*3.1.1.1.4 Scar sarcoid*

Scar sarcoid presents as erythematous, cutaneous, or subcutaneous swelling in the area of an old scar or beside a scar and the development of papules and nodules within the original scars [26]. Scar sarcoidosis can occur on skin sites damaged by a range of factors, including mechanical injuries, venipuncture,

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**Figure 3.** *Lupus pernio.*

*Clinical Manifestations of Sarcoidosis and Granulomatous Disorders*

intramuscular injections, inoculations, tattoos, and infections such as herpes zoster [27] (**Figure 4**). Foreign material within the scar, deposited by external factors including those stated above, is a possible cause of epithelioid granuloma [28]. The specific skin lesions that occur and the resulting sarcoidosis may be associated with the severity and duration of the disease, with scar sarcoidosis often being accompanied by systemic involvement [29]. Alterations, such as further damage or stress to the existing scars, often prompt worsening of sarcoidosis [30]. Scar sarcoidosis can appear at the onset of disease and must be looked for whenever a diagnosis of sarcoid is considered [8]. However, more commonly it is associated with longstanding pulmonary and mediastinal involvement, uveitis, peripheral lymphadenopathy,

Peak incidence of subcutaneous sarcoid is the fourth decade of life; females are more affected than male; these are asymptomatic to slightly tender subcutaneous lesions typically involving the upper extremities. Majority lessons are erythematous (57%), followed by skin colored (30%), hypopigmented, or violaceous [7, 10] (**Figure 5**). Lesions are usually multiple, clustered, and bilaterally asymmetrical [32]. There are autoimmune disease associations in a subset of patients. There is strong association with a systemic disease component at the outset of disease, notably bilateral hilar adenopathy; noninfectious panicular sarcoidal or epithelioid granulomas with minimal lymphocytic inflammation; and a favorable response to

A wide variety of different cutaneous manifestations have been reported, and

**Annular sarcoid.** Annular lesions are well-recognized forms of cutaneous sarcoidosis, amounting to around 8% of all skin lesions according to a recent study

*DOI: http://dx.doi.org/10.5772/intechopen.92236*

bony cyst, and parotid infiltration [9, 11].

oral corticosteroid therapy [31].

*3.1.1.1.5 Subcutaneous sarcoid (Darier-Roussy sarcoid)*

*3.1.1.1.6 Less common form of cutaneous sarcoidosis*

the clinical pictures are highly heterogeneous.

**Figure 2.** *Plaque sarcoid.*

*Clinical Manifestations of Sarcoidosis and Granulomatous Disorders DOI: http://dx.doi.org/10.5772/intechopen.92236*

intramuscular injections, inoculations, tattoos, and infections such as herpes zoster [27] (**Figure 4**). Foreign material within the scar, deposited by external factors including those stated above, is a possible cause of epithelioid granuloma [28]. The specific skin lesions that occur and the resulting sarcoidosis may be associated with the severity and duration of the disease, with scar sarcoidosis often being accompanied by systemic involvement [29]. Alterations, such as further damage or stress to the existing scars, often prompt worsening of sarcoidosis [30]. Scar sarcoidosis can appear at the onset of disease and must be looked for whenever a diagnosis of sarcoid is considered [8]. However, more commonly it is associated with longstanding pulmonary and mediastinal involvement, uveitis, peripheral lymphadenopathy, bony cyst, and parotid infiltration [9, 11].

#### *3.1.1.1.5 Subcutaneous sarcoid (Darier-Roussy sarcoid)*

Peak incidence of subcutaneous sarcoid is the fourth decade of life; females are more affected than male; these are asymptomatic to slightly tender subcutaneous lesions typically involving the upper extremities. Majority lessons are erythematous (57%), followed by skin colored (30%), hypopigmented, or violaceous [7, 10] (**Figure 5**). Lesions are usually multiple, clustered, and bilaterally asymmetrical [32]. There are autoimmune disease associations in a subset of patients. There is strong association with a systemic disease component at the outset of disease, notably bilateral hilar adenopathy; noninfectious panicular sarcoidal or epithelioid granulomas with minimal lymphocytic inflammation; and a favorable response to oral corticosteroid therapy [31].

#### *3.1.1.1.6 Less common form of cutaneous sarcoidosis*

A wide variety of different cutaneous manifestations have been reported, and the clinical pictures are highly heterogeneous.

**Annular sarcoid.** Annular lesions are well-recognized forms of cutaneous sarcoidosis, amounting to around 8% of all skin lesions according to a recent study

**Figure 4.** *Scar sarcoid.*

from India [32]. Papular lesions may coalesce or be arranged in annular patterns, usually with a red-brown hue. Lesions are indurated, have central clearing with hypopigmentation, and atrophy and scarring may occur (**Figure 6**). Usually, the photo-exposed areas that are affected have a predilection for the face, forehead, and neck. Alopecia may occur in the center of the lesions [32, 33].

