trichoscopy as a clue to the diagnosis of scalp sarcoidosis

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Pharmacology and therapeutics Trichoscopy as a clue to the diagnosis of scalp sarcoidosis Fernanda Torres 1 , MD, Antonella Tosti 2,3 , MD, Cosimo Misciali 2 , MD, and Sandra Lorenzi 2 , MD 1 Department of Dermatology, Institute of Dermatology of Rio de Janeiro (IDERJ), Rio de Janeiro, Brazil, 2 Department of Dermatology, University of Bologna, Bologna, Italy, and 3 Department of Dermatology & Cutaneous Surgery, Miller Medical School, University of Miami, US Correspondence Fernanda Torres, MD Institute of Dermatology of Rio de Janeiro (IDERJ) St Alexandre Ferreira 206 Lagoon Rio de Janeiro CEP 22.470-220 Brazil E-mail: [email protected] Conflicts of interest: None. Abstract Background Sarcoidosis is an idiopathic systemic granulomatous disease, in which non- caseating granulomas formations can occur in any organ. Although rare, involvement of the scalp can occur, which might lead to cicatricial alopecia. Dermoscopic features of scalp sarcoidosis had not been reported. Methods Clinical, dermoscopic, and histopathological features of two patients with scalp sarcoidosis were reviewed. Results Two Caucasian female patients aged 22 and 60 years old presented with diffuse folliculitis-like lesions and patchy alopecia, respectively. Dermoscopy of the lesions showed decreased hair density associated with perifollicular and follicular yellowish to pale orange round spots in the first case and diffuse orange discoloration with prominent telangiectasia in the second patient. Few dystrophic hairs were seen in both cases. Conclusions The orange spots seen at trichoscopy of lesions in scalp sarcoidosis may represent a clue to the diagnosis of this condition. Dystrophic hairs may indicate granulomatous activity. Sarcoidosis is an idiopathic systemic granulomatous dis- ease in which noncaseating granulomas formations can occur in any organ. 1 The skin is affected in around 25% of cases, the majority of whom are African-American. 1 Involvement of the scalp is rare and may lead to cicatricial alopecia as a result of the destruction of the hair follicles by the granulomatous formation. 2–5 Clinically, sarcoidosis may present as papules, nodules, or plaques, in some cases resembling discoid lupus erythe- matosus or lipoidic necrobiosis. 6–12 Dermoscopy has been described as an important tool in the diagnosis of hair and scalp disorders. 13–20 The use of this technique for the diagnosis of scalp sarcoidosis has not been described in the literature. We report two patients presenting with scalp sarcoido- sis and discuss their dermoscopic patterns. Clinical, dermoscopic, and histopathological features of two patients with scalp sarcoidosis were reviewed. Dermoscopic images were obtained with a computerized, polarized-light videomicroscope (FotoFinderdermoscope Ò ; Teachscreen Software GmbH, Bad Birnbach, Germany) with lenses at magnification factors of ·20–70 set at ·10 increments. The epiluminescent mode was used. Spring water (Eau Thermale Avène, Boulogne, France) was used as the interface solution. Case reports Case 1 A 20-year-old woman presented with a three-year history of itching folliculitis-like lesions of the scalp. At clinical examination, she presented with small alopecic patches and diffuse hair thinning on the occipital and left parietal regions, scalp erythema, and crusts (Fig. 1). Dermoscopy of the lesions showed decreased hair density, perifollicular and follicular yellowish to pale orange round spots, perifol- licular scaling, few dystrophic hairs, and telangiectasias (Fig. 2). A 5-mm punch biopsy was performed. Vertical sections revealed superficial sarcoidal granulomas consisting of lym- phocytes at the periphery of collections of a few epithelioid histiocytes in the middle dermis, prominent edema, and vasodilatation of the papillary dermis (Fig 3a). Horizontal sections at the isthmus level showed the destruction of follicular units by sarcoidal granulomas (Fig. 3b). Periodic acid-Schiff stain was negative for hyphae and spores. Alcian blue stain at pH 2.5 showed no mucin deposits. Chest x-ray, abdominal ultrasonography, and blood examination were normal. The patient was prescribed in- tralesional steroids and showed moderate improvement at the three-month follow-up. 358 International Journal of Dermatology 2011, 50, 358–361 ª 2011 The International Society of Dermatology

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Pharmacology and therapeutics

Trichoscopy as a clue to the diagnosis of scalp sarcoidosis

Fernanda Torres1, MD, Antonella Tosti2,3, MD, Cosimo Misciali2, MD, andSandra Lorenzi2, MD

1Department of Dermatology, Institute

of Dermatology of Rio de Janeiro

(IDERJ), Rio de Janeiro, Brazil,2Department of Dermatology, University

of Bologna, Bologna, Italy, and3Department of Dermatology &

Cutaneous Surgery, Miller Medical

School, University of Miami, US

Correspondence

Fernanda Torres, MD

Institute of Dermatology of Rio de

Janeiro (IDERJ)

St Alexandre Ferreira 206

Lagoon

Rio de Janeiro CEP 22.470-220

Brazil

E-mail: [email protected]

Conflicts of interest: None.

