A 20–year old female Moroccan patient with a history of a photosensitivy, presented lipoartophic hyperpigmented plaques in the abdomen, limbs buttocks and back. The lesions had appeared 12 months earlier. The dermatological examination revealed subcutaneous skin-colored nodules measuring 2-3cm in the upper limbs and buttocks, bilateral cervical and inguinal lymphadenopathy, a dyschromic plaque alopecia (7*4.5cm) with yellow dots, erythema and vellus hair, and one exclamation point at dermoscopy suggestive of alopecia areata. However a deep scalp biopsy and the skin biopsy of the lipoatrophic plaque of the limb down to the hypodermis showed an interface change, a dermal mucin deposit, epidermal atrophy with a lobular lymphocytic infiltrate with a positive direct immunofluorency (deposit of IgG, IgM and C3), these findings were consistent with a lupus erythematosus panniculitis (LEP). She was subsequently treated with hydroxychloroquine 350mg per day. After one-year therapy, we noticed no improvement and the patient tends to present more lesions. We decided to stop the hydroxychloroquine and do a second biopsy in a new nodule, and repeat the biopsy in the scalp, the histopathological examination revealed lymphocytes rim adipocytes, atypical perivascular and perilobular lymphocytic and histiocytic infiltrate, lymphocytes were least mildly enlarged and exhibit nuclear contour irregularity with a CD3+, CD2-, CD7+, CD8+, Granzyme B+,CD56-, CD20- on immunochemistry, Ki-67 stain exhibited a marking rate of approximately 15%. The whole body positron emission tomography (PET) scan showed subcutaneous hypermetabolic foci in the abdominal wall, thighs and the lower part of the back (SUV 5), the bone marrow and the lymphadenopathy biopsy were normal. As for the immunological investigations: the antinuclear antibodies were positive 1/160, DNA antibodies were positive at 28 (>10), her hemoglobin level was 10.4 g/dl, leukocyte count 3220/mm3 (leukopenia) with lymphopenia 1000/mm3, platelet count 197 000/mm3, her liver and renal function were normal. Based on the age, clinical presentation and the histopathological features and immunochemistry, we confirmed the diagnosis of a primary subcutaneous panniculitis-like T-cell lymphoma alpha-beta (SPTCL) associated with lupus erythematous panniculitis (LEP) and alopecia areata. We treated the patient with oral steroids 0.5mg/kg/day and topical minoxidil for her alopecia areata, After 4 months the patient didn’t present any new lesions and the subcutaneous nodules were completely resolved with area of regrowing hair in the scalp.
We present a rare case of SPTCL associated with lupus panniculitis. This case report illustrates the importance of renewing biopsies in patients with lupus panniculitis especially in the absence of disease control.