Terra firma-forme dermatosis – a dirty dermatosis: report of two cases

Terra firma-forme dermatosis is characterized by ‘dirty’ brown-grey cutaneous patches and plaques that can simply be eradicated by forceful swabbing with alcohol pads. The pathogenesis has been attributed to abnormal and delayed keratinization. Although affected patients present with typical lesions, the disorder is not well-known by physicians. From a clinical point of view, we lay emphasis on its unique expression and diagnosis/treatment. From a histological perspective, we highlight its resemblance to dermatosis neglecta and speculate on the role of ‘neglect’ in a patient with seemingly adequate hygiene. The role of urea containing emollients in the development of this disorder remains to be determined

Neurofibromatosis type 1 with localized unilateral hyperhidrosis: A rare association

Unilateral hyperhidrosis is a rare entity with primary and secondary causes. So far in literature, the associations documented with this entity rarely include neurofibromatosis. This association, however has been reported previously and perhaps is a more frequent incidence than is being recognized. Here we report a case to emphasize this association

Primary idiopathic systemic amyloidosis – rare classical cases with fatal outcome

Primary systemic amyloidosis is a rare condition. We report two cases of primary systemic amyloidosis. Both the cases were without any hematological abnormality. Cutaneous features were the predominant presenting symptoms in these patients. The patients presented with typical waxy lesions on face and macroglossia. Diagnosis was confirmed by histopathology with haematoxylin and eosin staining and Congo red stainin

Vitamin D-Dependent rickets Type II with alopecia: A rare case report

Vitamin D-dependent rickets type II is a rare hereditary disorder. It occurs due to mutations in the gene chr. 12q12-q14, which codes for vitamin D receptor. End-organ resistance to 1,25-(OH)2vitamin D3 and alopecia in severe cases are the characteristic features. We report a case of a 4-year-old boy with loss of hair over the scalp and body – first observed after 1 month of birth. The boy also developed difficulty in walking at the age of 2 year. On analysis, reduced serum calcium level (7.5 mg/dL) and elevated alkaline phosphatase level (625 IU/L) were reported. Initially, the treatment included intramuscularly administered single dose of 600,000 IU vitamin D, followed by 400 IU of vitamin D along with 1 g of supplemental calcium every day. Periodic follow-up was conducted for 2 months. Improvement was observed in the biochemical parameters and X-rays of the distal radial and ulnar metaphyses, although no improvement was observed in alopecia

Miescher granulomatous macrocheilitis: A case report

Cheilitis granulomatosa (CG) is a chronic swelling of the lip due to granulomatous inflammation. It is a rare inflammatory disorder first described by Miescher in 1945. It is a monosymptomatic form or an incomplete variant of Melkersson-Rosenthal syndrome. As the etiology remains unknown, treatment of CG is challenging. We present a case of CG in a 20-year-old male patient presented to us with diffuse swelling of the lower lip of seven years duration. On examination, there was gross enlargement of his upper-lip. A histopathological specimen from the lower lip showed non-caseating granulomas. We treated our patient with intralesional triamcinolone acetonide and oral clofazimine. The present case highlights the importance of thorough clinical examination, history & investigations in the diagnosis of this lesion as the findings mimic many other granulomatous conditions in dermatology

Correlation of serum antithyroid microsomal antibody and autologous serum skin test in patients with chronic idiopathic urticaria

Background: About 25-45% of patients of chronic urticaria (CU) have been stated to have histamine releasing autoantibodies in their blood. The term autoimmune urticaria is increasingly being accepted for this subgroup of patients. Review of the literature suggests high autologous serum skin test (ASST) positivity and presence of antithyroid microsomal antibodies in patients with autoimmune urticaria. Aims: To study prevalence of ASST positivity and antithyroid microsomal antibodies in chronic "idiopathic" urticaria and to study the correlation between the two parameters. Methods: All patients of chronic idiopathic urticaria satisfying inclusion/exclusion criteria were enrolled in the study after written informed consent. Patients of CU secondary to infections and infestations, physical urticaria including dermatographism, mastocytosis, urticarial vasculitis and those on treatment with immunosuppressive drugs for urticaria were excluded from the study. In all of these patients, complete blood count; ASST, serum T3/T4/thyroid stimulating hormone levels, antithyroid microsomal antibody (AMA) levels were done. Statistical analysis was done by Chi-square test, Fisher exact test and Kappa statistics. Results: Study included 24 males and 26 females with mean age of 39.54 years. Majority of patients belonged to 20-40 years of age. Females showed more ASST positivity. A total of 12 out of 50 (24%) patients showed positive ASST. A total of four out of 12 (33.33%) had positive ASST and raised AMA levels. Conclusion: Only 25% of patients of chronic idiopathic urticaria had positive ASST. ASST and AMA levels were positively correlated in our study. Further studies are required to authenticate this association

Malignant Mediterranean spotted fever

Fever with rash is one of the most common causes of referral to a dermatologist. A plethora of conditions need to be considered in the differential diagnosis. They may be broadly classified into infectious causes, drug reactions, and autoimmune disorders. Here we present a rare case of rickettsial fever with cardiac involvement in an elderly male patient with no comorbidities

Livedo reticularis: A review of the literature

Livedo reticularis (LR) is a cutaneous physical sign characterized by transient or persistent, blotchy, reddish-blue to purple, net-like cyanotic pattern. LR is a benign disorder affecting mainly middle-aged females, whereas livedo racemosa (LRC) is pathologic, commonly associated with antiphospholipid antibody syndrome. This article aims to review the causes of LR and LRC along with the evaluation and management

Amoxicillin and clavulanate potassium related Leucocytoclastic vasculitis

Leukocytoclastic vasculitis (LCV) is a small-vessel vasculitis with a reported incidence rate of 30 cases per million persons per year. It usually presents as a palpable purpuric skin rash on legs, though any part of the body can be affected. LCV rash may have an associated burning sensation or pain and in some cases may involve internal organs. In some cases, LCV rash may present as nodules, recurrent ulcerations or asymptomatic lesions. The diagnosis of LCV is usually made on skin biopsy. Etiological triggers may not be identified in as many as half of the cases. Treatment is usually conservative and includes identification and removal or treatment of the etiological trigger except in cases with internal organ involvement where systemic steroids and immunosuppressant may be necessary. In this article we present a case of Amoxicillin and Clavulanate potassium associated LCV that improved with discontinuation of the offending agent and treatment with systemic corticosteroids