Bernice R. Krafchik

Subcutaneous Fat Necrosis of the Newborn: A Review of 11 Cases

Abstract: Subcutaneous fat necrosis (SFN) of the newborn is uncommon and usually occurs in the first weeks of life following a complicated delivery. The frequency with which hypercalcemia develops as a complication is uncertain. We report the clinical features of SFN in 11 patients seen between 1991 and 1998. Ten were born by cesarean section and fetal distress was present in the majority. It was not possible to distinguish SFN from sclerema neonatorum by time of onset or related infant diseases. Hypercalcemia developed in four infants up to 7 weeks after the onset of SFN. Infants with this condition should be carefully monitored for hypercalcemia.

Eosinophilic Pustular Folliculitis In Infancy

Abstract: Five infants under 1 year of age were reported with a syndrome of recurrent crops of pruritic papulopustules of the scalp. In three children there were also intermittent outbreaks on the trunk and extremities. Cultures showed the pustules to be sterile. Biopsies of scalp and skin tissues showed eosinophilic folliculitis. Some patients had eosinophilia during outbreaks of pustules. These cases are similar to the eosinophilic pustular folliculitis reported in a few adult patients with the exception that there was predominant scalp involvement in the children. We propose that eosinophilic pustular folliculitis of infancy is a distinct pustular dermatosis.

Genital warts and sexual abuse in children

Genital warts in children indicate the possibility of sexual abuse. If sexual child abuse is recognized or suspected, then the law in all states requires that the physician report this to the appropriate authority. The dermatologist may be the first person to suspect the problem and can be of great help in protecting the child from future harm.

Mercury Intoxication: It Still Exists

Abstract:u2002 A 3‐year‐old boy presented to the Hospital for Sick Children with systemic symptoms and oropharyngeal and peripheral extremity changes suggestive of Kawasaki disease. He was found to have severe hypertension. Investigation for a catecholamine‐secreting tumor was negative. Toxins were considered when the patients 20‐month‐old brother presented with similar symptoms, and the boys were subsequently diagnosed with elemental mercury poisoning. We review the literature on mercury intoxication and discuss the historical context, clinical syndrome (acrodynia), treatment, and radiologic findings of this unusual diagnosis.

Multiple cutaneous granular cell tumors in children

Abstract: Multiple cutaneous granular cell tumors are lare in childhood. We cared for two children with 15 and 6 tumors respectively. The tumors were asymptomatic nodules in the skin. Histologically, they consisted of large cells containing granules that were positive on periodic acid‐Schiff stain and diastase resistant.

Transverse Striate Leukonychia Associated with Cancer Chemotherapy

Mees lines are transverse bands of leukonychia first described in patients suffering from arsenic intoxication (1). Similar nail changes have since been reported as an isolated finding, and in association with a variety of medical diseases and, less commonly, cancer chemotherapy (Table I). None of the last has involved pediatric patients. This report concerns transverse striate leukonychia in a child who received chemotherapy for an osteogenic sarcoma.

The unidentified parasite: a probable case of north american cuterebrid myiasis in a pediatric patient.

To the Editor: I read with great interest the article by Clifton et al (1) regarding immunotherapy of warts with mumps or Candida antigen. Cassone et al (2) used the term “microbial biological response modifier” to describe the immune modulatory effects of infectious microbes such as Candida albicans . They reported that Candida mannoprotein fragment F2 (MP-F2) is one of the strongest attractants of neutrophils. MP-F2, a powerful immunomodulatory fraction from the cell wall of C. albicans , is an active inducer of interleukin (IL)-8, tumor necrosis factor (TNF)α , IL-6, and IL-1 β production by human polymorphonuclear (PMN) cells and monocytes (2). Further, MacPhee et al (3) recovered CD4 + and CD8 +

