74. 3. Katta R. Cutaneous sarcoidosis: A dermatologic masquerader. Am Fam Physician 2002;65:1581‑4. 4. Takahashi H, Mori M, Muraoka S, Sato T, Dempo R, Shiratori A, et al. Sarcoidosis presenting as a scarring alopecia: Report of a rare cutaneous manifestation of systemic sarcoidosis. Dermatology 1996;193:144‑6. 5. H a i m o v i c A , S a n c h e z M , J u d s o n M A , P r y s t o w s k y S. Sarcoidosis: A comprehensive review and update for the dermatologist: Part I. Cutaneous disease. J Am Acad Dermatol 2012;66:699.e1‑18. 6. House NS, Welsh JP, English JC 3rd. Sarcoidosis‑induced alopecia. Dermatol Online J 2012;18:4. 7. Fernández‑Sánchez M, Saeb‑Lima M. Cutaneous sarcoidosis. Case report. Rev Med Inst Mex Seguro Soc 2012;50:541‑4. 8. Nonomura Y, Otsuka A, Miyachi Y, Kabashima K. Sarcoidosis of the scalp presenting as patchy alopecia; analysis of transforming growth factor‑ß expression in the affected area by immunostaining. Eur J Dermatol 2013;23:115‑6. 65
Ghosh, et al.: Sarcoidosis presenting as cicatricial alopecia 9. Katta R, Nelson B, Chen D, Roenigk H. Sarcoidosis of the scalp: A case series and review of the literature. J Am Acad Dermatol 2000;42:690‑2. 10. Baker H. Atrophic alopecia due to granulomatous infiltration of the scalp in systemic sarcoidosis. Proc R Soc Med 1965;58:243‑4. 11. Douri T, Chawaf AZ, Alrefaee BA. Cicatricial alopecia due to sarcoidosis. Dermatol Online J 2003;9:16. 12. Ball NJ, Kho GT, Martinka M. The histologic spectr um of cutaneous sarcoidosis: A study of twenty‑eight cases. J Cutan Pathol 2004;31:160‑8. 13. Smith SR, Kendall MJ, Kondratowicz GM. Sarcoidosis - A cause of steroid
66
responsive total alopecia. Postgrad Med J 1986;62:205‑7. 14. Mahajan VK, Sharma NL, Sharma RC, Sharma VC. Cutaneous sarcoidosis: Clinical profile of 23 Indian patients. Indian J Dermatol Venereol Leprol 2007;73:16‑21.
How to cite this article: Ghosh A, Sengupta S, Coondoo A, Gharami RC. Single lesion of sarcoidosis presenting as cicatricial alopecia: A rare report from India. Int J Trichol 2014;6:63-6. Source of Support: Nil, Conflict of Interest: None declared.
International Journal of Trichology / Apr-Jun 2014 / Vol-6 / Issue-2
Copyright of International Journal of Trichology is the property of Medknow Publications & Media Pvt. Ltd. and its content may not be copied or emailed to multiple sites or posted to a listserv without the copyright holder's express written permission. However, users may print, download, or email articles for individual use.
