Indian Journal of Pathology and Microbiology

: 2023  |  Volume : 66  |  Issue : 1  |  Page : 211--213

Subcutaneous sarcoidosis with a rare presentation of multiple nodules in lungs

Huiming Zhang, Xin Huang, Ying Zhou, Yi Zhan 
 Department of Dermatology, The Second Xiangya Hospital, Central South University, Changsha, Hunan; Hunan Key Laboratory of Medical Epigenomics, Changsha, Hunan, China

Correspondence Address:
Yi Zhan
Department of Dermatology, The Second Xiangya Hospital, Central South University, Changsha, Hunan - 410011
Ying Zhou
Department of Dermatology, The Second Xiangya Hospital, Central South University, Changsha, Hunan - 410011

How to cite this article:
Zhang H, Huang X, Zhou Y, Zhan Y. Subcutaneous sarcoidosis with a rare presentation of multiple nodules in lungs.Indian J Pathol Microbiol 2023;66:211-213

How to cite this URL:
Zhang H, Huang X, Zhou Y, Zhan Y. Subcutaneous sarcoidosis with a rare presentation of multiple nodules in lungs. Indian J Pathol Microbiol [serial online] 2023 [cited 2023 Mar 31 ];66:211-213
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Full Text

Dear Editor,

Subcutaneous sarcoidosis is an uncommon and special subgroup of cutaneous sarcoidosis.[1],[2] Patients with subcutaneous sarcoidosis usually don't involve other organs and cause severe complications.[3] Lung involvement by subcutaneous sarcoidosis is rarely reported in English literature. Here, we reported a rare case of subcutaneous sarcoidosis in which the lung and mediastinum are involved.

A 49-year-old female in good general health presented with a 2-month history of multiple subcutaneous nodules on her extremities. The patient developed swelling and pain in her left hand 2 months ago with no obvious inducement. Some painless nodules appeared after the swelling subsides, followed by several similar nodules arose on the back of her right hand, right wrist, right elbow, and right lower extremity. She denied cough, shortness of breath, fever, or any other systemic symptoms, and she had no positive family history. Her medical history was remarkable for hypertension and hypercholesterolemia.

Physical examination revealed multiple indurated subcutaneous nodules and mass on the dorsal aspect of two hands, extensor aspect of the right wrist, right elbow, and right thigh, measuring about 0.5–1.5 cm in size. The overlying skin was normal or slightly erythematous [Figure 1].{Figure 1}

Laboratory evaluations were notable for C-reactive protein 37.20 mg/L (reference range: 0–8 mg/L), erythrocyte sedimentation rate 34 mm/h (reference range: 0–20 mm/h), cholesterol 5.56 mmol/L (reference range: 2.9–5.2 mmol/L), Low Density Lipoprotein (LDL) 3.63 mmol/L (reference range: <3.12 mmol/L), and a normal level of angiotensin-converting enzyme (ACE). Other tests, including blood count, Interferon Gamma Release Assay (T-SPOT.TB), rheumatoid factor, antinuclear antibody, extractable nuclear antigen, anti-cyclic citrullinated peptide antibody, glomerular basement membrane antibody, myeloperoxidase antibody, proteinase 3 (PR3) antibody, rapid plasma reagin test, hepatitis B virus, hepatitis C virus, electrolytes, and liver and kidney function tests, were negative. High-resolution computed tomography (CT) scan of the chest revealed multiple small nodules in lungs. Enlarged lymph nodes were observed in the hilum of right lung and mediastinum [Figure 2].{Figure 2}

Histologic examination of a nodule obtained from right elbow showed numerous varying-sized epithelioid granulomas in the deep dermis and fat lobules, most of which are naked sarcoidal granulomas with scattered multinucleated giant cells [Figure 3]. No caseating necrosis was observed, and foreign bodies were excluded under polarized light microscopy. The acid-fast stain performed to exclude mycobacteria was negative.{Figure 3}

