A Case of Lupus Miliaris Disseminatus Faciei After Allogeneic Hematopoietic Stem Cell Transplantation
Lupus miliaris disseminatus faciei (LMDF) is a rare granulomatous skin condition characterized by the presence of small, reddish-brown papules predominantly on the face. The etiology of LMDF remains unclear, with various theories suggesting associations with Mycobacterium tuberculosis infection, granulomatous rosacea, and immune-mediated reactions to pilosebaceous units. This article presents a detailed case of LMDF occurring in a 43-year-old man following allogeneic hematopoietic stem cell transplantation (HSCT), highlighting the clinical presentation, diagnostic workup, histopathological findings, and therapeutic management.
The patient, a 43-year-old man, was diagnosed with acute T-cell lymphoblastic leukemia and underwent allogeneic HSCT using a 6/6 human leukocyte antigen (HLA)-matched donor, his sister. To prevent graft-versus-host disease (GVHD), he received cyclosporine A for five months. Three months after discontinuing cyclosporine A, he developed multiple small, dome-shaped, reddish-brown papules on the central area of his face, extending to both cheeks, lower eyelids, and chin. Notably, there were no extra-facial lesions. The clinical presentation is illustrated in Figure 1A.
A thorough physical examination was conducted, followed by dermoscopy, which revealed dotted and linear vessels arranged horizontally and vertically, forming a polygonal pattern. The dermoscopic mode used was polarized light, non-wetting, as shown in Figure 1E. Diascopic examination of the papules demonstrated an apple-jelly appearance, a characteristic feature of granulomatous lesions.
Histopathological analysis of the skin biopsy revealed dermal epithelioid cell granulomas with central areas of caseation necrosis and Langhans giant cells surrounded by a moderate lymphohistiocytic infiltrate, as depicted in Figures 1C and 1D. An acid-fast stain was performed on the pathology samples, and no mycobacteria were detected. This finding ruled out tuberculosis as a potential cause of the granulomas.
Laboratory investigations were conducted to further evaluate the patient’s condition. These included a full blood count, erythrocyte sedimentation rate, serum calcemia, serum angiotensin-I-converting enzyme level, blood chemistry, tuberculosis infection T cell spot test (T-SPOT), and a chest X-ray. All results were either normal or negative, reinforcing the diagnosis of LMDF and excluding other systemic granulomatous diseases such as sarcoidosis or tuberculosis.
Interestingly, both the patient and his sister, the HSCT donor, had a history of rosacea and LMDF. This familial predisposition raises questions about the potential genetic or immune-mediated factors contributing to the development of LMDF. The patient’s condition emerged after the discontinuation of cyclosporine A, suggesting that the immunosuppressive therapy may have played a role in modulating the immune response and preventing the onset of LMDF.
The pathogenesis of LMDF is not fully understood, but several hypotheses have been proposed. One theory suggests that LMDF may be a variant of Mycobacterium tuberculosis infection, although no definitive evidence supports this claim. Another hypothesis posits that LMDF is a form of granulomatous rosacea, but it differs from typical granulomatous rosacea in its lack of vascular symptoms, extra-facial involvement, and self-limiting course with scar formation. The granulomas in LMDF are thought to result from an immune response to pilosebaceous units, possibly triggered by an allergic reaction to foreign bodies or follicular contents such as keratins and sebum. In 2015, Nishimoto et al. proposed that cell-mediated immunity to Propionibacterium acnes (P. acnes) may contribute to granuloma formation. According to this theory, P. acnes enters the dermis following the destruction of hair follicles, leading to a granulomatous reaction.
In this case, the histopathological findings of dermal epithelioid cell granulomas with central necrosis and lymphohistiocytic infiltrate, combined with negative tuberculosis tests, support the hypothesis of an immune-mediated reaction rather than an infectious etiology. The development of LMDF after HSCT further suggests that immunological derangements following transplantation may play a role in the pathogenesis of this condition.
The patient was treated with a combination of oral isotretinoin (10 mg/day) and topical tacrolimus (applied twice daily). Additionally, he received intramuscular injections of 1 mL of compound betamethasone once a month for the first three months. This therapeutic regimen resulted in significant clinical improvement, as shown in Figure 1B, where the papules had mostly disappeared, leaving behind scars.
The management of LMDF remains challenging due to its unclear etiology and variable response to treatment. Various therapeutic approaches have been attempted, including intra-lesional or systemic corticosteroids, tetracyclines, metronidazole, erythromycin, dapsone, anti-tuberculous antibiotics, chloroquine, and clofazimine, with mixed results. In this case, the combination of oral isotretinoin and topical tacrolimus proved effective, highlighting the importance of individualized treatment strategies based on the patient’s clinical presentation and response to therapy.
In conclusion, this case report underscores the complexity of LMDF and its potential association with immunological alterations following allogeneic HSCT. The patient’s history of rosacea and LMDF, along with the familial predisposition, suggests a possible genetic or immune-mediated component in the pathogenesis of this condition. The successful treatment with oral isotretinoin and topical tacrolimus provides valuable insights into the management of LMDF, although further research is needed to elucidate the underlying mechanisms and optimize therapeutic approaches.
doi.org/10.1097/CM9.0000000000000411
Was this helpful?
0 / 0