Mutilating Facial Sarcoidosis

Dumitrescu, S; Schwartz, Robert A.; Baredes, Soly; Whitworth, James; McDonald, Reynard J.; Zarbin, Marco A.; Langer, P. H.; Ho, A. F.; Lambert, W. Clark

doi:10.1159/000018262

Cited by 10 publications

(6 citation statements)

References 13 publications

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“…Mutilating sarcoidosis appears to be a severe form of lupus pernio, with large centrofacial tumors and extension into the oral and upper respiratory tissue that resemble Wegener granulomatosis and malignant pleomorphic lymphoma. 61 One prospective study of 54 South Africans with skin sarcoidosis found that one quarter of patients presented with hypopigmented (Fig 4), ichthyosiform, lymphedematous, mutilating, ulcerative, verrucous, and other atypical forms. 62 Psoriasiform (Fig 5), lichenoid (Fig 6), verrucous (Fig 7), and angiolupoid (Fig 8) are variants of papular or plaque sarcoidosis that can be confused with psoriasis, lichen planus, warts (or lichen simplex chronicus), or lupus erythematosus, respectively.…”

Section: Cutaneous Disease Key Pointsmentioning

confidence: 99%

Sarcoidosis: A comprehensive review and update for the dermatologist

Haimovic

Sanchez

Judson

et al. 2012

Journal of the American Academy of Dermatology

209

269

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Section: Cutaneous Disease Key Pointsmentioning

confidence: 99%

Sarcoidosis: A comprehensive review and update for the dermatologist

Haimovic

Sanchez

Judson

et al. 2012

Journal of the American Academy of Dermatology

209

269

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“…45,46 The most frequent clinical manifestation of the great imitator is its papular or nodular form (Fig. 53 Ulcerative sarcoidosis shows a predilection for young adults and most often affects women and individuals of African or Japanese descent. The patient's face rarely remains unaffected.…”

Section: Cutaneous Manifestationsmentioning

confidence: 99%

“…Its differential diagnosis includes leprosy, granulomatous rosacea, Wegener's granulomatosis, and malignant pleomorphic lymphoma. 53 Ulcerative sarcoidosis shows a predilection for young adults and most often affects women and individuals of African or Japanese descent. Ulcers tend to arise within existing papulonodules on the lower legs but may occur on previously normal skin with a generalized distribution.…”

Section: Cutaneous Manifestationsmentioning

confidence: 99%

Cutaneous sarcoidosis: an intriguing model of immune dysregulation

et al. 2014

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Sarcoidosis is a systemic granulomatous disease characterized by the presence of non-caseating granulomas. Its etiology remains obscure. A plausible hypothesis suggests that a complex interplay of host factors, infectious processes, and non-infectious environmental factors, matched with a susceptible genetic background, results in a pathway that leads to systemic granulomatous inflammation. Although presentations of sarcoidosis vary enormously, multi-organ involvement is a common feature. Cutaneous involvement occurs in about 25% of patients with protean manifestations and variable prognoses. Skin manifestations are divided into specific lesions with histopathologically evident non-caseating granulomas and nonspecific lesions arising from a reactive process that does not form granulomas. A peculiar form of cutaneous sarcoidosis is represented by sarcoidal lesions at sites of trauma that has caused scarring. The pathogenesis of scar sarcoidosis remains unknown. Scar sarcoidosis is also associated with herpes zoster infection, surgery, and tattooing. Such heterogeneous events, along with those at the sites of chronic lymphedema, thermal burns, radiation dermatitis, and vaccinations, occur on areas of vulnerable skin labeled "immunocompromised districts". Numerous options are available for the treatment of cutaneous sarcoidosis. Although corticosteroids remain the treatment of choice for initial systemic therapy, other nonsteroidal agents have proven effective and therefore useful for long-term management. Tumor necrosis factor-α antagonists such as infliximab may have a role in the treatment of cutaneous sarcoidosis, especially in refractory cases that are resistant to standard regimens. Elucidation of the relationship of sarcoidal granulomas with malignancy and immunity may facilitate a better understanding of some pathomechanisms operating in neoplastic and immunity-related disorders.

