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  Table of Contents  
CASE REPORT
Year : 2018  |  Volume : 9  |  Issue : 4  |  Page : 256-258  

Polymorphous cutaneous sarcoidosis associated with peripheral vascular disease and its dermatoscopic findings


1 Department of Dermatology, Christian Medical College, Vellore, Tamil Nadu, India
2 Department of Pathology, Christian Medical College, Vellore, Tamil Nadu, India

Date of Web Publication2-Jul-2018

Correspondence Address:
Balakrishnan Nirmal
Department of Dermatology- Unit 1, Christian Medical College, Vellore, Tamil Nadu
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/idoj.IDOJ_315_17

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   Abstract 


Sarcoidosis is a multisystem disorder with cutaneous involvement with myriad of morphological presentations, often leading to diagnostic dilemma. We report a case of 31-year-old male with peripheral arterial disease who presented with three morphological forms of sarcoidosis simultaneously, namely, papular, psoriasiform, and pigmented purpuric dermatosis-like lesions. Dermatoscopy of cutaneous lesions showed yellow-orange globules, red dots, linear vessels, and white crystalline structures depending on the clinical forms. Histopathology of all three morphological types of skin lesions demonstrated sarcoidal naked granulomas.Sarcoid specific lesions of more than one morphological type presenting in a same patient is rare. Association of peripheral vascular disease with sarcoidosis is also seen rarely.

Keywords: Dermatoscopy, polymorphous, sarcoidosis, vascular


How to cite this article:
Nirmal B, George R, Telugu RB. Polymorphous cutaneous sarcoidosis associated with peripheral vascular disease and its dermatoscopic findings. Indian Dermatol Online J 2018;9:256-8

How to cite this URL:
Nirmal B, George R, Telugu RB. Polymorphous cutaneous sarcoidosis associated with peripheral vascular disease and its dermatoscopic findings. Indian Dermatol Online J [serial online] 2018 [cited 2020 May 28];9:256-8. Available from: http://www.idoj.in/text.asp?2018/9/4/256/235715




   Introduction Top


Sarcoidosis is a granulomatous disease that involves multiple organ systems. Cutaneous lesions are seen in around one third of the patients and are classified as specific and nonspecific lesions based on the presence or absence of sarcoidal granuloma, respectively. Specific lesions include macule, papule, plaque, subcutaneous nodules, ulceration, psoriasiform, lichenoid, ichthyosiform, vasculitic, verrucous, erythrodermic, lupus pernio, annular, and pigmented purpuric dermatosis-like lesions. Nonspecific lesions are reactive lesions which include erythema nodosum, erythema multiforme, prurigo, and calcification. The disease can masquerade as a variety of clinical presentations, and hence, is called a great imitator.


   Case Report Top


A 31-year-old male from Bangladesh presented with itchy hyperpigmented macules over both forearms and legs for three years, erythematous scaly plaques over abdomen and groins for two years, and skin colored papules over chest for one and half years. He also complained of claudication pain in the left calf and foot for past seven months. There was no history of discoloration of the toes, transient ischemic attacks, amaurosis, stroke, angina, abdominal pain, dyspnoea, or erectile dysfunction. He had no claudication of the upper limbs or right leg. He had been a smoker for 15 years. On physical examination, there were three types of skin lesions, viz., papules over chest, psoriasiform lesions over abdomen and groins, and pigmented purpuric dermatosis-like lesions over extremities. Peripheral pulse was absent on left popliteal and dorsalis pedis arteries. Ankle brachial pressure index for right and left legs were 1.06 and 0.46, respectively. Chest X-ray was unremarkable. Mantoux test was negative. Serum angiotensin converting enzyme level was raised. (152 U/l, normal range: 8–52 U/l). Serum calcium, phosphate, homocysteine, prothrombin time, activated partial thromboplastin time, and D-dimer levels were within normal range. Color Doppler showed complete block of left distal sapheno-femoral artery.

