Skin Manifestations of Addison Disease: Understanding Dermatological Signs

Introduction

The endocrine system interacts with the other systems, causing severe dermatologic illness. Therefore, these lesions can indicate endocrine illness and cause significant morbidity. Generalized hyperpigmentation in Addison's disease patients is especially noticeable in sun-exposed areas due to high levels of melanocyte-stimulating hormone (MSH). Addison's disease, also known as adrenal insufficiency, is a rare ailment that arises when the body fails to produce enough specific hormones. In Addison's disease, the adrenal glands produce insufficient cortisol and sometimes insufficient aldosterone. Adrenal gland damage in Addison's disease is often caused by autoimmune illness, which occurs when the immune system targets the body's cells and organs. Infections can also cause Addison's disease.

What Is Addison Disease?

Addison’s disease is also known as primary adrenal insufficiency and is usually caused by autoimmune causes. It accounts for 15 percent of primary adrenal insufficiency in children. Addison's disease can occur as a stand-alone disorder or as part of the autoimmune polyglandular syndromes (types 1 and 2). This illness includes demonstrating abnormally low cortisol secretion, determining ACTH (adrenocorticotropic hormone) status to differentiate primary and secondary adrenal insufficiency, and imaging the adrenal glands. The common laboratory abnormalities in Addison's disease are as follows:

1. Hyponatremia (decrease in sodium).

2. Hyperkalemia (increase in potassium).

3. Hyperchloremic metabolic acidosis (increase in ionic chloride).

What Is the Pathophysiology of Addison Disease?

• Cortisol production from injured adrenal glands is reduced, resulting in a loss of negative feedback on the hypothalamus-pituitary axis.

• This causes a rise in the production of corticotropin-releasing hormone and, as a result, proopiomelanocortin, a precursor to the biologically active hormones ACTH, melanocyte-stimulating hormone (MSH), and others.

• MSH levels that are elevated cause epidermal melanocytes to produce melanin.

What Is the Etiology of Addison Disease?

Addison’s disease occurs due to the adrenal cortices' failure to produce appropriate adrenocortical hormones. There are two types of adrenal insufficiency:

1. Primary - Intrinsic adrenal gland dysfunction caused by an autoimmune response (both cortisol and aldosterone deficiency).

2. Secondary - Hypothalamic-pituitary dysfunction caused by persistent glucocorticoid administration (only cortisol deficiency).

What Are the Signs and Symptoms of Addison Disease?

Initial Symptoms:

• Fatigue.

• Generalized weakness.

• Weight loss.

• Nausea.

• Vomiting.

• Stomach discomfort.

• Dizziness.

• Tachycardia (increased heart rate).

What Is Hyperpigmentation?

It is widespread and almost always observed in patients. It is frequently pervasive and evident in areas exposed to the sun and under high pressure. Elevated ACTH and melanocyte-stimulating hormones are contributing causes. The pigmentation-causing melanocyte receptors are thought to bind to ACTH. More hyperpigmentation can be seen in the palmar creases, gingival mucosa, nail beds, elbows, knuckles, posterior neck, breast areola, and lips (particularly the vermilion border). There is no hyperpigmentation in secondary insufficiency because the ACTH and MSH levels are low. Nonetheless, numerous fresh nevi could develop. Female patients may also have diminished or sparse pubic and axillary hair.

What Are the Skin Manifestations of Addison Disease?

• Widespread skin hyperpigmentation.

• Axillary and pubic hair loss.

• Hyperpigmentation of the hair.

• Nail bands with longitudinal hyperpigmentation.

• Hyperpigmentation of the mucosa.

Due to increased melanin synthesis, almost all adults and 67 percent of pediatric patients develop generalized golden hyperpigmentation. It is most noticeable in sun-exposed areas and trauma and pressure sites. Furthermore, the axilla, areolas, perineum, and palmar wrinkles frequently affect body parts. Pigmentation in certain children can be somewhat diffuse, and comparing children to other family members may aid in determining clinical findings. For example, those with Addison's disease may have hyperpigmented scars, hair darkening, and longitudinal bands on their nails.

Furthermore, uneven or streaking blue-black or brown hyperpigmentation of mucosal surfaces, most commonly affecting the tongue, inner surface of the lip, and buccal and gingival mucosa, may develop. Patients may also notice discoloration of pre-existing nevi. Vitiligo, caused by the autoimmune loss of cutaneous melanocytes, affects ten to twenty percent of autoimmune adrenalitis patients. In certain persons, combining Addison's disease with vitiligo may result in a striking combination of pigment loss and hyperpigmentation. Aside from color changes, women with Addison's disease may experience axillary and pubic hair loss, which is influenced by adrenal androgen production.

What Are the Risk Factors for Addison Disease?

Several autoimmune disorders are risk factors for the autoimmune Addison's disease:

• Graves' disease (thyroid condition).

• Pernicious anemia (type of anemia).

• Chronic thyroiditis (inflammation of the thyroid).

• Type I diabetes.

• Hypoparathyroidism (decreased thyroid levels).

• Vitiligo (patches on the skin).

• Hypopituitarism (decrease in pituitary hormone).

• Dermatitis herpetiformis (viral infection affecting the skin).

What Is the Treatment for Addison Disease?

Addison's disease is treated by replacing glucocorticoids and mineralocorticoids that the adrenal gland cannot produce. The clinical situation determines the dosage of glucocorticoids.

Main Treatment: Acute adrenal crisis patients need the following:

• Intravenous (IV) normal saline fluid resuscitation to replenish intravascular volume.

• To treat hypoglycemia, use dextrose correction of glucocorticoid and mineralocorticoid hormone insufficiency.

Continuity Phase: Hormone replacement therapy must be used for the rest of the life. Therefore, maintaining an average level of glucocorticoids and mineralocorticoids is the goal of maintenance therapy. These are the typical dosages:

• Glucocorticoids: 5 to 25 mg/day (milligrams per day) of Hydrocortisone (can be divided into two or three doses).

• Prednisone: 3 to 5 mg each day.

What Are the Other Endocrine Conditions With Skin Manifestations?

• Acromegaly: Glycosaminoglycan deposition leads to skin and soft tissue thickening resulting in coarsening and expansion of face and acral features.

• Hyperthyroidism: Stimulation of the thyrotropin receptor causes mesenchymal tissue growth and subsequent pretibial myxedema; other related cutaneous characters, brittle hair, and hyperhidrosis.

• Hypothyroidism: Patients have chilly, dry skin, brittle hair, and a jaundice-like look due to carotene overabundance.

Conclusion

Addison's disease is a relatively uncommon disorder with disastrous consequences if not diagnosed correctly. Unfortunately, patients are frequently misdiagnosed until an acute adrenal crisis develops because of the disease's insidious nature. The course of treatment for Addison's disease skin symptoms is typically the same as that for the condition's other symptoms. First, the patient needs hormone replacement treatment to restore or substitute insufficient hormones. Moreover, the patient can be given oral Cortisone acetate, Prednisone, or Hydrocortisone pills. Patients who cannot swallow or digest pills may occasionally need a corticosteroid injection. The Addison crisis is a serious endocrine emergency that must be identified and treated immediately.