**Angiolupoid sarcoidosis.** Angiolupoid sarcoidosis is an infrequent variant of the disease, affecting 8% of patients with cutaneous sarcoidosis [32]. It manifests clinically as single plaques with central hypopigmentation, which eventually acquire annular shape with prominent telangiectasias, preferentially located on the face, ears, or scalp [32] (**Figure 7**). It is usually present in women [34]. The cutaneous manifestation may be the first sign of a systemic sarcoidosis with the lung (stage 2) and gland involvement that are refractory to several conventional drug therapies [34].

**Hypopigmented sarcoid.** Dermal nodules with surrounding hypopigmentation and macular hypopigmented areas occur predominantly on the limbs. They may be tender but have no associated anesthesia [35].

In the nodules, the pigment is retained in the center where the color is dark redbrown. The lesions are ill-defined, and perifollicular pigment is usually retained at the periphery. Nodules are unattached to underlying structures and tender when pressed or squeezed [35]. Sarcoid granuloma present in all nodules but in half of macules [36].

**Morphea-like lesions.** Clinical features are indistinguishable from those of true morphea, and the cutaneous lesions may precede or arise years after the extracutaneous sarcoidosis [37]. Lesions are coalescing hyperpigmented atrophic plaques, may be indurated, usually in limbs, mainly lower limbs [38] (**Figure 8**).

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**Figure 6.** *Annular sarcoid.*

**Figure 5.**

*Subcutaneous sarcoidosis.*

*Clinical Manifestations of Sarcoidosis and Granulomatous Disorders*

*DOI: http://dx.doi.org/10.5772/intechopen.92236*

*Clinical Manifestations of Sarcoidosis and Granulomatous Disorders DOI: http://dx.doi.org/10.5772/intechopen.92236*

**Figure 5.** *Subcutaneous sarcoidosis.*

**Figure 6.** *Annular sarcoid.*

**Figure 7.** *Angiolupoid sarcoid.*

**Psoriasiform sarcoidosis.** It is a rare morphologic manifestation of sarcoidosis. The appearance of its lesion are psoriasiform and infiltrated (**Figure 9**); some were annular, and others followed the natural lines of cleavage of the skin [39]. This type of sarcoidosis is usually peculiar to the dark skin [40].

**Lichenoid sarcoidosis.** About 1–2% of all cases of skin sarcoidosis are in this variety. It is characterized by abundant pinhead-sized yellowish lesions closely grouped in round or oval clusters, slightly scaling, mimicking lichen planus [41]. Lesions can occasionally show superficial scaling (**Figure 10a, b**). Sites commonly involved include trunk, limbs, and face [42]. It appears symmetrically and in crops and is associated with the eye and joint complications [41, 43]. The dermoscopy findings usually reveal circular or oval yellowish brown lesions with the absence of Wickham's striae. This feature is not specific for sarcoidosis, but such homogeneous appearance of lesions indicates a granulomatous skin disease [44].

**Ulcerative sarcoidosis.** Ulcerative sarcoidosis usually develops from the papulonodular sarcoid lesions by ulceration, but some arose de novo. Ulcers also developed in psoriasiform, atrophie, lymphedematous, erythrodermic, verrucous, suppurative, and elephantine lesions [45]. Lesions usually occur in the lower limb, and the upper limb may also be affected; sometimes lesions are generalized [45, 46]. Ulcers tended to heal with scarring [46] (**Figure 11**). Women are affected three times more than men, and blacks are affected slightly more than whites. Ulcer is a presenting feature in nearly 30% of patients, half of them having an initial lesion of other types [45], and cutaneous lesions are usually the presenting sign of sarcoidosis [45].

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**Figure 9.**

*Psoriasisform sarcoidosis.*

**Figure 8.**

*Morpheaform sarcoidosis.*

*Clinical Manifestations of Sarcoidosis and Granulomatous Disorders*

*DOI: http://dx.doi.org/10.5772/intechopen.92236*

*Clinical Manifestations of Sarcoidosis and Granulomatous Disorders DOI: http://dx.doi.org/10.5772/intechopen.92236*

**Figure 8.** *Morpheaform sarcoidosis.*

**Figure 9.** *Psoriasisform sarcoidosis.*

**Figure 11.** *Ulcerative sarcoidosis.*

**Verrucous sarcoidosis.** All reported patients of verrucous sarcoidosis have been of African descent with a longstanding systemic disease. Usually, systemic sarcoidosis of other internal organs is present [47]. Lesions may be multiple exophytic, extensively verrucous yellowish-to-whitish lesions with a reddish-brown circinate border, size ranging from 1.0 to 2.0 cm in diameter [48].
