Dear Editor,
Sarcoidosis is a granulomatous disorder of unknown etiology with protean manifestations. Its bewildering range of cutaneous presentations has earned it the name of a “great imitator” in dermatology. The skin is the second most commonly affected organ after the lungs. The other organs affected are the eyes, heart, central nervous system, kidneys, and to a lesser extent, the liver, spleen, and upper respiratory tract.[1]
A 49-year-old female presented with multiple pruritic skin lesions with scaling of 11 years duration and a recent history of exertional dyspnea. On examination, there were multiple discrete, well-defined erythematous papules and plaques, some with an annular configuration, with white scales and perifollicular hyperpigmentation distributed on the anterior and posterior trunk, upper limbs, and upper thighs [Figure 1a and b]. The largest plaque measured 10 cm × 15 cm extending from the lower neck to the upper posterior shoulder [Figure 1b]. On palpation, there was grade 2 sclerosis (indurated but pinchable) of all the skin lesions. There was no sclerosis of the intervening normal skin. Auspitz’s sign was negative. There were no mucosal lesions, and the systems were within normal limits. Diascopy was non-contributory. The clinical differential diagnoses considered were psoriasis, generalized morphea, and mycosis fungoides.
Figure 1.
(a). Multiple discrete erythematous scaly plaques with sclerosis on the anterior trunk, (b). Large scaly plaque on the lower neck and upper posterior shoulder
Blood hemogram, liver, and renal function tests were normal. Viral markers and serology for syphilis were negative. Anti-nuclear antibody (ANA) profile was negative. Serum calcium was 9.7 mg/dL (Normal 9 mg%–11 mg%) and serum angiotensin-converting enzyme (ACE) was elevated at 51 IU/L (Normal 5–33 IU/L). Chest X-ray showed reticular shadows and hilar lymphadenopathy. Pulmonary function test was normal. Mantoux test was negative. Sputum for acid fast bacilli (AFB) and CB-NAAT (Cartridge-based nucleic acid amplification test) for tuberculosis were negative. Skin biopsy demonstrated multiple discrete and confluent round, non-caseating, non-necrotic epithelioid and Langhan giant cell granulomas with paucity of peripheral lymphocytes (“naked granulomas”) confined to the upper and mid dermis [Figure 2a and b]. The mid and lower dermis showed homogenization and degeneration of the collagen suggestive of sclerosis. Special stains for fungi and AFB were negative. Culture for fungus and AFB was negative. High resolution computerized axial tomography of the thorax showed multiple small pulmonary nodules and enlarged, homogenously enhancing lymph nodes involving the bilateral upper and lower paratracheal, pre-vascular, subcarinal, paraaortic, subaortic, and right hilar stations, suggestive of pulmonary sarcoidosis Grade 1 [Figure 3]. We made a final diagnosis of morpheaform sarcoidosis (MS) with pulmonary sarcoidosis. The patient was started on 50 mg prednisolone and 400 mg hydroxychloroquine, with topical clobetasol cream and white soft paraffin. The patient showed a prompt response to therapy after 1 month and is on a tapering dose of prednisolone [Figure 4]. Three months after therapy, all the skin lesions healed with atrophic scarring. The respiratory symptoms improved, and a repeat computerised tomography (CT) Scan demonstrated that the lymph nodes had significantly reduced compared to the previous scan.
Figure 2.
(a). Skin biopsy showing multiple discrete and rounded “naked” epithelioid granulomas (vertical arrows) and homogenized, degenerated collagen in the lower dermis (horizontal arrow), H and E x100, (b). Langhan giant cell with paucity of peripheral lymphocytes (arrow), H and E x400
Figure 3.
High resolution CT scan showing enlarged lymph nodes suggestive of pulmonary sarcoidosis – Grade 1
Figure 4.
