Type of publication:
Journal article

Author(s):
Kakroo S.N.; Khan M.A.; Beg M.A.; *Kakroo B.

Citation:
Journal of Pakistan Association of Dermatologists. 33(4) (pp 1562-1568), 2023. Date of Publication: October 2023.

Abstract:
Background Skin is frequently involved in small-vessel vasculitis. Based on immunofluorescence studies, it can be IgA type (Henoch-Schoenlein purpura) or IgA-negative type i.e. leukocytoclastic vasculitis. Clinically, palpable purpura is the hallmark of the disease. Both subtypes can be associated with variable systemic involvement. Objective To compare the clinical, histopathological and immunological profile of patients with IgA and IgA-negative vasculitis with special reference to renal involvement. Methods Seventy-five patients presenting to the outpatient clinic of a tertiary care hospital with a clinical diagnosis of palpable purpura were enrolled in the study. All patients had a thorough clinical examination and detailed history, and results were documented on a pre-made proforma. Results On direct immunofluorescence findings, 40 patients had IgA vasculitis and 35 IgA-negative vasculitis. The mean age of presentation was 38.8 years in IgA vasculitis and 54.3 years in IgA-negative vasculitis. IgA vasculitis presented with frequent cutaneous (n=27, 67.5%, itching or pain) and systemic symptoms (n=18, 45%, abdominal and joints) seventeen (42.5%) as compared to IgA-negative subgroup (25.4% and 11.4%, respectively). Morphologically, palpable purpura was seen in both groups, but 11.4% patients in IgA-negative vasculitis group presented with ulcerated lesions. Patients of both groups had vessel wall fibrin deposition and necrosis, inflammatory infiltrate in the vessel wall, erythrocyte extravasation, unclear dust and endothelial swelling in descending frequencies. Complement 3 (C3) was the commonest immunoreactant (n=71; 94.7%) followed by fibrinogen (n=59; 78.7%) and immunoglobulin A (IgA) [n=40; 53.3%]. Abnormal urine microscopy findings were detected in (n=28; 70%) patients with IgA vasculitis and in (n=2; 5.7%) with IgA-negative vasculitis. Serum creatinine was raised in 12 (30%) patients with IgA vasculitis and 7 (20%) patients with IgA-negative vasculitis. Other laboratory tests had similar frequency in two groups. Conclusion IgA vasculitis was seen in a relatively younger age group in comparison to IgA-negative vasculitis. C3 was the commonest immunoreactant staining the blood vessel wall followed by fibrinogen and IgA. Renal involvement occurred more frequently in the IgA vasculitis.

Type of publication:
Conference abstract

Author(s):
*Jacob M.A.; *Jain D.; *Shaw R.;

Citation:
British Journal of Dermatology. Conference: 102nd Annual Meeting of the British Association of Dermatologists. Glasgow United Kingdom. 187(Supplement 1) (pp 100), 2022. Date of Publication: July 2022.

Abstract:
Frontal fibrosing alopecia (FFA) is a scarring disease characterized by a receding frontotemporal hairline associated with varying degrees of eyebrow alopecia. It is usually seen in postmenopausal women. FFA has occasionally been reported in men, and can be confused with androgenetic alopecia, and therefore present quite late to the clinician. Although, histopathologically, FFA is similar in both sexes, there are a few differences clinically and with regard to treatment. We aimed to highlight these characteristics. A 51-year-old male presented with hair loss of 3 years' duration involving the eyebrows, sideburns and beard, followed by frontotemporal recession of the hairline. He reported a similar history in his mother and maternal aunt. In addition, he was known to have testosterone deficiency with low levels of total and free testosterone and sex hormone binding globulin, and was on replacement therapy. His remaining serology, including thyroid profile, was within normal limits. Clinical examination showed patchy alopecia of the eyebrows, eyelashes, sideburns and beard. There was frontotemporal recession of hairline along with loss of follicular orifices and perifollicular erythema. Additionally, he had diffuse thinning of the hair over the vertex and nonscarring alopecia over his extremities. A punch biopsy showed end-stage scarring alopecia with perifollicular fibrosis and 'backing off' of the surrounding lichenoid inflammatory infiltrate consistent with lichen planopilaris. A clinical diagnosis of FFA was made and the patient was started on hydroxychloroquine tablets along with 1% topical pimecrolimus twice daily over the patches of alopecia on the face. Common sites of hair loss in men include the sideburns, beard, eyebrows, eyelashes and occipital hair, and facial hair loss may precede eyebrow loss, as was the case in our patient. Facial papules, androgenetic alopecia and loss of body hair are more often observed in men with FFA than in women. In addition, the loss of body hair and eyelashes and the presence of facial papules have been associated with more severe forms of FFA and can be used as a prognostic indicator. Autoimmune conditions like thyroid disease and lichen planus are uncommon in males, although androgen deficiency has been linked to FFA in males in various studies. In addition, hormone-blocking drugs can contribute to FFA. Therefore, it is very important to keep this in mind during history and examination and perform relevant blood tests. In addition, treatment with minoxidil may provide better results in males.

