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SUMMARY A man born with a unilateral epidermal naevus on the right side of the abdomen developed at puberty symmetrical benign acanthosis nigricans in the body folds. Tbe symmetrical eruption later disappeared completely. Tbe unilateral epidermal naevus and tbe symmetrical eruption showed identical bistological features, i.e. tbose of acantbosis nigricans. At tbe age of 32 be developed a muco-epidermoid cancer of the left parotid gland. A decision wbetber tbe association of unilateral epidermal naevi witb malignant tumours occurs witb unusual frequency requires (a) a long follow-up of the patient with the unilateral naevus, (b) a differentiation of the unilateral eruption from unilateral malignant acan-thosis nigricans and (c) statistical evidence. more...

Pigmentary changes of the skin are present in almost all cases of phenylketonuria, alkaptonuria, pellagra and hemochromatosis. An appreciable percentage of patients with various other internal diseases may show cutaneous pigmentary changes that are diagnostically significant, e.g., freckles and seborrheic keratoses, unilateral epidermal nevi, incontinentia pigmenti, lentiginosis centrofacialis, and poikiloderma. The different types of acanthosis nigricans are discussed. more...

A. n., eine benigne Hautkrankheit, kommt in 2 Formen vor. Die Form, die mit einem internen Krebs kombiniert ist, wird “maligne” genannt. Die Form ohne diese Kombination heist “benigne”. Der in der Kombination anzutreffende Krebs ist sehr maligne. Bei “maligner” A. n. aktivieren Krebsstoffe, bei “benigner” A. n. Sexualhormone das Hautleiden. “Benigne” und “maligne” A. n. sind als Genodermatosen zu betrachten. Es ist auf Grund von Familiengeschichten, dem Studium der Eigenschaften dieser Krebse und der Beziehungen zwischen ihnen und A. n. anzunehmen, das eine genetische Beziehungen zwischen A. n. und Krebs vorliegt, und das die mit A. n. vorkommenden Krebse eventuell genetisch bedingt sind. more...

In 1943 Miescher (1) reported peculiar atrophic changes of the skin of body and scalp in a 7 year old Swiss girl. There were spots of atrophic alopecia on the scalp, and on the extremities and trunk irregular zones in which dimple-like depressions were found at the place of follicular orifices. The child was very small and had thoracic kyphoscoliosis, lumbar lordosis, luxation of the right hip, a considerably shortened right leg, and a moderately shortened left arm. Five years earlier, Burckhardt (2) has described the skeletal condition of the same child, then 2 years old, as chondrodystrophia foetalis calcarea. In the English speaking countries this disease is called chondrodystrophia calcificans congenita, chondro-osseous dystrophy with punctate epiphyseal dysplasia, or similar variations. more...

A family examined by Curth and Macklin in 1952 and found to suffer from generalized or localized forms of ichthyosis, was reexamined in 1970. The concept that the disorder had been conditioned by a dominant gene which manifested itself with varying degrees of severity in the various family members, seemed proved again. more...

In a previous paper by one of us (H. O. C. 1 ) the separate character of benign and malignant acanthosis nigricans was stressed. Differences between the two types exist with regard to the age of the patient at the onset of the dermatosis, the course, and the activator of the disorder. It was also pointed out that there may be in the genetic background of the two types significant differences which could furnish additional evidence for the assumption that benign and malignant acanthosis nigricans are separate diseases. The result of our genetic studies has confirmed our early impression. Since some controversy over the necessity of recognizing different types of acanthosis nigricans has arisen, we shall briefly define benign and malignant acanthosis nigricans before reporting our genetic data on these forms of the dermatosis. Pseudo acanthosis nigricans, a third variety, will also be described, and the reasons more...

