Adam Reich, Beata Wiśnicka and Jacek C. Szepietowski

Department of Dermatology, Venereology and Allergology, Wroclaw Medical University, Ul. Chalubinskiego 1, PL-50-368 Wrocław, Poland. E-mail: adi_medicalis@go2.pl

Accepted February 25, 2008.

Sir,

We read with great interest the case report by Carducci et al. (1) about nail findings in pemphigus vulgaris (PV). Nail lesions in PV are only sporadically reported in the literature; in our opinion this problem is underestimated. In the study presented by Schlesinger et al. (2) 30 of 64 patients (47%) with PV had concomitant nail abnormalities. In the control group only 2 of 64 subjects demonstrated nail lesions (2). Based on this report it seems that nail lesions are much more common in patients with PV than was previously thought (3). We suggest that the presence of some subtypes of nail abnormalities in PV patients may be connected with a more severe course of the disease and could therefore be useful in estimating the prognosis of PV subjects. In support of this theory, we describe here a patient with severe PV who developed haemorrhagic nail lesions during the exacerbation of PV (4).

A 57-year-old man was admitted to our department with large haemorrhagic blisters and erosions within the skin and oral mucosa. The diagnosis of PV was confirmed by the direct immunofluorescence test, which revealed deposits of IgG and C3 complement within the epidermis and by the detection of circulating pemphigus antibodies (1/640 titre). Therapy with intravenous cyclophosphamide (500 mg once) and intravenous methylprednisone (1000 mg/day during 3 consecutive days) was started. However, development of new skin lesions was noted. Moreover, subungual haemorrhages/haematomas of the proximal parts of the nails appeared. Nine toenails and 2 fingernails were affected. Haematomas were also found within the nail folds, especially on the great toe. Due to the progression of skin lesions over a period of one week, the treatment was changed to a combination of oral prednisone (1 mg/kg/day) and intramuscular methotrexate (30 mg once weekly). However, no improvement was observed and the patient died one week later because of severe electrolyte abnormalities and coagulation disturbances.

Based on this case history, we believe that haemorrhagic nail abnormalities may be a poor prognostic indicator for patients with PV. This theory is supported by another report of a patient with severe course of PV, who also developed multiple subungual and intraungual haemorrhages during disease exacerbation (5). However, as there are no relevant clinical studies confirming our observations, and as our suggestion is based on only 2 case reports, further investigations are required.

The authors declare no conflicts of interest.

References

1. Carducci M, Calcaterra R, Franco G, Mussi A, Bonifati C, Morrone A. Nail involvement in pemphigus vulgaris. Acta Derm Venereol 2008; 88: 58–60.

2. Schlesinger N, Katz M, Ingber A. Nail involvement in pemphigus vulgaris. Br J Dermatol 2002; 146: 836–839.

3. Engineer L, Norton LA, Ahmed AR. Nail involvement in pemphigus vulgaris. J Am Acad Dermatol 2000; 43: 529–535.

4. Szepietowski JC, Różycka B, Baran E. Subungual haemorrhages in fatal pemphigus vulgaris. J Eur Acad Dermatol Venereol 2001; 15: 87–88.

5. Bockers M, Bork K. Multiple gleichzeitige Hämatome der Finger- und Zähennegel mit nachfolgender Onychomadesis bei Pemphigus vulgaris. Hautarzt 1987; 38: 477–478.

Note: The authors of the original article (Carducci et al.) were given the opportunity to comment in response to this letter, but chosed not to do so.