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Collodion baby and harlequin fetus are two clinical entities that have characteristic appearances that mark their diagnosis. Yet, this is only the beginning of unraveling the congenital ichthyosiform dermatosis causing it, as they are mere phenotypic forms, not disease entities. Autosomal recessive ichthyosiform diseases are responsible for a majority of collodion babies. Both syndromic and nonsyndromic ichthyosis disorders cause collodion in babies; thus, these babies need to be thoroughly evaluated during hospital stay and during the follow-up period as well. The course of a collodion baby can range from self-healing to significant morbidity. It is more worse in the harlequin fetus. Neonates with both conditions need neonatal intensive care unit hospitalization. The collodion membrane gradually sheds in the majority, revealing the ichthyosis in time. Skin barrier maintenance is extremely important, as fragility of the skin leads to a sequelae of complications. Otherwise, treatment modalities depend on general care and temperature regulation, and retinoids have shown promising results. Both conditions are described, and the approach needed in evaluation of the collodion baby is highlighted.

We present a clinical case of ichthyosis hystrix, a rare genetic ichthyosiform dermatosis. Specifics of the disease are related to the complexity of differential diagnosis and verification of the diagnosis, because clinical manifestation of ichthyosis hystrix is similar with that of verrucous epidermal nevus. Clinical particulars of both nosologies are characterized by bizarre and widespread skin lesions of spicular hyperkeratotic growth, located along Blaschko lines. The exclusive feature of the clinical case described is a long-term (20 years) clinical and laboratory monitoring of the patient. The literature review provides brief information on etiology, pathogenesis, clinical features, differential diagnosis, and pathomorphology of ichthyosis hystrix and verrucous epidermal nevus.

