In adrenal insufficiency, recognition of the disease in its earlier phases may present a real challenge. The disease, if unrecognized and untreated, carries an almost uniformly poor and frequently fatal prognosis. We report a rare case of congenital adrenal aplasia accompanied by hyperpigmentation on the whole body surface. The diagnosis was confirmed by ACTH stimulation test and autopsy findings.
Adrenal Insufficiency ; Adrenocorticotropic Hormone ; Autopsy ; Diagnosis ; Hyperpigmentation* ; Prognosis

solated ACTH deficiency is a very uncommon cause of hypoadrenocorticism, with less than 200 cases reported in the literatures. The clinical presentation can be similar to that of primary adrenal insufficiency, but there is a greater tendency for hypoglycemia and absence of hyperpigmentation. The diagnosis is established by demonstrating hypocortisolism with undetectable serum levels of ACTH, normal adrenal responsiveness to prolonged ACTH infusion, and an absent ACTH response to insulin-induced hypoglycemia. Other endocrine function is normal.We experienced a case of isolated ACTH deficiency in 34 years old female who was admitted due to fever and drowsy mentality.So we present this case with a review of literatures.
Addison Disease ; Adrenocorticotropic Hormone ; Diagnosis ; Female ; Fever ; Humans ; Hyperpigmentation ; Hypoglycemia

We report two cases of lichen planus pigmentosus (LPP) that developed in a unilateral linear pattern. The patients presented with unilateral linear brown macules on the extremities. Skin biopsy showed orthokeratosis, basal hydropic degeneration with scarce lymphohistiocytic infiltrates, and numerous melanophages in both patients. These patients, to the best of our knowledge, are the first cases of LPP presenting with a linear pattern. LPP should be considered in the differential diagnosis of linear hyperpigmented skin lesions.
Adolescent ; Adult ; Biopsy ; Female ; Human ; Hyperpigmentation/diagnosis/pathology ; Lichen Planus/*diagnosis/pathology ; Skin/pathology

BACKGROUND: Syringoma is a relatively common benign appendageal tumor derived from the intraepidermal eccrine ducts. It occurs predominantly in women at puberty or later in life. The lesions usually are multiple and may be present in great numbers. Histopathologically, numerous small ducts are embedded in a fibrous stroma and their walls are lined by two rows of epithelial cells. OBJECTIVE: The aim of this study is to investigate clinical and histopathologic features of syringoma in Koreans. METHODS: Total 32 patients were included in this study. Data complied were sex, age, onset of disease, symptoms, family history, localization, distribution, and some histopathologic findings including acanthosis, basal hyperpigmentation, proliferation of fibrous stroma, vacuolization of cells, keratin-filled cyst, and characteristic tad-pole appearance. RESULTS: 1. It was observed predominantly in females as the sex ratio of 31:1. The mean age at diagnosis was 37.5-years-old. 2. The age of onset was in the 2nd decade in 9 patients, 3rd decade in 10 patients, 4th decade in 7 patients, 5th decade in 5 patients, and 7th decade in 1 patient. 3. Only two patients complained of mild itching. 4. Family history was noted in 14 patients. 5. It occurred most frequently in the upper and lower eyelids(28 cases), and followed by forehead(5 cases), genitalia(3 cases), temple(2 cases), cheek(1 case), and neck(1 case). 6. The distribution was bilateral except two patients. 7. The color of lesion showed skin-colored(23 cases), yellowish(6 cases), and brownish (3 cases). 8. Twelve cases of acanthosis, 11 cases of basal hyperpigmentation, 26 cases of proliferation of fibrous stroma, 13 cases showing vacuolization of inner cell layer of ducts, 8 cases showing keratin-filled cysts and 15 cases showing characteristic tad-pole appearance were observed. CONCLUSION: According to our study, syringoma predominantly occurs in eyelids of women as bilateral asymptomatic skin-colored papules and histopathologic findings frequently shows keratin-filled cysts.
Adolescent ; Age of Onset ; Diagnosis ; Epithelial Cells ; Eyelids ; Female ; Humans ; Hyperpigmentation ; Pruritus ; Puberty ; Sex Ratio ; Syringoma*

Nail-patella-elbow syndrome is a relatively rare autosomal dominant disorder characterized by nail and skeletal defects, especially of the patella. The principal manifestations include hypoplastic or absent patellae, dysplastic finger-nails, dislocated radial heads with limited elbow motion, and often iliac horns. A less common but not infrequent manifestations are hereditary nephropathy and hyperpigmentation of the pupillary margin of the iris ("Lester iris"). A 17-year-old man was presented with abnormalities of both the 1st and 2nd finger-nails since birth. He had triangular lunulae and hypoplastic nail plates. Two months ago, the diagnosis of subluxation of both elbow joints was made as limitation of motion and radiologic findings of both knee joints revealed hypoplastic patellae. Routine laboratory studies were all within normal limits. The patient's father had triangular lunulae and hypoplastic nail plates on both 1st finger-nails. We report a case of nail -patella syndrome with the review of literature.
Adolescent ; Animals ; Diagnosis ; Elbow Joint ; Elbow* ; Fathers ; Head ; Horns ; Humans ; Hyperpigmentation ; Iris ; Knee Joint ; Parturition ; Patella*

