The term terra firma-forme dermatosis arises from the Latin phrase terra firma, meaning dry land (dirt), thus implying dirt-like dermatosis. The authors highlight five cases of patients with terra firma-forme dermatosis presenting to our dermatology center between 2012 and 2013. All patients presented to the dermatologist for persistent reticulated brown patches on the skin. These patients ranged in age from 6 to 22 years. All patients had tried various cleansing soaps and agents but were unable to remove the patches. The condition was cosmetically unacceptable to the patients and parents. Clinically, these patients had reticulated brown patches. Rubbing 70% isopropyl alcohol wipes on the affected areas demonstrated clearance of the brown pigmented patches in all cases. The diagnosis of terra firma-forme dermatosis (TFFD) was confirmed by forceful rubbing with a gauze pad immersed in 70% isopropyl alcohol or ethyl alcohol. Patients should be reassured about the benign nature of TFFD and educated about the cleaning procedure. Recognition of this condition can assist physicians in making a diagnosis and therapy with a simple alcohol wipe, preventing further unnecessary tests for patients.
We report a study of the sensitization rate to sesquiterpene lactone (SL) mix at the National Skin Centre, Singapore. This is a retrospective review of 1256 patients who were patch tested to SL mix (0.1%) from January 1995 to December 1997. 18 out of 1256 patients (1.43%) reacted positively to SL mix. Their ages ranged from 13 to 58 years (average 33.8 years). There were 15 Chinese, 2 Indian and 1 Malay patients. There was a female preponderance, with a female: male ratio of 3.5:1. The most common clinical presentation were dermatitis of the hands (8; 44%), face (5; 28%), and generalized involvement (5; 28%). After extensive evaluation, only 2 patients had a positive patch test reaction that was of current clinical relevance. 13 patients had concomitant sensitization to other allergens. The 3 commonest concomitant allergens were nickel, balsam of Peru and fragrance mix. In our series, SL allergy is observed to be more common in females and the mean age is lower compared to the classical Compositae dermatitis. The sensitization rate is comparable to other studies, although the clinical relevance is only 11%.
Cutaneous plasmacytosis (CP) is a rare skin disorder characterized by multiple reddish brown nodules with polyclonal plasma cell proliferation. It has most often been reported to affect the trunk but is also known to affect the face and extremities in adults and is predominantly seen in Asians. The etiology is poorly understood, and there is no consensus on treatment methods.Five cases diagnosed to have CP were collated from our institution. Their clinicopathologic features and treatment outcomes were reviewed.Four of the 5 patients presented with lesions that affected multiple sites of the body including the trunk, axillae, face, and limbs. The remaining patient had lesions localized to his axillae. The lesions were generally asymptomatic. All patients had hypergammaglobulinaemia but only one had a faint monoclonal band detected on immunofixation. Common findings in the biopsy results for all patients were perivascular plasma cell infiltrates without light chain restriction on kappa/lambda staining, as well as mast cell infiltrates. Partial remission of cutaneous lesions was observed in 3 of the patients, with 2 of them responding well to psoralen and ultraviolet A radiation therapy.CP presents with distinctive clinical features and characteristic histological features including polyclonal perivascular plasma cell infiltrates. The axilla seems to be a frequent and characteristic site of involvement and may be a useful clinical clue to the condition. In the management of patients with CP, it is important to exclude secondary causes of plasmacytic infiltrates. While there are no clearly established treatment modalities for CP, psoralen and ultraviolet A radiation therapy may be a viable option in view of the clinical improvement observed in our patients who received it.
Abstract Background Keratitis–ichthyosis–deafness (KID) syndrome is a rare ectodermal dysplasia characterized by generalized erythrokeratotic plaques, sensorineural hearing loss, and vascularizing keratitis. Cutaneous changes and hearing loss typically present in early childhood, whereas ocular symptoms present later. Mutations in the connexin (Cx) 26 gene, GJB2 , are now established to underlie many of the affected cases, with the majority of patients harboring the p.D50N mutation. Methods A rare patient demonstrating features of incomplete KID syndrome associated with an uncommon Cx26 gene mutation is described. Results The patient presented late in adolescence with partial features of KID syndrome. There was limited cutaneous involvement and the rare association of cystic acne. Both hearing impairment and ophthalmic involvement were mild in severity. Genetic mutation analysis revealed a previously described, rare mutation in GJB2 , resulting in a glycine to arginine change at codon 12 (p.G12R). Conclusions This report describes a patient exhibiting characteristics suggestive of a late‐onset, incomplete form of KID syndrome with the GJB2 mutation (p.G12R). The p.G12R mutation has only been described in one other patient with KID syndrome, whose clinical presentation was not characterized.
Abstract Toxic epidermal necrolysis (TEN) is a severe, immune‐mediated, mucocutaneous reaction resulting in extensive keratinocyte apoptosis. High‐dose human intravenous immunoglobulins (IVIG) have been proposed as an effective treatment for TEN. Retrospective data from 8 patients with TEN and 4 patients with Stevens‐Johnson syndrome‐toxic epidermal necrolysis (SJS‐TEN) overlap treated with high‐dose IVIG were analysed. The total dose of IVIG administered was 2 g/kg body weight, with the exception of 2 patients who received a total dose of 1.5 g/kg body weight. Their mean age was 49.9 ± 18.8 years (range, 19 to 70 years). The mean time from the first sign of skin lesion or mucosal or epidermal detachment to commencement of IVIG was 8.7 ± 5.5 days (range, 3 to 22 days). Of the 11 patients who survived, the mean time to objective response was 3.6 ± 1.9 days (range, 2 to 8 days). The length of stay (LOS) in hospital was 20.4 ± 8.0 days (range, 10 to 37 days). The survival rate was 91.6%. One patient developed permanent mucocutaneous sequelae following TEN. There were no adverse reactions to IVIG. We conclude that high‐dose IVIG may be a safe and effective therapy for Asian patients with TEN.
Malignant atrophic papulosis, commonly known as Degos disease, is a rare vasculopathy encompassing both benign, cutaneous and lethal systemic variants. We report a case of chronic cutaneous lupus erythematosus in a 41-year-old male presenting with prominent Degos-like skin lesions. Multiple atrophic, porcelain-white, scar-like papules and plaques with dusky, erythematous borders, suggestive of malignant atrophic papulosis, were noted on the patient's back. Additional cutaneous findings included photo-distributed facial erythema and discoid lupus-like plaques on the face, shoulders, and arms. Clinicopathological correlation supported a diagnosis of chronic cutaneous lupus erythematosus; hydroxychloroquine was initiated with good clinical response. No new or active lesions were observed at the sixteen-month follow-up. This case highlights a rare skin finding associated with chronic cutaneous lupus erythematosus and underscores the importance of ruling out primary autoimmune disease, particularly lupus, before a diagnosis of malignant atrophic papulosis can be made.
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