Abstract

Background Sarcoidosis is an idiopathic systemic granulomatous disease, in which non-

caseating granulomas formations can occur in any organ. Although rare, involvement of the

scalp can occur, which might lead to cicatricial alopecia. Dermoscopic features of scalp

sarcoidosis had not been reported.

Methods Clinical, dermoscopic, and histopathological features of two patients with scalp

sarcoidosis were reviewed.

Results Two Caucasian female patients aged 22 and 60 years old presented with diffuse

folliculitis-like lesions and patchy alopecia, respectively. Dermoscopy of the lesions showed

decreased hair density associated with perifollicular and follicular yellowish to pale orange

round spots in the first case and diffuse orange discoloration with prominent telangiectasia

in the second patient. Few dystrophic hairs were seen in both cases.

Conclusions The orange spots seen at trichoscopy of lesions in scalp sarcoidosis

may represent a clue to the diagnosis of this condition. Dystrophic hairs may indicate

granulomatous activity.

Sarcoidosis is an idiopathic systemic granulomatous dis-ease in which noncaseating granulomas formations canoccur in any organ.1 The skin is affected in around 25%of cases, the majority of whom are African-American.1

Involvement of the scalp is rare and may lead tocicatricial alopecia as a result of the destruction of thehair follicles by the granulomatous formation.2–5

Clinically, sarcoidosis may present as papules, nodules, orplaques, in some cases resembling discoid lupus erythe-matosus or lipoidic necrobiosis.6–12

Dermoscopy has been described as an important tool inthe diagnosis of hair and scalp disorders.13–20 The use ofthis technique for the diagnosis of scalp sarcoidosis hasnot been described in the literature.

We report two patients presenting with scalp sarcoido-sis and discuss their dermoscopic patterns.

Clinical, dermoscopic, and histopathological features oftwo patients with scalp sarcoidosis were reviewed.Dermoscopic images were obtained with a computerized,polarized-light videomicroscope (FotoFinderdermoscope�;Teachscreen Software GmbH, Bad Birnbach, Germany)with lenses at magnification factors of ·20–70 set at ·10increments. The epiluminescent mode was used. Springwater (Eau Thermale Avène, Boulogne, France) was usedas the interface solution.

Case reports

Case 1

A 20-year-old woman presented with a three-year historyof itching folliculitis-like lesions of the scalp. At clinicalexamination, she presented with small alopecic patchesand diffuse hair thinning on the occipital and left parietalregions, scalp erythema, and crusts (Fig. 1). Dermoscopyof the lesions showed decreased hair density, perifollicularand follicular yellowish to pale orange round spots, perifol-licular scaling, few dystrophic hairs, and telangiectasias(Fig. 2).

A 5-mm punch biopsy was performed. Vertical sectionsrevealed superficial sarcoidal granulomas consisting of lym-phocytes at the periphery of collections of a few epithelioidhistiocytes in the middle dermis, prominent edema, andvasodilatation of the papillary dermis (Fig 3a). Horizontalsections at the isthmus level showed the destruction offollicular units by sarcoidal granulomas (Fig. 3b). Periodicacid-Schiff stain was negative for hyphae and spores.Alcian blue stain at pH 2.5 showed no mucin deposits.

Chest x-ray, abdominal ultrasonography, and bloodexamination were normal. The patient was prescribed in-tralesional steroids and showed moderate improvement atthe three-month follow-up.358

International Journal of Dermatology 2011, 50, 358–361 ª 2011 The International Society of Dermatology

Case 2

A 60-year-old woman presented with asymptomatic pat-chy alopecia of two months’ duration (Fig. 4). She had ahistory of hilar lymphadenopathy for the last seven yearsand had submitted to repeated chest scan examinationswithout a definitive diagnosis. Clinical examinationshowed five infiltrated alopecic patches in the vertex andleft parietal region. The skin overlying the patchesappeared to be atrophic with an orange discoloration.

Dermoscopy of the lesions showed absence of follicularostia, diffuse yellowish to pale orange discoloration,perifollicular scaling, dystrophic hairs, and prominenttelangiectasias (Fig. 5).

A 5-mm punch biopsy was performed. Vertical sectionsrevealed the almost complete absence of follicles and con-fluent sarcoidal granulomas in the dermis and hypodermis(Fig. 6a). The papillary dermis showed severe edema andvasodilatation. Horizontal sections at the isthmus levelshowed scarring alopecia with the presence of scatteredminiaturized anagen follicles surrounded by epithelioidgiant cells (Fig. 6b). The dermis contained numeroussarcoid granulomas at all levels.

The patient was referred to a pneumologist for furtherfollow-up, and a systemic process was detected, withinvolvement of the lungs and lymphadenopathy. She wastreated with systemic steroids for lung sarcoidosis withno improvement of the alopecia.