Clubbed fingers have been known since the fifth century B.C., when Hippocrates 1 described this sign in patients suffering from empyema. Hippocratic fingers were originally regarded as being secondary to certain intrathoracic disorders, but in the 20th century a hereditary type of clubbed fingers was recognized. Our observations on a family indicate that the disorder can exist without accompanying visceral disease and may occur in various degrees of severity. The Affected Family Members The proband * is a 41-year-old Negro † who is 5' 1" tall. He was born on St. Kitt's, British West Indies, and has had deformed nails since he can remember. They have not changed over the years. The nails of all fingers (Fig. 1) and both big toes are large and convexly curved in the longitudinal and transverse axes. The thumb nails measure 3.3 cm. at their greatest width and 2.3 cm. at their greatest length. The more...

Mit Kenntnis des Melanocytensystems und der chemischen Natur des Melanins ist man der Losung des Problems der Melanogenese einen grosen Schritt nahergekommen.

To the Editor.— Prolonged use of oral contraceptive drugs can be associated with the appearance of acanthosis nigricans in the axillae and other body folds. It is important to be aware of this possibility, since the affected individuals are middle-aged and may be suspected of having malignant acanthosis nigricans. They will, therefore, be subjected to unnecessary laboratory studies for an internal malignant tumor. Moreover, the dermatosis may not resolve unless the medication is discontinued. Report of a Case.— A 36-year-old woman, the mother of two children, was seen at the Vanderbilt Clinic in 1968 with a dermatosis of the body folds. She also had extremely dry skin, particularly on the lower extremities. The patient had been taking various oral contraceptives since 1960. Initially, norethynodrel with mestranol (Enovid) had been used, and later, norethindrone with mestranol (Ortho-Novum), and progestin, norethindrone, and mestranol (Norinyl 1 + 50) had been given. The eruption in more...

• Follicular atrophoderma apparently does not occur alone but is associated with other abnormalities. It is present in three groups: (1) If it is associated with chondrodystrophia calcificans congenita, it occurs only in girls and is due either to an X-chromosomal gene causing the changes in the heterozygote (ie, in the female sex) and prenatal death in the hemizygous state (ie, in the male sex), or to an autosomal dominant gene with expression almost wholly limited to the female sex; (2) If it is associated with Bazex's syndrome, it occurs in male and female persons in successive generations and is due either to an autosomal dominant gene or to an X-linked dominant gene; and (3) If it occurs in association with keratosis palmoplantaris dissipata, keratosis follicularis, and hyperhidrosis palmoplantaris, it may be due to a recessive gene or a mutation. (Arch Dermatol114:1479-1483, 1978) more...

The purpose of this report is to call attention to an interesting lesion of the skin which occurs in association with characteristic lesions of the bones. Only a few cases of this disease have been reported, but the lesions are so inconspicuous that it is possible that many cases have been overlooked. OSTEOPOIKILOSIS In 1915 Albers-Schoenberg1described the case of a man, 22 years old, in whom roentgen examination revealed numerous minute lesions in all bones except the skull, spine, scapula, patella and clavicle. They were located chiefly in the epiphyses, though isolated lesions were found in the diaphyses. The lesions appeared as minute oval areas of condensation, usually with the long axis in the direction of the long axis of the bone. Their size varied from that of the head of a pin to that of a pea. They were sometimes so numerous as to make the bone more...

Under the name Parinaud's conjunctivitis a peculiar inflammation of the conjunctiva of one eye with subsequent swelling of the regional preauricular and submaxillary lymph glands has been described. The various organisms that have been accused as possible causative agents include the tubercle bacillus (human and bovine), Bacterium tularense, Spirochaeta pallida, the glanders bacillus, Sporothrix, Streptococcus and Leptothrix. The virus of venereal lymphogranuloma has also been listed among the etiologic organisms. In this paper we are adding to the small number of recorded cases another case of conjunctivitis due to the virus of venereal lymphogranuloma. The French ophthalmologist Morax 1 was the first author to call attention to the etiologic relationship of the virus of venereal lymphogranuloma to some cases of Parinaud's conjunctivitis, although he had no case of his own to prove his point. In the case of Levaditi, Bollack, Basch and Desvignes, 2 marked unilateral conjunctivitis of the bulb more...

