1560 https://www.journal-imab-bg.org J of IMAB. 2017 Apr-Jun;23(2)

ABSTRACT:Background: Sarcoidosis is a chronic multisystem

granulomatous disease of unknown etiology, characterisedbytheformation of noncaseating granulomas in the involvedorgans, such as the skin, lung, lymph nodes, eyes, joints,brain, kidneys and heart. Cutaneous involvement is about25% with different clinical presentations, including papules,nodules, plaques, and infiltrated scars. The lichenoid pat-tern is one of the rarest types of cutaneous sarcoidosis.

Purpose: To present 50 –year-old woman diagnosedwith Sarcoidosis cutis lichenoides.

Material and methods: We present a 50-year-oldwoman in good general condition, with skin rashes local-ised on upper limbs. The disease started four months ago.From physical examination, pathological cutaneous lesionsaffected right and left forearms and cubital folds, presentedby papules of various sizes, pink in colour, smooth and shiny.Complete blood count, biochemistry, urine analysis andmicrobiology were in normal ranges except elevated levelsof alpha-1 globulin and gamma- globulin. X-rays examina-tion and abdominal ultrasound were normal too. Mantouxtest was negative. Histopathological examination demon-strated granulomas located in the papillary dermis andcomposedof epithelioid and giant cells, a few lymphocytes,without necrosis. Consultations with pulmonologist andophthalmologist showed no abnormalities.

Results: The diagnosis Sarcoidosis cutis lichenoideswas based on the medical history, general status, and mor-phological characteristics of skin lesions, histopathologicalexamination and consultations. In the absence of organ in-volvement, systemic therapy with Methylprednisolone andtopical treatment with Flumetasone pivalate 0.2mg/Salicylicacid 30mg/g was started. On the 5th day of treatment, werevealed initial resorption of papules. After discharging thepatient from the hospital, systemic therapy with Methylpred-nisolone and topical treatment with Tacrolimus 0.1% oint-ment was conducted. There were no new lesions 3 monthsafter the end of the therapy with Tacrolimus 0.1% ointment.

Conclusions: We present a rare case report of Sar-coidosis cutis lichenoides.

Keywords: Sarcoidosis cutis lichenoides, lichenoidpattern, tacrolimus.

INTRODUCTION:Sarcoidosis is a rare systemic multi-organ disease with

unknown aetiology. It is characterized bygranulomas,withoutcaseous necrosis,in the affected tissue ofthe lung, lymphnodes, brain, kidneys, heart, eyesand joints. Skin involve-ment is observed approximately 25% to 35% of cases andcan be detected at any stage of the disease, most commonlyoccurring in the early stages. Because of the variety of le-sions described in cutaneous sarcoidosis, diagnosis is a chal-lenge. Exclusion of many other diseases is required as thereis no single diagnostic test to prove this granulomatosis[1].We present a clinical case of cutaneous sarcoidosis in a50-year-old woman with lichenoid skin lesions. The finaldiagnosis of Sarcoidosis cutis lichenoides was based on theclinical and histological findings.

CASE REPORT:A 50-year-old woman was observed in the Department

of dermatology and venereology with a history of skin rashon her upper limbs. The skin eruption started 4 months be-fore hospitalisation and number and size of the lesions gradu-ally increased. The patient does not report itching. She ishaving pain from palpation of the rash. The patient has sec-ond degree chronic venous insufficiency, as an underlyingdisease. Physical examination does not reveal any pathologi-cal changes.Pathological skin changes haveaffected left andright forearms and cubital folds. They were presented bysmooth and shiny,pink coloured papular lesions with a di-ameter of 1-3 mm (Fig. 1, 2). The size and number of thepapules increased with diagnostic process. There wåre nopathological changes in the mucous membranes and skinappendages. Peripheral lymph nodes wåre not palpated.There was no evidence of synovitis and arthritis from themusculoskeletal system examination. Increased values ofthe following laboratory parameters were found: erythrocytesedimentation rate (32/70 mm), C-reactive protein (6.98 mg/ l), Alpha 1 globulin (8%), Gamma- globulin (21%) as wellas the ratio of T-helper / T-suppressors. From total bloodcount Stam-granulocytes was elevated (77.4%) and lympho-cytes were lowered (15%). There were no pathologicalfindingsfrom lung and heart X-ray and abdominal ultrasound.Mantoux test was negative. Histopathological examinationof the skin revealed atrophy of the epidermis andmultiple,superficially and deeply located, well-demarcated, non-caseating granulomascomposed of epitheloid histiocytes,

A CASE REPORT OF SARCOIDOSIS CUTISLICHENOIDES

Desislava Tsvetanova1, Ivelina A.Yordanova1,Verka Pavlova1, Tatyana Betova2,Dimitar K. Gospodinov1

1) Department of Dermatology, Venereology and Allergology, Faculty ofMedicine, Medical University Pleven, Bulgaria2) Department of General and Clinical Pathology, Faculty of Medicine, MedicalUniversity Pleven, Bulgaria

Journal of IMAB - Annual Proceeding (Scientific Papers). 2017 Apr-Jun;23(2):Journal of IMABISSN: 1312-773Xhttps://www.journal-imab-bg.org

https://doi.org/10.5272/jimab.2017232.1560

J of IMAB. 2017 Apr-Jun;23(2) https://www.journal-imab-bg.org 1561

eosinophils, plasma cells, lymphocytes and Langhans giantcells,in the dermis. (Fig. 3, 4).There were no pathologicalchanges from the consultations with pulmologist and oph-thalmologist. The dermatological, laboratory, radiographicand histological findings listed above have been consistentwith the diagnosis of the skin sarcoidosis. In the absence oforgan involvement, the patient initiated systemic therapywith Methylprednisolone at a dose of 40mg /daily for seven

days and topical treatment with Flumetasone pivalate 0.2mg/Salicylic acid 30mg/g. On the fifth day of treatment, we re-corded initial resorption of the papules. After the discharge,the patient continued the following treatment: systemicallywith Methylprednisolone tablets (for 14 days) and topicallywith Tacrolimus 0.1% ointment. There were no new lesionsafter three months of treatment with Tacrolimus 0.1% andthe old ones were completely resolved (Figure 5).