Figure 2 Dermoscopy shows well-delimited, perifollicularand follicular, yellowish to pale orange round spots. Notethe presence of few dystrophic hairs. (Dermoscopic image;original magnification ·70)

(a)

(b)

Figure 3 (a) Vertical section: superficial sarcoidal granulomain the middle dermis consisting of lymphocytes at theperiphery of collections of a few epithelioid histiocytes.[Hematoxylin and eosin stain (H&E); original magnification·10.] (b) Horizontal section at the isthmus level: destructionof follicular unit by sarcoidal granulomas. (H&E stain; ·20)

Figure 1 Case 1. Small alopecic patches and diffuse thinning

ª 2011 The International Society of Dermatology International Journal of Dermatology 2011, 50, 358–361

Torres et al. Trichoscopy of scalp sarcoidosis Pharmacology and therapeutics 359

Discussion

Sarcoidosis may affect the scalp and may lead to scarringalopecia as a result of the destruction of the hair folliclesby the granulomatous formation.3–5 These two casesdemonstrate two different stages of scalp involvement insarcoidosis. The first illustrates initial disease with local-ized scalp involvement, and the second presents a latediagnosis with systemic involvement.

Although scalp involvement in sarcoidosis has beenreported to be more common in African-Americans,2 ourtwo patients were Caucasian.

In the patient with initial disease (case 1), sarcoidosispresented with folliculitis-like lesions that appeared

as well-defined, yellowish to pale orange, round spots atdermoscopy. These spots differ from the yellow dots ofalopecia areata as they are larger (at least three times thesize of the yellow dots) and do not correspond to follicu-lar openings. At pathology these orange spots corre-sponded to the round, well-formed granulomas in thesuperficial dermis.

In the patient with systemic involvement (case 2), scalpsarcoidosis presented with patchy alopecia. In this case,pathology showed follicular destruction by a granuloma-tous process affecting the deep dermis and hypodermis.

Figure 5 Dermoscopy shows the absence of follicular ostia,diffuse yellowish to pale orange discoloration, dystrophichairs, and prominent telangiectasias. (Dermoscopic image;original magnification ·70)

(a)

(b)

Figure 6 (a) Vertical section showing multiple inflammatorygranulomas in the papillary and middle dermis. The granulomascontain epithelioid histiocytes surrounded by a moderatelydense infiltrate of lymphocytes. Note prominent edema anddilated vessels in the papillary dermis. [Hematoxylin andeosin stain (H&E); original magnification ·10.] (b) Horizon-tal section at the isthmus level showing numerous sarcoidgranulomas and cicatricial alopecia with few remainingscattered miniaturized anagen follicles. (H&E stain; ·4)

Figure 4 Case 2. Infiltrated alopecic patch on left parietalregion

International Journal of Dermatology 2011, 50, 358–361 ª 2011 The International Society of Dermatology

Pharmacology and therapeutics Trichoscopy of scalp sarcoidosis Torres et al.360

Dermoscopy showed diffuse orange discoloration, and theindividualized round spots were less evident. In bothcases, dermoscopy showed prominent telangiectasias inthe scalp, which corresponded to vasodilatation in thepapillary dermis.

The presence of ‘‘orange spots’’ at dermoscopy in cuta-neous sarcoidosis was recently described by Pellicanoet al.,21 but there are no data about dermoscopy in scalpsarcoidosis in the literature.

The presence of dystrophic hairs was also interestingand probably correlates with the activity of the disease.

Clinical differential diagnosis in both cases includedcutaneous lymphoma, mucinosis follicularis, scalp discoidlupus erythematosus, Jadasshon nevus, xanthomatosis,and possible other causes of granulomatous formation,such as lipoidica necrobiosis, elastolytic granuloma, andinfectious diseases.1 Thus, this dermoscopic feature, desig-nated ‘‘orange spots’’, represents a clue to the diagnosisof scalp sarcoidosis.

Conclusions

The orange spots seen at trichoscopy of lesions in scalpsarcoidosis may represent a clue to the diagnosis of thiscondition. Dystrophic hairs may indicate granulomatousactivity.

References

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Acad Dermatol 2000; 42: 690–692.3 Golitz LE, Shapiro L, Hurwitz E, Stritzler R. Cicatricial

alopecia of sarcoidosis. Arch Dermatol 1973; 107: 758–760.

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5 Takahashi H, Mori M, Muraoka S, et al. Sarcoidosispresenting as a scarring alopecia: report of a rarecutaneous manifestation of systemic sarcoidosis.Dermatology 1996; 193: 144–146.

6 Henderson CL, Lafleur L, Sontheimer RD. Sarcoidalalopecia as a mimic of discoid lupus erythematosus.J Am Acad Dermatol 2008; 59: 143–145.

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13 Ross EK, Vincenzi C, Tosti A. Videodermoscopy in theevaluation of hair and scalp disorders. J Am Acad

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19 Inui S, Nakajima T, Shono F, Itami S. Dermoscopicfindings in frontal fibrosing alopecia: report of four cases.Int J Dermatol 2008; 47: 796–799.

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Dermoscopy of cutaneous sarcoidosis. Poster presentationat the Second Congress of the International DermoscopySociety, November 12-14, 2009, Barcelona.

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Torres et al. Trichoscopy of scalp sarcoidosis Pharmacology and therapeutics 361