A Soft-Coated Wheaten Terrier with an ichthyosiform dermatosis is described. Patches of thick, scaly skin were apparent from birth and progressed in extent as the dog aged. At 3 months of age, the scaling was generalized and was accompanied by a greasy exudation which matted the haircoat. The skin was greasy and there was generalized scaling, but the skin of the head and pinnae of the ears was most affected. There was minimal involvement of the foot-pads. Microscopically, the skin had patchy areas of ortho-and parakeratotic hyperkeratosis, follicular keratosis, superficial perivascular dermatitis, and variable hypergranulosis. Electron microscopical findings were consistent with proliferative hyperkeratosis. The dog did not respond to treatment with benzoyl peroxide shampoos, humectant sprays, and isotretinoin. Comparative features of skin diseases similar to this canine ichthyosiform dermatosis are discussed. Resume— Une dermatose ichtyosiforme est observee sur un Soft-Coated Wheaten Terrier. Des zones cutanees squameuses et epaissies apparues des la naissance s'etendent avec l'âge. A l'âge de 3 mois, l'etat squameux est generalise et associe avec une seborrhee grasse qui collent les poils entre eux. Les lesions sont generalisees avec toutefois un predilection pour la face et les pavilions auriculaires. Les lesions podales sont peu importantes. Les lesions histopathologiques sont caracterisees par une hyperkeratose orthokeratosique et parakeratosique, une keratose folliculaire, une dermite perivasculaire superficielle et une hypergranulose variable. La microscopic electronique montre une hyperkeratose proliferative. Le chien n'a pas repondu aux shampooings au peroxyde de benzoyle, aux sprays hydratants et a risotretinoi'ne. Les aspects compares de cette dermatose ichtyosiforme sont discutees. [Helman, R. G., Rames, D. S., Chester, D. K. Ichtyosiform dermatosis in a Soft-Coated Wheaten Terrier. (Dermatose ichtyosiforme sur un Soft-Coated Wheaten Terrier.) Veterinary Dermatology 1997; 8: 53–58.] Resumen Se describe una dermatosis ictiosiforme en un Soft Wheaten Terrier. Desde el nacimiento presentaba areas de piel descamativa y engrosada que se extendieron con la edad. A los tres meses, la descamacion era generalizada y se acompanaba de un exhudado oleoso que enmaranaba el pelo. La piel se encontraba oleosa y habia descamacion generalizada, siendo la piel de la cabeza y pabellones auriculares la mas afectada. Los cojinetes se encontraban poco afectados. Microscopicamente la piel presentaba areas dispersas de hiperqueratosis orto-y paraqueratotica, queratosis folicular, dermatitis perivascular superficial e hipergranulosis variable. Los hallazgos ultraestructurales fueron compatibles con una hiperqueratosis proliferativa. El perro no respondio a un tratamiento con champu de peroxido de benzoilo, esprais humectantes e isotretinoina. Se discuten aspectos comparativos de enferemedades cutaneas similares a esta dermatosis ictiosiforme canina. [Helman, R. G., Rames, D. S., Chester, D. K. Ichthyosiform dermatosis in a Soft-Coated Wheaten Terrier. (Dermatosis ictiosiforme en un Soft Wheaten Terrier.) Veterinary Dermatology 1997; 8: 53–58.] Zusammenfassung— Es wird eine ichthyosiforme Dermatose bei einem Soft Coated Wheaten Terrier beschrieben. Von Geburt an waren Bezirke mit dicker, schuppiger Haut sichtbar, die sich in der Ausdehnung mit zunehmendem Alter der Hundes vergroserten. Im Alter von drei Monaten war die Schuppenbildung generalisiert und wurde von einer schmierigen Exsudation begleitet, die das Haarkleid verklebte. Die Haut war schmierig und das Schuppen generalisiert, jedoch war die Haut am Kopf und den Ohren am starksten betroffen. Die Pfotenballen waren nur minimal miteinbezogen. Bei der mikroskopischen Untersuchung zeigte die Haut herdformige Bezirke von ortho-und parakeratotischer Hyperkeratose, follikularer Keratose, oberflachlicher perivaskularer Dermatitis und variabler Hypergranulose. Die elektronenmikroskopischen Befunde gingen mit der proliferativen Hyperkeratose konform. Der Hund sprach auf die Behandlung mit Benzoylperoxid-Schampoos, feuchigkeitsspendenden Sprays und Isotretinoin nicht an. Vergleichbare Erscheinungen von Hauterkrankungen, die dieser caninen ichthyosiformen Dermatose ahneln, werden diskutiert. [Helman, R. G., Rames, D. S., Chester, D. K. Ichthyosiform dermatosis in a Soft-Coated Wheaten Terrier. (Ichthyosiforme Dermatose bei einem Soft Coated Wheaten Terrier.) Veterinary Dermatology 1997; 8: 53–58.]

Two males and a female in a family of seven children from consanguineous parents showed a generalized ichthyosiform dermatosis characterised by spiney hyperkeratosis. Bilateral neurosensory deafness affected only the older child, aged 10 who, in addition, was totally blind. His 1–year–old brother developed photophobia and severe corneal ulcers, and his 5–year–old sister suffered from dry eyes due to scarcity of tears. The three patients showed hair defects, with different degrees of alopecia and alterations of the hair–shaft, including eliptical cross–section tike woolly–hair. We stress the clear recessive inheritance of this association, its wide clinical variability and the less known hair shaft alterations.

Summary An unusual ichthyosiform dermatosis with circumscribed areas of blistering hyperkeratoses as well as scaly areas was observed in five consecutive generations of a German family. Light and electron microscopical studies revealed oedema of the keratinocytes in the upper epidermis but no defects of tonofilaments and keratohyalin granules. We suggest that this type of ichthyosis exfoliativa inherited as an autosomal dominant trait represents a new and distinct entity.

Keratitis-ichthyosis-deafness (KID) syndrome is a genetically determined disorder of keratinization, a congenital ichthyosiform dermatosis with additional extracutaneous features (keratitis and deafness). Because of the association of ichthyosis or ichthyosiform dermatosis with central and/or peripheral nervous system involvement, KID syndrome has been (and is) traditionally defined as a neuroichthyosis or neurocutaneous disorder.

