Palisaded neutrophilic granulomatous dermatitis in systemic lupus erythematosus with a butterfly rash-like lesion

ARTICLE

Auteur(s) : Noriyuki Misago¹, Hisako Inoue¹, Takuya Inoue², Kohei Nagasawa², Yutaka Narisawa¹

¹Dermatology, Department of Internal Medicine, Faculty of Medicine, Saga University, Nabeshima 5-1-1, Saga 849-8501, Japan
²Rheumatology, Department of Internal Medicine, Faculty of Medicine, Saga University, Saga, Japan

A 31-year-old female presented with a relapsing fever of 39 °C and a rash of one week’s duration after sun-exposure. She had no history of medication. She displayed a butterfly rash-like lesion with infiltrated, erythematous plaques and papulosquamous lesions that were symmetrically distributed around the nose (figure 1A). She also presented with erythematous maculopapular lesions and plaques, which were symmetrically distributed on the lateral and extensor aspects of her upper arms (figure 1B). Laboratory investigations revealed an elevated anti-SSA/Ro antibody titer of 31.4 U/mL (normal 0-10). However, other immunological data were normal or negative. A biopsy specimen from an erythematous plaque on the right upper arm showed a diffuse or nodular infiltrate throughout the reticular dermis (figure 1C), which was primarily composed of histiocytes demonstrating interstitial and palisaded patterns with piecemeal fragmentation and sclerotic collagen (figure 1D). A substantial number of neutrophils were intermingled with the histiocytes. These histopathological features were diagnostic of palisaded neutrophilic granulomatous dermatitis (PNGD); however, mild to moderate interface dermatitis showing vacuolar, hydropic degeneration with scattered lymphocytes and melanophages in the papillary dermis was also present (figure 1E).

With the diagnosis of PNGD associated with unclassifiable autoimmune disease, the treatment consisted of rest without medication and all symptoms, including PNDG, spontaneously resolved after one week. Five years after her first visit, the patient presented again with a fever of 39 °C and a rash after sun-exposure and overwork. She displayed similar clinical features to those present at her first visit, namely, a butterfly rash-like lesion and symmetric, erythematous maculopapules and plaques on the upper arms. A biopsy specimen from a lesion on the upper arm once again showed the diagnostic features of PNGD associated with moderate interface dermatitis. However, laboratory investigations revealed leucocytopenia (2.3 × 10³/μL), positive antinuclear antibodies (1:160) with a speckled pattern, a decreased C4 of 7 mg/dL (normal 13-35), and the presence of anti-Smith (244 U/mL, normal 0-10) and anti RNP antibodies (169 U/mL, normal 0-10), which confirmed the diagnosis of SLE. A decreased C3 level was later seen during the follow-up. The fever and rash, including recurrent PNGD, did not respond to treatment with 20 mg/day of prednisolone, but resolved with 40 mg/day.

PNGD is a rare dermatological condition which shows various clinical features [1-3]. PNGD is characteristically associated with systemic diseases [1-3], particularly autoimmune conditions. There are a few reported cases of PNGD in patients with SLE [4-6], and PNGD is considered to be a non-specific cutaneous manifestation of SLE rather than an SLE-specific cutaneous manifestation (such as a butterfly rash). The butterfly rash-like lesion, which developed twice with PNGD in this patient, is interesting. Two possibilities have been considered, namely an SLE-specific butterfly rash associated with PNGD, or PNGD clinically manifesting as a butterfly rash-like lesion. As no skin biopsy was taken from the face lesion, it is impossible to distinguish between these two possibilities. It is interesting that the recurrent PNGD on the upper arm showed an association histopathologically with interface dermatitis, demonstrating that the lesions in this patient developed under combined (PNGD and SLE) pathogenic pathways. Although specimens of the recurrent PNGD were not available for immunofluorescence staining in this case, a previous case of PNGD associated with SLE demonstrated linear deposits of C3 at the basement membrane [4].

Whether the appearance of PSGD in an autoimmune disease is associated with a worse therapeutic response and/or a poor prognosis has not yet been clarified [4-6]. This case was notable for the presence and continued activity of an underlying disease, which was finally clearly identified as SLE.

Acknowledgements

References

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