Dermatoscopic examination of skin lesions was done under 10× magnification using a pocket dermatoscope (Dermlite DL3, 3Gen Inc., USA) using liquid paraffin as interface fluid. Dermatoscopy of papular lesions showed yellow globules [Figure 1]. Psoriasiform plaque showed pale orange globules, white crystalline scar-like structures, linear vessels, and red blotches on dermatoscopy [Figure 2]. Pigmented purpuric dermatosis-like lesions showed yellow globules with accentuation of normal reticular pigmentary pattern around the globules and red dots within them [Figure 3]; [Table 1].
Figure 1: (a) Skin-colored papules over chest (papular); (b) dermatoscopy of papular lesion (10×) showing yellow globules (black arrow)

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Figure 2: (a) Erythematous plaque over abdomen (psoriasiform); (b) dermatoscopy of psoriasiform lesion (10×) showing pale orange globules (black arrow), white crystalline scar-like structures (star), linear vessels (white long arrow), and red blotches (black long arrow)

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Figure 3: (a) Hyperpigmented macules over lower limbs (pigmented purpuric dermatosis-like), (b) dermatoscopy of pigmented purpuric dermatosis-like lesion (10×) showing yellow globules (black arrow), red dots (white arrow), and accentuation of normal reticular pigmentary pattern (star)

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Table 1: Dermatoscopic findings of various cutaneous forms of sarcoidosis

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Skin biopsy from all three types of cutaneous lesions on histopathological examination showed epidermal atrophy with epitheloid granulomas in the superficial dermis comprising epitheloid histiocytes, Langhans type giant cells, and few lymphocytes without necrosis. Erythematous plaque from the abdomen in addition mild perivascular and focal periadnexal lymphocytes, histiocytes, and few multinucleate giant cells in the mid and deep dermis [Figure 4],[Figure 5],[Figure 6]. No acid fast bacilli was seen on special stain. Reticulin stain showed Preserved reticulin framework was revealed on reticulin stain.
Figure 4: Histopathology of papular lesion demonstrating ill-defined epithelioid granulomas in papillary dermis just beneath epidermis (a, H and E, ×40; b, H and E, ×100)

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Figure 5: Histopathology of psoriasiform lesion demonstrating ill-defined epithelioid granulomas in papillary dermis just beneath epidermis (a, H and E, ×40; b, H and E, ×100)

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Figure 6: Histopathology of pigmented purpuric dermatosis-like lesion demonstrating discrete non-caseating epithelioid granuloma in papillary dermis with epithelioid histiocytes and multinucleated giant cells (a, H and E, ×40; b, H and E, ×100)

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Medicine and vascular surgery consultations were sought to rule out systemic associations of sarcoidosis and expert management of left leg claudication. Patient was started on oral hydroxychloroquine sulfate 200 mg/day.alongwith oral prednisolone 30 mg/day which was tapered over 3 months with Inj Alprostadil 500 microgram infusion was administered once monthly through intravenous route alongwith oral aspirin 75 mg daily for his vascular complaints. Three months after initaiation of treatment, skin lesions resolved completely with improvement of claudication pain.


   Discussion Top


Sarcoidosis is a multisystem disorder with several distinct cutaneous findings. There are very few reports of different morphological types of cutaneous lesions in the same patient. Krasowska et al. reported three types of skin lesions, namely, subcutaneous nodules, annular plaques, and erythema nodosum-like skin lesions with multisystem involvement in a 56-year-old woman.[1] Pandhi et al. reported a 30-year-old woman with eyelid papules, palmar nodules, scar, and tattoo sarcoid.[2] Our patient had three specific presentations of sarcoidosis, namely, papules, psoriasiform plaques, and pigmented purpuric dermatosis-like macules. Of these three, pigmented purpuric dermatosis is one of the rarely reported morphological variant of cutaneous sarcoidosis.[3],[4]