Ichthyosis hystrix is a term used to describe an autosomal dominant rare form of an ichthyosiform dermatosis which is characterized by hyperkeratotic spiny scales. We present a healthy 22-year-old man with lesions which clinically and histopathologically corresponded to the rare inherited disorder ichthyosis hystrix.

Ichthyosis hystrix is a term used to describe an ichthyosiform dermatosis which is characterized by hyperkeratotic spiny scales mainly over extensor aspects of limbs with palmoplantar keratoderma and occasionally associated with deafness and neurological deficit. It is a rare autosomal dominant form of ichthyosis and very few cases are reported in literature. We are presenting a 46 years-old-male patient of ichthyosis hystrix with unusual presentation. He had lesions mainly over the face and scalp with palmoplantar keratoderma and significant nail changes.

A 2-year-old male with KID syndrome was presented. Family history was negative for similar skin disease. The patient showed generalized ichthyosiform erythroderma, vascularizing keratitis, and neurosensory deafness. Additional clinical features included nail dystrophy, hypotrichosis, anhidrosis, and recurrent cutaneous bacterial and fungal infections. Histologic examination disclosed a basket-weave pattern of hyperkeratosis and irregular papillomatous configuration of the epidermis. Electron microscopic examination showed an excessive amount of glycogen in the cytoplasm of erector pili muscle cells. This is the first case reported in Japan.

The clinical, light microscopic, and fine structural features of an unusual case of ichthyosiform dermatosis are presented. Clinically, this 44 year old Caucasian male had been affected by stable ichthyosis hystrix-like lesions on his extremities since infancy. In middle age, clinically different, but histologically similar lesions developed in the form of scaly plaques all over his trunk as a second phase. Microscopically, the epidermis consisted of abnormally large prickle cells, which faded without formation of a distinct granular layer into a thick and mainly orthokeratotic stratum corneum. The cytoplasm of the prickle cells showed division into inner and outer zones separated by a peculiar shell. While the exoplasmatic zone had fairly normal structures including well developed tonofilament-desmosome complexes, the inner zone was devoid of tonofilaments, but rich in mitochondria, endoplasmic reticulum, and other organelles. The shell appeared to consist of disorganized tonofilaments associated in higher layers with small keratohyalin granules. While no structured membranes divided the zones, there seemed to be a phase boundary which prevented keratohyalin granules and tonofilaments from entering the endoplasm. The term biphasic ichthyosiform dermatosis thus seems applicable on the clinical as well as on the fine structural level.

• Keratoderma hereditaria mutilans (KHM), or Vohwinkel's syndrome, is a rare genodermatosis consisting of hyperkeratosis of the palms and soles with a characteristic "honeycomb" appearance, keratotic structures taking the shape of a starfish and/or knuckle pads on the dorsal surfaces of the hands, and constricting bands (pseudoainhum) encircling digits of the hands and feet. We describe three cases of a variant of KHM with an associated ichthyosiform dermatosis in a pedigree consisting of 19 affected individuals through six generations. An autosomal dominant inheritance pattern for KHM was confirmed. One of the patients was successfully treated with isotretinoin, 0.6 mg/kg/day orally. We offer five hypothetical genetic models to account for the simultaneous expression of palmar-plantar keratoderma and ichthyosiform dermatosis. ( Arch Dermatol 1984;120:1323-1328)

• Acquired ichthyosis was recognized in four patients, in whom the associated malignant disorders were carcinoma of the lung, breast, and cervix. This skin lesion may be a valuable clinical sign in recognizing the existence of a malignant condition. Although the emergence of an ichthyosiform dermatosis may have serious prognostic implications, it may also be useful in monitoring the course of the malignant disorder and the patient's response to therapy. The relationship to nonlymphoproliferative malignant neoplasms may be more common than has been previously recognized. ( Arch Dermatol 111:1446-1447, 1975)

Five patients in a large pedigree with autosomal dominant palmoplantar keratoderma and/or an ichthyosiform dermatosis were studied. Biopsies of the keratoderma, a remote hyperkeratotic plaque on an arm and the ichthyosis all revealed the same histopathologic changes including hyperkeratosis with round retained nuclei. All 5 patients had elevated serum beta-glucuronidase activity. The significance of beta-glucuronidase in this and other skin diseases is discussed.

