Erythrokeratodermia Variabilis Present at Birth: Case Report and Review of the Literature

Abstract: A healthy boy had the distinctive lesions of erythrokeratodermia variabilis (EKV) at birth. Twenty-eight patients described in the literature had EKV that presented in childhood. Nine of the 28 were said to have had a rash since birth, but none were distinctive of EKV. To our knowledge this is the first well-documented case describing a child born with the skin manifestations of EKV. We conclude that patients with EKV are infrequently born with a rash, and that only very rarely when the rash is present is it suggestive of the disorder.     

VARICELLA BULLOSA ASSOCIATED WITH MEASLES VACCINE

A bullous eruption complicating varicella is described. It is suggested that concurrent infection with measles vaccine virus was the cause of the unusual rash.     

Psoriasiform reaction to atenolol

A 61-year-old woman developed a psoriasiform rash 18 days after starting atenolol. The eruption resembled that described with other beta blockers and subsided 10 weeks after withdrawing the drug. Cutaneous reactions to atenolol are rare and the development of this rash has not been previously reported.     

Digital image analysis of facial erythema over time in persons with varied skin pigmentation

Background/purpose: Selected chemotherapeutic agents used for the treatment of cancer are known to cause skin toxicities. One group of agents, epidermal growth factor receptor (EGFR) inhibitors, characteristically precipitates an acneform rash. Currently, no standard of care exists for the management of the rash resulting from EGFR inhibitors. In order to objectively evaluate any management strategy, a method to quantify the rash is required. The purpose of this paper is to describe a method to quantify the erythema of a facial rash through the use of digital photography and image analysis. Methods: A Canfield OMNIA System using a Canon PowerShot Pro1 camera was used to obtain high‐resolution digital images of facial rashes. Digital images were recorded in Joint Photographic Experts Group format, corrected for brightness and white balance and color. A method was developed to analyze digital images of erythema independent of the range of skin pigmentation. Results: Two examples are given to illustrate the method developed and its utility. Conclusion: An inexpensive and portable method is described for objectively monitoring the development of facial erythema in subjects of the full range of skin pigmentation. This method can be used clinically to examine the development and resolution of facial rash erythema in response to treatment.     

Persisting cholinergic erythema: a variant of cholinergic urticaria

A new variant of cholinergic urticaria is described. Four patients each had a similar persistent macular skin rash distributed maximally over the upper limbs and upper trunk. Though the rash was persistent, individual macules were of short duration but new macules continually appeared at adjacent sites. Exercise and hot baths exacerbated pruritus and provoked lesions in previously unaffected areas. Topically applied benzoyl scopolamine blocked the appearance of the lesions after challenge. Tests of cholinergic function were normal, apart from an exaggerated pupillary response to arecoline in one patient.     

Nonthrombocytopenic purpura induced by carbromal.

A case of carbromal-induced purpura is described and the typical clinical features are presented. The diagnosis was confirmed by patch tests with 1 percent and 5 percent carbromal in propylene glycol and the positive results of a macrophage migration inhibitory factor test for carbromal. The rash gradually subsided following withdrawal of the drug.     

Fixes Arzneimittelexanthem auf Terbinafin mit charakteristischem Verteilungsmuster eines Baboon-Syndroms

A 26 year old patient developed a fixed drug eruption located on his hands, inguinal and gluteal areas following oral treatment of onychomycosis with terbinafine. The rash showed the characteristic distribution of the "baboon-syndrome", so-named because of the red perianal region of the baboon. Although epicutaneous testing revealed no positive reaction, the rash could be induced in identical sites by oral administration of terbinafine. As the underlying pathomechanism for the "baboon-syndrome" a systemically induced allergic contact dermatitis has been suggested. In addition to the described substances, e.g. mercury, amoxicillin, ampicillin, heparin and nickel, this is the first report of "baboon syndrome" induced by terbinafine.     

Cutaneous reaction to diltiazem resulting in an exacerbation of angina

A 77-year-old woman developed a widespread erythematous rash after treatment with the calcium antagonist, diltiazem, which resulted in an exacerbation of the angina for which the drug was originally described. Drugs which may give widespread cutaneous side-effects should be used with caution in patients with compromised cardiac function.     

Oral 3,CHILD syndrome with unusual cutaneous features

We report a case of a rare epidermal naevus genetic disorder which presented with unusual cutaneous features which to our knowledge have not previously been described.
A 5-year-old girl of Indian origin was noted at birth to have a shortened left leg, left arm and left index finger. A rash was first noted at 3 months of age with a red scaling eruption in the left groin and medial thigh which resolved over a few weeks leaving a residual area of macular hyperpigmentation on the thigh area. The groin eruption has since recurred. A rash with a similar morphology appeared at the age of 6 months affecting the perianal area (predominantly left sided) and this rash has persisted. The fingernails of her left hand were noted to be dystrophic at the age of 18 months as were several toenails (bilateral but not universal). In addition, she has developed hyperkeratotic areas on both soles and at the left popliteal fossa. Several of these areas have a linear morphology. There is no history of consanguinity or any family history of similar skin problems. Genetic analysis revealed a mutation in the NSDHL gene, which is responsible for CHILD syndrome. Her mother did not exhibit this mutation.     

Paracetamol-induced fixed drug eruption with positive immunofluorescence findings

A 93-year-old woman with an 8-year history of episodes of fixed drug eruption due to paracetamol is described. The rash was characterized by bullae, crusted lesions, hyperpigmented and erythematous patches as well as lesions resembling toxic epidermal necrolysis. Direct immunofluorescence revealed deposition of IgG and C3 in the epidermal intercellular cement substance of lesional skin only; the pathogenetic significance of this finding is discussed.     

