Nail unit involvement during severe initial pemphigus vulgaris development

ARTICLE

Auteur(s) : Zoi Apalla, George Chaidemenos, George Karakatsanis

First Dermatologic Department, Aristotle University Thessaloniki, 124.Derfon str., 54643 Thessaloniki, Greece

We describe the case of a 70-year-old woman who presented with a 2-month history of painful confluent oral erosions, denuded epithelium and moist crusts on the upper and lower lips, gingival, buccal and palatine mucosa. Erosive, partially crusted lesions and intact vesicles and bullae were present on her neck, inframammary creases and periauricular area. Inflammation, suppuration, pemphigus-like blisters and erythematous fluctuant swelling at proximal and lateral nailfolds of all 4 extremities were observed. The big toenails were more intensely affected (figure 1). Yellow discoloration of the nail plate, foul-smelling nail bed and onycholysis resulting in shedding of nails were also noticed. The anal, vaginal and ocular mucosa were not affected. According to her medical history, nail alterations preceded the skin and oral lesions, which manifested one week later. A Tzanck smear from the oral mucosa revealed the presence of acantholytic cells. A skin biopsy from the affected periungual area demonstrated suprabasilar acantholysis typical of pemphigus vulgaris. Direct immunofluorescence from the same region reported an intercellular epidermal deposition of IgG and C3. High titers of IgG circulating intercellular antibodies were detected using indirect immunofluorescence techniques. Direct microscopic examination and cultures obtained from the nail fold exudate were negative for fungus or bacteria. The clinical and laboratory investigations confirmed the diagnosis of pemphigus vulgaris. Treatment with immunosuppressive agents, including a combination of systemic corticosteroids at an initial dose of 80 mg/day and cyclophosphamide at a dose of 100 mg/day, resulted in a complete remission of both cutaneous and nail lesions, over a 4-month-period.

Pemphigus lesions on the skin of distal digits surrounding the nail plate are not unusual and are often mentioned in standard textbooks [1]. However, primary, acute nail changes as a part of the disease initiation constitute a rare phenomenon that has been infrequently reported [2]. Fingernails, especially the nails of the thumbs and index fingers, are more often involved than toenails. Onychomadesis, onycholysis, cross-ridging, pitting, Beau’s lines, subungual hemorrhage, paronychia, nail dystrophy, and nail plate discoloration are the abnormalities more often seen [2-4]. Shedding of the nail plate can occur for two possible reasons: an impairment of the nail matrix may prevent new nail formation and thus loosen the existing nail plate, or a subungual blistering process, as a result of acantholysis, may detach the nail plate from the underlying nail fold [4]. The nail unit involvement can be a part of the initial presentation of the disease or occur during a flare, along with other lesions. According to the results of recent clinical investigations, nail changes in patients with pemphigus vulgaris do not correlate to the duration or severity of the disease [5].

Laboratory investigations, including biopsy of the nail bed, matrix, or folds for routine histological examination and DIF, are usually helpful to confirm the diagnosis. However, taking into account that these procedures cause significant discomfort to the patient, they are recommended only in cases of absence of other skin lesions or when the only clinical signs of the disease are the nail alterations. In addition, cultures of nail samples are useful in order to exclude other causal factors or the presence of a secondary, fungal or bacterial infection. It remains unclear which factors determine the location of pemphigus lesions, but it may correlate with a different distribution pattern of pemphigus vulgaris antigen [6]. It is possible that the nail bed may represent a distinctive antigenic target region for the circulating auto-antibodies. Systemic therapy is warranted, as topical treatment is insufficient, and in the majority of cases, results in complete nail recovery, with no permanent damage or disfigurement.

Acknowledgements

Références

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6 Ioannides D, Hytiroglou P, Phelps RG, Bystryn JC. Regional variation in the expression of pemphigus foliaceous, pemphigus erythematosus, and pemphigus vulgaris antigens in human skin. J Invest Dermatol 1991; 96: 159-61.