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“…Lupus pernio is characterized by violaceous or erythematous indured papules, plaques, or nodules that are primarily distributed on the central face [138]. The alar rim of the nose is often affected.…”

Section: E Lupus Perniomentioning

confidence: 99%

“…The alar rim of the nose is often affected. Lupus pernio is more common in women than in men and is associated with chronic disease and extrapulmonary involvement [138,139]. Without treatment, the lesions progressively increase in thickness, size, and induration, eventually resulting in considerable cosmetic disfigurement.…”

Section: E Lupus Perniomentioning

confidence: 99%

Clinical Manifestations of Sarcoidosis

Jara‐Palomares¹,

Caballero-Eraso²,

Gutiérrez³

et al. 2013

Sarcoidosis

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Type of involvement Comments Mucosal erythema and edema Nonspecific finding* Granular mucosa Nonspecific* Cobblestone mucosa More common in lobar and segmental bronchi* Mucosal plaques (yellowish) Also occurs in other disorders* Obstructive sleep apnea, which occurs in about 5% of the general population, seems to occur with increased frequency in patients with sarcoidosis, especially in patients with lupus pernio [18]. Sarcoidosis of the upper airways has been suggested as one of the possible mechanisms for sleep apnea in patients with sarcoidosis and lupus pernio, although the overwhelming majority of obstructive sleep apnea in sarcoidosis is most probably related to obesity from corticosteroids. Sarcoidosis of the supraglottic airways in children is rare [19]. 2.2. Larynx Laryngeal sarcoidosis often occurs as an isolated phenomenon and is usually attributed to asthma [20]. Occasionally, laryngeal sarcoid can lead to progressive life-threatening airway obstruction [15]. Laryngeal sarcoidosis is uncommon [21]. The incidence of laryngeal sarcoidosis is estimated to be about 1.2%. Laryngeal sarcoidosis could be treated with systemic and intralesional injections of a corticosteroid, surgical intervention, carbon dioxide laser ablation, and external beam radiation [22, 23]. Paralysis of the left vocal cord and hoarseness can occur from compression of the left recurrent laryngeal nerve by enlarged lymph nodes [24, 25]. Systemic corticosteroid therapy has resulted in resolution of the hoarseness [24]. 2.3. Central airways The trachea and main bronchi are less frequently affected than the lobar, segmental, subsegmental, and distal airways. Sarcoid granulomas of trachea, main carina, and major bronchi by themselves seldom produce significant obstructive symptoms or airway dysfunction [26, 27]. Cough is the main symptom. Symptoms, clinical examination, flow-volume curves, and bronchoscopy help in assessing the severity of the central airway stenosis [28]. Mainstem bronchial stenoses as well as segmental stenosis have been described in patients with sarcoidosis [29]. Disabling inspiratory and expiratory airflow limitation mimicking fixed upper airway obstruction has been reported [27]. Bronchoscopy may demonstrate other changes as: mucosal erythema, edema, friability, granularity, fine cobblestoning, and sarcoid nodules. The characteristic yellow waxy nodules typical of sarcoidosis are less likely to occur in the trachea and main bronchi, but when seen in these areas, they tend to be sparsely distributed. Extrinsic compression of the central airways by the enlarged mediastinal and hilar lymph nodes is uncommon. Right middle lobe syndrome caused by extrinsic compression and intraluminal sarcoidosis has been described [30]. 2.4. Distal airways Sarcoidosis could affect lobar, segmental, subsegmental, and more distal bronchi as well as bronchioles, which is manifested as mucosal inflammation, endobronchial granulomas, stenosis, extrinsic compression, distortion, bronchiectasis, bronchiolitis, airway hyperreactivity, and streak...

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Mutilating Facial Sarcoidosis

Cited by 10 publications

References 13 publications

Sarcoidosis: A comprehensive review and update for the dermatologist

Sarcoidosis: A comprehensive review and update for the dermatologist

Cutaneous sarcoidosis: an intriguing model of immune dysregulation

Clinical Manifestations of Sarcoidosis

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