To the Editor.— In the report "Nevus Unius Lateralis, Brain Tumor, and Diencephalic Syndrome," which appeared in the May issue of theArchives( 95 :501, 1967), Capt Meyerson stresses the association of a neurological disorder with a dermatological condition. He does not, however, discuss the association of a unilateral epidermal nevus with a tumor. Such an association was also present in this patient and was previously reported by Pack and Sunderland ( Arch Surg 43 :341, 1941) and myself ( Postgrad Med 41 :447, 1967). Pack and Sunderland saw unilateral epidermal nevi associated with a mammary adenocarcinoma, and epidermoid esophageal cancer, and an epidermoid cancer of uncertain origin in three patients, respectively. It is possible that in one instance the authors were dealing with unilateral malignant acanthosis nigricans. I observed a patient who had benign symmetric acanthosis nigricans at puberty, a unilateral epidermal nevus indistinguishable from acanthosis nigricans since birth, and a more...

To the Editor.— Altman and Stroud,1recently reported on two patients with typical features of both ichthyosis linearis circumflexa and Netherton's syndrome. In 1962 I presented a 22-year-old Negro woman with ichthyosis serpentina, bamboo hair, periodic loss of scalp, axillary, and pubic hair, atopic eczema, pulmonary tuberculosis, and asthma.2A 2-year-old sister (among seven normal siblings) had begun to show broken hair shafts. Ichthyosis serpentina, a mild form of ichthyosis vulgaris, therefore, seems to be another variant—not necessarily psoriasiform—of ichthyosis associated with trichorrhexis invaginata (bamboo hair). Since atopy has been present in some of the reported cases but has been missing in others, this component does not seem to be a necessary part of the syndrome. more...

FIFTY per cent of cases of acanthosis nigricans, of which 420 cases have so far been reported,1are associated with internal cancer. Acanthosis nigricans provides an unexpected and helpful natural experiment for cancer research. Strong2recommended the study of linkage to investigators of the origin of neoplastic tissue: "If one can demonstrate that the tendency to develop carcinoma is linked to ( that is, carried on the same chromosome with) any hereditary color or morphological character, then one could prove that the new condition, i. e., the production of spontaneous carcinoma, is definitely inherited." In the problem confronting us my colleagues and I are dealing with human beings and not with experimental animals. Instead of hereditary color and morphologic character we are presented with discoloring and verrucous cutaneous disease which in a high percentage shows signs of genodermatosis. i. e. dermatosis originating from the germ plasm. The advantages and more...

The classification into inflammatory and noninflammatory dermatoses which led, to cite a few examples, to the listings of exfoliative erythroderma, lichen planus, and mycosis fungoides as noninflammatory, and those of epidermolysis bullosa and pemphigus as inflammatory dermatoses, is controversial. Apart from this questionable classification, however, the two volumes can be highly recommended to American dermatologists. The roster of authors is international. Seborrheic dermatitis is discussed (in English) by Paul Gross (with J. T. McCarthy), the bullous and pustular dermatoses, and the deposits of the body's own metabolic products, by Walter F. Lever. Stephan P. Brunauer is (with Sulzberger and March) the co-author of the chapter on the Sulzberger-Garbe disease. Gross, Lever, and Brunauer had their dermatological training in Europe before becoming acquainted with American dermatology, and with their contributions we are getting the best of two worlds. Sulzberger, one of the two original describers of the Sulzberger-Garbe disease, is co-author more...

The familial incidence of incontinentia pigmenti and its almost exclusive occurrence in females have been explained by two theories. One theory (Pfeiffer 36 ) suggests that the disease is due to an autosomal, dominant gene, which is sex-limited in its expression, producing the defect only in females. The other theory (Lenz 3 ) suggests that incontinentia pigmenti is due to a sex-linked gene carried on the X-chromosome. This gene acts as a dominant in females and is lethal in males. The pros and cons of each theory are discussed. The Franceschetti-Jadassohn type of incontinentia pigmenti is clinically and genetically different from the classic type. A third of "transitional" cases occurs in boys and girls. Its genetic mechanism has not yet been determined. more...

