Haber's Syndrome Research Articles

Two Sporadic Cases of Haber's Syndrome: First Case Report from India.

Two Sporadic Cases of Haber's Syndrome: First Case Report from India.

A case of reticulate acropigmentation of kitamura: dowling degos disease overlap with unusual clinical manifestations.

Reticulate hyperpigmentary disorders are a group of rare genetic pigmentary abnormalities which includes reticulate acropigmentation of Kitamura (RAPK), Dowling-Degos disease (DD), reticulate acropigmentation of Dohi (RAPD), Haber's syndrome, and Galli-Galli disease. A 25-year-old male presented with asymptomatic dark-colored lesions on his hands and feet with light-colored skin lesions involving the trunk since three years. Dermatological examination revealed hyperpigmented macules in a reticulate pattern involving the dorsa of the hands and feet, front and sides of the neck, axillae, periorbital region, and groin. Multiple pits were present over both palms, with breaks in dermatoglyphics. He also had multiple nonacne facial scars predominantly on the nose and malar areas. The patient had overlapping features of RAPK and DDD. In addition, he also had hypopigmented macules and acneiform facial scars. Such an overlap of features of reticulate pigmentation has not been previously reported in the literature.

The spectrum of reticulate pigment disorders of the skin revisited

Within the group of classical reticulate pigment disorders of the skin, Galli-Galli disease (GGD), Dowling-Degos disease (DDD), Kitamura's disease (RPK), Haber's syndrome (HS), and reticulate acropigmentation of Dohi (RAD) are included and distinguished clinically and histopathologically. The clinical appearance of the reticulate pigment disorders of the skin is similar, with slight differences in age of onset and associated disorders. The histopathologic features of reticulate pigment disorders of the skin are comparable, with the exception of the unique hallmark of suprabasal acantholysis, which can be observed exclusively in GGD. Based on a critical discussion, we recommend using major and minor defining criteria for diagnosing skin lesions compatible with the reticulate pigment dermatoses of the skin. Herein we discuss a unifying nosological concept to provide straightforward diagnoses of the reticulate pigment disorders of the skin with a therapeutic impact.

Maladie de Bowen et carcinome épidermoïde au cours d’un syndrome de Haber : deux cas

Haber's syndrome is a rare form of autosomal dominant genodermatosis. Clinically, it is associated with rosaceiform dermatosis of the face that begins in childhood, and profuse keratotic lesions resembling seborrheic keratoses, seen predominantly on the trunk, the tops of the limbs and the scalp. We report two cases of Bowen's disease and cutaneous epidermoid carcinoma in Haber's syndrome patients. A 67 year-old woman with Haber's syndrome and with a familial history consulted for a budding lesion on the abdomen, histological examination of which confirmed epidermoid carcinoma. A 77 year-old woman presented a clinical picture consistent with Haber's syndrome, with three infiltrated erythematosquamous abdominal lesions. Histological examination of a biopsy sample confirmed the clinical diagnosis of Bowen's disease. The patient was successfully treated with imiquimod. These two cases appear to indicate the existence of an association between Haber's syndrome and the presence of cutaneous carcinomatous lesions. We propose the hypothesis of transformation of the keratoses seen in seborrheic keratosis. These lesions may be considered as pre-cancerous. Association with skin carcinomas requires regular monitoring of these patients. The use of imiquimod to treat lesions in patients with Bowen's disease resulted in complete cure.

Familial Syndromes with Skin Tumor Markers

A number of interesting syndromes have been described in which skin tumors are markers of heritable disorders. In Cowden's disease, Muir-Torre's syndrome, and Gardner's syndrome, benign skin tumors accompany and sometimes precede the development of internal visceral malignancy. The association of skin cancers with other abnormalities is found in nevoid basal cell carcinoma syndrome, Bazex syndrome, Rombo syndrome, xeroderma pigmentosum, dysplastic nevus syndrome, and epidermodysplasia verruciformis. Other genetic syndromes in which benign skin tumors herald the existence of systemic diseases include neurofibromatosis, tuberous sclerosis, Haber's syndrome, and Buschke-Ollendorff syndrome. Diagnosis of one of these syndromes may be ascertained by taking a thorough family history. Recognition of the skin tumor may trigger the proper questions regarding family medical history. Diagnosis hinges upon the physician having a high enough index of suspicion to link the appearance of the skin lesions to the diverse manifestations accompanying these disorders. Recognition will also set the stage for appropriate genetic counseling.

(35) Dowling-Degos disease with rosacealike eruption (Haber's syndrome)

(35) Dowling-Degos disease with rosacealike eruption (Haber's syndrome)

Haber's syndrome or Dowling-Degos disease?

Haber's syndrome or Dowling-Degos disease?

Haber's Syndrome or Dowling-Degos Disease?

<h3>To the Editor.—</h3> The report of three cases of Haber's syndrome (HS) in the June 1981Archives(117:321-324) by Kikuchi et al denigrates Dowling-Degos disease (DDD) as a possible alternative diagnosis. Clinically, the Japanese cases and, in particular, case 2 had axillary, black, verrucous lesions, "open comedo-like or milium-like papules," and "black papules" that closely resemble the dark dots and the diffuse seborrheic keratoses of the flexures of DDD. Dowling-Degos disease does not always have a reticulate pattern, and follicular lesions may well be the main manifestation.¹Even the histopathologic features shown in the article are strikingly similar to the delicate downgrowths encasing the enlarged follicles of DDD.¹ Actually, DDD does not show the central facial telangiectatic erythema described in HS, but facial erythema and burning sensations on wind exposure are not surprising features in youngsters with the dry skin and the "ichthyosis-like lesions over the lower extremities"

Haber's syndrome or Dowling-Degos disease?

Haber's syndrome or Dowling-Degos disease?

Haber's Syndrome or Dowling-Degos Disease?-Reply

In Reply.— Thank you for your interest in the article. Although there are several similarities between the cases diagnosed as Dowling-Degos disease and our cases, especially histologically, I think HS and DDD are different entities. Our patients did not have reticulate pigmented anomalies of the flexures but did have black warts and red faces. Haber's syndrome may be a disease of hereditary seborrheic keratoses. Rosacea, which is not found in DDD, is a striking feature of HS. One of our patients had used adrenal steroid ointments frequently, but the other two patients had not. However, there are indeed some similarities between HS and DDD that should be studied further. Another condition that should be differentiated from HS is familial, eruptive milia. The patient described by Heard et al 1 (not the first to be described) had multiple nonpigmented papules ranging from 1 to 3 mm in diameter on the face,

Haber's Syndrome

Three Japanese brothers had the rosacea-like facies and multiple intraepidermal epitheliomas that characterize Haber's syndrome. They also had xerosis of the lower extremities. The facial lesions were dry, warm, erythematous, and indurated, with enlarged follicular orifices and telangiectasia. One patient had disturbing "burning" of the face, which was relieved by short courses of steroid therapy that were given for systemic effects.

Haber's syndrome. Familial rosacea-like dermatosis with keratotic plaques and pitted scars.

A persistent rosacea-like rash affected the face of a 61-year-old Japanese man. The eruption began in early life with redness of the forehead, cheeks, chin, and nose. There are small follicular papules, scaly nodules, and depressed scars on the trunk and limbs. The microscopic features are irregular acanthosis of the epidermis and the presence of multiple epidermal cysts. There is a peculiar branching proliferation of basal cells from the underside of the epidermis and the pilosebaceous apparatus. A family history revealed that 15 members of his family have had the same sort of skin lesions since childhood.