Year : 2006 | Volume
: 51 | Issue : 4 | Page : 265--268
Nail unit in collagen vascular diseases: A clinical, histopathological and direct immunofluorescence study
PA Nabil, Raghavendra Rao, Shrutakirti D Shenoi, C Balachandran
Department of Dermatology, Kasturba Medical College, Manipal - 576104, India
Department of Dermatology, Kasturba Medical College, Manipal - 576104
Background: Abnormalities of the nail unit are common in patients with connective tissue diseases. Clinical examination of the nail unit, coupled with biopsy of proximal nail fold offers an additional advantage in the diagnosis. Purpose: Our aim was to record clinical changes of the nail unit in connective tissue diseases and to study the histopathological (both H and E and periodic acid Schiff and direct immunofluorescence (DIF) findings of nail-fold biopsy. Materials and Methods: Thirty-eight confirmed cases connective tissue diseases attending skin OPD were enrolled in the study. After detailed clinical examination of the nail unit, a crescentric biopsy was taken from the proximal nail fold (PNF). Histopathological and DIF studies were was carried out. Findings: Nail changes could be demonstrated in 65% connective tissue diseases. Specific histopathological (H and E) and immunofluorescence findings were also encountered in many patients. Conclusion: Clinical examination of the nail unit offers additional clue in the diagnosis of connective tissue diseases. Though DIF of PNF biopsy is useful in the diagnosis, it is not an ideal site for H and E study, as the yield is very low. Limitations: Lack of adequate comparison group and non-utilization of capillary microscopy for the detection of nail fold capillary abnormalities.
|How to cite this article:|
Nabil P A, Rao R, Shenoi SD, Balachandran C. Nail unit in collagen vascular diseases: A clinical, histopathological and direct immunofluorescence study.Indian J Dermatol 2006;51:265-268
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Nabil P A, Rao R, Shenoi SD, Balachandran C. Nail unit in collagen vascular diseases: A clinical, histopathological and direct immunofluorescence study. Indian J Dermatol [serial online] 2006 [cited 2020 May 27 ];51:265-268
Available from: http://www.e-ijd.org/text.asp?2006/51/4/265/30291
Examination of the nail apparatus in patients with connective tissue diseases may provide important information as they may have broad spectrum of nail changes. Because of the translucency of the nail plate, the proximity of its vasculature and the fact that the nail complex frequently manifests changes associated with connective tissue diseases, the nail should be scrutinized routinely when examining the patient. The common changes include periungual erythema, nail fold telangiectasia (NFT), ragged cuticle, finger tip scarring etc. Thus, nail findings give an important clue to the diagnosis.
Materials and Methods
Thirty-eight consecutive patients of connective tissue diseases (diagnosis confirmed by biopsy and serology) attending skin OPD were chosen for the study. After a detailed clinical examination of the nail unit, a crescentric biopsy was taken from the proximal nail fold (PNF); either from the involved skin or apparently normal skin.
Informed consent was taken and the biopsy site was anaesthetized using 2% lignocaine. A spatula was inserted under the PNF and the tissue loosened from the underlying nail plate over approximately 2-3 mm from the free edge of the nail fold on the midline decreasing gradually towards the lateral nail folds. A crescent - shaped section of the proximal nail fold was then cut. It was divided in to two halves, one stained for histopathology (H and E and PAS) and the other for direct immunofluorescence (DIF).
Out of 38 patients, four were males and 34 were females. The diagnostic distributions of the patients are shown in [Table 1]. Nail changes were observed in 25 (65.8%) patients [Table 2]. The most common nail changes observed were the presence of NFT and longitudinal ridging (28.9% each). Other changes observed include, nail-fold hyperpigmentation (21.1%), nail-fold erythema (18.4%), ragged cuticle and discoloration (15.8%), fingertip scar and chronic paronychia (7.9%), nail fold depigmentation (5.3%), pitting, cuticular hypertrophy and digital gangrene (2.6% each) [Table 3].
Out of seven female patients with scleroderma, four had diffuse cutaneous type while the rest had limited cutaneous type. Nail changes were observed in five patients (71.4%); four with diffuse cutaneous type and one with limited cutaneaous type. The most common change noted was NFT (four patients; 57.1%). Other changes observed were ragged cuticle (three patients; 42.86%), fingertip scars (three patients; 42.86%), longitudinal ridging (two patients; 28.6%).
Mixed connective tissue disease (MCTD)
Nail changes were observed in six patients (85.7%). NFT was the commonest finding (four patients; 57%), followed by longitudinal ridging, cuticular hypertrophy, discoloration of nail plate, nail fold depigmentation and hyperpigmentation. Digital gangrene was observed in one.