One case of malignant acanthosis nigricans associated with internal malignancy has been presented and the suhject of malignant acanthosis nigricans has been revieived. The patient who was 50 year old man had been affected generalized hyperpigmentation with pnpillomatosis especially on forehead, neck, axilla, umbilical area, anogenital area, palm and soles and associated with highly malignant adenocarcinoma of stomach. Diagnosis was confirmed by characteristic clinical appearance, explorative laparatomy and histopathologically
Acanthosis Nigricans* ; Adenocarcinoma ; Axilla ; Diagnosis ; Forehead ; Humans ; Hyperpigmentation ; Middle Aged ; Neck ; Stomach

Laugier-Hunziker syndrome (LHS) is a rare acquired benign macular hyperpigmentation of the lips and oral mucosa which is often associated with longitudinal melanonychia. LHS is known to be an entirely benign condition with no systemic manifestations, which requires patient reassurance as the only intervention. The significance of this condition is due to its inclusion in differential diagnoses of pigmentary disorders of the oral mucosa, especially Peutz-Jeghers syndrome. We report a case of Laugier-Hunziker syndrome in a 66-year-old woman who presented with longitudinal pigmented bands on her fingernails and multiple, pigmented macules on the lip and tongue.
Aged ; Diagnosis, Differential ; Female ; Humans ; Hyperpigmentation ; Lip ; Mouth Mucosa ; Nails ; Peutz-Jeghers Syndrome ; Tongue

BACKGROUND: Partial unilateral lentiginosis (PUL) is a rare pigmentary disorder characterized by grouped multiple lentigines on otherwise normal skin that histologically have the typical features of lentigo. This entity has been only rarely reported in the Korean population. OBJECTIVE: The purpose of this study was to evaluate clinical and histopathologic characteristics, association with other disorders, and differential diagnosis of PUL. METHODS: We reviewed our experiences of thirteen cases of PUL which had been collected in our dermatology clinic during the 6-year period between 1993 and 1998. RESULTS: Twelve patients were female and one was male. In 3 patients the lesions appeared after the age of 20 years. Ten patients had the lesions on the upper part of the body, the neck being the most common location. No bias was shown in terms of the side of the body affected. Cafe-au-lait macules (one to three) were found in six patients, axillary freckles were observed in two. Histopathologic examination of biopsy specimens commonly showed hyperpigmentation of the basal layer, elongation of rete ridges, and an increased number of melanocytes. There ,was no evidence of associated disorders or family history. CONCLUSION: Based on this data, we confirmed that PUL is a benign, idiopathic lentiginosis with no commonly associated abnormalities. Furthermore, we believed that PUL is not uncommon in Korean people.
Bias (Epidemiology) ; Biopsy ; Dermatology ; Diagnosis, Differential ; Female ; Humans ; Hyperpigmentation ; Lentigo ; Male ; Melanocytes ; Melanosis ; Neck ; Skin

Female ; Gastrectomy ; Humans ; Hyperpigmentation ; diagnosis ; Middle Aged ; Nerve Compression Syndromes ; diagnosis ; Obesity ; surgery ; Peripheral Nervous System Diseases ; diagnosis ; Postoperative Complications ; diagnostic imaging ; Pruritus ; diagnosis ; Weight Gain ; Weight Loss

We report a case of atypical eruption due to chemotherapeutic agent in a 60-year-old man who presented with asymptomatic, erythematous, 0.5cm in diameter, confluent, and elevated papules and plaques confined to the face. The patient was previously diagnosed with small cell carcinoma of the lung with liver metastasis. Two months after the diagnosis, a first course of chemotherapy including etoposide was started. Five days after starting the chemotherapy, the patient developed a facial eruption. Histopathologic examination demonstrated increased epidermal mitotic figures, cells in metaphase arrest, basal cell layer hyperpigmentation, prominent dyskeratosis, and squamous atypia. The most distinctive histologic feature was the presence of starburst cells, which are markedly enlarged pale staining keratinocytes containing small basophilic fragments of nuclear debris haphazardly scattered throughout the cytoplasm in a starburst pattern.
Basophils ; Carcinoma, Small Cell ; Cytoplasm ; Diagnosis ; Drug Therapy ; Etoposide ; Humans ; Hyperpigmentation ; Keratinocytes ; Liver ; Lung ; Metaphase ; Middle Aged ; Neoplasm Metastasis