THE SYNDROME PAGE

A 12-year-oId female had atypical, mild ichthyosis and whorled hyperpigmentation, cicatricial alopecia, foUicular atrophoderma, and slight stunting of growth (Figs. 1^). What is your diagnosis? Answer: X-linked dominant chondrodysplasia punctata (Conradi-Hunermann syndrome), Chondrodysplasia punctata has many variations, but usually occurs in three main genetically distinguishable types—recessive, X-linked dominant, and autosomal dominant. It is characterized by small focal calcifications in the cartilage associated with anomalies ofthe facies, eyes, and skin. The recessive variety (rhizomelic dwarf) is the most easily recognized. Infants are born with ichthyosis, cataracts in 75% of cases, and proximal limb shortening (rhizomelia). The ichthyosis occurs as dry, erythematoos, scaling skin with occasional bullae. It has a bizarre patterning reminiscent of incontinentia pigmenti. The hyperkeratotic bands fade in two to three months, leaving residual, whorled hyperpigmentation. The skeletal deformities arise from calcification in the epiphyses causing disruption of enchondral bone formation. The nasal bridge is flattened and the palate high-arched. Most children with this condition die before age 2 or survive with psychomotor retardation and spasticity. The dominant and X-linked dominant varieties of chondrodysplasia punctata (Conradi-Hunermann) are difficult to distinguish from one another. The former disease is usually confined to females (? lethal to males) and exhibitis variable asymmetric skeletal involvement, A generalized erythema and scaling, distributed in a linear whorled pattern, are present at birth but disappear over a period of up to three months. Other skin findings that aid in the diagnosis are a specific type of foUicular atrophoderma resulting from previous areas of hyperpigmentation and inflammation, and cicatricial

Commentary on Nagaraja, Kanwar, Dhar, and Singh: Frequency and Significance of Minor Clinical Features in Various Age-Related Subgroups of Atopic Dermatitis in Children

In this issue of Pediutric Dermutolo~y, Nagaraja, Kanwar, Dhar, and Singh attempt to evaluate the minor clinical features of atopic dermatitis (AD) as to frequency and significance in various age groups. The sensitivity and specificity of the minor signs have been evaluated by a number of investigators (1-3), with discrepancies in the findings, including lack of complete agreement between this and a previous study by the same authors (3) . One hundred patients and 100 controls were studied, and 24 features were evaluated in each patient and each control. Additional features were infraauricular fissuring, diffuse scaling of the scalp, and Hertoghe sign (absence of the outer eyebrows). The information was gathered both by history and physical examination. Of the 24 features, 15 were highly significant. Of the additional three features, two were significant: nonspecific scaling of the scalp and infr~uricular fissuring. The authors considered pruritus, typical morphology and distribution, chronic relapsing course, and personal and family history of atopy as the major criteria, which is in agreement with most other studies (4-6). The only problem that I can see with these criteria is a family history of atopy. Altho~gh there is no doubt that a strong family history of atopy is often associated with AD, atopy is so prevalent in the general population that this particular feature should perhaps be more clearly categorized. Atopic dermatitis was clinically delineated by Hill and Sulzberger in 1935 (7). The diagnosis is made by physicians all over the world, with a frequency of up to 12% of the population. Sulzberger is quoted in the 1955 book by Rudolf Baer as saying, “It appears to me that the morphology, the distribution and the other classic features that characterize the cutaneous manifestations of atopic dermatitis are so well known that every skin specialist and practically every physician, not to say a great number of intelligent laymen, recognize the condition as an entity no matter what name they may give to it” (8). In the same chapter he goes on to describe the classic major symptoms and signs of the disease. In 1980 Hanifin and Rajka (9) recognized that the majority of doctors look at children with a dermatitis and clinically diagnose AD by pattern recognition. There were no uniform, standardized diagnostic criteria then, and there are still no specific diagnostic laboratory markers. These authors developed a list of criteria that would help in diagnosing AD. Hanifin and colleagues subsequently recognized that the original list was inappropriate for infants and children and twice developed a modified list of criteria (43). In 1985 Mevorah et a1 evaluated the diagnostic significance of minor clinical features in AD (2). When these were compared with the minor criteria assessed by the present authors in their original ar-

Hutchinson-Gilford syndrome.

The Hutchinson-Giiford syndrome (progeria) is an extremely rare disorder characterized by growth retardation and acceierated degenerative changes of the cutaneous, muscuioskeletal. and cardiovascular systems. In recognition of the prematurely aged appearance of these patients, Gilford (I) proposed the term progeria, which is derived from the Greek word for aging, geras. Since the first report by Hutchinson in 1886 (2), approximately 73 cases have been reported (3), with an estimated incidence of 1 in 8 miliion births (4). Most reported patients are Caucasian, with an equal sex distribution (4). Aithough the pathogenesis remains unclear, the disorder is considered to be a manifestation of either a random dominant gene mutation or an inherited autosomal recessive gene (5).