To the Editor:— It is of the greatest importance to differentiate malignant acanthosis nigricans, which is one of the best external clues to internal adenocarcinoma, from benign and pseudo acanthosis nigricans, which do not threaten the patient's life. The paper by Winkelmann, Scheen, Jr., and Underdahl (The JournalOct. 29, 1960, p. 1145), however, does not decrease the difficulties of differentiating the various types. Winkelmann et al . apply to the benign type, which is present at birth or begins in childhood or at puberty, the term "congenital," which they do not use in its strict sense, meaning present at birth, but as "referring to the state [of acanthosis nigricans] having been present since infancy." What they call benign post-pubertal acanthosis nigricans is either malignant or pseudo acanthosis nigricans, since the benign type does not begin after puberty. It seems difficult to connect, as they do, acanthosis nigricans with so many more...

THIS is a report on cases of a recently (1937) described clinical entity of recurrent ulcerations of the oral cavity and genitals and of repeated attacks of uveitis which, in later stages, is associated with hypopyon. The disease of the eyes may lead to complete loss of vision. Cases of this syndrome had already been reported before 1937. Bluthe1in 1908, Adamantiades2in 1931 and Dascalopoulos3in 1932 observed patients with this triad. In 1937, Behcet4gave the syndrome the name of triple symptom complex, when he recognized the interrelationship of various pathologic processes in one patient. More cases of the triple syndrome or of part of it will, in all probability, be found in the literature reported by dermatologists or gynecologists as cases of recurrent ulcus vulvae acutum or, erroneously, of chancroids, by internists and dentists as cases of recurrent oral aphthae and by more...

The association of acanthosis nigricans and cancer in a great number of cases is well known. This association occurs in 45 per cent of the patients with acanthosis nigricans, according to Mukai;1in 45.3 per cent, according to Kuttner,2and in 54.5 per cent, according to Moncorps,3who included cases of definite and probable cancer. The form of acanthosis nigricans which is associated with cancer is called malignant.4 Patients with the benign type of acanthosis nigricans do not suffer from cancer. This negative fact alone, not any difference in the macroscopic or histologic features of the two forms, determines the diagnosis of the benign type. It is true that only in one or two cases has the malignant form been observed in infancy or childhood and that it is the benign type which usually appears in early life, but no sharp division can more...

To the Editor.— I have for years been interested in the relationship between benign dermatoses and malignant internal processes. The list of these associations is steadily growing. Not every dermatosis occurring in a patient suffering from malignant disease, however, is causally related to the malignant disease, and several of the following criteria must be fulfilled before the association of the two conditions can be considered causal. These criteria are: (1) The dermatosis and the malignant tumor begin at about the same time. (2) The two disorders follow a parallel course. (3) The tumor may be uniform or distinctive. (4) In certain syndromes a genetic basis is responsible for the two conditions (and criteria 1 and 2 need not be fulfilled). (5) There is statistical evidence for the association of the two disorders. In Kiely and Perry's patient (Arch Derm 100 :63-65 [July] 1969) none of the above criteria (perhaps with more...

To the Editor:— In the September issue of theArchives of Dermatology and Syphilology( 44 :349, 1941) Leslie Nicholas, describing an interesting case of acanthosis nigricans, stated: "In no instance heretofore, however, has it [acanthosis nigricans] been described in association with and immediately overlying deep metastatic malignant growths of the skin." There is, however, a case reported by W. Dubreuilh ( Ann. de dermat. et syph. 7 :67, 1918) in which metastases of a cancer of the stomach were found in the skin underlying acanthosis nigricans. This is the case of a 22 year old French soldier, who died about four months after onset of severe gastric symptoms and eight months after acanthosis nigricans made its first appearance. At autopsy generalized acanthosis nigricans, ulcerated carcinoma of the pyloric region and metastases in the deep skin or on the border of the hypoderm, in many lymph glands and in the lymph vessels more...