Systemic lupus erythematosus (SLE)
Out of 11 (61.1%) patients with nail changes, six had nail-fold erythema (54.5%) [Figure 1]. Other changes noted were longitudinal ridging, ragged cuticle, NFT, pitting, discoloration, chronic paronychia, nail-fold hyper and depigmentation.
NFT was seen in one patient of DM. One patient with DLE had chronic paronychia with discoloration of the nail, while nail changes in the form of longitudinal ridging, thinning and nail-fold hyperpigmentation was seen in one patient of rheumatoid arthritis.
Raynaud's phenomenon was seen in eight patients (21.05%). This included three cases each of SLE and MCTD and two cases of scleroderma. All of them showed nail changes.
Specific H and E changes of connective tissue diseases were seen in seven patients (18.4%). Homogenization of the collagen was seen in four patients with scleroderma [Figure 2] while basal cell degeneration was seen in three samples of which two had SLE and one MCTD.
PAS positive cuticular deposits [Figure 3] were present in 10 (26.3%) nail-fold biopsy samples, out of which 8 had nail changes. They were more common and were more extensive in scleroderma (three patients; 41.9%); they were also seen in SLE (3 patients; 41.9%), MCTD and DLE (two patients each; 33.3%).
DIF of nail-fold was positive in 27 (71.05%) samples. Lupus band (with immunoglobulin, C3 and fibrinogen) at dermo-epidermal junction was positive in 22 (57.8%) samples [Figure 4]. This included 13 (72.2%) samples of SLE, five samples of scleroderma (71.4%), three samples of MCTD (42.9%) and one sample of DLE (33.3%). This was more common with IgM than with other FITC conjugates. Other DIF changes noted were blood vessel deposits (C3, fibrinogen) in 9 (23.7%) patients (MCTD - 3, SLE - 2, DM-2, RA and DLE - one each), epidermal nuclear stain in three cases, (two cases of MCTD and one case of SSc) and IgM colloid bodies (DLE, SLE - one case each).
Nails may be affected either directly or indirectly in various connective tissue diseases. The vast majority of these onychopathies represent non-specific reaction pattern. But a few changes are more specific and help to establish the diagnosis. Hence a thorough examination of the nail unit is mandatory in all patients with connective tissue diseases.
Commonest nail change observed in our patients with SLE was nail-fold erythema. This is in contrast to the previous study by Urowitz et al . who reported onycholysis to be the commonest change. They also found that the incidence of Raynaud's phenomenon and mucous membrane ulcerations were common in SLE patients who had nail changes. Other reported signs like red lunula, bluish-black discoloration of the nail plate were not seen in our patients.
NFT was seen most frequently in scleroderma and MCTD patients in the study. Previous studies have found an association between the degree of nail fold capillary abnormality (as detected by capillary microscopy) and internal organ involvement.,, Systemic organ involvement in the form of interstitial lung diseases was noted in two of our patients with scleroderma who had NFT. Maricq et al . have observed that long term follow-up of patients with Raynaud's disease is necessary when they have associated NFT as they are likely to develop systemic manifestations later in the course of the disease. Thus examination of the proximal nail-fold gives useful diagnostic and prognostic information in patients with connective tissue diseases.
Out of two patients with dermatomyositis, only one had NFT. Thickening and hyperkeratosis of the cuticle are the other common nail changes that were not seen in our patients. One patient of rheumatoid arthritis in our study had nail changes in the form of thinning, hyperpigmentation and longitudinal ridging. Other characteristic nail changes associated with rheumatoid arthritis like red lunula, splinter hemorrhages, periungual vascular lesions were not seen in our patients.,
There is paucity of reports on nail-fold biopsies in the literature. This may be partly due the concern that biopsies of this site could result in disfigurement of the nails. Also, there may be concern regarding the poor healing of the biopsy site in scleroderma patients. However it is a well-established fact that compared to 'spontaneous' ulceration, biopsy site heal surprisingly well. Our study further supported this fact as all our patients had normal healing. Specific histopathological (H and E) changes were observed in 18% while eosinophilic PAS positive cuticular deposits were noted in 26% of the samples. This cuticular deposit occurs due to increased vascular permeability resulting in the extravascular deposition of serum proteins. Scher et al. have observed that the severity of cuticular deposits correlates with the degree of in vivo capillary abnormalities. DIF of the nail-fold showed typical lupus band in 57.8% of our patients. Chen et al. have described a specific granular epidermal intercellular immunoglobulin deposition and a unique cytoplasmic staining pattern in dermatomyositis. We did not encounter such findings.
Nail-fold biopsy is a simple procedure that can be performed with minimal or no disfigurement of the finger. Moreover, equal halves of the same biopsy specimen can be used for both DIF and histopathological (H and E and PAS) evaluation.
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