CEDT Journal

1.       Introduction 

Cutaneous findings are observed in an estimated 30-40% of patients with sarcoidosis [1].  On exam, brown, red, or skin-colored infiltrative papules and plaques without overlying scale are the most often encountered lesions. However, a host of other clinical presentations exist either individually or in combination. As such, cutaneous sarcoidosis can often mimic a different disease process. We report a case of a 51-year-old female with cutaneous sarcoidosis with different clinical presentations. 

2.       Case Report 

A 51-year-old Caucasian female originally presented to the rheumatology clinic with a three-week history of painful swelling and “Knots” on her bilateral forearms and hands. The patient was referred to dermatology for evaluation. Her past medical history was significant for type II diabetes mellitus, Hashimoto’s thyroiditis and retinoblastoma of the right eye requiring enucleation as a child. 

On clinical examination, she was noted to have erythematous to violaceous indurated, large edematous plaques on the bilateral extensor arms and distal lower legs as well as deep firm nodules (Figure 1). The differential included morphea vs panniculitis vs eosinophilic fasciitis vs other deep dermal inflammatory process.

A punch biopsy was performed on the left proximal forearm and revealed granulomatous dermatitis with minimal surrounding inflammation (Figure 2). Fungal and mycobacterial stains were negative, and no polarizable foreign material was appreciated. Subsequently, a tissue culture was performed and was negative for organisms. 

Further work up included a chest x-ray which showed mild bibasilar atelectasis. She did have a slight elevation in her angiotensin converting enzyme level. A chest computed tomography scan was performed and showed mild septal thickening with 1-2 mm ground glass nodular opacities scattered throughout the bilateral lungs. There were also numerous enlarged mediastinal, hilar, and periportal lymph nodes. She was later evaluated by pulmonology who recommended pulmonary function tests. She was started on Plaquenil 200 mg twice daily. 

Two months later at clinic follow up, she developed the appearance of new erythematous papules diffusely scattered on her dorsal hands, forearms, and upper arms (Figure 3). Also noted, of new onset, were erythematous papules along an old scar on the left frontal hairline (Figure 4). She continued to complain of fatigue and joint pain in the wrists, knees, and hands, although reported to have improvement in the indurated plaques and nodules that she presented with initially. Biopsy of the new eruption also showed a granulomatous dermatitis with negative infectious stains.

The patient was continued on Plaquenil and also was given a three-week taper of prednisone. This almost completely cleared the papular eruption on the forearms. She continues to use topical Clobetasol for the remaining areas of induration. 

3.       Discussion 

The vast spectrum of morphologies associated with cutaneous sarcoidosis remains a diagnostic challenge (Table 1). The most common type of lesions is maculopapular with reddish-brown hyperpigmentation. Lupus pernio is characterized by chronic, violaceous papules or plaques affecting the central face. Lesions may also manifest as an enlarging, previously inactive scar.  Plaque-type sarcoidosis tends to be symmetric on extensor surfaces, often exhibiting thick scale that mimics psoriasis. Hypopigmented patches are seen with a central red papule giving them a “Fried-egg” appearance. Flat-topped, lichenoid lesions are rare, but predominant in children. Ulcerative forms may cause previous lesions to ulcerate or new lesions to appear on the lower extremities. Additionally, subcutaneous nodules, ichthyosiform, morpheaform, and erythrodermic manifestations have been reported [2]. The presence of non-caseating granulomas on histopathology will help confirm the diagnosis [3].

Prognosis, severity, and chronicity of sarcoidosis changes markedly depend on the type of lesion. Marcoval, Mana, and Rubio analyzed 506 patients with systemic sarcoidosis [4]. 80% of the patients had skin findings prior to or at the time of systemic disease diagnosis. Persistent, life threatening illness and increased need for systemic corticosteroid therapy was more likely involved with plaque type sarcoidosis. The patients that presented with papules in the study resolved in a mean 5.44 months, most clearing spontaneously. Interestingly, although their study had low power, there was a noticeable association between lupus pernio and bone involvement seen in 50% of cases. Additionally, patients with subcutaneous sarcoidosis showed a relatively good prognosis usually resolving in a mean of 10.10 months. Erythema nodosum, although non-specific, is associated with good prognosis. 

There are also specific constellations of findings that are seen in certain clinical variants of sarcoidosis. Chronic lung involvement, uveitis, and bone cysts must be considered with the appearance of violaceous infiltrative lesions of lupus pernio on the nose, cheeks and earlobes. Lofgren’s syndrome, linked with acute sarcoidosis, must be considered when erythema nodosum occurs simultaneously with hilar adenopathy, acute iritis, migrating polyarthritis and fever [1]. Blau syndrome, an autosomal dominant form of sarcoidosis caused by mutations in the NOD2 gene, initially presents with a scaly or nodular granulomatous dermatitis, but continues on with a clinical course featuring arthritis, uveitis, synovial cysts and camptodactyly [5].

In conclusion, sarcoidosis can present with several clinical morphological presentations and, as such, may pose a diagnostic challenge. Herein, we reviewed the clinical cutaneous presentations of this condition. Histopathology reveals non-caseating “naked” granulomas and is helpful in confirming the diagnosis. Given its systemic nature, diagnosis of cutaneous sarcoidosis is very important and may facilitate and guide further diagnostic workup and therapeutic planning.

Figure 1: View of an erythematous to violaceous plaque on the left proximal forearm with peaud’orange epidermal change. The biopsy site is marked in ink.

Figure 2: 4x views of deep granulomatous inflammation extending into subcutaneous tissue present on initial biopsy of sclerodermoid plaque from patient’s left forearm. Subsequent stains and tissue cultures were negative for infection.

Figure 3: Diffuse papular eruption on bilateral forearms and dorsal hands. Biopsy site marked in ink.

Figure 4: Small eruption of papules on the left frontal hairline around previous traumatic scar.

Table 1: Select Types of Cutaneous Sarcoidosis [1,2].

1.       Jain S (2012) Dermatology: Illustrated Study Guide and Comprehensive Board Review. New York, New York: Springer.

2.       Mañá J, Marcoval J (2012) Skin manifestations of sarcoidosis. Presse Medicale 41: 355-374.

3.       Vasaghi A, Kalafi A (2012) Unusual manifestation of cutaneous sarcoidosis: a case report of morphea form sarcoidosis. Acta Med Iran 50:  658-651.

4.       Marcoval J, Mañá J, Rubio M (2011) Specific cutaneous lesions in patients with systemic sarcoidosis: relationship to severity and chronicity of disease. Clinical and experimental dermatology 36: 739-744.

5.       Blau Syndrome. U.S. National Library of Medicine: Genetics.