Prompt response that can be ascribed to prednisolone. She was prescribed prednisolone, hydroxychloroquine and topical clobetasol cream
MS is a specific cutaneous manifestation of sarcoidosis, as the histopathology shows non-caseating granulomas.[1] MS can be confused with morphea clinically due to the sclerosis, but the presence of granulomas in histopathology clinches the diagnosis, as granulomas are not seen in morphea or any other sclerosing disorders. Psoriasis does not present with sclerodermatous plaques and has specific histological features. Mycosis fungoides (MF) can present with sclerodermatous plaques. Clinically, these plaques are severely pruritic. Histopathology shows epidermotropism, Pautrier’s microabscess, and atypical cells in the dermis, none of which were seen in our patient. Sometimes, serial biopsies may be required to diagnose MF. The prompt response of the cutaneous lesions and pulmonary symptoms to systemic steroids ruled out MF in our case. The presentation of morpheaform lesions with systemic involvement should warrant investigations for sarcoidosis, as generalized morphea does not present with systemic symptoms. It is postulated that activated T-lymphocytes and macrophages from the sarcoid granulomas releases chemokines, which in turn causes secondary collagen degeneration and homogenization, leading to MS.[2] Females are more affected than males with MS, as the present report and other reports demonstrate. Ichthyosis, erythroderma, subcutaneous lesions, hypopigmented lesions, and ulceration are other rare cutaneous manifestations of sarcoidosis. A case of MS with verrucous morphology has also been reported in literature.[3] Once a diagnosis of cutaneous sarcoidosis is made, investigations should be done to detect systemic involvement, as systemic involvement requires steroids and other immunosuppressive therapy. A literature search revealed that the most common association of MS is with pulmonary sarcoidosis and polyarthritis.[4] In our case, the patient had pulmonary sarcoidosis. However, the presentation with MS cannot be related to an increased risk of pulmonary sarcoidosis or polyarthritis due to the small number of cases reported, unlike lupus pernio and plaque sarcoidosis, which are significantly associated with systemic involvement.[2,3] Whatever be the cutaneous manifestation of sarcoidosis, systemic involvement can occur later, and therefore periodic surveillance is required. The relation of MS with disease prognosis is also not known due to the paucity of case reports. MS is the rarest presentation of sarcoidosis. We came across only 11 previous reported cases in English literature [Table 1].[5,6,7] The therapy for MS is the same as for other cutaneous manifestations of sarcoidosis. Therapy is also dependent on the presence of systemic involvement. The skin-targeted therapies are clobetasol, tacrolimus, and intralesional triamcinolone. The systemic therapies are prednisolone (40 mg/day–60 mg/day), methotrexate, cyclophosphamide, mycophenolate mofetil, hydroxychloroquine, minocycline, pentoxifylline, thalidomide, leflunomide, allopurinol, isotretinoin, Psoralen Ultraviolet A (PUVA), infliximab, adalimumab, and recently, melatonin.[5,6,7] We are reporting a case of MS—the rarest presentation of sarcoidosis.
Table 1.
Case reports of morpheaform sarcoidosis in the literature
| Year | Author | Clinical features | Treatment |
|---|---|---|---|
| 1990 | Hess, et al. | Sclerosed plaques on the trunk and limbs + polyarthritis and pulmonary sarcoidosis | Prednisolone |
| 1998 (3 cases) | Burov, et al. | Sclerosed plaques on the thighs and buttocks + polyarthritis | Prednisolone |
| 2006 | Ginarte, et al. | Linear sclerosed plaques on the left lower leg | Prednisolone + hydroxychloroquine |
| 2010 | Choi, et al. | Sclerosed plaque on the left forearm | intralesional triamcinolone |
| 2010 | Jee, et al. | Sclerosed plaque on the forearm | Topical clobetasol |
| 2012 | Vasaghi, et al. | Indurated plaques on the face and forearm + pulmonary sarcoidosis | Prednisolone + hydroxychloroquine |
| 2021 | Hashemi, et al. | Atrophic plaque on the right lower leg and arthritis | Prednisolone + hydroxychloroquine + methotrexate |
| 2023 | Gowda, et al. | Linear sclerosed plaques on the limbs | Prednisolone + hydroxychloroquine |
| 2024 | Rau, et al. | Sclerosed verrucous plaques on the scalp, trunk, and limbs + pulmonary sarcoidosis, panuveitis | Prednisolone, methotrexate. clobetasol |
| 2024 | Present case | Sclerosed psoriasiform plaques on the trunk and limbs + pulmonary sarcoidosis | Prednisolone + hydroxychloroquine |
Conflicts of interest
There are no conflicts of interest.
Funding Statement
Nil.
References
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