A 42-year-old man, presented to the Dermatology clinic with a history of a pruritic rash that started on his upper chest and spread to his head in a photodistributed pattern. He also had severe bone pain, unexplained recurrent fevers, right upper quadrant pain, muscle aches, ankle joint pain, mild subjective exertional dyspnea and drenching night sweats that began 9-10 months after the onset of the rash. He reported that the eruption was exacerbated by sun exposure. Previous treatment included two, 2-week courses of 50 mg of prednisone. Partial improvement of the rash, occurred each time. Multiple courses of topical permethrin to treat suspected scabies did not help. Past medical history was remarkable only for alcohol-induced cirrhosis and psoriasis. His medications were venlafaxine, naproxen, and pantoprazole. He did not have any industrial chemical or radiation exposures, and his family history did not include any autoimmune diseases.
On presentation, he had photodistributed well-defined red-brown, annular and ovoid non-scaly plaques mainly involving the head, neck, shoulders, and upper chest (Figure 1(a)). There were also subtle well-defined, non-scaly red-brown macules on the abdomen and thighs. The tibia and ulna were tender to palpation bilaterally. There was no overt joint inflammation and no adventitial breath sounds. The differential diagnosis included granuloma annulare, subacute cutaneous lupus, infectious granulomas including leprosy, actinic granuloma, and mycosis fungoides.
Notable laboratory findings included a negative antinuclear antibody test, diffuse reactive hypergammaglobulinemia with no monoclonal bands and mild transaminitis with elevated gamma-glutamyl transferase. It was felt his abnormal liver enzyme tests were most likely secondary to his alcohol use. Skin biopsy of the lesions revealed superficial and deep non-necrotizing granulomatous dermatitis with scattering accompanying lymphocytes. There were no atypical lymphocytes, diffuse neutrophilic/lymphocytic infiltrates, dermal edema, mucin, or epidermal change (Figure 1(b)). The skin biopsy (Figure 1(c)) and a blood smear were negative for leprosy. A tuberculosis skin test was non-reactive. On imaging, a computed tomography (CT) of his chest and abdomen showed non-calcified pulmonary granulomas, while a bone scan demonstrated cortical uptake in the tibia suggestive of osseous sarcoidosis. Further testing of pulmonary function excluded restrictive lung disease. An echocardiogram and ophthalmological exam were unremarkable.
In light of his investigations, he was diagnosed with systemic sarcoidosis with photodistributed cutaneous involvement. Prednisone 40 mg daily with a slow taper and hydroxychloroquine 200 mg twice daily were started. Resolution of his cutaneous involvement, arthralgias, and bone pain occurred over 3 months. He is followed by a general internist to monitor for progression of his pulmonary disease and his other systemic manifestations. He has not had any adverse side effects and has had a sustained remission on antimalarial monotherapy.