To the Editor.— I read with great interest the article written by Cram and associates in theArchives(115:467-471, 1979), describing two children who shared a similar constellation of defects including an atypical ichthyosiform dermatosis, neurosensory deafness, vascularization of the corneas, anhidrosis, and abnormalities of the hair and nails. The authors also referred to 14 other patients described previously who displayed the same pattern of malformation. An isolated but important abnormality not mentioned in the article by Cram et al that occurred in one of those 14 individuals was reported by Baden and Alper. 1 One of the two patients they described was noted at age 7 years to have a nodule of the tongue that, on biopsy, proved to be a squamous cell carcinoma; there was no evidence of either regional or distant metastases and the tumor was treated with irradiation. There has been no recurrence in eight years

To the Editor.— I believe that there are more reported cases of the syndrome of ichthyosiform dermatosis and deafness as discussed by Baden and Alper (Archives113:1701-1704, 1977). Very recently, Senter and colleagues 1 added another case; they included in their summary a similar case of Salamon et al 2 and three cases of Tay 3 who clearly lack some of the predominant features. Still another patient who was described in 1975 by Pincus et al 4 seems to belong to this group. In addition to the ichthyosiform skin disorder and neurosensory deafness, her patient demonstrates several of the other features common in this syndrome: loss of vision owing to progressive vascularization of the corneas; abnormalities of the hair, teeth, and nails; postnatal growth deficiency; and neuromuscular defects and recurrent skin infections. The patient in Pincus's case report is now 8 years old and has had hyperkeratotic and erythematous skin from

• Four members of a consanguineous family showed a congenital disorder characterized by an ichthyosiform dermatosis, sclerosing palmoplantar keratoderma, and multiple keratotic papules arranged in bands with a linear, cordlike distribution. This association seems to represent a distinct entity. The differential diagnosis is described. (Arch Dermatol1989;125:103-106)

In Reply.— I read with interest the letter by Dr Silvestri and agree that the patient described by Senter et al has the same disorder on which we reported. The other three case reports I believe represent different disorders, although they have certain similarities to our cases.

Two sisters and two unrelated patients with an ichthyosiform dermatosis resembling nonbullous ichthyosiform erythroderma were found to have lipid accumulations in the granulocytes of the peripheral blood, in the granulocyte precursors in the bone marrow, and in the liver. In each case, the blood anomaly was readily detected on examining a peripheral smear. One patient showed many of the features seen in Refsum syndrome. This association of findings in four patients may constitute another neurocutaneous lipidosis.

Two patients had an unusual ichthyosiform dermatosis, neurosensory deafness, and vascularizing keratitis. There was no family history of the disorder and no consanguinity. A squamous cell carcinoma of the tongue developed in one child. The association of such an eruption and deafness in previously reported cases is reviewed.

Historical Considerations Porokeratosis is a disease characterized by sharply circumscribed, kerato-atrophic areas in the midst of unchanged skin occurring in sites of predilection. The lesions are usually multiple—rarely single; familial incidence is frequent, with males being affected twice as often as females. Although Majocchi1was the first to recognize porokeratosis as an entity (1883), naming it "linear and hystrix ichthyosiform dermatosis," his successor Mibelli described the condition in such detail (1893) that it bears his designation (porokeratosis). Independently of Mibelli, Respighi described it and named it "hyperkeratose figuree centrifuge atrophicante." Gilchrist, in the United States, emphasized the hereditary aspects of the dermatosis.1 Porokeratosis is a rare disease, yet Bopp2encountered 16 cases in one year. Although there are numerous reports of individual cases in the literature, detailed and exhaustive studies of the disease are nevertheless lacking. In their report published in 1943, Bloom and Abramowitz3prophesied