Fig. 1 and Fig. 2. Right and left forearms – lichenoid papules in cubital folds of right and left forearms.

Fig. 3. Skin sarcoidosis, Hematoxylin and eosin stain(HE) magn. x 25. Atrophy of the epidermis. Inflammatorygranulomatous reaction in the dermis with multiple well-demarcated, uniform in size and shape, superficially anddeeply located, noncaseating granulomas.

Fig. 4. Skin sarcoidosis, Hematoxylin and eosin stain(HE) magn. x 40. An epithelioid granuloma composed ofepitheloid histiocytes, plasma cells, few lymphocytes andLanghans giant cells.

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Fig. 5. Right forearm –completely resolving of thepapules three months after treatment with Tacrolimus ung.0.1%

with the absence of Wickham‘s striae, even if this patternis not specific for sarcoidosis. These homogeneus patchesindicate for a granulomatous skin disease [6]. Lichenoidlesions have rarely been reported in young children, oftenin combination with ocular and joint complications, butrespiratory system involvement is usually absent [7, 9].

The histological examination of Sarcoidosis revealsgranulomas without caseous necrosis, with epitheloid his-tiocytes, a different number of Langhans cells and maturemacrophagesin the skin, lungs and lymph nodes [8,10].They are surrounded by a small number of lymphocytic in-filtrates composed mainly of CD4 + T-lymphocytes andseveral CD8 + lymphocytes [11]. It is essential to use spe-cial stains in histological preparations and various culturesto exclude bacterial and fungal infections. The reason isthatsarcoid granulomas have no unique histological character-istics to differentiate them from other non-caseating granu-lomatous diseases [12].

Various therapeutic approaches are described inthetreatment of cutaneous sarcoidosis including local, intrale-sional and systemic corticosteroids, anti-malaric treatment,Methotrexate, Tetracycline, Thalidomide, Allopurinol,Isotretinoin and Infliximab. In the last decade, interest inthe use of topical calcineurin inhibitors in thetreatment ofcutaneous sarcoidosis has increased [7, 13, 14]. Tacrolimus,0.1% ointment, is an immunosuppressive agent, a macroli-deisolated from Streptomyces tsukubaensis. Tacrolimus hasa modulating effect on the T cell-mediated response andhasthe ability to suppress both hapten-induced productionof Th1 cytokines and the production of TNF-alpha [9, 15].Several cases of cutaneous sarcoidosis treated withTacrolimus 0,1 % ointment have been reported in the lit-erature [17,18,19]. Only one of them has reported alichenoidpattern [16].

CONCLUSION:In conclusion, we presented a rare clinical case of a

50-year-old woman with Sarcoidosis cutis lichenoides.There was no other organ involved in the patient. Treat-ment with Tacrolimus 0.1% ointment gave excellent results.The patient is under observation now.

DISCUSSION:Sarcoidosis is a chronic granulomatous multisystem

disease with unknown aetiology. The disease was describedforthe first time from Besnier in 1889. The author reports apatient in whom the relationship between red lesions onthe face and nose and swelling of the fingers is examined[2].

This diagnosis occurs with a slightly higher inci-dence in women than in men. Usually, patients are between20 and 40 years old[3]. Skin involvement was observed in25% of cases with different clinical manifestations [4].Skinrashes in sarcoidosis can be classified into two groups: spe-cific and non-specific [2].The lichenoid form ofSarcodidosis is in the group of specific lesions. This ex-tremely rare skin manifestation of the disease occurs in 1%-2% of cases of skin sarcoidosis[5]. Clinically, thelichenoid type is presented by multiple 1-3 mm, erythema-tous or violet maculopapular lesions, covered withfinesquams.Most common rashes affected extensor areas ofthe face, trunkand limbs andthe lesions could be singleor grouped, as in the case here presented. The dermoscopyusually revealedcircular or oval yellowish-brown lesions

1. Zhang JX, Kerbleski JF, GottliebAB. Manifestations of cutaneous sar-coidosis: a case report of an AfricanAmerican woman. J Natl Med Assoc.2009 Jun;101(6):614-7. [PubMed]

2. Besnier E. Lupus pernio de laface: Synovitesfongueuses symétri-ques des extrémités supérieures. AnnDermatol Syphil. 1889; 10:333-336.

3.Ishihara M, Ohno S, Ishida T,Ando H, Naruse T, Nose Y, et al. Mo-lecular genetic studies of HLA class IIalleles in sarcoidosis. Tissue Antigens.1994Apr;43(4):238-241.[PubMed]

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Address for correspondence:Desislava Tsvetanova,Department of Dermatology, Venereology and Allergology, Faculty of Medi-cine, MedicalUniversity - Pleven,91, Gen. Vladimir Vazov str., Pleven, BulgariaE-mail:desi_tzvetanova@abv.bg

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Please cite this article as: Tsvetanova D, Yordanova IA,PavlovaV, Betova T, Gospodinov DK. A case report of Sarcoido-sis cutis lichenoides. J of IMAB. 2017 Apr-Jun;23(2):1560-1563. DOI: https://doi.org/10.5272/jimab.2017232.1560

Received: 20/01/2017; Published online: 23/05/2017