Coexisting pustular and ulcerative pyoderma gangrenosum

ARTICLE

ejd.2011.1594

Auteur(s) : Sanae Nakano, Naoki Oiso naoiso@med.kindai.ac.jp, Akira Kawada

Department of Dermatology, Kinki University Faculty of Medicine, 377-2 Ohno-Higashi Osaka-Sayama, Osaka 589-8511, Japan

Pyoderma gangrenosum (PG) is a rare, non-infectious neutrophilic dermatosis that is frequently associated with a systemic disorder. It includes the classic ulcerative form and atypical pustular, bullous, and superficial forms. Pustular PG usually occurs as discrete painful pustules with a surrounding halo of erythema [1]. Acne fulminans (AF) is a rare severe form of acne vulgaris associated with systemic symptoms. AF develops suddenly with fever as highly inflammatory tender and ulcerative nodules covered with hemorrhagic crusts, mainly on the upper chest, back, shoulders, and face in young men [2]. Here, we describe a 34-year-old Japanese man showing pustular and ulcerative PG.

A 34-years-old Japanese man was referred to us in May 2010 with painful pustules on the face, upper chest, shoulders, and back, and subsequent painful ulcers on the left cheek and right chest. At the age of twenty-two, he was hospitalized because of multiple pustules on the jaw, nape, and trunk and a high fever. Fosfomycin calcium and cinamikacin sulfate were not completely effective. Dapsone 75 mg or 50 mg per day halted the eruptions for more than ten years. His medical history was otherwise unremarkable. His family history was negative for any chronic skin or other inflammatory disorders.

A physical examination revealed (i) painful swollen erythema with a central ulcer surrounded by pustules on the left cheek, (ii) a painful ulcer 12 cm in diameter with dark reddish undermined edges on the right chest, and (iii) discrete painful pustules with a surrounding halo of erythema on the face, upper chest, shoulders, and back (figures 1A-B). He had a high temperature, 39.0 ̊C.

Laboratory examination showed white blood cells 28,800/mm³ (neutrophils 88.4%, lymphocytes 2.3%, monocytes 6.0%, eosinophils 3.0%, and basophils 0.3%), platelets 51.4×10⁴/mm³, red blood cells 5.10×10⁶/mm³, and C-reactive protein 20.015 mg/dL. Repeated bacterial cultures showed no growth. A biopsied specimen from the pustule showed a massive neutrophilic infiltrate with suppurative abscess formation through the dermis to the epidermis (figures 1C-D). Systemic examination detected no arthritis, inflammatory bowel disease, monoclonal gammopathy, or suppurative hidradenitis.

Administration of imipenem hydrate and cilastatin sodium, and subsequently amikacin sulfate and fosfomycin calcium, was unsuccessful. On the basis of clinical and histological evidence, this patient was believed to have pustular and ulcerative PG without a systemic disorder. The administration of predonisolone 30 mg per day was effective.

The diagnosis of pustules was crucial. The developing lesions were consistent with AF. However, a recurrence of AF is extremely rare [2]. The evolution from pustules to ulcer may occur in pustular PG [1]. Dapsone has limited value in AF [2], but may be effective for PG [1]. The clinical and histopathological appearances, the age at recurrence, and the effectiveness of dapsone for more than ten years indicated the coexisting pusular and ulcerative PG.

Previously, Kurokawa et al. described a 30-year-old Japanese man who had a five-month history of AF and PG-like eruptions developed about 2 years later [3]. AF lesions were characterized by severe, painful, papulonodular eruptions on the face and upper trunk and PG-like lesions were shown as the peripherally enlarging ulcerative appearance on the bilateral lower legs [3]. The clinical and histological similarities are present in Kurokawa's and our cases. These two conditions may be a very close spectrum in the same disorder. Further accumulation of cases would elucidate if AF and pustular PG can be categorized in the same entity or overlapping disorders.

In summary, we describe a rare case showing the evolution from pustular to ulcerative PG. Immediate and accurate diagnosis is required to prevent the progression of pustular PG.

Acknowledgements

Financial support: none. Conflict of interest: none.

References

1. Cox NH, Jorizzo JL, Bourke JF, Savage COS. Vasculitis, neutrophilic dermatoses and related disorders. Pyoderma gangrenosum. In: Burns T, Breathnach S, Cox N, Griffiths C (eds). Rook's textbook of dermatology. Vol. 3. 8^th edn. West Sussex, Wiley-Blackwell John Wiley & Sons, 2010; 50.64-50.74.

2. Zaba R, Schwartz R, Jarmuda S, et al. Acne fulminans: explosive systemic form of acne. J Eur Acad Dermatol Venereol 2011 ; 25 : 501-507.

3. Kurokawa S, Tokura Y, Nham NX, et al. Acne fulminans coexisting with pyoderma gangrenosum-like eruptions and posterior scleritis. J Dermatol 1996 ; 23 : 37-41.