Bleomycin-induced flagellate erythema

A 56-year-old woman who developed widespread pruritus and flagellate erythema after attempted pleuredesis with bleomycin is described. The raised linear lesions of flagellate erythema could not be reproduced by scratching, and histopathological examination revealed a lymphocytic vasculitis. The rash faded spontaneously over several weeks to leave streaks of post-inflammatory melanoderma which remained for 6 months. The role of scratching and dermographism in the pathogenesis of the bleomycin-specific eruption is discussed.     

Diaper Dermatitis: Frequency and Severity Among a General Infant Population

The frequency and severity of diaper dermatitis was measured among a midwestern suburban population of 1089 infants ranging in age from 1 to 20 months. No diagnosis of specific etiology was made. Fecal samples were collected and analyzed for Candida albicans, and information on family characteristics, infant diet, general health, history of rash, and diapering habits and practices was collected by questionnaire. The distribution of the severity of observed diaper rash can be described as a logarithmic-normal function, implying several multiplicative causative factors. Within the total severity range, there appear to be three subcategories of diaper rash, differing in some manner, perhaps reflecting different etiologies. The frequency of observed diaper rash was a function of the maturity of the infant, reaching a maximum around 9 to 12 months of age. The prevalence of severe rash correlated with the presence and level of fecal C. albicans. Infants diapered exclusively in disposable diapers showed less rash (P less than 0.001) than those diapered exclusively or sometimes in cloth diapers.     

6-mercaptopurine-related hand-foot syndrome in a four-year-old child

BACKGROUND: Hand-foot syndrome (HFS) is a dose-dependent cutaneous side effect of cytostatic chemotherapy. It has also been described under the names of acral erythema and palmoplantar erythrodysesthesia. We report a case of HFS during treatment of acute lymphoblastic leukemia with 6-mercaptopurine (6-MP) (Purinethol((R))) in a four-year-old child. PATIENTS AND METHODS: A four-year-old boy treated for acute lymphoblastic leukemia developed dry and painful palmar and plantar erythema with fissures. The rash began three weeks after up-titration of 6-MP. There was no past history of cutaneous disease and no other potential trigger factors. The rash ceased after 6-MP withdrawal. DISCUSSION: To our knowledge, this is the first case of HFS due to 6-MP therapy in a child. 6-MP is a major reference drug for the management of acute lymphoblastic leukemia. Numerous cytostatic drugs have been involved in such eruptions.     

Cutaneous vasculitis associated with long-term thiouracil therapy

Many rashes have been attributed to thiouracil therapy, but severe vasculitis is rare. A distinct pattern of cutaneous vasculitis affecting limbs, face and ears has been described with propylthiouracil, and is particularly associated with treatment of more than 18 months duration. A positive Antinuclear Antibody (ANA) in the absence of LE cells is often demonstrated. We describe the first such case due to methylthiouracil therapy, the bullous haemorrhagic rash having the histology of pyoderma gangrenosum, and we review the available literature regarding this pattern of vasculitis.     

Pityriasis lichenoides chronica presenting as hypopigmentation

Seven black or dark skinned patients with pityriasis lichenoides chronica (PLC) are described, who presented with widespread hypopigmentation. The evidence on which a diagnosis of PLC was made is presented and the differential diagnosis discussed. The hypopigmentation was noted in some patients to be especially marked on the proximal parts of the limbs and axillary folds. A diagnosis of PLC should be considered in all dark skinned patients with a widespread hypopigmented rash.     

Reticular erythematous mucinosis syndrome in a patient with polyarthritis

A patient with seronegative oligoarthritis who developed the reticular erythematous mucinosis (REM) syndrome is described. This syndrome is considered to be a dermatological entity unrelated to systemic disorders. Aggravation of the rash by exposure to sunlight and a good response to anti-malarial agents suggest a relationship with rheumatological disorders, e.g. rheumatoid arthritis and systemic lupus erythematosus. Dermatologists consulted by a patient with the REM syndrome should be aware of the possibility of an associated rheumatological disease.     

Oral mucosa lesions as atypical manifestation of adult-onset Still’s disease

Adult-onset Still’s disease is a systemic inflammatory disorder of unknown etiology, characterized by skin rash, spiking fever, arthralgias or arthritis, and leukocytosis. The typical skin rash is evanescent, salmon-pink, nonpruritic and maculopapular, predominantly on the extremities. It is considered one of the major Yamaguchi’s criteria in adult-onset Still’s disease. However, atypical skin lesions are also described. Here, a 61-year-old woman with sore throat, spiking fever, polyarthritis and evanescent salmon-pink nonpruritic maculopapular skin rash on the extremities was diagnosed with adult-onset Still’s disease. In addition, atypical brown macules on oral mucosa, localized on the inner lips and tongue were also observed. Biopsy revealed a neutrophilic infiltrate. Despite treatment and improvement of the adult-onset Still’s disease, the atypical oral mucosal lesions persisted.     

Cutaneous findings in a case of psittacosis

A patient had severe pneumonia, respiratory failure, and disseminated intravascular coagulation caused by Chlamydia psittaci. Cutaneous findings included a maculopapular blanching rash, acrocyanosis, superficial venous thromboses, and splinter hemorrhages under the fingernails. The history of exposure to potential vectors of psittacosis and the presence of some or all the cutaneous findings described herein may lead to the early recognition of psittacosis.     

Sparing of leprosy macule in ampicillin hypersensitivity rash

A case of tuberculoid leprosy is reported in a middle aged female, who presented with generalised erythematous maculopapular rash which followed ampicillin therapy. In spite of extensive and severe involvement of the skin all over the body by erythema and rash, the hypopigmented patch of leprosy on the face did not show any erythema or rash and stood out more clearly as an island of pale area in the midst of large area of erythema on the face. The possible mechanism of non development of erythema and rash in the patch is discussed.