To the Editor:— "Acanthosis Nigricans and Pituitary Tumors" ( 198 : 619, 1966) by J. Brown, R. K. Winkelmann, and R. V. Randall discusses a multitude of conditions associated with acanthosis nigricans without classifying them beyond a division into malignant or benign types. However, classification into several types is necessary since great differences with regard to prognosis, inheritance, and reversibility exist between acnathosis nigricans in the various associations. I suggest the following classification: Type I: Malignant acanthosis nigricans, associated with an internal adenocarcinoma, occurring usually after puberty, usually fatal, and not proved to be genetically determined. Types II to IV: Not associated with an internal adenocarcinoma and therefore "benign" in a broad sense of the word. Type II: True benign acanthosis nigricans, present at birth or beginning in childhood or at puberty, resembling ichthyosis hystrix, inherited in an irregularly dominant manner, and not associated with endocrine disease. Type III: Pseudo- (secondary) acanthosis more...

To the Editor:— In a clinical note inThe Journalfor Sept. 18, 1954, page 242, Dr. Rothman described pituitary basophilism in the juvenile type of acanthosis nigricans in a 41-year-old man weighing 345 lb. The author states that patients with disease of the benign or juvenile type are "usually excessively obese." Obesity is not a common feature in the benign type. Among 30 patients whom I examined, 28 were of normal weight and only 2 were slightly overweight. The features that distinguish the benign from the malignant type of the dermatosis may be summarized as follows. Benign acanthosis nigricans starts at birth, in childhood, or at puberty. After the onset of the dermatosis the process remains stationary or spreads. Extension occurs most frequently at puberty, but the dermatosis remains stationary or regresses after that period. Malignant acanthosis nigricans may start at any age. It may occur in children, but more...

Acanthosis nigricans, rather widely discussed in dermatologic handbooks and journals, 1 has been strangely neglected in medical and surgical literature and in publications dealing with cancer. This is the more difficult to understand since in a great number of cases the occurrence of this characteristic cutaneous disease in itself suggests the presence of a coexisting internal cancer in the patient. The association with cancer occurs in 49.74 per cent of all cases of acanthosis nigricans. So far 384 cases of the disease have been reported. The study of a noncancerous disease associated with cancer under definite conditions offers great advantage to cancer research, which becomes wider by the additional investigation of the associated phenomenon and of its relationship to cancer. It is particularly fortunate that this associated phenomenon is represented by a well defined disease of the skin visible at early stages of development. DEFINITION Acanthosis nigricans is a benign more...

To the Editor. —In their excellent paper entitled ``Bullous Variety of Incontinentia Pigmenti (Bloch-Sulzberger)'' (A. M. A.Arch. Dermat. & Syph. 65 :557-567 [May] 1952), Epstein, Vedder, and Pinkus state that they saw no signs of follicular atrophoderma upon examination of their patient at the age of 16 months. The authors, however, noted agenesis of the skin appendages in the histologic pictures of specimens taken at the ages of 19 days and one year. Agenesis of skin appendages was noted in the histologic pictures of follicular atrophoderma of two girls observed by me (Curth, H. O.: Follicular Atrophoderma and Pseudopelade Associated with Chondrodystrophia Calcificans Congenita, J. Invest. Dermat. 13 :233-247 [Nov.] 1949) at the ages of 8 and 9 years, respectively. In the 9-year-old child and her 4-year-old sister incontinentia pigmenti was also present. I saw my patients with incontinentia pigmenti and their 37-year-old mother for the first time at more...

ACANTHOSIS nigricans is significant not only to dermatology; its ramifications and associations extend to many branches of medicine. This paper elaborates on aspects of acanthosis nigricans which are of interest to various medical disciplines. Two types of acanthosis nigricans, a dermatosis benign in itself, are observed. The type associated with cancer of an internal organ is because of this association called "malignant." The type of the dermatosis not so associated is called "benign." The quotation marks denote that it is illogical to differentiate the two types by such qualifications. The terms "malignant" and "benign" have, however, been retained for practical reasons. These designations are far more satisfactory than the terms "adult" and "juvenile," since children may suffer from and die of "malignant" acanthosis nigricans, while otherwise healthy, older persons have suffered from "benign" acanthosis nigricans for many years. "Benign" acanthosis nigricans begins at birth or in childhood or more...