Type of publication:
Conference abstract

Author(s):
*Shaw, Roberta; *Oliwieckim Simone

Citation:
British Journal of Dermatology. Conference: 102nd Annual Meeting of the British Association of Dermatologists. Glasgow United Kingdom. 187(Supplement 1) (pp 206), 2022. Date of Publication: July 2022.

Abstract:
A 15-year-old boy presented to the paediatric dermatology clinic with an unusual bilateral intermittent rash to his lower legs, which he had noticed for many years. The patient demonstrated multiple irregular white macules and urticated papules on a background of cyanosis of the legs when standing for prolonged periods, such as in the shower, and which was relieved by sitting. The mother believed that the rash began after a flu-like illness, and aside from this he was usually fit and well, with no significant past medical history, family history or regular medications. The patient had a full blood screen, including coagulation screen, which were normal. The patient described an intermittent lightheaded sensation on standing. Therefore, while in clinic the patient had a lying and standing blood pressure, which demonstrated a significant systolic drop from 137 mmHg lying to 109 mmHg standing, while his heart rate remained stable. The clinical findings suggested a diagnosis of BASCULE syndrome. BASCULE syndrome is a benign vasomotor dermatosis first described in 2016. The clinical findings described include Bier anaemic spots, cyanosis and urticaria-like eruption. The pathogenic mechanism remains unknown but is thought to be associated with transient tissue hypoxia through arteriolar vasoconstriction causing a paradoxical urticated rash that is worse on standing. Although BASCULE syndrome has been described as a separate entity, there have been links to POTS (postural orthostatic tachycardia syndrome) and orthostatic acrocyanosis, and so these diagnoses should be considered when investigating a patient with similar features.

Type of publication:
Conference abstract

Author(s):
Ieremia E., *Mudaliar V., *Kelly S., Grech B., Rodriguez P., Martin B., Calonje E.

Citation:
British Journal of Dermatology, July 2014, vol./is. 171/(90), 0007-0963 (July 2014)

Abstract:
Dermal hyperneury is defined as the presence of increased and hypertrophic myelinated and nonmyelinated nerve fibres in the dermis. Cutaneous nerve hyperplasia is rare and can be seen in lesional skin in multifocal or localized forms. When multifocal, it can be present in a pure cutaneous or mucocutaneous form or it may have syndromic associations. It is fascinating that it is present in the normal skin of patients with multiple endocrine neoplasia type 2b (MEN2b) and Cowden syndrome, but also in the lesional skin of those patients, as well as in neurofibromatosis (type 2), attenuated forms of MEN2b and in medullary thyroid carcinoma with macular amyloidosis. Localized, it may be encountered in areas of trauma, nodular prurigo, notalgia paraesthetica, neurocristic hamartoma and rarely in cases of chronic rubbing/scratching (Schaffer JV, Kamino H, Witkiewitcz A et al. Mucocutaneous neuromas. An underrecognised manifestation of PTEN hamartoma- tumor syndrome. Arch Dermatol 2006; 142: 625-32; Winkelmann RK, Carney JA. Cutaneous neuropathology in multiple endocrine neoplasia, type 2b. J Invest Dermatol 1982; 79: 307-12). We present six cases spanning through the spectrum of conditions described. We describe four patients with multiple cutaneous papules, variably symptomatic. Extensive investigations did not reveal any syndromic associations. Furthermore, we include two localized forms: one case of notalgia paraesthetica and one case of trauma. It is interesting to note that PTEN and RET mutations seen in Cowden and MEN2b syndromes, respectively, are implicated in common pathways of the growth and development of neural-crestderived and nerve tissue. We would like to propose dermal hyperneury as a distinct rare entity specifically in those cases presenting with multiple lesions confined to the skin and no syndromic stigmata, and therefore, no associated risk of malignancy.

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