The patient received hydroxychloroquine 100 mg bid, thalidomide 50 mg bid, tripterygium glycosides 20 mg bid, and glycyrrhizin tablet 75 mg bid since the patient refused glucocorticoid therapy due to the side effects. The subcutaneous nodules became softer and smaller after 1-month treatment, whereas no improvement of the multiple small nodules and lymphadenopathy in lungs detected by CT scan was observed. After 3 months of treatment, all subcutaneous nodules disappeared. Due to the Corona Virus Disease 2019 (COVID-19) pandemic, the patient was followed up by telephone, and there was no recurrence for nearly 2 years.

Sarcoidosis is a multisystemic, noncaseating granulomatous disorder with unknown etiology. It mostly affects the lung, skin, and lymph nodes. Skin lesions occur in approximately 9–37% of patients. Cutaneous manifestations are diverse, including macules, papules, plaques, nodules, lupus pernio, erythema nodosum, vasculitic, psoriasiform, erythrodermic, scar-like, and subcutaneous sarcoidosis. Among them, subcutaneous sarcoidosis is an uncommon and special subtype, accounting for 1.4–6% of all cutaneous lesions.[1],[2] The first case was reported by Darier and Roussy in 1904; it is also known as Darier–Roussy sarcoidosis. The skin lesions in subcutaneous sarcoidosis mainly occurred on the extremities, rarely located on the head and trunk. They typically present as multiple, mobile, painless, firm, round subcutaneous nodules with intact overlying epidermis. The lesions can be single or multiple, with an average size of 0.5–2 cm.[1] Most past cases reported were middle-age female (78.3%).[4] Up to 68.3% of patients presented with elevated serum ACE levels,[4] which is considered as an index reflecting the activity of the disease and may serve as a potential marker for systemic diseases.[5] However, the ACE level lacks sufficient specificity and is not always correlated with the activity of the lung involvement.[6] Therefore, ACE is currently regarded as a reference for diagnosis. Differential diagnosis of subcutaneous sarcoidosis includes epidermal cysts, multiple lipomas, and rheumatoid nodules, which present similar subcutaneous nodules; therefore, histopathologic examination should be performed.[1] Subcutaneous sarcoidosis shows multiple noncaseating granulomatous nodules seated deeply in the subcutaneous. Infectious granulomatous diseases, such as mycobacterium and fungal infections in these contexts, should be excluded by special staining such as acid-fast staining, Periodic Acid-Schiff staining (PAS) staining, tuberculin skin test, and Interferon Gamma Release Assay (T-SPOT) test. Subcutaneous sarcoidosis is regarded to be the early stage of systemic sarcoidosis frequently associated with the other organ, but its association to lung involvement is rarely reported.[3] Systemic involvement including the lungs, the eyes, heart, and nervous system should be excluded. Patients with painless subcutaneous nodules usually don't have severe complications of sarcoidosis, such as pulmonary fibrosis, bone problems, and cysts. Even though it showed a good prognosis, long-term follow-up is still recommended.[2]

Our patient presented with multiple painless subcutaneous nodules in the extremities with rear findings of lung involvement. The serum ACE level, calcium, and T-SPOT test were all within normal limits. The personalized treatment for sarcoidosis should be based on the proceeding and the severity of the systemic organ involvement. For patients with severe systemic involvement or disfiguring skin lesions, systemic corticosteroid therapy should be considered. In patients with only cutaneous involvement, intralesional injection of corticosteroids is one of the options, but usually patients have limited improvement. Alternative therapies have been reported to be beneficial, such as methotrexate and hydroxychloroquine. Considering the side effects of corticosteroid, this patient was treated using hydroxychloroquine, thalidomide, tripterygium glycosides, and glycyrrhizin, and satisfactory results were shown.

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

The study was funded by the National Natural Science Foundation of China (No. 81703134) and Hunan Province Nature Science Foundation of China (2018JJ3772).

Conflicts of interest

There are no conflicts of interest.


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