Dermatoscopy of sarcoidosis is characterized by yellow-orange globules or structureless areas, linear vessels, and white lines between the orange globules.[5] The yellowish orange areas correspond histopathologically to granulomas and are also found in other granulomatous disorders such as lupus vulgaris, necrobiosis lipoidica, and cutaneous leishmaniasis. Although dermatoscopy may be helpful in ruling out other clinical mimickers of sarcoidosis, differentiation among the granulomatous disorders might be difficult with dermatoscopy alone. Orange translucent areas, white scar-like depigmentation are more suggestive of sarcoidosis, while a combination of pink, white, and yellow background suggests necrobiotic granuloma.[6]

Vascular system associations of sarcoidosis include cutaneous vasculitis involving large vessels, superficial thrombophlebitis, and venous thromboembolism.[7],[8],[9] The association of sarcoidosis with these vascular disorders can be either attributed to their common immune mediated etiology or vascular disease arising as a complication of sarcoidosis. As most previous reports have relied on vascular imaging rather than histopathology for diagnosis, definite reason behind this association remains elusive.,[10] The salient features of this case were the presence of polymorphous sarcoid specific cutaneous lesions in same patientand the association of peripheral vascular disease with sarcoidosis, both of these findings being rarely reported previously.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.



 
   References Top

1.
Krasowska D, Schwartz RA, Wojnowska D, Maćkiewicz B, Czelej D. Polymorphous cutaneous and chronic multisystem sarcoidosis. Acta Dermatovenerol Alp Panonica Adriat 2008;17:26-30.  Back to cited text no. 1
    
2.
Pandhi D, Sonthalia S, Singal A. Mortimer's Malady revisited: A case of polymorphic cutaneous and systemic sarcoidosis. Indian J Dermatol Venereol Leprol 2010;76:448.  Back to cited text no. 2
[PUBMED]  [Full text]  
3.
Mittal RR, Singh SP, Gill SS. Psoriasiform sarcoidosis associated with depigmentation. Indian J Dermatol Venereol Leprol 1996;62:103-5.  Back to cited text no. 3
[PUBMED]  [Full text]  
4.
Sezer E, Yalçın Ö, Erkek E, Şahin S. Pigmented purpuric dermatosis-like sarcoidosis. J Dermatol 2015;42:629-31.  Back to cited text no. 4
    
5.
Pellicano R, Tiodorovic-Zivkovic D, Gourhant JY, Catricalà C, Ferrara G, Caldarola G, et al. Dermoscopy of cutaneous sarcoidosis. Dermatology 2010;221:51-4.  Back to cited text no. 5
    
6.
Ramadan S, Hossam D, Saleh MA. Dermoscopy could be useful in differentiating sarcoidosis from necrobiotic granulomas even after treatment with systemic steroids. Dermatol Pract Concept 2016;6:17-22.  Back to cited text no. 6
    
7.
Rowland Payne CM, McGibbon DH. Sarcoidosis presenting as widespread thrombophlebitis. Clin Exp Dermatol 1985;10:592-4.  Back to cited text no. 7
    
8.
Goljan-Geremek A, Geremek M, Puscinska E, Sliwinski P. Venous thromboembolism and sarcoidosis: Co-incidence or coexistence? Cent Eur J Immunol 2016;40:477-80.  Back to cited text no. 8
    
9.
Liu Y, Li S, Cao J, Wang YX, Bi YL, Xu ZJ, Huang H. Concurrence of Sarcoidosis and Takayasu Aortitis. Chin Med J 2015;128:851-2.  Back to cited text no. 9
[PUBMED]  [Full text]  
10.
Ri G, Yoshikawa E, Shigekiyo T, Ishii R, Okamoto Y, Kakita K, et al. Takayasu arteritis and ulcerative cutaneous sarcoidosis. Intern Med 2015;54:1075-80.  Back to cited text no. 10
    


    Figures

  [Figure 1], [Figure 2], [Figure 3], [Figure 4], [Figure 5], [Figure 6]
 
 
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